Peter's Progess

20 days later, TPN is coming back

2012-01-27T22:03:00.000-05:00

January 6 was Peter's last night of TPN... we hoped. Tomorrow night he'll start back on it again. He has been losing weight steadily since his surgery, which was good since he was overweight on TPN and fluids, but only good up to a point. His ideal weight is likely 47 to 48 pounds, but he is now 44 1/2 pounds and decidely skinny and bony... not just thin, but frankly bony. It is normal to lose some weight when transitioning off TPN, but he is dropping too much weight too fast.

The immediately apparent issues that have complicated Peter's success off TPN are unstable daily ostomy outputs that can vary from just under 600mL/day to over 1200mL/day and then the responsive action to take away his enteral feeds and place him on Pedialyte, which has considerably fewer calories than his formula. He is now on six Imodium capsules a day, and some days it seems to help, other days it doesn't seem to do a thing. At this point it is unclear whether the formula he is on needs to be substituted out for another one, or whether this is an issue with his bowel. As his ostomy does slow down considerably with Pedialyte, the output amount is likely due to the formula. We will be looking further into all of this over the next couple of weeks, perhaps going onto a different formula or substituting out some of the formula for something else.

People have asked why he is on formula instead of real food. The typical protocol in coming off TPN is to be on formula, as formula is more nutrient and calorie dense than real food, so a person can take in less formula than food. This gives the bowel time to gradually adapt to a more normal intestinal functioning. Peter does eat some real food, so it is possible we might have success feeding him more real food in place of some of his formula, but he frankly doesn't eat enough to sustain without formula or TPN. Unfortunately, with the reinstitution of TPN, we also know his appetite will go down.  It is a difficult catch to be on TPN - it can prevent or treat malnutrition, but it can also reduce the desire to eat. Everyone has held out as long as possible in putting him back on TPN, but at this point we all agree that he needs to regain some of the lost weight and definitely not lose any more. The goal is to still get back off TPN as soon as possible though.

Peter is disappointed that he needs to go back on TPN, as are we, but he is only going to be on three nights a week and will likely go down to two nights a week once he gains a little of this lost weight back. We are, however, happy that he will get back on Omegaven again, as he had to stop Omegaven once he stopped TPN. We had tried enteral fish oil after he ended Omegaven and that did not fare well with his ostomy output, so Peter has not had the benefits of those great omega 3 fatty acids in the 20 days he's been off TPN. We had a great break from the tedium of TPN set-up, but honestly, the last two weeks have been rather harried in keeping up with the dealings of his fluctuating high ostomy output: calculations, phone calls, emails, and boluses of Pedialyte, sometimes every half hour, in an effort to keep him off TPN. Resuming TPN may be the less complicated option at this point.

We decided to give Peter a "bonus" to restarting TPN by telling him we will have another celebration when he comes back off TPN. He is already looking forward to it.  "Will I get to open more presents?" he asked tonight. I just love the thought process of a six year old!

A little video of Peter's party

2012-01-09T21:51:00.000-05:00

We tried to take some video of Peter's little party for coming off TPN. Most of what was captured is mayhem, but this one at least captures the excitement that he and his siblings have about this special milestone.

The significance of the watusi gift is only this - Peter has a great appreciation for animals and he has a huge collection of plastic animals at this point. After he got home from his long hospital stay in Sept-Oct, we took him to the zoo, where he saw a watusi. It was an impressive animal! He has been wanting a plastic watusi since that time, but there is just no plastic watusi to be found. The day of his party, his sister came across this animal, which actually is a Texas Longhorn, but awfully close in appearance to a watusi, and felt that if we cut the tag off, Peter might just be convinced it is indeed his long sought-after watusi. So far, he is convinced that it is!

Peter is OFF TPN - hooray!

2012-01-09T21:37:00.000-05:00

The big news is that Peter is now officially off TPN! In less than four months after his surgery in September, he has gone from 7 nights per week of TPN dependency to zero dependency on TPN! He is taking in almost 1300 ml of formula daily, along with 120 to 200 ml of yogurt and pureed baby foods, and about 120 to 250 of water a day. A great thing with Peter being six years old is that he has been able to understand why I am feeding him formula several times a day even though he doesn't care to drink it. He has been able to decide that he wants to be free from TPN and to understand that for him to accomplish that goal, he needs to drink the formula. We had a little party for him to celebrate his end of TPN and he was so excited. He decided that he wanted sugar cookies (reduced in sugar for him) and that they were to be shaped cookies, not drop cookies, and he wanted to help make them. He also wanted strawberries for his party. We then added some surprises for him with crepe paper streamers, a banner, a candle to blow out, and some unexpected gifts and cards. He had a wonderful time and the celebration gave him some well-deserved recognition for the effort he has put into coming off TPN.

Peter has been on TPN since April 2006. The most he was ever able to come off was three nights a week for a few months, and then the fistula started putting out huge volumes of output and he had to go back to 7 nights per week of TPN. It almost doesn't feel real that he is actually off TPN since we have been using it for so many years. I am posting some photos of what his nightly set-up on TPN was. We will miss the great health benefits that we know Omegaven gave to Peter, but will certainly not miss the twice-per-night changing out of the old Omegaven bottle for the new one and resetting of the pump. His nightly set-up now is just a little IV push medication, setting up his tube feedings, and hooking up his ostomy to a drainage bag. He, and we, are enjoying the freedom that comes with being off TPN.

Supplies needed to set up TPN and Omegaven. I forgot to include the 50mL bag of saline which we hang at 6am to "chase" the remainder of Omegaven down the IV tubing. Below are photos of what the set-up looks like after injecting the multivitamins and connecting the tubes.

Omegaven

This yellow bag is what the TPN looks like after the multivitamins have been added. This yellow bag and little pump are what is inside the maroon bag below.

This was Peter's nightly set-up with TPN, Omegaven, and enteral feeds. The tiny little white and teal pump and pink bag behind it are all that will be remaining on his IV pole, as they delivery his near-700 milliliters of enteral feedings overnight. The ostomy drainage bag is still there too, but hanging off the bottom of his bed. That will go away whenever the ileostomy gets surgically reversed.

Hopefully this is goodbye to TPN for good, but if not, we'll enjoy the freedom while it lasts!

To Boston and back yet again

2011-12-07T21:53:00.000-05:00

Dr. Jennings has been great about looking over emailed photos of Peter's wounds the last few weeks, and ultimately felt it was time to surgically fix them. Peter had retained some of the sutures from his surgery in September instead of them dissolving, and the skin around them had some localized infection surrounding the retained sutures. Some of those sutures had come out on their own, but the rest needed some assistance, as they were painful and not healing. Peter did well with the brief surgery, but it took him five hours after the surgery to wake up! The wound cultures are growing out a Staph Aureus species, so it looks like Peter will need to begin a course of antibiotics, which in Peter's case is not always such a simple thing. Ideally he would be given enteral antibiotics, but of the four antibiotics Peter has had reactions with, three of them have been administered enterally. He has done OK with two of those three in IV form though. So, we are waiting to see what the dosing will be and hope that it will be a schedule that is not too crazy to keep up with.

Peter is now up to 1000mL of formula per day and there is talk of taking him completely off TPN in the next week or few and just giving him some IV fluid for hydration on the nights he would have needed TPN. From there it should be a pretty fast switch to IV fluids just as needed, I think. Hopefully Peter will not have any side effects from the antibiotics and will continue to make these great strides in getting off TPN. We are reluctant to take him off TPN from the standpoint that he will no longer be on Omegaven, but if he gets off TPN his liver should get healthier and his appetite should increase. He has been on TPN since Spring 2006 and on Omegaven since late December 2007. It is exciting a few nights off TPN each week; I can hardly imagine NO TPN. I'll post when it becomes official.

Pics from the zoo and Halloween

2011-11-19T22:30:00.000-05:00

Guess who Peter was for Halloween? Dr. Kamin! He was so proud to play the role of Dr. Kamin as he trick-or-treated around the neighborhood while everyone oohed and aahed at his selection of costumes, most of them knowing how playing the role of doctor has more meaning in it for Peter than it would have for most children. The red backpack was carrying his wound vac, which he became quite adept at carrying.

Birthday, wounds, and TPN progress

2011-11-19T22:11:00.000-05:00

Many updates on Peter since the last post. Peter finished with the wound vac November 4. The vac was on longer than anticipated, but did the job of closing up the wound. Peter has some pyogenic granulomas on his wounds which are making them not fully heal, but we are hopeful that treatment with silver nitrate sticks can heal them. Sadly, that treatment hurts as it is being applied, as it is essentially burning off the granulomas. It does not hurt once the dressing is put on, but this poor kid has been through so much it is frustrating that this treatment hurts him. We could give a pre-medication, but with a systemic med he then gets drowsy and dopey for a long time after the procedure. We are looking into a topical anesthetic for the area, but unsure if it can be used on an open wound without becoming systemic inadvertantly. We will do this procedure for a few more days, and then if this does not work, he will need surgical revision of both surgical wounds, since they are both affected.

Overall, though, he handled that big surgery phenomenally well, much better than many expected, and we are glad that this surgery was performed. The final report on the intestinal surgery indicates that all of the removed bowel was diseased, and if he had not had this big surgery performed, we would still be dealing with intestinal fluid losses from the fistula and still stuck on TPN seven nights a week.

So due to the success of that surgery, we have amazing news. Peter had his CAIR appointment this past week, and he has grown so much that he is now going to be off TPN for four nights a week! This is the most he has been off since TPN was initiated on him in late Spring, 2006. He is working hard on taking in as much formula as he can, and once we are off TPN, we will work on switching over to a more palatable diet. He has gone from seven nights on TPN when we left in October, down to three nights of TPN in only a month and ten days! The CAIR team was quite impressed with his progress!

Peter also had his sixth birthday. We took him to the local zoo and he had a terrific time seeing many of the animals that he has seen in his books and that he has copies of in plastic. Going to a zoo was his big request while we were inpatient in Sept through Oct, and it was such fun watching him take it all in.

Home!!

2011-10-12T22:51:00.000-04:00

On Friday evening, October 7, we received the good news that Peter's wounds are healing enough to come home. We arrived home Saturday evening.

Peter still has a wound vac on the midline incision, but we don't expect that he'll need it more than a week or two. The vac dressing sits on top of his belly, with a tube attached to the dressing, and then a pump attached to the tube, which all work together to provide suction to create negative pressure to promote wound healing. I wasn't sure how we would manage having a five-year-old attached to a wound vac. It is a three pound machine, about the size of a small lunch box, and the tubing is about five feet. Much to my surprise, he has been remembering to take the machine with him when he wants to walk around, and gathering up the tubing so that he doesn't trip over it.

Dare I say that life without the fistula is dramatically different already? I am still holding my breath that some new problem does not emerge, but so far I am astonished by the difference in his care. Once the incisions heal up, I think that difference will be even more profound. I know it doesn't sound like much to go from emptying two ostomy bags to just emptying one, but multiply that times three or four times a day, times seven days a week and it adds up, especially to a five year old who has to stop playing when mom says it is time to get his bags emptied, 800 to 1000 ml a day from that fistula! Then add to that the additional time difference between a ten minute ostomy bag change twice a week, versus an hour-long change for both the ileostomy and fistula which was occurring two to four times a week.

The main reason for the surgery of course was not to simplify life, even though that has been the benefit I find most dramatic at this early stage post surgery. The real purpose of that very big, risky surgery was to allow this child to eat and get off TPN. The fistula has repeatedly made it impossible to get feeds high enough to get off TPN, because it would leak more fluid as the feeds increased, throwing electrolytes off and fluid status off. The true dramatic changes from this surgery are yet to unfold as we hopefully finally get Peter off TPN over the next year or so. Yes, he still has many risks with liver fibrosis, portal hypertension, low white count, low platelets, and enlarged coronary arteries, but I expect that some or much of this will resolve as he moves away from TPN dependency as well. Omegaven halted the progressive liver damage so that we could get to this stage without a need for transplant, but with Peter's bilirubin having been so high for so long, the only way to heal the liver from its fibrosis is to get off TPN. So, now we have a chance to do that!

Another bleed, but...

2011-10-06T22:38:00.001-04:00

Today is Day 28 post surgery. Peter has been on the regular floor since Sunday, four days after the big bleed. Yesterday he bled again, but his timing was impeccable. The surgery team was actually just a few doors down from his room and came in as soon as the nurses came rushing in. Dr. Kamin followed in within five minutes, and Dr. Jennings within ten minutes or less. And this time, we were able to see exactly where the bleeding was coming from and fix it!!

Peter has a small "hole" on his ileostomy stoma, which I noticed on Monday. Jen, the nurse who held pressure on the stoma with last week's bleed, noticed a clot in that same spot last Wednesday after the bleeding stopped. Drs. Kamin and Jennings both were made aware of the "hole" and the clot, and said that if we were to see another bleed and witness the bleeding from there, then we would know that it was the source of the big bleed last week, as we have all felt a little unsettled that the esophagus was the bleeding source given there was no blood in the g-tube and the stools that followed had no trace of blood in them for days.

So, yesterday, exactly one week from the first bleed, Peter bled again, but with his timing of it, we were all able to see the exact source of the bleed, stop it early so he did not need a transfusion or to go to the ICU, and to get it sutured closed. How perfect is that scenario?! Truly, we are all amazed! Chris and I continue to fully believe there is a Divine plan to all of this, which we expect we will never know, but to call the timing of this a coincidence seems way too random to both of us.

In terms of where this leaves us for future bleeds, we will need to pay attention for bleeds like we did years ago, and hope that this too shall pass. There is a good chance that with a little more time away from surgery and with the resuming of enteral feeds, the portal hypertension will decrease. However, as has always been the case, there is a possibility that the portal hypertension will increase too, and if it does, then we'll begin talks as to ways to decrease it.

The wounds are improving, but the midline vac is still on. I can see Peter's belly button again, and despite the surgical scars on his belly, seeing that little belly button where the fistula used to be just makes me want to smile and cry both at the same time.

Peter gets another wound vac dressing change tomorrow and Dr. Jennings will decide if Peter can go home yet. His lateral wound is looking really good now, and hopefully will close completely in another week or so.

As an aside, Flashes of Hope was at the hospital the other day and did a free photo shoot of Peter. Per their website, http://www.flashesofhope.org/about/mission, "Flashes of Hope is a nonprofit organization that changes the way children with cancer and other life threatening illnesses see themselves through the gift of photography and raises money for pediatric cancer research." I am really looking forward to seeing the photos when they arrive in a few weeks!

Day 23

2011-10-01T22:08:00.000-04:00

Peter had his scope yesterday afternoon and extubated well. Dr. Kamin is still not entirely sure what caused the bleed, but banded some newly-enlarged varicose veins in Peter's esophagus in case they were the cause. He scoped as far into the small bowel as he could and as far up the ileostomy as he could, and did not see any other areas likely as cause. Peter has been on an octreotide drip and a pantoprazole drip since we came to the ICU with the bleed, and they will be weaned off shortly.

The surgical incisions are improving slowly, and there is still one area that is being watched carefully. We'll be here for a little while longer to keep watch with both the bleeding and the incision healing, but overall the news seems postive. The plan is to move back to the regular floor tomorrow. Peter is in very good spirits and increasingly playful.

Day 18 - back to the ICU

2011-10-01T21:57:00.001-04:00

Our darling Peter decided to give us a real scare yesterday and we are actually back in the ICU. He had a sudden 750 mL bleed from his ileostomy. It was an intense, scary time between when I noticed it and when we were confident that it had stopped. He needed two units of blood and monitoring and tomorrow he will have a GI scope to look around to see if we can see any varicose veins that would be the cause. I have seen him do this type of bleeding before, but it has been years since we last saw it, and the concern is obviously to make sure we understand why it happened this time and how to have it not happen again. It could be increased portal hypertension (high blood pressure in the liver/spleen/intestine circulation) from the operation (unwanted side effect), or there is a very remote possibilty it could be related to the antibiotic he was on. We expect to know more after tomorrow's scope which will be performed in the late afternoon. They will also change his vac dressing then, instead of today, as he will be under anesthesia and it will be easier to really look at the incision sites without hurting or upsetting him.

At this pace, we may end up not needing the vac when we go home. In retrospect, I am grateful that we were still here when the bleed happened, which would not have happened if the wounds had been healing as expected.

Day 17 since surgery

2011-09-25T16:55:00.000-04:00

We are still inpatient, but have been on his "regular" floor for 8 days now. Peter is doing very well physically, but the two surgical wounds are not. We tried wet-to-damp dressings for a few days, then silver impregnanted Aquacel, and then on Wednesday, the surgeon decided we needed to go to wound vacs. Peter had two wound vacs placed over the surgical sites on Wednesday evening, and we are here to make sure that they actually do get the wounds to turn around and heal enough to go home. Peter's wounds not only did not heal, but the skin began to open back up and there was yellow slough covering the wound beds, despite measures to keep that under control. Both wounds were producing a fair amount of drainage and ended up opening up all the way down to the fascia in two areas, and each time we would change the dressings (twice a day) the wounds would look the same or worse, no progress.

Dr. Jennings is quite apologetic that the wounds are not healing, feeling like the issue may be as a result of the wound catheters that were used for pain management. He needn't apologize though, as we all thought the wound catheters were a good idea, as a wound catheter is what made Peter have so little pain after his surgery in April. He also mentioned Peter's ascites as possible cause for the wounds to open as they did. Peter has had an abdominal binder on since he was extubated, and his ascites has gone down quite considerably, but for awhile he was HUGE and it was difficult to get the fluid off him. A thirteen hour surgery is a lot on any body, so swelling post-op is expected, but as Peter is so prone to ascites, he stayed big there even once the fluid began to leave the rest of his body.

Peter has healed quite well with his last two surgeries, so there is not a reason to think he will not heal from this one. His liver numbers had a few days of a rise, but they have been trending down nicely over the last two weeks, as has his BUN and creatinine (indicators of kidney health status) which went up a bit right after the surgery. Nutritionally, his TPN has been loaded with extra protein, zinc, and vitamin C. He is on Cipro IV for some bacteria in the wounds, however the bacteria should not have caused this much tissue damage, and is likely due to the probing and poking that was done after we noticed the wounds not healing. Tomorrow, Dr. Jennings will take the vac dressings off and we will get to see what the wounds look like for the first time since Wednesday.

This admission has been hard on Peter. He has been asking to go home for days and days. His daddy came up here with two of his siblings to surprise Peter (and me) over the weekend. The visit was such a boost to Peter's morale! He has been smiling and back to some of his silly antics again, which I really had not seen so much of before their visit. Hopefully there will be huge progress in the wounds when the dressings come off tomorrow, and we can plan our trip home!

Still in the ICU but improving

2011-09-15T19:46:00.000-04:00

Peter extubated two days ago. His CO2 was high so he needed bi-pap to help him breathe off the CO2. Yesterday he went to just an O2 mask, and today he is on room air. He was playing a little bit in bed today, blowing bubbles and doing a little coloring with markers. It was good to see him smile.

He is going through a little bit of withdrawal from the narcotics he had to keep him sedated while intubated. His pupils were huge yesterday morning and he was quite fidgety, but today he is much more at ease, not needing any narcotics for pain or agitation except once for his dressing change.

He still doesn't realize that the fistula is gone. I tried to tell him today, but it didn't sink in. His abdomen is covered by an abdominal binder, so he doesn't see that there is a dressing there instead of a bag. As he lies down for ostomy bag changes, lying down for a dressing change doesn't seem to be anything different either. He is agitated during the dressing changes post surgery, unlike at home, but othewise, I suppose it all seems the same to him in his current state of mind. It will be interesting to see his reaction when he realizes that the fistula is finally gone.

Still intubated

2011-09-11T19:40:00.006-04:00

Peter is still intubated, disappointingly. He has accumulated about 5000ml of fluid since surgery, despite conservative measures with his fluid intake, and he is puffy all over. As such, it is not surprising that his lungs have a little fluid on them as well, which is why he is being kept intubated right now. The doctors started him on a Dopamine drip to support the kidney's blood perfusion and gave him albumin and he is now on a Lasix drip.

It is hard to see him lying sedated in the bed with the tubes and vent. He has nine IV pumps going continually, a urine foley, a tube up his nose to drainage, his G-tube attached to a drainage tube, his ileostomy attached to drainage tube. Some of this is his nighttime "usual:" two to three drainage tubes, two to three IVs. The sedation and the vent and his large size are what makes it hard to see him like this, as well as the addition of meds and pumps, instead of their removal. The concern that this was too big of a surgery for him to handle plagues me. I don't regret doing the surgery, and we still feel that it made sense to do this surgery now. This surgery and its risks have been a conversation many times over the years, so the decision was not a hasty one, although the opportunity presented itself unexpectedly.

I have been reassured by everyone who comes in that this is not unusual for such a big operation, even for a healthier child. It is admittedly difficult to not look at him and have flashbacks to his ICU days back in 2006. The priest came to visit him today and said some prayers over him and encouraged me to remember that Peter is not in that same situation and to not worry yet if the doctors have told me that things are still normal. He told me that if Peter pulled through his illness back in 2006, to be hopeful that he will do it again, especially as he is healthier now than he was then.

Please keep Peter in your prayers! Thank you.

Fistula surgery

2011-09-09T20:53:00.001-04:00

The fistula is GONE!! It took thirteen hours in surgery, but it is finally gone!

The intestine which was connected to it was bad tissue. It was leaking chyle and had no necessary function since it was taken out of the GI tract in March. It was continuing to cause sodium imbalances and limited his ability to feed since it seemed to go up in excretion volume as we would increase his feeds. Ultimately, there was no way to continue to increase feeds and get off TPN without removing that intestinal tissue.

Peter was called to surgery at 6:30 am and taken back into the OR at 7:30 am. At 9:15 the first incision was made. There had been a lot of prep work to do before making the incision, but he was already getting sedated at 7:30 am. At about 2:30, Dr. Jennings and Dr. Kamin met with us and Dr. Jennings told us that he could continue to remove the intestinal tissue on the other side of the fistula and then close it, or just stop where he was and close up, knowing that the main source of trouble had been taken out, but with the chance that we could still see problems with that portion of fistula and tissue that was still left. We all agreed that it seemed a good idea to continue while he was doing so well. Dr. Jennings said he was up for continuing the surgery another six to eight hours.

At 8:30 pm, Dr. Jennings came to say that the surgery was finished and Peter's fistula is gone. Peter's abdominal muscles have been moved almost all the way back to midline. (They have been off to his sides since July 2006, when he had abdominal compartment syndrome and the abdominal incision had been left open to heal without being sutured back together.) He lost a liter of blood, which was not much considering he has portal hypertension. Dr. Jennings said his liver looks pretty healthy in color, no nodules and is firm but not hard. We can thank Omegaven for that!

Peter has a ways to go to recover from such a big surgery, but so far he is doing well. We still can't believe that the fistula is actually gone. This should have big potential for his overall recovery to a life without TPN, and for his physical ability too now that his muscles are so much closer to the correct place they should be.

Peter's recent trips to Boston

2011-08-14T21:33:00.000-04:00

Peter had two seven hour trips to Boston over the last two weeks. One was for his CAIR visit, which went well, and then the other was an admission for an illness. At the CAIR visit, we established that Peter gained four pounds in two months and were able to decrease his TPN by 15% per night. This is good news in terms of the health of his GI tract, as the weight was gained by what he has been taking in by mouth and G-tube. During that same trip, Dr. Kamin scoped Peter's fistula and established that it is chyle that is leaking from the bowel. A follow up test in nuclear medicine shows that the chyle leak is contained just to the intestine that is connected to the fistula, and that there is not a leak in the "good" parts of his intestine. Dr. Jennings has therefore stated that Peter should come back soon for surgery to remove the intestine that is connected to the fistula. I realize most people would not be happy to hear that their child has to go for intestinal surgery. Peter is losing 1200 mL of fluid a day (from the fistula!) and has to get weekly labs to try to maintain sodium and fluid balance, so surgery sounds like less risk than trying to maintain things as they currently are. We are at a standstill with increasing enteral feeds because the intestine attached to the fistula only increases in output as the feeds go up.

At that same visit we had Peter's face, neck, ears, and chest lasered to remove the telangectasia spots and spider veins that have emerged over the years. Peter was the first patient at Children's Hospital Boston to have a laser treatment done in the OR. The dermatologist was telling us that they have wanted to get the OR equipped to handle the laser for some time, but it has been a slow process. In fact, when we first talked about doing this back in March at his Vascular Anomalies Clinic visit, the OR still wasn't equipped to handle the laser (electrical needs, I believe). There were some kinks in getting the laser to the hospital from the satellite clinic so that Peter could get lasered, and then another problem in communication as to which OR rooms were equipped to handle the laser. As such, Peter's scope and accompanying laser were delayed for six hours. Dr. Kamin was very kind to continue to postpone the scope until the laser arrived. After the procedure I was very worried that we had made a mistake in doing the laser, as the lasered areas truly were a dark purple afterwards and casued more heads to turn that the original spider veins and telangectasia did. However, two-and-a-half weeks later, we are very glad with the results and feel that it was a good move after all.

CAIR was Thursday, the scope was Friday, nuclear medicine was all day Monday and Tuesday morning, and then we drove home. Wednesday night Peter spiked a temperature, ultimately reaching the dreaded number 100.5, which means cultures need to be drawn to be sure that there is no line infection. A line infection can quickly lead to sepsis, so I called Boston Thursday morning. By late Thursday morning, Peter was acting mostly better, and in the late afternoon he went for a brisk walk with me and came home and jumped on his little trampoline. It seemed all was well, minus a decrease in hunger and some bloating of the abdomen. However, his CBC came back with a white count of 0.4 and platelets of 37. The plan was to redraw the labs the next morning and hope that the CBC numbers were erroneous. Thursday night the fever returned and hit 101.7. There was an even bigger pit in my stomach then. He was fussy when he woke up and actually put himself back to bed. Another call to Boston and another set of cultures and CBC drawn. We talked with Dr. Kamin and made a plan to head up to Boston with the possibility of the CBC still being the same, and the understanding that if it was good, we'd just return home and wait out the return of the blood cultures. As we got closer to Boston, Dr. Kamin called and told us to just head to the ER and plan to be admitted because the numbers were still the same. Concerns ranged from a simple virus, to a bloodstream infection or line infection, to peritonitis, to chyle now leaking into the abdominal cavity post the scope. After admission, he never did spike another fever, but he slept a lot, his belly remained big, his hunger and thirst were way down, and his white count went down to 0.37 and neutrophil count down to the 100 range. The platelets stayed down about 47,000. After two more sets of cultures and 72 hours of prophylactic IV antibiotics, Peter seemed well enough, no bacteria found, and the white count and neutrophil count improved, at least a little bit. We spent another two nights up there off antibiotics just to be sure nothing returned. The final impression is that he likely came down with a virus which hit him but no one else in the family. It hit him very hard to say the least. He is due for labs on Tuesday and the hope is that his numbers have returned to his normal. His normal is always abnormal, but more like 2.5 white count (normal is about 5) and platelets of 80,000 (true normal would be 150,000-400,000) and neutrophil count of 1,000 to 14,000 (which I can't recall is truly normal or not). Dr. Jennings said that the neutrophil count must be at least 500 for surgery or else the risk of infection is too great after surgery. A low white count makes a person more susceptible to illness and infection because the body cannot generate enough of a white cell response to attack illness/infection.

Peter has been FULL of energy since we left the hospital, and even before we left was practically running in the halls. His recollection of our trips is that he had fun playing with the toy animals on 10 South and that he is looking forward to playing with the animals the next time we are admitted. God is good.

Reciting the alphabet - forwards and backwards

2011-07-07T23:16:00.000-04:00

Peter will be six on November first, so he is only five years and eight months old. Tonight he recited the entire alphabet BACKWARDS! He has long known the entire alphabet and the phonic sounds associated with each letter, but out of the blue he started with Z and went all the way to A consecutively, without missing a letter. Wow!

Peter lost his fifth baby tooth a few days ago. So far, all four bottom front permanent teeth are brown from tip to botton. I can't tell what color the new top front tooth will be, but had a glimpse of white when brushing his teeth tonight, a brief moment of excitement, and then realized that it was only toothpaste, not the new tooth. We continue to wait and prayerfully hope that the other permanent teeth will be white, or at the very least, not MORE discolored than the baby teeth are.

Peter can now swing on the monkeybars. He hangs on one bar and swings back and forth from that one bar a couple of times, but he is propelling himself, not screaming in fear, and is rightly proud of his new feat. One of these days, I'll post that he can finally jump... perhaps a video to go with that one!

Fistula increases again

2011-06-27T21:19:00.000-04:00

Peter's fistula continues to go up in volume. He is putting out a little over a liter a day from his fistula. His output is almost as high as it was before the surgery. The docs are discussing how to handle it and may try a new medication to see if that will slow it down, but ultimately he needs more surgery and it is a question of how best to surgically correct it and when. He is still eating, but he is limited in what he is taking in still, as there is some suggestion that there is a correlation between the volume he ingests and the volume put out the fistula. There is not a connection to the fistula and the GI tract anymore, the surgery fixed that, but as there is still a good deal of intestinal tissue connected to the fistula, that intestinal tissue still thinks it needs to secrete liquid when Peter eats. That is my understading of the reason for so much output, anyway. That, and the chyle leak that continues alongside this problem.

I must say that it is very difficult to find low-fat foods that are not high in sugar or fiber. We have about 5 boxes of cereal for him to eat, baby foods which he is beginning to tire of since he has been eating them for years, and some meltable Gerber Puffs which he likes but have no caloric value. He has enjoyed dipping pretzels into ketchup and mustard, and baked chips this way as well. He won't eat noodles or bread, though they would be good for him. He did eat a few nibbles of pizza today though. It wasn't low fat, so the fistula output became more milky after that snack, but how do you say no to a kid who is finally trying another new food that any other time would be a milestone? Dr. Kamin is OK with an occasional fattier snack, as he has reminded us of the importance of considering quality of life as well. I wonder if there is pizza-flavored baked chips?!

The survey says... chyle

2011-05-31T17:04:00.000-04:00

We are back from a fast trip to Boston two weeks ago. Tests seem to point that the fluid leaking out is chyle, and that it is limited to the fistula versus the entire bowel. We tested Peter with a 12 hour fast, which was easily accomplished overnight. Since he is still on TPN 7 nights a week, it was even easier. Then we collected his ileostomy and fistula outputs for testing. After he woke up, we gave Peter 3 ounces of whole milk with olive oil in it, waited an hour and a half, and collected both outputs. Then we gave him the same drink a second time, waited an hour and a half, and collected both outputs again. The crazy part of the second collection is that due to the timing of collection, it needed to be done in a public restroom and then placed in a box with ice alongside of the other specimens, all the while trying to remain clean about the process, and quick, as we had already checked in to the waiting area to see the doctor.

The fistula output turned very cloudy after the fatty meals, suggesting chyle, and the fistula electrolytes suggest chyle. The ileostomy had a lot of undigested sugars, which may be due to the fatty meal or due to his condition of short gut, or could be pancreatic insufficiency, so we are now testing the pancreatic enzymes in his ileostomy outputs. Some questions arise as to why the chyle in the fistula is not high in protein, and why the ileostomy triglycerides were elevated after a fatty diet, but Peter's tests usually lend to results that need interpretation versus "going by the book." So, with much discussion and interpretation, the impression is that it is a chyle leak in the intestine that is connected to the fistula, but that the rest of the GI tract seems to not have a chyle leak. It is good news that the chyle leak seems to be isolated to the fistula.

Further good news of all of this is that it looks like surgery will be the answer to stopping the chyle leak. No one wants to take Peter off all feeds for 4 to 6 weeks to attempt to have the leak close on its own, as his liver is already taxed on the amount of TPN he receives. Feeding the gut helps to keep the liver happy, even if the feeds are small in amount. We will actually try to increase feeds slowly, keeping to low fat, and then in several months hopefully surgically remove the fistula and the chylous intestine that is attached to it. Details of course need to be hammered out and this plan is only in the beginnings of talk, but Peter did so well with the last surgery that it may be feasible to get rid of the fistula altogether despite the concerns that have come up about doing so in the past. Our earlier talks for the next surgery were for a take-down of the ileostomy, so I don't know yet if the fistula surgery would take precedence over that or if they would be combined surgeries.

A chyle leak

2011-05-04T08:34:00.001-04:00

The predominant thought is that Peter is leaking chyle out his fistula. Per the information at http://www.medicine.virginia.edu/clinical/departments/medicine/divisions/digestive-health/nutrition-support-team/nutrition-articles/practicalgastro-may04.pdf, "Chyle is an alkaline, milky, odorless fluid that provides about 200 kcal/liter. It contains greater than 30 g/L of protein, 4–40 g/L of lipid (mostly triglyceride) and cells consisting primarily of lymphocytes (1). Chyle leaks are a rare complication; they can occur for a variety of reasons after injury to the intra-abdominal lymphatics."

Usually chyle leaks go into the abdominal cavity or thoracic cavity, so we are fortunate that this is not the case. It can be a complication post surgery, but there is some question if Peter has had this for a long time, and it was masked by the leakage of food out of the fistula. The usual treatment is conservative, and begins with a low-to-no-fat diet to decrease the amount of lymph (chyle) flow. Peter switched to a formula called Tolerex for the last ten days, but it does not seem to be helping. The fistula started going up in volume prior to the switch to Tolerex, but rather than going down or holding steady, it has continued to climb to the point he is now putting out 850 mL of drainage from the fistula per day. Along with the ileostomy increase that we did see correlate with the change to the new formula, Dr. Kamin suggested that we just stop the commercial formulas altogether for a week to see if that helps. Peter can eat and drink no fat, low sugar drinks and foods still, but we will eliminate the formula to see if maybe Peter responds better to a real-foods approach. Although it can be difficult to get enough calories to get off TPN without a commercial formula, the idea for now would be to see how well Peter's intestines respond to this type of diet and then go from there.

The next step if this doesn't help is possibly to eliminate all food/drink for awhile, and/or to begin some more invasive procedures to try to look inside and isolate where the leak is coming from. We may also be collecting fluids to determine with more certainty that it is a chyle leak versus some other complication. The fistula collection two weeks ago highly suggests chyle leak, but a collection of the ileostomy may help see if the whole intestine is affected, and a new collection of the fistula may help to see if there has been a change in the content make-up. A biopsy may also be considered to see if there is a food allergy that is causing these symptoms. Dr. Kamin will take this week to consult with Drs. Jennings and Fishman again to see what tests and approaches seem best to determine what is going on and how to manage it.

"Quality of life" is a phrase that gets used from time to time with Peter, and it is difficult to reconcile that he really does walk a line with his health when I see him climbing the equipment at the playground, playing and laughing with his siblings, learning the things that a five-year-old learns. Really, the term "quality of life" isn't a term that is used in any other situation than one where someone has to weigh medical treatments and conditions with the enjoyment of life itself. As usual, Peter acts just fine, despite the craziness of the things going on inside him. He remains our miracle child. Thank you to all who continue to pray for him!

The fistula, the fistula

2011-04-18T17:27:00.000-04:00

Again, we are having trouble with the fistula. As we are feeding Peter more formula, the fistula output is getting cloudy, like a watery milk color, and is going up in volume. We gave him blue food dye in his formula today to see if it is formula coming out of his fistula. The good news is that no blue food dye came out the fistula; we saw it all exit the ileostomy. The bad news is that there is likely something going on that needs to be addressed. We will be collecting some of the fistula output for analysis. Details about what the problem is and how to manage it are still to follow. I don't think the docs feel it is something terrible, but it does seem to be a setback in our goal of getting him off IV nutrition.

Reconnected!!

2011-04-04T22:48:00.001-04:00

Well, Peter is partially reconnected, anyway. On March 23, he spent 7 hours in surgery to bypass the enterocutaneous fistula in his jejunum. Dr. Jennings (surgeon) and Dr. Kamin (GI) worked together in the OR to locate the good portions of bowel above and below the fistula, and dissect them from the fistula and resect them to each other to make the bowel continuous all the way down to the ileostomy. Dr. Jennings made the incision and painstakingly worked through the scar tissue surrounding the intestine until he was near the place for resection. Then Dr. Kamin came in and used an endoscope to navigate the bowel and precisely locate where Dr. Jennings should make the cuts for resection. In doing this, Dr. Jennings left the fistula in the abdomen, but essentially cut it off from the rest of the GI tract, leaving just a loop of bowel going nowhere internally and exiting out the mid-abdomen as it has been for years. Someday, Dr. Jennings will remove that fistula entirely, but it is expected to be a huge operation and he would like Peter to be entirely off TPN for a long time, with his liver as strong as it can be, before he tackles it.

How well Peter did in the OR and how quickly he has recovered from surgery has everyone relieved and saying prayers of thanks. The pre-surgical letter that Dr. Jennings had sent to Peter's pediatrician and cc'd to us had this statement in it, "Of course the risks are considerable [and] with his portal hypertension and previous surgery this could be a lethal event." The letter also discussed why we all felt it would be beneficial to take this risk, and that given his current good health and recent minor surgeries, we "think we can make this a relatively safe operation for him." Dr. Jennings told us that the OR was very well stocked with blood products and emergency supplies, but that he only needed to give Peter one transfusion, more as a buffer than out of dire necessity, as Peter's blood loss was only 200mL. For a person with portal hypertension, this little amount of blood loss is remarkable. Dr. Jennings came out of the seven hour operation with a big smile, and ours soon matched his as he told us how well Peter did.

Peter had very little pain after the surgery.  Often, a person with portal hypertension is not a good candidate for an epidural, unless the spinal circulation in not compromised, and we do not know that with Peter as we have not imaged those vessels. The anesthesiologist, Dr. Sparks, and Dr. Jennings, both decided that a wound catheter would be the best device for pain management. It is essentially an epidural catheter that is placed in the wound instead of in the spinal canal, using continual delivery of a nerve anesthetic (ROpivicaine was used for this). Though his operative site was right below the diaphragm, which is apparently a very painful site because the diaphragm moves every time you breathe and therefore the wound would move too, Peter needed just a few doses of additional pain medication the first day-and-a-half, and then none after that. He may not have needed any additional pain meds beyond the wound catheter if we had known that morphine would cause him to get irritable and itchy. His mouth hurt from the endotracheal tube and the endoscope, and we all thought that a little morphine would keep him comfortable, especially as the meds from surgery began to wear off. As he continued to be agitated and complain, we thought the pain was really breaking through, but eventually we realized the morphine was the cause.   He kept the wound catheter in for five days, and was up and walking around on the second day after the operation. He walked a mile in the hallways on Sunday, just four days post-op. He continued walking at least a mile a day in the hallways until we discharged on Friday April 1! Did I mention that he was out of the ICU and on the regular floor in less than 24 hours?! And he was home again 10 days after his operation.

The only thing that really went wrong for Peter was that he gained a lot of ascites. He left the hospital three pounds heavier than when we were admitted, and with a belly 10cm larger in circumference. Fortunately, Peter tolerates ascites well from a respiratory standpoint; many people with ascites develop an increased work of breathing and can accumulate secretions in their lungs. Peter very easily puts on ascites when his sodium is too high, and once the fistula was diverted, the amount of sodium in his TPN was not reduced fast enough. Peter's serum sodium was within normal limits for most people, but for Peter, when his serum sodium is within normal range, he will third-space it and begin to put on ascites in his abdomen. The sodium in his TPN has been reduced and we just had labs drawn today to see where his serum sodium lies now.  He has lost a little of the weight and abdominal girth, but has a good ways to go. It will remain difficult to feed him until more of the ascites goes away, as per his history of when he has put on ascites in the past.

Though we won't know with certainty until we get to really feeding him, it appears that the surgery was successful. The fistula output is different in color, nature, and amount, and the ileostomy has gone up in volume as would all be expected.

Dr. Jennings talked to us about the next surgery being the take-down of Peter's ileostomy. The humorous part of that is he brought it up right after coming out of the OR to tell us that Peter was headed for recovery and how well the operation went. He literally had just spent seven hours working on Peter's abdomen, doing a surgery that could have gone very wrong, and was so optimistic post-op that he started discussions about the next surgery.  He was/is so enthusiastic about fixing Peter, yet very humble about his talent in making it possible to surgically correct Peter. The plan for the next surgery will unfold over the next few months after Peter gets to really eating more.  Peter apparently has an ileostomy that is fairly high up the ileum, so by taking down the ileostomy, Peter will have more ileum for digestion and then also will have use of his colon to reabsorb liquid and decrease his need for IV fluid replacement.

Dr. Jennings and Dr. Kamin have our deepest gratitude for their continued help in getting Peter better.  The nurses and Playroom Ladies (Kirsten and Amanda) have our sincere thanks as well.   And as our priest said to us as we told him about Peter doing so astonishingly well in surgery and with his recovery, "Thanks be to God. Truly, thanks be to God!"

Cheeseburger!

2011-02-23T23:20:00.000-05:00

About two months ago I meant to post this after it happened, but as things have been busy I am posting it now.

We were discussing dinner plans in the morning and Peter overheard us say that we would have hamburgers for dinner. He piped up, "Can I have a cheeseburger for dinner?" To put this in context as to why this would stand out for us, Peter's dinners consist of Stage 1 Gerber baby meat, a pureed vegetable, and a token amount of crackers/Cheerios/chips/other table food. This request was an unusual one for him. It is also one of the wishes that I have had for him for the past couple of years... to see his ability to eat expand so much that I will one day see him eat a burger.

Of course we said that we would make him a cheeseburger, and went on with the morning. Several times throughout the day he remarked that he wanted his very own cheeseburger, so I started thinking about how to make that happen. Usually we will give him a piece of what he asks for and he will lick it, taste it, nibble it, and maybe eat it. I didn't know what he would do with a whole burger, but did wonder what he would do with a whole burger too. As dinnertime approached, it hit me that I could make a tiny one and use part of a hotdog bun to make a slider-sized cheeseburger. Peter was so excited about this that he volunteered to set the table too.

Dinner came and he wanted ketchup and mustard on the burger. He examined the burger, then held it up and took a bite out of it. We were all on the edges of our seats, camera out. He loved the burger! Being short-gut, on TPN 7 nights a week, with limited food intake which is mostly pureed or liquid, he only ate about 1/4 of the burger, but he absolutely enjoyed himself. We told him we would save the leftover part for him and we did. The next morning he asked for that burger again and ate another 1/4. Lunchtime came and he wanted it again. In the end, he ate almost all of that burger.

Dinner

Breakfast

Lunch

He hasn't asked for another burger yet, but has gone on to try a little bit of ham, kielbasa with seeded mustard, hotdog with ketchup and mustard, and the Indian spinach dish, palak paneer. Boy do we get a kick out of him eating.... medical grade formula, baby food, and then break out the seeded mustard and kielbasa. His feeding therapists who worked with him from last Spring through late Fall, have enjoyed hearing of his new conquests as well.

MRI, vascular clinic, and surgical decisions

2011-02-23T22:46:00.004-05:00

Peter has his MRI last week. The MRI shows that Peter has 3 lesions in the liver, but the likelihood is that they are most likely related to the cirrhosis/fibrosis, as opposed to cancer. It is possible they have been there for years, as we haven't had an MRI of the liver since 2006 (I think), and it was apparently difficult to see even one on ultrasound. Peter moved during the MRI at a crucial point, so at some point he will need another imaging of the lesions, but for now the doctors are very comfortable that we can proceed with surgery and that Peter is not entering some kind of rapid decline. We think that it is unlikely that his liver disease has gotten worse, as the function does not suggest it is at all as bad as the biopsy or radiology tests indicate. Peter is probably borderline cirrhosis/fibrosis.

That all said, we discussed the risks of surgery and how it could affect the liver. Since our last visit, Dr. Kamin consulted with the liver docs and everyone is in agreement that Peter's liver has at least a 90% chance of pulling him through surgery and recovery without much insult to the liver. Great odds in Chris's and my opinions! There are of course real risks with that 10%, as Dr. Jennings reminds us, but there are also dangers to doing nothing or transplant as well.

The plan for surgery will be as follows. Both Dr. Jennings (surgeon) and Dr. Kamin (GI) will be in surgery. The surgery will connect the good portion of bowel above the fistula with the good portion of bowel below the fistula, so Peter can pass food all the way through without losing some along the way. That should in turn slow down the fistula enormously, as there won't be a direct passage to it. It will then truly be a "mucus fistula" and drain only what it produces itself. Dr. Jennings will have the whole OR day reserved for Peter in the event it is necessary, but this procedure should be fairly straightforward. It is the bleeding from portal hypertension and past surgical adhesions that are the big concerns during surgery, and I suppose post-op as well. The liver health comes into play during post-op recovery.

The Vascular Anomalies Clinic (VAC) also went well. The doctors feel the spider veins and red spots are most likely due to liver health versus a separate vascular condition. They are not 100% sure of that, and were very upfront about that, but for now we are going with the thought that they are related to the liver. The larger arm is likely a compromised blood flow to the arm, but as it causes no problem other than size there is nothing necessary to do for it. The hand getting bigger post PICC seems to point to a small clot or pinching of the vessel. If they can get clearance to use the laser in the OR, they may laser the spider veins on his face when he has the abdominal surgery.

Good news, good visit, ...... and good weather this time too!

Liver concerns and another cancelled surgery

2011-02-08T22:39:00.000-05:00

We recently returned from another visit to Boston which entailed more than our usual CAIR visit. This visit also involved an abdominal ultrasound to look at the liver and kidneys, a CT to look at the chest, and endoscopies of the esophagus, fistula, and ileostomy.

The ultrasound suggests that we need to follow up with a liver MRI to help further establish whether Peter's liver is declining. There is a lesion on ultrasound, which hopefully is nothing, but could be showing that the liver is entering the stages of cirrhosis, versus staying in his current stage of fibrosis. The ultrasound of the kidneys was to see if kidney stones are developing, as Peter has high levels of calcium in the urine now. His nephrologist at CHOP feels that the IGA nephropathy is still in remission, and that the calcium is likely due to the high levels of IV sodium needed to compensate for the fistula losses, or due to too much vitamin D. We hope to get the cause identified and removed shortly, and fortuately we do not see calcium build up in the kidneys.

The CT of the chest was ordered because the Vascular Anomalies Clinic physicians saw enlarged chest vessels on a portion of an old abdominal CT and want clarity if he has enlarged pulmonary vessels. Per the CT, he does. A "quick" trip over to cardiology established that the cardiologist is not alarmed, and the cardiac echo and EKG now are the same as a year ago. However, the chest may need a follow up MRI as well.... to be determined soon.

The endoscopy was the big test that we were waiting for on pins and needles, as it was to reveal whether Dr. Jennings would be able to close the now high-output fistula next week. The disappointing news is that the bowel is more complicated than hoped. He has two "chambers" in his bowel below the stomach, which both have connections to the fistula, and the fistula is physically necessary for draining a loop of bowel that goes nowhere. That leads us back to the idea of needing a bigger surgery on the bowel, trying to determine if the liver is healthy enough for recovery from a larger surgery, and whether a shunt to decompress the portal hypertension is needed or not. We essentially have 3 options it seems:
1. do nothing (but is Peter headed for liver failure now, and we are unable to advance feeds on Peter without the fistula going up in output),
2. take a chance on the surgery (but Dr. Jennings does not want to do that without establishing a reasonable chance that the liver can handle the surgical recovery, hence the liver imaging we did and are still planning), or
3. do transplant (but multivisceral transplants come with their own big set of problems and serious risks, and we are not clear yet if we could do an isolated liver transplant on Peter).

We will be returning to Boston soon for the MRI, the actual meeting with the Vascular Anomalies Clinic, and hopefully leaving with answers as to whether this child is going to have surgery to get his bowel fixed enough so that we can resume advancing feeds and begin weaning him off TPN like we did a little over a year ago.

Back on Protonix again

2011-01-11T19:21:00.000-05:00

Our trial run off Protonix hit a wall and Peter is back on it again. He did not have typical reflux symptoms while off it, but instead had increasing amounts of stomach air that would make him feel terrible until we were able to vent the air out of his G-tube. We were venting him multiple times a day, over a period of 14 days. The doctor is not certain what exactly would cause that type of symptom without further exploration, but suggested that we try Protonix again just to see if that helped Peter to feel better and get rid of the stomach gas. It worked like magic! The day before we resumed, I had to vent his stomach eight times in 10 hours, venting up to 250mL of air out of his tummy. I gave him his IV Protonix at bedtime and the next morning he woke up and said with glad surprise, "Mommy, my tummy doesn't hurt." Sure enough, we did not need to vent his G-tube at all, and his appetite was huge the entire day. He has only needed two ventings of air, minor amounts, since we resumed the Protonix four days ago.

The side effects of Protonix are what led us to want to try to take him off the medication. Osteopenia from bedrest, TPN, and hospital-life (no sunshine for vitamin D) led to his broken leg back in CHOP a few years ago, and one of the potential side effects of Protonix is osteopenia. There are other possible side effects as well, but my biggest concern at this point is osteopenia. Also, Protonix is not well-studied in children. After seeing the profound improvement that Peter had once going back on the medication, it seems obvious he needs to be on it though. I am hoping the doctor can weigh in more on the atypical symptoms when we go back up to Boston.

The vascular anomolies team is to review Peter's case tomorrow evening. We wait with mixed emotions. Of course, Boston is expecting a major snow storm tomorrow with 15 inches of snow and huge winds, so it may unfortunately be postponed.

The fistula is now putting out an average of 1300 mL of fluid a day. It is a challenge to keep the fistula empty enough as to not have the adhesive on the bag wear out too quickly, and to respect Peter's wishes to empty the bag as infrequently as possible. We empty it 5 to 6 times a day, and even then the wear on the bag has been shortened from 3 to 5 days of wear down to 1 to 3 days of wear. The doctor and we agree that trying to increase feeds would be imprudent, as that could further provoke the fistula into putting out more. So once again we are at a standstill in our ability to wean Peter off TPN, even though we had success with getting off 3 nights a week back in 2009. We remain prayerful and hopeful that the surgeon will figure out how to close off the fistula soon.

Making plans

2010-12-03T23:34:00.002-05:00

Peter's recent CAIR appointment went as hoped. We met with Dr. Jennings and Dr. Kamin together and the topic of surgery for the fistula was revisited. Peter' fistula continues to put out 3/4 to a full liter of fluid a day. Dr. Kamin is rightfully concerned that we will end up back in the hospital for sodium issues if we are not careful, and that playing hospital at home is tricky and not without serious risk, so the feeds will remain at a standstill as to keep the fistula from continuing to rise in output. Dr. Jennings is in agreement then that we should revisit surgery if we are in such a tenuous place in the medical management of the fistula. Of course the same concerns about surgical risk exist as they did before, mainly centered around post-op recovery. Dr. Jennings came up with another idea as to how to close the fistula as he was examining Peter, which would be to close the skin over the fistula, as it seems that the drainage would continue to travel further down through the intestine if it were just not allowed to exit. If I understand correctly, he suspects that the intestine where the fistula is presses up against the abdominal wall, and that we could use the abdominal wall to close the fistula instead of actually trying to cut out the part of the intestine which contains Peter's very vascular fistula. This would greatly reduce the risk of bleeding since Peter has a huge collateral vein that connects to the fistula, and it would be a less complicated surgery by far. The downside is that there would be a good chance that the fistula would end up coming back at some point. The plan is to admit Peter for more abdominal examination which would involve scoping the fistula and a more thorough manual , evaluation of the fistula. Drs. Kamin and Jennings would team up together to evaluate and decide if this would potentially work. We are hoping for this to occur sometime soon in the new year.

Additionally, Peter continues to have small red spots on the left side of his chest, his left arm and hand are noticably bigger than the right, and the spider veins he has on his face seem to be more in number on the left than the right. The spots have been continuing to slowly develop over the last year, and there seems to be one now on his lower left eyelid margin. Dr. Kamin has suggested that we consult with the vascular clinic at Children's Boston, which is world-renowned. We have agreed that this sounds like a good idea and are waiting to hear from them as to what steps we need to take to investigate if this is a vascular abnormality or just a strange phenomenon for Peter whose medical progress doesn't follow the norm anyway.

Apparently the American Academy of Pediatrics has just come out with an officialy statement that probiotics should not be used in children with a central line, due to infection risk. Dr. Kamin brought that up as he felt it necessary that I know that, however he was also appreciative that Peter has been on probiotics for two years and has had no infection, so he is in agreement with our continuing to give Peter probiotics to keep intestinal bacterial overgrowth at bay.

We are also attempting to take Peter off Protonix. He has been on it for years, but there are risks to staying on proton-pump inhibitors, with one of the risks being to bone health. He does not show signs of needing to stay on it for reflux anymore, so we are starting a slow reduction and hopefully successful cessation of the medication.

Will post more about the plans for surgery and vascular clinic as we know more.

Running!

2010-11-12T23:19:00.002-05:00

Peter's nurses, therapists, and family have put countless hours into Peter's physical therapy, and look at the results! He is having such a fun time running too!

Happy 5th Birthday Peter!

2010-11-12T23:17:00.000-05:00

An overdue update

2010-11-12T23:12:00.003-05:00

I can't believe the last update was in July. Time has flown by! The biggest news is that Peter had his FIFTH birthday last week! It was wonderful, and our favorite part was his understanding and excitement about having a birthday. He had some healthy anticipation and did much of the planning for his big day, including making the decision that he would prefer to have his candles placed on a cake instead of the food he prefers to actually eat, which is mint-chocolate-chip ice-cream. He also wanted to go to Mass and to Longwood Gardens, which we gladly honored. Peter had a great time opening cards and gifts this year too, including one from the Coram team that prepares his TPN week after week after week!

The fistula output has returned with more volume than he had last Spring when we were talking about doing surgery to stop it. Putting on the huge amount of ascites is what likely slowed it down over the summer, but now that the ascites is back off, the output has gone up, even with less feeds than he had in the Spring. Peter has been on weekly labs for the last two months or more, as we try to keep electrolytes in balance. We are supposed to meet with the surgeon again when we go back up to Boston later this month, to see if we should revisit the idea of surgery. I don't know what else we can do besides surgery, as there seems to be no way to get Peter off TPN if we can't advance feeds due to the fistula output. The last discussion about surgery ended with a plan where he would be scheduled once he is off TPN for several months, but we can't even get enough in enterally to stop TPN for one night, let alone 7 nights a week. We can actually see and/or smell the enteral formula exiting the fistula, off and on most days.

Peter also has developed more red spots on his left upper chest and neck. We had noticed these towards the end of last year, and now there are more, but no one is sure what they are. His spider veins that are along his lower jawline seem to be more apparent now that his summer tan is going away. Hopefully they are not actually increasing.

Now back to more good news: Aside from the fistula going awry and the spots and spider veins, Peter is doing amazingly well. He is being reevaluated by his therapists to see if we can decrease his therapies now, from once a week to only once or twice a month instead. He is now truly interested in things like Playdoh and trucks and coloring, whereas a few months ago it took a good bit of encouragement to spark even some interest in doing those things.

He also took his first bathtub bath since he was about five or six months old. He screamed the whole time, and the next time too, but afterwards stated that he liked it and is agreeable to another bathtub bath next week. We have a PICC line protector which prevents water from getting to his PICC line and makes a bath feasible. It has taken a long time to convince him to wear it so he can get wet, and to be honest, it took me a long time to feel comfortable with trying a bath with Peter, as the risks of infection go up dramatically if the central line gets wet.

Peter is also learning to ride a bike with training wheels, he is learning to swing, and he has started taking interest in using his little outdoor tools to help his daddy when they are outside together. He is also running now, and he likes it too! To top things off, and remember, he has just turned five, he knows all of the states, by shape and location, and their capitals.

My favorite part of all of the changes though, is that Peter has become very outwardly affectionate with us. He comes up a couple times a day just to give a hug, or to say, "Oh, Mom!" and grab my hand for a loving squeeze. I smile even just as I am thinking about it!

Some new photos, finally

2010-07-17T23:20:00.002-04:00

Peter has had a love for violins for the last year or more.

Seeing a violin stops him dead in his tracks and he will then

declare that he sees one and insist that you also verify it is such.

We decided that it was time to go ahead and get him one.

Now we need to find a violin teacher!

This summer (2010) Peter has truly enjoyed the outdoors so

much more than he did last summer. His tolerance for

heat is vastly improved and he is able to spend long amounts of

time outside. His passion this summer is

blowing bubbles... over and over again.

He looks for shapes in what he has blown as well:

an Elmo head, Ten apples up on top, a bear claw...

This is Peter with his contagious grin. It was taken in January 2010,

when his ascites was at its absolute lowest.

With his ascites (intentionally) up very high right now,

his cheeks are very large and his face is overall puffier.

Puffy face or not,

that smile is as genuine as they come.

Surgical plans postponed again

2010-07-15T19:07:00.003-04:00

Sigh. It is hard to know what to hope for - surgery or no surgery. And, it is hard to know whether to be happy that the decision to postpone surgery was made based on the fact that Peter is doing so well.

For a child with short gut, Peter IS doing well. He is out of the hospital, labs are stable, his ostomy outputs are stable, he acts like he feels good, he is energetic, and we are able to advance his enteral feeds again. However, he is still on TPN 7 nights a week, has a central line to administer the TPN (he fights the dressing changes terribly and there are very big health risks if the site gets infected or wet), we have to drain his stomach every night or else he vomits, and he definitely is "different" from other children his age both in appearance and behavior... not to mention the lesser issues that come up in managing two ostomies.

The goal of surgery at this time was to stablilize his fistula outputs (but not necessarily close the fistula) and allow for us to increase his feeds and decrease his TPN dependence. Once off TPN, his liver will likely begin to heal and he should be able to tolerate bigger surgeries to close the ostomies. Now that he is accepting more feeds and the ostomies have been stable through it, it seems that he is accomplishing the surgical goal without the need for surgery.... at least for now. So we will take off some of the ascites, but not all of it in the event that the ascites is what made the fistula settle out somehow (maybe by pushing on the bowel and altering the flow). The hopes would be that we keep increasing feeds and outputs stay low and we get back to where we were before January 2010's admission. If we end up back where we were in January and can't control the fistula, then surgery looks like it will be imminently necessary.

If we keep outputs stable, then Dr. Jennings feels that the bowel closures (getting rid of the ileostomy and the fistula/jejunal ostomy) can be planned for age 7 or 8. Boy does that sound a long way off! He has seen pediatric patients settle themselves out and redirect their issues before. We have seen this in Peter ourselves too. The duodenostomy was very much out of control about two years ago and then it just suddenly started closing off to a few milliliters out per day. Perhaps this is what Peter is doing with the fistula now. Only time and advancement of feeds again will help us know this.

Of note, the abdominal ultrasound today shows that the bowel is not adhered to the abdominal wall as was feared. That is good news for surgery whenever it occurs.

AND, we are going to try the Farrell bag by Corpak that Susan in Canada told me about in the comments on the last post. Hopefully that will help us to feed Peter and vent him overnight using the gastrostomy that he has. We've not been able to successfully feed him more than 15 mL ovenight since the ascites was put on, due to the need to vent instead. It looks like a great product.

Big belly, plans to unfold

2010-07-01T23:11:00.000-04:00

Peter hit his ascites goal weeks ago. We have been maintaining an abdominal circumference of 67 to 68cm, occasionally hitting 69cm. He is four and a half years old, and had been wearing size 5 overalls to accomodate his belly, prior to trying to increase his girth. He is now in a size 7/8 overalls due to the increase in his belly. He is still unable to be fed overnight, and needs to be vented through his G tube instead. His face is still puffy with the extra fluid. His hydroceles repair has withheld through all of this extra fluid, but he does appear to have two bilateral areas of swelling above the scrotum now, seen when he exerts. It will be good when we can stop needing him to hold onto his ascites.

The plan to do surgery is still uncertain. We are going up to Boston soon and will meet with Dr. Jennings, Dr. Kim, and Dr. Kamin to discuss exactly what to do. We are set up for surgery in late July if the best plan seems like it is surgery. Before that talk, we will have an abdominal ultrasound to assess where the ascites is accumulating in the belly. Hopefully the abdomial fluid is causing expansion in a way the the surgeon feels is helpful.

To add some confusion to the overall picture, Peter's fistula output has gone down since the ascites has gone up. To help understand this, we have now started trying to increase daytime feeds again to see if the fistula output goes up as we increase feeds. If the output does not go up much as we increase feeds, then it may cause the doctors to ponder whether surgery is necessary at this time. Recall that the increase in fistula output was the cause for the consideration of surgery at this time. I am guessing though, that if we lowered Peter's ascites again, the fistula would pick up again. What I mentally picture is a pocket of fluid that pushes on the intestines in a way that pinches off the fistula opening so that only a little fluid comes out, and when his ascites is less the opening opens up further. The docs seem to picture that possibility too, but we have not captured that in imaging tests, so it is hard to know. It could also be that Peter had some kind of GI bug that threw his gut out of whack back in December/January, and that with the reduced feeds and with time, it settled out and that is the cause for the fistula output going down. Hopefully we will get to advance the feeds enough before we go up to Boston, that we can see if the change in feeds affects the fistula. We are hoping that the surgery can happen during the summer when the cold and flu virsus levels are low. If we have to return home again and wait further to see how the fistula performs with addition of more feeds and reduction of ascites again, it looks like we will not have that summer advantage, and there will be more waiting as we watch to see how feeds and fistula output go.

We count our blessings that Peter is the pleasant, happy child that he is. It is hard for us to be patient with all of the uncertainty sometimes. In his sweet little ways he gets us through those times and reminds us to enjoy the time we have now. We went blueberry picking today and it was just great! (Of course, I left the camera at home though.... again.)

The Alternate Bowel Plan

2010-05-28T23:33:00.000-04:00

We discussed this plan initially while up in Boston, but I haven't posted about it because no one was sure if this was the approach we would take. The plan is to create more ascites in Peter's belly to prepare him for one of the abdominal surgeries that I described in the last post. Ascites is basically fluid accumulation in the abdomen, and it can make the abdomen protrude quite far. Usually we are trying to keep ascites off Peter. He is quite prone it, but by monitoring and adjusting sodium and fluid, we can keep it in check.

We are now trying to take advantage of his tendency to putting on weight in the belly so that there is extra abdominal skin when he goes for surgery. One of the many surgical challenges that Peter faces is the possibility of not being able to close the abdomen back up after surgery. Peter was unable to be surgically closed after he suffered from abdominal compartment syndrome back with his initial surgeries. His little body spent months growing skin over a mesh dressing that was placed over his intestines. That area is now a patch of scar tissue roughly three inches by five inches, and the muscles underneath are still about 4 cm apart, where they should actually be touching. The big issue with leaving the abdomen open like he had to, is primarily infection. That is a huge risk, especially when there is an ileostomy present as well. The development of a fistula is another issue, as we found out when Peter developed a fistula right in the middle of the scar tissue, where a little piece of mesh was poking out. If we can expand his abdomen enough now, then he should stand a better chance of post-operative closure.

It is a novel idea to do this using ascites, apparently. The development of full-blown ascites carries risks such as difficulty breathing, general discomfort in the abdomen, and infection of the ascites fluid itself. However, Peter has had more and less ascites all along, and generally he seems to weather it well. We are all watching him carefully as we begin trying to expand his belly now. He started at 61cm around about three weeks ago and he is now 64cm around.

It is hard to watch him get bigger in the gut and not want to take that fluid back off him. He will likely look more and more puffy in the face as this continues, and I imagine he will end up wearing nothing more than big T-shirts and his diaper, instead of his cute little overalls that cover up his ostomies and slightly big belly usually. But, as it has the potential to provide a big advantage to his surgical recovery... please pass the salt!

Surgical plans changed

2010-05-05T20:08:00.001-04:00

Dr. Jennings met with us today. Peter was scheduled for surgery tomorrow - a shunt procedure to decrease portal hypertension so that we could proceed with bowel closure several weeks later. That surgery is now cancelled, as a decision made together with him and us. The concerns with the shunt are that the liver may not receive enough blood for good perfusion, and that the liver may be taxed too hard with such a major surgery at this time. Peter's biopsy last month shows that Peter's liver disease has not progressed, but that he still has some bridging fibrosis. That makes his liver more vulnerable in recovery from major surgery.

Our plans now are going to involve a different surgical approach in a few months. Dr. Jennings will likely try to attach the lower section of bowel to Peter's stomach so that food can pass through the bowel and less come out the fistula. Or he may try to close the fistula, but the fistula has huge collateral blood flow and tying off the circulation may increase portal hypertension, adversely affecting the liver. (Hence one of the reasons we had been planning for a shunt procedure in the first place.) Both surgical approaches are on the drawing board at the moment, with a leaning towards the first approach.

The thought is that if we can utilize the bowel enough to get off TPN, the liver has a good chance of healing. Then the bigger surgeries required to finish rerouting the collateral circulation and closing the fistula may be performed at some point later on if possible. If the bowel closures cannot be performed, even if we can just get Peter off TPN it would ultimately help his liver and be better for him.

None of this is an easy solution, and plans of course may detour again, but we are all optimistic that this is all in Peter's best interest. We were again reminded by Dr. Jennnings that there is no other person in the world like Peter. Between the bowel, the liver, the portal vein clot... he is quite unique. The fact that he is not only alive, but is alive and thriving, is truly remarkable. Omegaven saved his liver and has undoubtedly extended his life on TPN. Without Omegaven he would have either needed to go on for transplant or would have died.

So, as plans for a different surgery unfold, more will be posted. For now, we will return home tomorrow and hopefully get around to posing some of the photos that are long overdue on this site.

March and April

2010-04-19T21:38:00.001-04:00

Lots of "boring" news, but the really exciting stuff will be coming soon when we go to Boston for SURGERY! Peter went up to Children's Boston last week for a liver biopsy and CT to help plan for abdominal surgery. The fistula continues to put out more and more amounts, requiring continued weekly electrolyte labs, often followed by yet another tweaking of his TPN (IV nutrition) recipe to keep on top of the electrolyte changes produced by the fistula outputs. Dr. Jennings, one of the surgeons at Children's, met with us when we went up in March, and it seems like surgery is the best solution for Peter at this point. It has been a long hope to reconnect the bowel, but for Peter a reconnection is not so simple. The first thing that will need to be done is the placement of a shunt which will bypass the clot in the portal vein, thereby decreasing the portal hypertension that Peter has in his abdomen. That will then assist the gut in its circulation and overall health and ability to digest food, as well as decrease the tendency to bleed as much during surgery. I don't yet know the plan from there, but discussions have included surgery to close the fistula as well; I am not sure of the timing on that yet.

The PICC line dressing was solved with another style of statlock that I found online. It has a deeper, wider indent at the top, which allows for more clearance room between the insertion site and the statlock. With that, we decrease the risk of contamination of the PICC site, as the statlock does not rub up near the insertion. If I get a chance to take and download a photo I will. (The photo below is from an earlier post and it has the old statlock shown.) The new statlock style, if anyone is looking at this and wanting to try it to resolve a similar situation, is PIC0220, made by Bard. The other thing we did to make the dressing change less subject to contamination, was to add another set of sterile gloves to our dressing change, so that the line is kept as clean as possible. I don one pair of sterile gloves during the removal of the old statlock, after removing the tegaderm with nonsterile gloves. Then I put on a new pair of sterile gloves to clean the line and put on the new statlock. We use a third pair on my husband who holds the catheter in place at the insertion site as the old dressing is being removed and I am changing gloves, but before it is cleaned with chloraprep, as Peter is still prone to wanting to move around whether the PICC line is secure or not.

On a non-medical front, Peter is very close to running now! He runs like a toddler at this point. It is more than a brisk walk, but not quite the run that a 3 or 4 year old would use. The look on his face as he runs is priceless. He is very pleased with himself and his "great speed." The cheers that his family and nurse give him certainly add to his satisfied self-image... as it should be.

I am having technical problems adding photos tonight, but hopefully can download his running video soon, as well as some new photos of Mr. Peter.

The new PICC photo

2010-03-01T22:56:00.001-05:00

We don't like the new PICC afterall

2010-03-01T22:51:00.002-05:00

There, it's official. We do not like the new PICC that Peter got in January. I do like a PICC line in general, but not this new one. The biggest frustration with it is the placement. This may be a very boring entry for anyone who does not have a PICC line, but for someone familiar with PICCs, perhaps it will be of interest. I do need to vent this frustration, so I am posting it.

For the last three years, Peter's PICC lines were secured with a little adhesive device called a Stat-Lock, which was located about an inch below the actual insetion site of the PICC line. It was a good set-up, as the Stat-Lock was capable of lasting up to a month and did not need to be kept sterile since it was so far away from the insertion site of the PICC.

The new line has the Stat-Lock actually just one or two millimeters from the insertion site, surrounding the site itself, and now needs to be changed every week with the dressing change. The Stat-Lock device has these little "doors" that keep the PICC line from popping back off the device, and these tiny movable posts on which the PICC cross post need to be landed. Somehow this is all to be kept sterile as we are opening and closing the little doors to remove the old Stat-Lock, and then opening and closing little doors as we put on the new Stat-Lock. It can be done on a mannekin arm or on an adult that stays still, but on a four-year-old child, it gets a little tricky, even with another adult immobilizing the arm and Peter's body. To remove the old Stat-Lock, we need to lift up the PICC line, which then tries to work its way out of Peter's arm while we frantically work on keeping it from sliding out, sterile gloves becoming dangerously close to non-sterile, if not truly non-sterile despite our best efforts. It is like trying to manipulate a wet noodle as it wants to slide out and we are trying to push on it to stay in. Once the entire site is cleaned, we can put the PICC line down and hold it more securely, but then we have to lift the PICC line again to place the new Stat-Lock on... trying to be sterile, landing it just a millimeter or two away from the sterile insertion site, wet noodle thing happening again.

The whole procedure is ridiculous in light of the set-up we used to have. We have had so many people remark on how well we maintained his old site and how remarkable it was that he didn't get PICC line infections. With this new line sliding in and out during dressing changes and with the Stat-Lock so close to the insertion site, I will be the one amazed if we are able to keep Peter's line infection free, or from just sliding on out during one of our dressing changes. We are hearing from some of Peter's nurses that this is the current procedure for PICC lines, with the Stat-Lock where our new one is. I will not be surprised to see that the rate of infection also rises with this current trend.

GI plans

2010-02-25T22:29:00.000-05:00

It looks like we will plan to do a fluoroscopy study late next month to find out what is going on inside Peter's GI tract. The fistula continues to put out more fluid than it used to, even with the feeds less than half of what they had been when we went up to Boston last time.

A fluoroscopy study on Peter is never easy because he has several places where the contrast fluid leaks out after it is put into his G-tube. The leakage of fluid into his dressing and into his ostomy bags then makes it impossible to see what is going on inside. I am not sure how we are going to work this out, but hope if it involves the removal of ostomy bags during the procedure that we are able to keep the PICC line clean!! We've managed it before, and with many prayers will hopefully manage to keep the line clean again. Dr. Kamin and Dr. Jennings will be there for the one to two hour procedure, and possibly Dr. Kim as well. Dr. Jennings and Dr. Kim are the surgeons who are familiar with Peter's anatomy.

Peter is still on TPN 7 nights a week, as we can't advance his feeds to where they had been before. We are managing to keep his sodium levels stable though, only needing to add a little 1/2 normal saline here and there when his outputs exceed his new baseline.

Please keep Peter in your prayers as we sort out his fistula issues. We are praying for test results that show us that it is time to close the fistula, as well as the doctors finding that it would not be as difficult to surgically close as feared.

An unexpected admission

2010-01-25T22:45:00.003-05:00

Peter's CAIR appointment went well, but what we weren't counting on was an admission! Peter's blood sodium was at a value considered critically low, so he was admitted for correction of that low sodium. Apparently correcting hyponatremia (low blood sodium) too rapidly can lead to swelling of the brain, so there needs to be a gradual increase back to normal value, done in an ICU setting, at least until the level is high enough to require less labs and less monitoring.

Peter had been acting well, so the low sodium was a surprise to EVERYONE. The only real change in him has been a fairly high increase in fistula output over the past few weeks, continuing even after we changed to Elecare again (discussed in the last post). Peter dropped two and a half pounds over the last month, some of it is likely true weight loss since we had decreased formula concentration and amount without going up in TPN. More of the loss, though, seems to be the ascites that has disappeared over the last several weeks as the fistula output went up. The fistula output seems to have a high amount of sodium, and that has undoubtedly been the main cause of his low serum sodium.

There are a few reasons that the fistula could be putting out more, but to diagnose the cause with certainty would involve radiation to image the bowel. The prudent thing then is to do some trial and error with feeds and labs to monitor the sodium. If that doesn't work, then we will need to do further testing.

The trick is to prevent another bout of this hyponatremia. As such, the plan now is to go on TPN seven nights a week again, unfortunately. The reason for adding back all three nights off of TPN is because we have had to drastically cut back on feeds in an attempt to get the fistula to slow back down. He is getting just 10 mL an hour of formula now, whereas he had been up to 850 plus of formula a day. The hope is to slowly advance each week and to keep the fistula output from advancing with the formula increase.

The plan also is to keep the ascites off Peter's abdomen now that it is gone. He has had ascites even before the AVM was found. It is one of the symptoms that you often see with liver disease. With ascites, there is too much fluid in the body's abdominal tissue, and sodium affects that. For Peter, "too much sodium" is actually what would be a normal value for most people. When we see his belly get larger, we can tell without labs that his sodium levels are rising. It will be tricky to get his sodium leveled out without his ascites returning. It is so good to see his body without the giant abdomen that he has had for so long, and we all feel it is best for him to keep it from returning... if we can.

I have to say that if ever there was a "better" time for this to have happened, it was during this trip. We had already planned to spend the night in Boston, so we did not have to turn around and drive several hours back. As much as it is stressful going to the ER at 9:30 at night with a four year old who is hooked up to TPN and another IV pump that is the size of a double toaster, and an enteral feeding pump, along with an infant, it would have been so much worse had we needed to drive several hours back or needed to go to some other nearby ER that didn't know him at all. Dr. Kamin graciously called in to make sure that things went smoothly and that all parties involved were following the same plan for Peter. Even on vacation he called in to make sure all was going well. Thank you, Dr. Kamin!

Something of interest to add, is that during our ER trip, we encountered a Fellow who had taken care of Peter when she was a Resident at CHOP. She was just as surprised to see Peter as we were to see her. She remembers when he was at CHOP early on when the AVM had not yet been found, as well as when he was in the ICU at CHOP having bleeds requiring emergency transfusions. And she recalled that we left for Boston from CHOP's ICU to Boston's ICU so that we could get Peter on Omegaven. Now he is four years old, talking, eating, and one happy kid. We emphasized that Omegaven was the medication that helped him be able to get to this point, and that having one physician continually oversee him versus rotating doctors, also got him to this point. Hopefully she will remember these things as she goes on in medicine, and maybe they will be helpful to someone she cares for down the line.

One last thing.... Peter got his new PICC. It is a beauty as far as PICCs go, a little more bulky with the clamp, but it is a single lumen this time, so less bulk overall. Let's hope this one lasts a very long time. It works, so we are off to a better start than with the last PICC.

Increased outputs and formula changes

2010-01-09T16:13:00.000-05:00

We are back to Elecare from Vital Jr in effort to see if Peter is having some intolerance to Vital Jr. Peter's fistula and ileostomy outputs have drifted up over the last couple of months, and he has had 3 bouts of really high outputs with 2 needing him to go on Pedialyte for 2 or 4 days until it settled out. We were emptying his fistula bag every 1 to 2 hours at some points!

The other questions as to why his outputs have gone up so dramatically would be whether he is having bacterial overgrowth (SBBO)or if this is an anatomical change. We see that the fistula's output is consistently higher than the ileostomy over the last few months, but that did not occur anywhere near the timing of the gastrostomy repair which ended the stomach losses. Peter doesn't display his SBBO symptoms from the past though, so hard to say it is that. We did withhold his probiotics for two separate weeks in December, so it could be overgrowth. We are adding kefir and daily plain Stonyfield yogurt to his diet to assist with the usual probiotics in the event that he needs an extra boost of probiotic. Peter's history of reactions to enteral antibiotics for SBBO keeps us all hesitant to treat him empirically with a round or two of antibiotics. If anyone wants to know what he uses, we are still on VSL#3 and florastor, rotating one week on one and one week on the other, usually without a break between.

The good news seems to be that with the caloric decline that occurred by going back on 26 calorie Elecare from 30 calorie Vital Jr and by decreasing the volume from 950 to 650 per day, is that Peter is HUNGRY! He is remarkably interested in nibbling on crackers now, and as such is working on his feeding skills without prompting. And, although he has lost about two pounds with the diarrhea and with the reduction in calories, his new-found hunger may be showing us that his gut has been working well absorbing Vital Jr all along, despite the increased outputs.

Because he has lost weight, is showing us that he is hungry, and is continuing to slow down with his fistula output, we are going up in Elecare volume over the next few days. Hopefully he will regain the weight and keep the fistula output down. We will reassess where he is when back up in Boston.

Peter's new PICC

2010-01-04T23:15:00.002-05:00

The week before Christmas Peter needed to have his monthly labs drawn from his PICC line. Routine labs, routine procedure. Not this time. This time the PICC would not give more than 2mL of blood for labs and we needed about 20mL. So after some talks with the homecare nursing team and Children's Boston, we agreed that Peter's line could be tPA'd in the home setting now that he is bigger and no longer having active bleeding episodes that he had in the past. It was successful, got the blood needed for the labs, and then the line stopped flushing completely, and upon closer inspection, it had little particles floating in it. Everybody's guess is that the hard crystallized build up that we have seen in the line since last Feb, must have reacted with the tPA and started to flake off. Ugh! Since he has a dual lumen PICC, we decided to use the other lumen for his TPN and to get the line replaced. Monday the 21st of December we went to CHOP for a replacement PICC. It was just to be another rewire, which should be a reinsertion of a guidewire into the old PICC, removal of the old PICC, and then insertion of a new PICC over the guidewire. It should just take an hour once he goes into Interventional Radiology. We have had this done a few times now. Well, things started by a delay of three hours before even getting taken into the IR room, but no one telling us that the delay was that long. We kept being told we would be going there shortly. Three hours of keeping a wiggly four year old entertained is exhausting, and there was me AND one of Peter's nurses there! Then once he had been in IR for well over an hour and we still hadn't heard how things were going, I asked the nurse. She called IR and then they told her that the IR doc was in the process of placing the PICC line into the other arm because the rewire had been unsuccessful. When Peter was brought to the recovery room, we were told by the doctor that she had tried very hard to rewire, but the vein that the PICC had been in started spasming and was narrowed, so it was time to give that vein a rest. Poor Peter had to be given more sedation for that longer procedure though, so we ended up staying at CHOP until 8:30 that night and were the last to leave the IR recovery area. He was beside himself on the ride home - tired, and very thirsty. None of us even thought about his fluid replacement over the day other than a 300 mL bolus of normal saline during the procedure. Fortunately we had some Pedialyte to offer and were able to pick up more at WalMart on the way home.

Now for the real bombshell... the new PICC line has a LEAK!! At first I thought I was just being careless and getting Peter wet when flushing the line. Then I thought that perhaps the cap was faulty. Finally, we took a long hard look at it and unbelivingly, watched a drop of the flush solution drip out of the line as we flushed it. We flushed again, and there was another drip of solution. We took off the dressing and found that the dressing edges were wet from this leak, and the stat-lock was holding little droplets of fluid inside. The 'good news' is that only one of the two lumens has a leak, so once again we can give TPN until we can get a new line put in. Boston agreed to our request that they redo the PICC for us when we are up there again for Peter's intestinal rehab visit. Meanwhile, the red lumen with the leak is clamped off tightly, to be unused and unflushed until we replace it in a couple of weeks.

Halloween and birthday photos

2009-11-27T17:30:00.006-05:00

Our miracle child celebrating his fourth year! There is much to celebrate!!

"Bob 'd Builder" for Halloween

Back from Boston

2009-11-21T21:29:00.003-05:00

NORMAL bilirubin - direct and indirect!
NORMAL albumin!
NORMAL hematocrit! (hemaglobin still a little low, but pretty close to normal!)

Wait, there's more....

Another night off TPN now, for a total of three nights off TPN per week!

We had a very good checkup in Boston... good labs too! Our biggest focus this time was that we still need to work on Peter's vitamin D status. Even though we have had him at twice the usual dose for the last 3 months or so, his stores still do not seem to be high enough. He will now go to 600 IU of vitamin D per day. During this trip we found out that Dr. Lo, one of the doctors that we see in CAIR clinic, has his PhD in vitamin D metabolism. That sure puts my mind at ease as we assess Peter's vitamin D, calcium, and overall bone status to assure he gets as far away from his past osteopenia as possible.

A new age, feeds, and the new tooth

2009-11-13T12:58:00.005-05:00

Peter has turned four years old! Peter's birthday is All Saints' Day. It was an exciting birthday for all of us, as this is the first year that he really seemed to understand and care that it was his birthday. He even had gift requests - books, puzzles, and chapstick (he is a mouth breather and his lips get very dry if we don't keep up with ointment and chapstick). He decided upon pumpkin pie as his "cake," since he has to have pureed foods still. Better than the pumpkin pie was the whipped cream, though. He has asked for whipped cream again.

Photos of birthday and Halloween to follow soon.

On the medical front, Peter has made good strides (great strides for Peter) with his feeding increase sice we were in Boston nearly two months ago. He has gone from two ounces of baby food daily to five ounces of baby food daily. Vital Jr. has increased from 650 mL per day to 850 mL per day. He is taking a little over half of the Vital Jr. formula overnight via pump, and the rest is by mouth. All of the baby food is by mouth. He is even eating Gerber Puffs and Gerber Li'l Crunchies now. He ate a whole can of Gerber Li'l Crunchies this week in only about 5 days. They look like cheese puffs, and he ate about 85 of them over 5 days!

The new tooth is beginning to emerge. The top is........ BROWN! We are disappointed, needless to say. We are praying that the rest of his teeth will look better. The dentist told us two years ago that the only way to "fix" the color of teeth stained by bilirubin is to veneer them. Bleach will not fix the color. Hopefully they will be strong... more important than color in the long run.

Lost a tooth!

2009-10-17T21:21:00.003-04:00

Peter just lost his first tooth... and he is two weeks shy of 4 years of age! It is his front lower left tooth, which makes its loss "normal" although it seems an early age to lose a tooth at all. We noticed it was loose only a handful of days ago, and when I brushed his teeth tonight, it just popped right out. We are praying that his permanent teeth will not have the gray, green, and brown colors that his baby teeth have from the longs months where his bilirubin levels were so elevated. No tooth is crowning yet, but I guess we'll find out soon.

Two nights off TPN per week!

2009-09-30T21:01:00.004-04:00

Peter had his Boston check-up last week. It was a very speedy up and back trip spanning about 40 hours total... would have been 38 but traffic got pretty bad around 10:30 pm on the return trip home. During the trip we stopped to see Great-Nana in Connecticut, which was quite nice. Once we got up to Boston, then we had one comlete ostomy change (entailed all 3 ostomy bags needing to be changed), another duodenotomy change, two enteral feeding administrations along with venting and pausing multiple times, and two TPN administrations, one of which was in the van - I do wonder what people think when we set up his TPN while sitting in our parked van in the middle of the parking lot at dusk! And in between there was the unloading and loading of a van full of ostomy and TPN supplies as well as clothing and food and siblings. A family vacation like no other!!

Peter's weight and height have both gone up enough that we are now able to have only 5 nights of TPN per week! Although we still have 2 nights per week of IV fluid, it feels so liberating to not have to mix the TPN bag or to worry about changing over the Omegaven bottle at midnight. And even better than losing another night of TPN is the fact that the weight gain is because of his gut working rather than TPN alone.

We did some other tweaking of his care, including removal of extra vitamin K and extra vitamin C from the TPN (two less meds in the TPN bags is another liberating move). The reduction in vitamin C was done to hopefully reduce the oxalate we see in his urine so that he will not develop stones in his urine. The vitamin K was removed since we have seen his bleeding times stable over the last year and a half. If we see bleeding times increase, then we can always add the vitamin K back in. We are also going to reduce the amount of calcium he receives in his TPN to decrease the calcium losses we see in his urine. We possibly are just giving him too much calcium and this would be an obvious fix if that is in fact the cause for the calcium in the urine.

The bad news from the trip is that his portal hypertension does seem like it is worsening. While the esophageal scoping done last trip showed that the varices are no worse, CT and ultrasound images of the abdomen show continued pockets of ascites and an enlarging spleen. The problem seems to be from the continued clot in the portal vein versus a worsening of the liver, which is better news than if it involved the liver. The thought seems to be that we will currently do nothing, but we can try a decrease in IV sodium to keep him at 2mEq/kg/day to see if that helps at all. If Peter's platelets continue to drop (they have lowered some over the last few months, but are not scary low) then we may see that we need to act surgically to lower the portal pressure with a shunt. This would help divert the pressure away from the spleen and ultimately then help keep the platelet numbers up. If we see intestinal bleeding return or are unable to advance feeds on Peter, that also may indicate that we need to address a surgical shunt for the portal hypertension. Otherwise we will just keep watch, as he has been doing very well with feeds and no bleeds. Since he has intestinal scarring (probably severe), portal hypertension, is missing part of his liver, and he is still only about as tall as a yardstick, surgery is very tricky, especially a shunt procedure deep inside the body.

We will have to address the portal hypertension before any reconnective surgeries. The plan for any surgery (including the shunt) becomes more contingent upon how truly necessary it is. With the ostomy bags now leaking about every 2-3days now, that news hits a little harder than it normally would. We had been hoping to start talks to close up ostomies in the spring, but that is more for convenience than out of necessity for digestion, so likely closing them up will have to wait. Is this terrible news? No. Disappointing, yes, but not terrible.

Peter certainly seems to be doing well despite the portal hypertension. He had at leaast 10 people in his little exam room for his clinic visit. Several came just to see how well this little miracle boy is doing because he does look that good. And Mr. Peter hammed it up well for his audience. We are blessed that this child does not realize that he has an illness and that he is very happy with himself and in general.

Pics from July Boston trip

2009-09-04T21:32:00.008-04:00

This is Peter in pre-op, looking at the smiley face Dr. Jennings drew on his index finger and his big toe. Those smiley faces produced three days of amusement for Peter!

This is the finished dressing with nursing pad and tegaderm covering the dressing pictured below.

This is the old dressing with bottle nipple over allevyn, over aquacel, over zinc paste.

When you consider that this dressing got changed one to three times a day b/c the leaking was that bad, you can see why we wanted to get that gastrostomy site fixed! Peter and we are all happy with the new, no-dressing-needed site!

Boston updates... from July!

2009-09-04T20:42:00.004-04:00

Peter went up to Boston at the end of July for SURGERY! These were not the intestinal closing surgeries that we hope for... one day. But they were surgeries nonetheless. The big deal about "surgery" for Peter is that we had been told in the past the he is a terrible surgical candidate due to his portal hypertension (great bleeding risk), elevated INR (another bleeding risk), and history of poor healing from earlier surgeries on the abdomen. Dr. Jennings has discussed Peter's surgical risks and possibilities with us before (see old posts). As such, we all felt it was a good idea to do these surgeries to help make Peter more comfortable as well as to see if he heals better then he has in the past and to see if the bleeding turned out to be terrible or not.

The surgeries that Peter had done were to move the gastrostomy to a new site, close the old gastrostomy, and close the bilateral hydroceles. This all came about because we were going to be putting Peter to sleep for an upper endoscopy (EGD)to evaluate his esophageal varices (which are due to portal hypertension). I will try to post pictures of his last gastrostomy site and how we had to dress it to keep it from pouring out. The old dressing consisted of aquacel, allevyn, zinc paste, part of a nipple from a baby bottle to stabilize the G-tube, a nursing pad over all of that, surrounded by tegaderm all along the borders. This would be changed one to three times a day, depending on leakage amounts. Our new beautiful G-tube only needs tape to keep it secure, no dressing, just a little skin prep or zinc to protect from scant G-tube ooze. The old site healed remarkably well with some stitches to close the stomach opening and wet-to-dry dressings to close the rest of the hole. It is just a tiny scar now. The hydroceles healed well too. There are two perfectly-healed thin scars from that procedure. Dr. Jennings and Dr. Kamin said that the amount of bleeding was not much, especially considering that he has portal hypertension. Also, the EGD revealed that there has been no increase in the size of the esophageal varices since the evaluation two years ago.

We have started nighttime pump feeds with Peter...very slow rate to begin with. (Elevating the head of a toddler bed-- to keep him from lying flat and getting formula in his lungs while he is tube-fed-- is not as easy as one would think, as the waterproof mattress is slippery and the sheet then moves quite a bit.) We are hoping then to not have to chase him around the house every hour to drink his formula, and to hopefully leave him more physiologically hungry during the day so he will want to work more on eating food. We are still only giving about two ounces of baby food daily, but aspire to increase that over the next few weeks.

Along with pump feeds overnight we are changing from Peptamen Jr to Vital Jr. They are similar products but the flavor of Vital Jr is better than Peptamen Jr and there is a prebiotic in Vital Jr as well.

We have increased his IV iron to see if he can pull out of his anemia. We went from 15mg iron dextran to 25mg. He had pulled out of the anemia before once we put him on IV iron, but he has gained weight and possibly just needs a bigger dose. I am greatly reluctant to put him on enteral iron b/c of the GI side effects that can come from enteral iron.

Back to Boston later this month. That will be just to check in with the Intestinal Rehab Clinic. We are thinking we will get another reduction in TPN as Peter is gaining weight well. He sure seems to feel good!

We have also started vitamin D drops. Endocrinology wants his vitamin D levels up at 30 or above given his istory of osteopenia. Despite going outdoors daily for one to two hours all Spring and Summer, his vitamin D level is only 22.

July photos

2009-08-23T20:15:00.010-04:00

Peter is moving on to toddler activities such as coloring. We are at a point now (August, despite the pics being from July) where we are able to get him out more and HE is now enjoying it more. It is heartening to see Peter feeling so good and wanting to do more and more things.

Do you see what is peeking out of Peter's shorts? Peter wears two ostomy bags - one for a mid-abdominal fistula and one for his ileostomy. They unfortunately are exposed during shorts season when Peter sits down. See below for a great solution!

I purchased two fabric ostomy bag covers online made by another mom whose child has an ostomy. Peter is wearing a navy blue one here and it really isn't too obviousm covering up what otherwise it quite obvious and rather a bit of a put-off to see poking out of his shorts. This mother's son uses the cover bag so that he can wear his ostomy bag over a pair of shorts. Peter's torso still isn't long enough where we can go to shorts and a shirt, but the cover bags also work well under a onepiece outfit like Peter wears. If you are interested in these bags, we found them at Gus Gear (www.gusgear.net). So far they have held up very well. Peter likes having the ostomy bags covered when we are out too... especially the one with zoo animals on it.

Kidney update

2009-06-16T21:24:00.002-04:00

We went to the nephrologist today. The protein in the urine is very slight at this point! We are reducing Peter's enalapril from 2.5 mg a day to 1.5 mg a day. We'll return to the nephrologist in 3 months for another check up. He feels Peter still has IGA nephropathy as the cause of glomerulonephritis, but that Peter has a very mild case of it. We will continue to monitor to make sure that it does not increase in severity, but otherwise the report is very, very good!

First night without TPN

2009-05-23T09:48:00.002-04:00

Last night was our first night with no TPN, no Omegaven. He is still on TPN 6 nights a week, but this was our first "night off" from TPN since Boston told us we could go to 6 nights per week instead of 7.

Peter was hooked up to a "simple" bag of 10% dextrose with some added sodium so that he would receive enough fluid to compensate for the volume of TPN he is accustomed to at night. We were not sure how he would do... BUT HE DID GREAT!!! He is very chipper this morning and not a drop of fluid retention is evident. In fact, his urine went up an extra 100mL overnight. Low blood sugar was another potential concern, but we see no signs of that either. Toddler energy should be able to be bottled up and given to parents!

I am curious to see if his desire to eat/drink goes up on the day following no TPN. He is liking the choice in formulas to drink since Boston gave us some samples to try. It looks like we will be going now with Vital Jr. Vanilla, Vital Jr. Strawberry, and plain Elecare. We still get some protesting as we try to fit in 10 feeds of 60mL of formula a day, but the protests seems to be lessening with a choice of what he should drink. He is really enjoying his dinnertime feeds of baby food now too... again with an improvement when given a choice versus just being presented with what he is to eat. And of course the obvious thing is, why wouldn't he want a choice??!! Don't we all want some say in what we are given?!

Boston visit May 2009

2009-05-18T22:27:00.002-04:00

We went up this time for the usual Omegaven/CAIR check-in as well as for an endoscopy to evaluate the improvement or worsening of Peter's esophageal varices which were not very bad in August 2007. We were also going to get Peter's gastrostomy site fixed that it may stop needing a fortress of dressing to keep the leaks under control.

7 hours in the car on Monday, 8 hours in pre-op testing/meetings on Tuesday, and then he got sick on surgery-day which was Wednesday. Although disappointing, surgery was ultimately cancelled b/c he should be in tip-top shape for these elective procedures. Instead, we had blood cultures drawn with one positive culture in the red lumen. However, repeat cultures before antibiotics were started are revealing no infection at all. The decision is to stop the antibiotics after only 5 days. He has had nasal congestion for over two weeks now and possibly he was clearing a mild infection on his own between Wed an Thurs, or somewhere along the line there was contamination of one of the culture tubes. It is hard to be sure the exact reason there was nothing growing with the second set of cultures, but it is relieving that we can stop the antibiotics now and avoid another set of cultures.

Thursday we had CAIR and of course Peter was back to himself, and then we drove back home after that, pulling in to the driveway at 1:45am. Peter could not stop smiling once he got into his bed at home.

The things we found out from our visit to Boston are many, and as follows:

1. Echo of Peter's heart indicates that the enlarge coronary arteries have not changed since the last echo over a year ago. Good news!

2. PICC line is in correct placement still per chest x-ray. Confirmation of correct placement in a growing child is a good thing.

3. Dr. Jennings feels that Peter's gastrostomy may be so prone to leaking partially b/c there is very little muscle supporting the G-tube MICkey button. He is going to close up the original site and create a new gastrostomy altogether. I'll have to post a photo of the crazy dressing we have to do now to keep the current site contained. It is an infection waiting to happen b/c it creates a moist, dark environment, but the only other alternative has been to let the site leak all over the place, letting a good portion of his feeds leave shortly after he is fed. A new site should allow for minimal dressing, minimal leaks, and best of all should allow Dr. Jennings to see how well Peter is able to heal from a "simple" surgical procedure. Peter's portal hypertension, INR of 1.2, and unique bowel structure make surgery much more complicated than a child without all of that. How Peter heals from this will likely give us all some insight into future bowel surgeries for him.

4. Apparently Peter's hydroceles may be repairable at this point too. When we go back up for the surgery and endoscope, Dr. Jennings possibly will correct this problem too.

5. We are able to shave off one night of TPN a week now!! This will leave Peter with only 6 nights of TPN a week, and one night of replacement fluid since he can't currently drink enough to compensate for the fluid he will not receive overnight. The nutritionist said Peter's weight gain is nearly double what it should be at this point, so that allows for the loss of a night of TPN at this point. How exciting!!

6. We will add an extra 5 mg of iron to his TPN once a week since his iron levels are on the lower side again. He is biting and chewing on things now, not food, but things. While I thought that might be a good thing so he becomes more oral and interested in food, it also be a symptom called "pica" indicating that he is low on iron.

7. We were given some samples of other formulas to try in an effort to get Peter to stop resisting his elecare so often. He goes through periods of taking it just fine, and other times it is taking extreme bribing to get him to drink it. We are sampling EO28 Splash and Vital Jr. So far he seems to like them both about as much as he likes the Elecare. The variety allows us to at least give him a choice, but so far nothing is winning him over wholeheartedly.

8. Discussing why Peter tends to do so poorly with his feeds when he eats too closely to bed-time or when he has to sit for long periods of time (in the car or waiting in the hospital), Dr. Kamin feels that it is likely that Peter's digestion is somewhat positional. The duodenum is very scarred and likely has a tendency to balloon with feeds. We notice that Peter eats much better on days he is more active. He is more prone to vomiting when he eats and does not have the opportunity to to move around much. We also notice that he vomits more when he is holding onto fluids and getting more ascites in the abdomen. I didn't ask about the why's on this, but wonder if it could be that as he has more fluid in the abdomen there is less room for movement of the bowel as he is digesting. We are having some issues with feeds now as he recovers from so many days of sitting and possibly from side effects of the antibiotic. As much as Peter enjoys the trip to Boston, we usually have to deal with feed and/or fluid issues for a bit until he settles back out.

I think I have recalled all of the main points from the trip. We feel it was a really encouraging visit!!

Oh, by the way, Peter is in a "big boy bed" now. We replace his crib with a toddler bed a month ago. He has not missed his crib one little bit!

Spelling Genius??

2009-03-19T21:53:00.018-04:00

I don't know that we can declare genius status yet, but we decided to see just how many words Peter is spelling these days, and we get at least 50! He is only 3 years and 4 months old and can spell several 2 letter, 3 letter, 4 letter, 5 letter, 6 letter, and even an 8 letter word accurately. OK, he still isn't jumping or running, and he still isn't eating table food, but what a curiosity is his spelling capability.

The other neat thing we see is how much he has progressed in what he can do physically. We finally captured the backwards bear walk on video before he moves on to some other trick and leaves this one behind. He is now independent on stairs if there is a railing or something else to hold onto. We still hover nearby to catch him if he should stumble, but his confidence and skill are gaining. We are in the process of attempting to learn how to pedal a tiny "Big Wheel" so he can ride in the driveway with his siblings. He is also walking around the block twice a day on many days, versus just once a day, for a total of a mile and a half a day!

One last hurrah is that we are up to 100mL of elecare every two hours now. He can drink the 100mL all in one sitting. He takes 5 feeds like that per day, and then nothing enterally overnight. He is still on TPN and Omegaven 7 nights a week for 12 hours per night, but we are having dreams of reducing some of that now that he is up so far in feeds and still not needing to be vented at night via G tube.

Spelling:

Backwards bear walk: (forgive the protesting halfway through, and the overly-exuberant-mom-talk, I goofed on the first three attempts at videotaping and he was ready to be done the bear walk by the time I finally got the video recording going properly.)

February's Boston Visit

2009-03-16T22:00:00.005-04:00

Peter had the best check-up to date when we went up in late February. He went in spelling words - "a-p-p-l-e is apple," "J is for Jill," "v-i-o-l-i-n is violin," and so on. I have never seen a three year old spell like he does, but Dr. Puder and Kathy Gura say that the Omegaven kids all seem to be really smart, so perhaps it is that good fish oil at work yet again.

After the spelling impressed everyone we went on to focus on his growth and behold, Peter has grown in height and weight, and not just following the curve this time. He grew almost 4cm in two months, and the weight gain is not fluid weight, it is real weight. Dr. Kamin felt his abdomen and we all agree that his abdomen doesn't have the feel it used to. Instead of feeling a little spongy with fluid along the sides, it feels more solid.

Peter had essentially been able to keep the G tube closed for the last month and a half also, which was huge progress compared to our December visit where Peter was needing the G tube put to drainage overnight every night in November and December.

We dropped the dextrose in the TPN a little bit and made plans to advance feeds further. He was and still is at 26 calorie elecare and it was felt he can stay there. We have been working on changing his feeds from 45 mL every hour to 90 mL every two hours and we just reached that goal in the last week. So far, so good. When we get to 115mL every two hours we get to drop a little TPN and begin to try our hand with baby food again.

Currently he takes 10mL of stage 1 baby fruit with his probiotics, but we have not pushed foods further since the focus has been on advancing elecare. Peter is the type of child that does better with one change at a time versus multiple changes, so we can figure out what causes problems more easily (usually more easily said than done though). We are stocked with stage 2 foods in anticipation, but also know that the last two times we fiddled with baby food he blocked up. Hopefully he'll do better this time... we think we'll be able to try the baby foods in another week or so. This time he is on probiotics, so we'll see if that is helpful with the baby foods advancing. Maybe a happier intestine will be less likely to block up. Blockage could also be from stricture, so we'll see as we go along.

More photos to come along soon. :)

Playing in the snow

2009-02-22T17:35:00.007-05:00

The photos are a month old, but here they finally are. Peter had a great time sledding and being out in the snow... until one of his brothers threw snow at him and it went down into his coat. Ah, siblings! One of these days Peter will have his revenge snowball throw.

OK, Peter didn't really clear any of the driveway, but he at least held the shovel for a while and smeared a bit of the snow around.

Thanks for taking me sledding and out in the snow Kristy! (Kristy is one of Peter's wonderful homecare nurses.) I'm sure it is obvious, but the girl in the photo is not Kristy. This is Peter's sister in the photo.

After an hour plus in the snow, it was time for a nap... anywhere!

Glomerulonephritis

2009-02-05T16:33:00.002-05:00

The verdict on Peter's kidneys is that he has glomerulonephritis. from Medline, "Glomerulonephritis is a type of kidney disease caused by inflammation of the internal kidney structures (glomeruli), which help filter waste and fluids from the blood." There can be different reasons for the condition, and it can be mild, moderate, or severe in nature. We think that Peter has had this for many months and that he is presenting as a mild case. The nephrologist feels Peter's cause is likely IgA nephropathy versus some of the other causes that could be the culprit, but the only way to make a definite diagnosis of the cause would be to do a kidney biopsy. Peter is not the ideal candidate for biopsy of any organs due to portal hypertension, so unless he gets acutely worse or does not respond to medication, we will just ride on the fact that he has glomerulonephritis and remain watchful.

He has started on vasotec (enalapril), which is a blood pressure lowering medication. He does not have high blood pressure, which is a blessing since it can go hand-in-hand with kidney disease, but the medication also has the property of protecting the kidney from scarring. Prolonged inflammation of the kidneys from glomerulonephritis can eventually cause scarring of the kidneys and long-term damage. He has been on this medication for several days now and seems to be tolerating it well. We will hope for his labs to show that he handles the medication well and will return to the nephrologist in two months. Peter will likely need to stay on the medication for a good long time, possibly for life. The nephrologist said that sometimes people clear the condition, and sometimes they do not. We are on a wait-and-see approach to see where things end up, but it all sounded encouraging and manageable.

Peter's Probiotics Regime -Part Two

2009-02-02T21:41:00.002-05:00

After posting about Peter's probiotics, I got to thinking about the central line issue that makes many doctors, nurses, and other SBS'ers worry about infection. I thought I would revisit this subject with additional details for other parents/caretakers who are dealing with bacterial overgrowth (BO) and the question of whether to add probiotics or antibiotics.

When we first began treating BO sometime in mid 2007, we went straight for antibiotics given Peter's proneness to intestinal bleeding from portal hypertension (possibly the BO as well) and his recent hospitalizations for a gut translocated organism in June if memory serves correctly, and the fact that he had only come out of the hospital in Feb 2007 after almost a year inpatient. The antibiotics worked very well... for a couple of rounds. We did flagyl for a week, augmentin for a week, and cipro for a week, and then either repeated or took one week off before repeating (can't recall). They were all administered enterally. As we grew accustomed to this routine over a couple of months, Peter began doing funny things while on the enteral antibiotics. The first issue was hives. After the third round of hives we came to recognize that they were occuring when he was on augmentin. We stopped augmentin and the hives left and haven't returned. We continued with just flagyl and cipro until we began to have mystery illnesses while on flagyl. I have written about that in this blog before - see posts from March and April 2008 where Peter was hospitalized and cultured and there was nothing discovered as far as illness. The third time the mystery illness occurred we realized that each time he had been on flagyl. We stopped the flagyl and the illness left the next day. Then all we had left was cipro. We were looking for patterns by this point and found one - enteral bleeding within a few days of starting cipro. (By this point Peter's enteral bleeds had pretty much resolved and were rare occurences compared to the year before, but when cipro came onboard it would predictably return.)

This is when we started the probiotic drink of kefir. It was good stuff, but eventually stopped working, likely due to the small amounts that we are limited to since Peter takes so little in enterally to begin with. Speaking with Peter's GI in Boston about the BO and where to go with it, he suggested that we could try probiotics, although it is considered risky in a child with a central line. Given that we have already been hospitalized because of antibiotics, it seemed worth the risk to try probiotics over antibiotics. The cautions I have been given are that probiotics are LIVE organisms. What has been emphasized to me, is that one of the culprits for infection in a line of a person using probiotics is likely a cross-contamination versus necessarily a gut translocation. In other words, a person may have probiotic on their hands, not realize it before handling the central line and thereby contaminate the line directly. That may not be the route of contamination in all probiotic line infection cases, but just like one would thoroughly wash hands after changing a dirty diaper or ileostomy bag before accessing the line, one should be extremely thorough in cleaning after probiotics before handling the line or accessories. Below are the tips that were given to me along with how we have adapted them to Peter's routine.

The germs, though "good" germs, are still "bad" germs if they get into the bloodstream, so wash hands thoroughly and make sure that the probiotics are handled with diligent care so that they do not get anywhere near the central line. Administer probiotics long before accessing the line or making TPN of handling IV meds. Do not administer or mix or store probiotics anywhere near where you make TPN or handle IV meds. Our routine is that Peter receives the probiotics only after his TPN is down and his lines have been flushed and wrapped in coban for the day. The probiotics are mixed only where food is prepared, not where TPN would ever be prepared, and is mixed over a large dinner plate to catch any excess powder that might fall onto the plate when it is being measured. The powder is mixed with a teaspoon of baby food in a small bowl that is on the plate. The plate is used then as a tray to keep the possibly dirty bottom of the bowl from contaminating another surface. Peter is fed the food containing the probiotic, not allowed to try to feed it to himself. Immediately after feeding it his mouth is wiped with a damp paper towel, the bowl and spoon and plate go into the dishwasher, and the person who administered the food and anyone else who handled the probiotic in anyway washes hands with soap and water.

Peter has been on this regime since late December and though the attention to maintaining strict cleanliness when using the probiotics seems a little strict and uptight, it seems to be working well for us. We'll keep posting updates about the success or lack of it as we go on.

Peter's probiotics regime - Part One

2009-01-28T16:34:00.005-05:00

Kids with short bowel syndrome (SBS) tend to have recurrent bacterial overgrowth (BO) in their intestines. We haven't had to deal with this in ages with Peter, but in December Children's Boston suggested that Peter's difficulty with feeds may be due to BO. ***UPDATE: I think the more frequent abbreviation is SBBO - Short Bowel Bacterial Overgrowth, versus just BO.***

We have had terrible luck with enteral antibiotics in the past, resulting in hives, mystery ailments ending Peter up for hospitalization, and intestinal bleeding. We had tried 3 different enteral antibiotics (flagyl, augmentin, and cipro), each presenting one of those problems. We had put Peter on kefir for several months after the antibiotics had failed and the BO odor was returning. That worked well for many months, but apparently did not work well enough to be the sole prevention for BO, possibly given the small amounts we had to give Peter.

Peter has now been on powdered probiotics since late December, and we think we are seeing a huge improvement! We started on 15-35 Probiotic which has 35 billion organisms per capsule, but it also has enough sodium in it that Peter ultimately began to refuse it due to taste. We moved on to Florastor, which is a yeast organism. He has been on this for 2 weeks and we will keep him on it one more week. The initial response on Florastor was that he smelled quite poopy most of the time, but at this point he does not and he is acting like he feels just wonderful. Perhaps the poopy smell was him clearing out the BO?? He takes the Florastor in a little baby food and is no longer refusing elecare. He is sleeping better at night too!

We will then go to VSL#3, which is quite similar to the 15-35 Probiotic, but no sodium. We'll give that for two weeks, go back to Florastor for two weeks, and then go for a two week break off all probiotics. Meanwhile we are hoping to begin increasing elecare again to see if he can tolerate it better now that the BO is out.

Still waiting to hear back from the nephrologist about what is going on with Peter's kidneys if anything.

Christmas photos 2008

2009-01-14T21:32:00.009-05:00

This year Peter understood that Christmas is baby Jesus' birthday. He understood that presents have gifts inside that you keep. He understood how to open the gifts. And he enjoyed himself! What a lovely Christmas gift and blessing to see the advances from last year to this year!

The endless cold and the fearless wrestler

2009-01-14T21:01:00.005-05:00

I have been meaning to post for the last 10 days but have been stuck with an endless cold rendering me essentially useless by the end of the day which is the best time for me to try to update things... after the kids are down and it is quiet. Interestingly, Peter had the bug for only a day and a half, though he has a persistently low white cell count in his labs and has for the last two years at least. It is a whopper of a cold though, striking every one of us plus one of the nurses that works in our house here. It seems to be rebounding on the kids including Peter today.

Peter's albumin yesterday was down from 2.9 to 2.2 and he acts like he is holding onto fluid in the abdomen in his breathing and gait. We hope it is just the cold! When he has been hit with a good illness in the past we would see his albumin get down below two and he would swell up, needing IV albumin and lasix to remove the fluid. I don't see the swelling taking over like in the past, so hopefully he is pulling out of this on his own already.

Prior to the cold we actually have seen some great progress with Peter physically. He still can't jump, but he is doing a whole lot more self moving when playing which imitates many of the positions PT wants him to work on. Also, what is really a big leap forward is that he has started to enjoy crashing into his brothers and rolling around on the floor with them in mock-wrestling moves. His speech is coming along beautifully and he is using full sentences with occasional pronouns and making conclusions about what someone may be feeling based on what they are doing - ie, if I am holding a cup, "Mommy is thirsty."

We are still perplexed with the urinalyses that we are getting over the last few weeks. There is still large amounts of microscopic blood and there is protein in the urine. We are to find a kidney consult locally to see what is happening. We also still are trying to understand why he holds onto fluid as he does. Children's Boston lowered the sodium in his TPN even lower, but so far we don't see much change. But again, perhaps that is because of the nasty cold virus and the proneness to holding fluid when ill. Maybe the kidney consult will give us some clues.

We are still at 30ml per feed with the elecare but have added a tiny bit of stage 1 fruit again. Peter can detect the probiotics in his elecare and we have been seeing an aversion to the elecare, seemingly out of fear that it will not be the pure elecare but will have the probiotic in it. Putting the probiotic in the baby food is so far working out well. We are now trying Florastor probiotic and have great hope that will work as well for him as it has in other "intestinal failure" patients. (I don't particularly like the term intestinal failure and almost panicked when I first saw it on Peter's chart, but it does not mean that his intestine is failing and has no chance, in case you were wondering.)

December is here and almost gone??!!

2008-12-22T20:47:00.006-05:00

I keep meaning to post updates but not sure what to update on these days. I want the news to be all good, but we are having ups and downs and ups and downs.

Fortunately, we have not had anything scary like in the "old days" where we getting admitted for a fever or having to schedule another blood transfusion. And actually, everyone, in Boston and locally, seems to be in agreement that Peter's liver is healing nicely. His last two total bilirubins were 1.0 and then 0.8 - both NORMAL!! And the direct bilirubins were 0.4 both times, also NORMAL!! Platelets on last draw were about 130,000 and the rest of his labs seem to be pretty good. It is still a mystery why the CRP continues to go up and down, but he gives no other indication of infection.

The downs really are more related to his feeding and his fluid balance. It seems like Peter gets more fluid positive for several days to even a couple of weeks if really gradual, then reaches a point that he begins to refuse feeds, vomits feeds, and by that route eventually loses more fluid until he is not so positive in fluid and weight. Then he begins to accept feeds again and we begin the cycle of gaining fluid weight and vomiting and refusing feeds all over again. We have increased his protonix to 12mg twice a day from 10 mg once a day (that is an acid blocker that should help with controlling GI secretions if these problems are due to too much acid).

At home we have also decided to increase his probiotics as well, since one of the side effects of changing the pH with protonix is an increased likelihood of bacterial overgrowth. The docs have considered that bacterial overgrowth could be already at play here with the problems in feeding, but Peter's history of allergies and strange reactions to enteral antibiotics leads us all to wait a little longer before prescribing antibiotics. The short-gut wiki and the Yahoo short gut group both have families that have put their children on probiotics to successfully ward of bacterial overgrowth. Peter has remained on the kefir, but dosing is hard to guess, so we are adding some powdered probiotic into his kefir. His tongue has had a coating on it again for months and his breath has been bad along with it, so we hope for a clearing of all of that like we have had in the past with kefir alone.

Feeds had gotten as high as 45ml of 28 cal elecare 10 times a day, which is 1/3 of what his daily needs would be, but shortly after the triumph, Peter stopped accepting that and we are somewhere around 350 to 400 in feeds a day, sometimes less on his "bad" days. Baby food came to a halt with the loss in elecare feeds in hopes that we can get the most nutrition from his feeds as possible. There have been many discussions with the docs up in Boston about increasing protein in TPN again and decreasing sodium, but they are a little at a loss because of the risks involved in going over the traditional limits in TPN.

Other thoughts the docs have about why he is having feeding difficulty, is that his intestines may be ballooning up with feeds versus sending them through in a constant flow. That would explain why Peter's ileostomy output remains constant despite the increase in feeds. We will likely schedule a follow up upper GI and possible endoscopy the next time we are up in Boston... unles Peter does what we hope and just pulls out of whatever it is he has been doing the last month and a half.

Another odd thing is that we see him urinate more when the protein in his TPN is kept at a higher number. This all seems puzzling and there is no answer yet. It could be a good thing as he flushes his kidneys more, or could be bad in that there is more urea and nitrogen waste that the kidneys perceive a need to handle as toxins. The suggestion is that we look into getting a consult from a urologist or nephrologist to assist with how to interpret why/how Peter is handling his fluids. It does not seem to make sense, but over and over we see him urinate more when the protein levels are higher, within a day or two of increasing the protein. And if we decrease it he urinates less, predicatably. We are looking into sodium as a culprit of his holding fluid as well. He really should not be prone to ascites as much with his liver doing so much better. But perhaps he is losing protein out his ostomies.

To check for protein loss out the ostomies we have submitted Peter's output fluids again. The original batch two months ago was mislabeled so we are trying this again. Results from those tests will hopefully guide everyone as to whether or not Peter needs extra protein or less sodium as well as whether he is actually digesting the elecare that he takes in. These fluids have travelled in the back of our van from PA to Boston and are now in transit to the Mayo. Amazing.

We had a random u/a come back showing calcium oxalate and some blood in the urine (not detectable with the naked eye). Those may indicate kidney stones. On ultrasound there are no stones seen fortunately. The plan is to decrease the calcium and phosphorus in the TPN and to put him on enteral calcium to bind the oxalate.

We have seen some growth in Peter, but unfortunately not leaps and bounds as we hoped and expected. I guess the dryer is shrinking some of his clothes, but really he does seem to be taller as we see his little face peering over the table top and couch. He did grow 2 cm over the last two months and he has put on some weight, but not as much as my scale would suggest. Of course, the day up to Boston and the day there he took in much less elecare than usual, and he had to have his G tube open to drainage the whole ride up, so I am guessing he did lose a little weight over those two days as well.

Once again I am posting a huge digest of information instead of steady little posts. Sorry about that. It is a lot to take in all at once.

The one thing I should part with is that Peter is definitely still making tremendous progress overall. There is no question that he is in a better spot now than he was a year ago when we decided to suspend his transplant listing. As Christmas approaches we are ever-thankful for the blessings that we have been given. They are numerous indeed!

PS

2008-11-08T23:10:00.002-05:00

We met another child with TPN (and Omegaven), G tube, short gut while we were in clinic. There is some really interesting piece of news that I have to share in case there is anyone else out there who is baffled by G tube back-ups. This child, Charlie, has to have his G tube vented every morning to let out air and residual gastric contents before being hooked up to feeds or else he will vomit. It is necessary for him that this be done every day. Here I thought that the occasional gastric back-ups that we have with Peter were abnormal and showing us that we were overstressing the bowel. I would cut way back on the feeds or even stop the feeds for a day or two and gradually begin to build him back up in feeds again. (Peter takes his feeds by mouth, but we would still just stop offering anything other than some water). Apparently needing to put the G tube to drainage for a while can be a normal event, and once that drainage is out feeds can be resumed as normal. I wish I had known that before. I am glad to have that understanding now.

By the way, the child I am referring to is the very first child that was put on Omegaven in hopes of living until a liver transplant was found. Omegaven not only decreased the liver damage but quickly got him off the transplant list. He looks wonderful!

Back from Boston, Halloween, Birthday

2008-11-08T22:06:00.010-05:00

Opening gifts all by myself this year!

Trick or treat!

Boston Commons... this vaguely looks a little like the cover on
one of the Beatles albums

I am getting behind in the updates. It has been busy around here as you can see by the title of this entry.

We went up to Boston a few weeks ago for another two month check up. Peter was in good form and showed off his increasing strength and speaking skills to everyone who saw him. EVERYONE at CAIR clinic commented on how very good he looks and how strong he is getting. The weight gain and growth are a little disappointing when we look at them on a graph and compare them with what he should be doing. He is behind on the charts as we expected, but he is not following the growth curve properly and that may be an issue of concern. We will investigate that further after the next check up if need be, but meanwhile his elecare is advancing and if the gut is working, that should help with the nutrition.

Peter's results are back from his methionine breath test that he took in June. He has had 4 of these tests now. The first 3 showed declining liver function. They were done in Jan 2007, Aug 2007 when we listed him for transplant, and sometime around Jan 2008. The one from June 2008 shows the function of a normal liver!! This is still a test that is in testing stages itself, with the hopes that it will be a non-invasive test to replace liver biopsy. However, the doctor who runs this test was ecstatic about the results as they certainly seem to correlate with his other labs and how well he is acting.

Then Halloween was here. Peter dressed up and liked it!! I really had my doubts that he would agree to an outfit, but we found one he liked and he wore it, and even the hat that accompanies it. He had a wonderful time outside in the dark. We used the flashlight and talked about the darkness. To try to keep him away from germs he didn't go door to door with the other kids but he didn't care about that anyway. We were out for over an hour, but when we came back to the house he was unhappy because he wanted to go out some more. He finally settled for playing ball in the house in his costume before he had to go to bed.

Peter's birthday is the day after Halloween, All Saints' Day. He is officially three years old now. Just like Halloween, he was much more involved in the holiday this year compared to last year. He tore into the cards and gifts and seemed to understand and enjoy the fact that it was his birthday. He has seen other people celebrate birthdays throughout the year and now it was his turn. We had to light the candles twice for him because he enjoyed them so much. Sadly, no cake for him yet, but like the Halloween candy, he didn't care that we were eating it and he wasn't. Instead, he licked the apple slices and gluten-free pretzels and crackers that were on his tray and was content with that... especially when daddy put the candles in the apples and relit them!

Good times

2008-10-18T10:27:00.003-04:00

We are back up to 55mL elecare per feed again! Peter's activity level is fabulous. He is taking little amounts of stage 1 baby fruits now as well. The key to getting him to willingly take the fruit by mouth was to CHILL it. We should have guessed, seeing as he will only take his elecare when it is cold.

We will be taking Peter to Boston in a few days. We hope to test his outputs to see how well his bowel is working. He has been having interesting labs the last two months. A month ago his CRP began to rise again. The CRP was 15 a month ago (up from less than 8 which is "normal" with the laboratory we use), and last week it hit 40. The mystery CRP rise is similar to what he did last spring, with a rise that has no accompanying symptoms. We don't think there is infection, so we will just watch him. His BUN (kidney lab) has been going up, so the doctors may have the lab examine his outputs to see if there is some tweaking necessary in the TPN to make the kidneys happier. Meanwhile, his bleeding times have improved again and his liver function tests are looking marvelous. Peter himself is behaving well, and that is the main thing. He is happy and active, and oblivious to the ups and downs of his labs this last month. Now, if he can get through his visit to Boston and back and have no funny incidents, that will be just super!

Peter's third birthday is coming up November 1 - All Saints' Day!!

new photos

2008-10-08T22:08:00.009-04:00

Peter went to the park for physical therapy today. We went with a canister of Germ-X wipes to wipe down the handrails and put him to the task of trying to climb stairs and the ladder. The reward was to walk across the bridge at the top of the playset and to slide down the various slides. He did quite well for his first real attempts on a playground set!!

Reply to Suz

2008-09-24T13:46:00.005-04:00

Hi Suz. I posted your comment with the last post, but have no way to respond to you other than this blog. I would be happy to share the things I have learned with you. You can email me directly at bambinosfour@yahoo.com and I can email back and forth with you. One good resource is the blog of Ellie Brogan, found at http://eleanorbrogan.blogspot.com. Her story is encouraging and her parents have links to several helpful resources on the internet, including other parents of children with short-gut, and informative sites with more tips.

As far as room set-up, when we came home from the hospital, we put Peter's crib in the living room and decided to sleep on a pull-out sofa bed in the room with him. We still have this set-up, as it has been the most efficient for us. Lugging all of his equipment up and down stairs each day would have been too difficult. It was and is still important to us to keep him in the thick of the activity in the house to keep him really stimulated. A year in the hospital (plus the additional ins and outs afterwards) left Peter fairly behind physically and socially. It seemed to be helpful for us to bring a stimulating environment to him as much as possible. He now is toddling around the house and exploring on his own, but it took a year and a half to get him to that point. Now we still stay in the living room with him because his supplies are all there if we need them, and because we can keep an eye on him easily when he goes to bed. We can look at him and know if he is fussing b/c he is cranky and tired or if there is a problem such as being tangled up.

Essentially our living room has become Peter's care room. We bought three plastic units to store his supplies. One has IV supplies, one has dressings for the ostomies, and one has toys but we use the surface for the diaper scale. The plastic is nice b/c it can be easily cleaned with clorox wipes. Under the crib we have placed mats of sturdy plastic carpet protector (the kind in a roll you find at the hardware store). It is very tacky but practical to keep Omegaven off the rug (just wipe off your spills) and to protect the rug from occasional foley bag drips when we drain the foley bags in the morning. One day we will regain our living room for its intended purpose, but for now it is Peter's room.

Also, we bought a small refrigerator to store Peter's IV meds and TPN. This keeps the meds away from food items that may contaminate his supplies. Many people do recommend this for storage of refrigerated IV meds.

Peter does not have a broviac. He has a PICC line. Ellie's site has other families with broviacs too. Broviacs are more common in the pediatric community than PICC lines. I am not familiar with the antibiotic regimen you mentioned, but someone else may be.

We change the tubing on the Omegaven daily. Omegaven runs overnight for 12 hours and comes down in the morning, tubing and all. In the evening, we use a Y extension set to connect it to the TPN line, with a cap on both ends of the Y extension versus having the tubing connected but not running. When he finishes the Omegaven we can take everything down to the cap (the same kind you use to cover the end of the central line) of the Y extension and let the TPN finish up with the portable pump. The pharmacist at our infusion company helped us work up this set-up. We have found that it is really helpful to get to know the pharmacist who makes the TPN for home use. The home infusion pharmacist can be instrumental in helping you get comfortable with the supplies you are using and to bring in new ones if you find you need something different.

Yahoo has some groups too. I haven't been to them yet. I just found out about them on the short-gut wiki which is put together by other parents of short-gut kids. That wiki site is http://grey.colorado.edu/shortgut/index.php/Main_Page. It is another excellent site to visit, but I don't know if you can ask questions there. The yahoo groups should provide you with a place to ask questions. You can also email me with additional questions if you would like.

Congratulations on getting home after a year! The initial transition can be hard, but it really does get easier once you adjust to life outside of the hospital again and once you get into a rhythm of your child's care.

Back in the saddle

2008-09-15T22:59:00.003-04:00

Peter seems to have completely pulled out of his obstructive type of episode. He is no longer needing to have his G tube open to drainage, and he is tolerating his Elecare again. He is appropriately asking for "milk" (Elecare) and is taking his goatmilk kefir again, and things seem to be getting down to the ileostomy again, looking digested. The duodenostomy bag has been replaced with a piece of gauze that is being changed two times a day, with less than 2 mLs of drainage per dressing. Peter is back to himself and we are ever grateful once again! We are starting the Elecare slowly though... 20 mL per feed today and we will gently work our way back up to the 55 per feed where we left off, and hopefully go on to even more feeds. We will avoid lamb for now though. Anything thicker than a drinkable fruit puree is going to be a "no" for now, nutritious or not.

No feeds for now

2008-09-10T21:56:00.005-04:00

We don't know if it was the Gerber stage 1 lamb or the change from a flat ostomy dressing to a convex one, or just something bound to occur, but something has changed with Peter and things are not really flowing through now. He started backing up and out his gastrostomy site more and more, and then vomiting, all around the time we finished up our three day trial of one tiny jar of baby lamb. We eventually had to put the old MIC gastrostomy tube in with a foley attached and just leave his gastrostomy to drainage full time. We still can't keep the tube closed off even for a few hours without gastric contents and bile leaking out around the tube. With all of this, the duodenostomy now puts out nothing at all.

His labs do not suggest infection and temp is fine and generally he acts fine. His electrolytes got slightly out of kilter with the extra drainage from the stomach and he is now on IV normal saline for 12 hours at night to balance that. The electrolyte imbalance may be why he was slightly off in bahavior the last few days.

It seems that a little of his body fluid passes through from the stomach and works its way to the ileo, but we are not giving him any Elecare or kefir now and definitely no baby foods. He is getting water for drink when he wants anything, but it mostly all comes back out the open gastrostomy tube. Right now we are just waiting to see what comes of this episode. It has been going on now a little over a week!

Stopping feeds and having to reattach another drainage tube is of course disheartening. However, we are extremely grateful that Peter is still home and that he is not in distress. Admittedly too, it is nice not having the problematic duodenostomy to deal with, and the G tube leaks very little around the dressing at the moment. How's that for a silver lining?? Hopefully the next post will be about the resuming of feeds... soon.

Still working on getting the Boston Commons photo up.

A protest to holiday weekends when my child is chronically ill

2008-08-30T17:32:00.010-04:00

I thought my dislike for holiday weekends was going to end after we left the hospital. The dislike came about with the realization that everyone who is important to your child's care goes away for the holiday, leaving the place short-staffed, under-supplied, and unfamiliar with what is going on with your child. It continues to be the case at home too.

The durable medical supplies we were to receive are missing and no one is at the company to help with that. The infusion supply company sent me no TPN additives with the order... fortunately someone called me back within the hour, but now someone has to go into work over the holiday weekend and another person has to drive the meds here. It's not my fault, but somehow I feel bad about the situation. We seems to have "events" occur on weekends and holidays, and unfortunately it happens enough that I feel responsible, even though I haven't been the cause 99% of the time. Maybe people just get too distracted by their holiday/weekend plans, or maybe since Peter has so many needs things get missed here and there and it just seems that there are problems often since there are is so much room for error. My strongest opinion is that the companies get too big and we just become another number in the system, and as such no one gives much thought to the total picture. Our IV nutrition company seems to be short on Peter's needed items often. I get a note in the box of supplies that this or that was not in stock about 40% of the time, or something just may be frankly missing. If it were the same person each time handling the ordering, stocking, and shipment of supplies, I think we would fare better. Right now there are too many people who handle his orders from start to finish.

To add to the lack of relaxing here, Peter's main doctor in Boston sent an email that he will be away and if anything happens to contact the GI fellow on call. I never like getting that email and certainly don't share that info with Peter who I am sure would like to take that as a challenge to see just who I would end up contacting then. Hopefully Peter got all of his really sneaky tricks out last week with the PICC line break. He is teasing me now with his leaky G tube and by staying awake very late into the night.

The G tube is leaking SOOOO very badly now. A sanitary napkin is just not doing the same as the allevyn and aquacel and nursing pad combo that was keeping the leaks more contained and a little less in amount. With no durable medical supplies arriving as they should have, MY holiday weekend is not such a holiday at all.

A busy last two weeks...

2008-08-29T23:24:00.005-04:00

The trip to Boston went well. Peter is a great traveller in the van! A little over a year ago he was needing to be air ambulanced to Boston. Now he is enjoyably riding in the van for 7 to 9 hours, and this time with the G tube clamped the whole time AND enjoying his ice-cold Elecare along the way. No gagging! Our biggest challenge in the trip was on the way up with the duodenostomy leaking twice. There we are in the parking lot of the gas station with Peter lying on the backseat changing the ostomy bag, mad at me because the sun was in his eyes, but we were unable to change that situation b/c the bag was already off before we realized the sun was the problem versus the cramped quarters and wetness. The second duodenostomy leak was corrected by removing the ostomy bag and applying an ABD pad and wrapping a diaper over that and then tackling the application of a new bag once we got to our hotel room a few hours later.

Our visit with the doctors and nursed in Boston went well. We were outpatient the whole time. Dr. Puder thought Peter looked very good - better than he ever has. We agree. Peter charmed many with his improved walking and impressive progress in vocabulary since his last visit two months ago. Peter has gained some weight and height, whereas at his last checkup he had plateaued. We are now able to stop his calcitriol (vitamin D), as it appears by labs that he is no longer having ongoing bone loss, but rather is now having good skeletal growth. Hooray!! We also are being encouraged to begin baby foods on Peter. I wasn't expecting that news at all. The idea at this point is mainly to help Peter get used to eating, versus gaining real nutrition from the food. He had his first baby food tonight, Gerber lamb, and he did quite well with it... just a few tiny spoonfuls, but seemed to enjoy it and tolerate it. Meanwhile we will also continue to go up in his Elecare feeds. When we get to 2 1/2 oz per feed then we will plan to test his outputs to see how well he is digesting what he consumes. We are at 50 mL per feed now, so we are 2/3 of the way towards that goal. If he can prove he is using what he eats, then we will discuss lowering the amount of TPN he receives each week. Also, we are getting to run the TPN over 12 hours now. It is so nice that he can run around that much longer free of something attached to his PICC line.

The upper GI went well. We didn't have any crazy ostomy bag repairs afterwards, which has happened in the past, but this time we were able to avoid. Maybe that is because I was prepared with every ostomy need I could think of and a side room available if we needed it, kindly arranged ahead of time by one of the nurse practitioners. The results show that things do flow in a linear fashion from the stomach to the ileostomy, without too much leakage from the other ostomy sites along the way. We will need to keep an eye on the duodenostomy that it doesn't begin to pouch or back up as we increase feeds. Peter does not seem to have developed any new fistulas and the stricture was unremarkable, which is also positive news. Dr. Jennings did point out that we still will need to know how Peter is circulating around the portal vein clot before any surgery could be considered, and to know more about the liver and bowel function. The gut still appears shortened even with use, so perhaps there is scarring causing the shorter gut versus just disuse. The bottom line seems to still be to keep feeding him and continue slow advances in the feeds, and we will continue these thoughts in two months when we are up again.

The leaky G tube still leaks... that is another story for later.

Then 36 hours after getting home the PICC line cracked!!! I don't know why, but it did. There was no trauma that I know of, but maybe it broke from the wear and tear of a toddler who occasionally tugs at it and who puts a whole lot of tension on it when he begins to walk away from his IV faster than one of us can catch up with the IV bag. We were able to get another PICC placed at CHOP two days later, and so far there is no sign of infection. Whew!!

I am hoping to post a photo or two from Boston soon. We finally made it to Boston Commons!!

Feeds. Gtube, and more toddlerhood

2008-08-12T22:37:00.007-04:00

We are up to 40 mL per feed, five times a day!! We bumped up the kefir too, with 10mL in the morning, and 10mL at night. The G tube has been clamped for a solid 60 hours now and he is tolerating this change beautifully.

Labs drawn today show a hemoglobin at 10.6 and hematocrit of 34.0 which is a normal hematocrit AGAIN. Platelets are 83. We didn't get liver numbers, but he looks good and acts good, so we'll assume all is well.

Peter has started trying to stand while he is in the highchair and in the stroller if we don't heed his call to take him out as soon as he deems he should be allowed to get out. We are now adding on the "extra" straps that go over the shoulders to keep him better secured in the highchair. I don't have that option with the stroller. Fortunately he can't scale the crib yet.

He can't stand the word "no" and behaves as if it is a challenge for him to attempt harder. I am sure that those of you with toddler experience know well of what I am speaking about. Hopefully we are not far from a stage of greater reason and aggreeability where we can tell him to stay still while I am accessing his PICC line and he will cooperate and stay still instead of trying to grab the line, or where we can say let's fix that and he will let us fix the dressing versus trying to rip it off. (You can stop laughing now. I know he is a toddler, but I can hope!)

Increasing feeds and back on kefir

2008-08-08T21:40:00.004-04:00

We are up to 165 mL elecare (24 calorie concentration)! Peter advanced to 30 mL per feed a few days ago, and today we have gone up to 35 mL per feed. He is still on the goatmilk kefir (10 mL) daily. We had tried goatmilk yogurt for a few days, as well as trying nothing with probiotics in it, and feel that kefir is the better choice. The bacterial overgrowth odors seem to be gone after about the fifth day, and the ileostomy looks like it is well-digested again.

We had a possible back up from the stricture about two weeks ago, where the G tube turned brown, then the fistula turned brown. (Two posts ago.) We didn't know what to make of it, but in talking with Dr. Kamin, it could have been the addition of safflower oil enterally. We are not going to pursue that further unless Peter's essential fatty acid profile tilts much further. His Omega 3 levels are high from the Omegaven, while the Omega 6 levels are low. This is expected on Omegaven, but should balance out once he gets on more feeds.

Also of great interest to us was finding out just what color is "normal" for ileostomy stool! Dr. Kamin said that on the elemental formulas like Neocate and Elecare, stool can be dark green, almost black. What we thought was old black blood (melena) and the black flakes that seem to pop up often seem then to be more likely to be what Elecare poop can look like. With additional reading online, it seems that consistency can vary, along with color too. Peter's ileostomy output has not increased, but we see such variation and know how easily he has bled in the past and have been told to still remain watchful and cautious as we advance feeds. With this knowledge and continued prayer and a feeling of calm now, we are increasing the feeds every 72 hours, by 5 mL per feed per day. He is fed five times a day, so that can add up in time.

Our mickey button is still a leaky mess. That of course doesn't help with getting all of the feeds into him. Both the mickey and the old G tube leak, so we went back to the mickey last week, hoping that its lack of hanging tubing would take some of the tension off the hole. Either device is leaky based on position. We have been told that we can try to shrink the hole by removing the device and putting on an ostomy bag, and then being watchful that the hole doesn't close. He has had this tract for two years, so the likelihood of a fast closure is not probable. We tried this method to shrink the hole just after the oil incident above and had a little success in shrinking the hole, but it is enlarged again. We are trying to avoid decompressing Peter at night anymore so there is no tension from the tube attached to the mickey button, and it is quite packed with aquacel and allevyn now to avoid movement. But Peter manages to move it enough when he rolls on top of someone or on top of one of his toys, so that it leaks and we lose some of the feeds. The hope for now is to keep pushing the feeds, stablize the mickey as best we can, keep up with the G dressing changes, and maybe with the extra nutrition he can try to heal that hole a little. If need be we can put the ostomy bag on him at night so he at least gets his feeds in, but I really don't want to be up several times in the night checking that the hole is still there either.

We go up to Boston in another week for an upper GI and an Omegaven check-up. Perhaps we will have more answers as to where we are headed with Peter... whether we need to go back to planning for transplant or whether we see some progress and continued hope in bowel rehabilitation.

Active toddler challenges

2008-07-28T21:44:00.003-04:00

In addition to our dreadful G-tube leaks, which are increased by Peter doing those toddler things like crawling, scooting on the belly, reaching, bending, not to mention pulling on the tube and picking at the dressing, we had some more fun tonight with the TPN line!

Peter decided that he was ready to take off as I was putting away the G-tube dressing supplies and the next thing I see is his TPN line and cap on the floor, UNATTACHED!!! SCREAM!!! Fortunately the cap that came off was from the Y extension and not the one directly connected to the PICC line, and fortunately he stopped when I told him not to move. Quick handwash post G-tube dressing change, clamp the line, breathe, yell for husband, breathe again. The TPN was salvagable, however in changing the tubing out got a ton of air in the bag which the pump refused to pump out, so I had to make a whole new bag of TPN and get rid of the bag he had been using. I understand TPN is several hundred dollars a bag. Then again, hospitalization and treating a line infection is thousands of dollars a day, and a much bigger health risk. Poor Peter's blood sugar started slumping by this point and we grabbed a popsicle to try to get him over the hump. It seemed to help. Once we got the new TPN bag up and running and Peter's blood sugar leveled out now that he was receiving the 42 mL/hour of dextrose again, he started pulling on the tube and on the caps.

I worry that he is plotting the removal of the tubes now that he saw that it could come off without mommy's help!?! I need to figure out how to outsmart him now. We have used a line cover that Ellie's family gave to us, but he has been prone to getting angry when it is on and pulling more at the line. I am wondering about taping over the caps, but he is a real picker and he may just pick more at them once he notices there is something to pick off. We have to use duct tape to tape his sleeve down over a cloth cover we have over the PICC to keep him from picking at the PICC dressing. He has figured out all of the other tapes and has only had mild success removing duct tape. It is time to search the web for other parent ideas.

Feeds and bleeds

2008-07-28T20:50:00.005-04:00

We had to open the G tube to drainage for most of the day yesterday.

We have been having a lot of G site leaking the past few weeks. A week-and-a-half ago we had moved to a mickey button which leaked 4 to 5 ounces a day, but then we ended up going back again to his old style G tube with five inches of hanging tube which leaks more around one ounce a day. (How I long for the days of no leaking and dressings that lasted for several days!)

During one of yesterday's G tube dressing changes, we saw brown drainage leaking out around the G tube instead of clear or milky with formula. While cleaning around the site it began to just pour out around the tube, so we hooked the G tube up to drainage and unclamped it, collecting another 80 mL of dark brown fluid immediately. Within half an hour we saw it move on down to the fistula, and by the end of the day saw it coming out the ileostomy. As it moved on down, the upper areas cleared up. The question is why it happened.

One possibility that stands out is that the stricture just above the ileostomy is not letting everything through. We had increased the volume of feeds the day before to 140 from 125. When we have tried to just increase the density of formula from 24 to 26 cal, we have seen black tarry stool, also indicative of bleeding in the GI tract. Can he only process ap to 125 mL of 24 calorie formula a day? It is odd though that we were able to bump him up so quickly to this amount, arbitrarily stopping there, and then this is by coincidence his balancing point.

OR we may be seeing this problem related to the addition of safflower oil enterally just under a week ago. Dr. Kamin had asked us to try giving Peter safflower oil enterally to try to balance out his essential fatty acid (EFA) profile. On Omegaven as his sole fat intake, he has had a much higher amount of omega 3 and really no omega 6, and there is some concern that he really should have a better balance of fats as a growing child. This may just be a theoretical concern, but Dr. Kamin said that there is also some evidence that long chain fat exposure to intestinal mucosa will provoke heightened adaptation response, so it seemed like it was worth a try. Since we have started it, we have seen the black tarry stool and this bleed yesterday. So, is it the oil? FYI, elecare does have safflower oil listed as its second ingredient... however it must be somehow reduced that it is in an elemental form. We have been giving him the extra safflower oil to drink poured right out of the oil bottle. I think we will hold that extra oil for now.

Or, is it that Peter just can't use his intestine and we will continue to see bleeds? Is this one of the symptoms that the label "intestinal failure" refers to? We have seen lots of bleeding in the past with Peter, and it seems like a lot of it revolved around trying to feed the gut. However, those bleeds often were more frank blood, with lab values suggesting that the liver was more at issue with higher bilirubin, elevated coags, and LFTs that were much more elevated. Peter's labs still are not totally normal, but this is expected because of the portal vein clot which is still responsible for portal hypertension which then still has an effect on the liver. The labs are remarably improved from where they were though. Bilirubin around 2 vs in the teens or 20s, coags down from 1.8 to 1.2, LFTs near normal but not completely normal. He is not needing transfusions anymore and we have seen his hemoglobin actually rise on its own since we have added iron to his meds. There is a link between feeding the gut when the liver is sick, which makes the liver more sick, and Dr. Puder kept emphasizing that before Peter's feeding tube was removed. At this point where Peter's bilirubin has dropped so low and is staying low, he should be able to use the gut though without further injury to the liver. We think. Routine labs were drawn earlier today and should help.

Maybe Peter's very delicate, newly awakened intestine is really just not ready for anything more, much like a newborn's, and it will just take more time??? Is this part of the "Peter is writing his own book" that is going to continue to unfold.

We resumed feeds again this afternoon. Peter really never seemed distressed by the bleed yesterday or with the melena before that. He was really not very happy with me telling him he could not have any more "milk" (elecare) yesterday afternoon or earlier this morning.

Yogurt and/or kefir are still part of our routine... 1 tsp yogurt a day or 2 tsp kefir, both made of goatmilk. I don't think they are the culprit. I did tell Dr. Kamin about adding it in and he didn't comment either for or against it.

Peter's stool looks digested and more like his usual again tonight.

Kefir

2008-07-20T22:58:00.004-04:00

Last week we added goatmilk kefir to Peter's enteral care. I just told Dr. Kamin about it via an email conversation tonight. I hope he is OK with it.

Peter's ileostomy and fistula had begun to get the usual odor of bacterial overgrowth, which then leads to a need for enteral antibiotics. I am 99% convinced that it was cipro that caused the bleeding two weeks ago. Peter has demonstrated hives and mystery illnesses on other enteral antibiotics for bactrial overgrowth, so we would really like to avoid more enteral antibiotics.

In prior discussions with Dr. Kamin, he is in favor of probiotics in general, but because there is still some risk of translocation of that good bacteria into the bloodstream, Children's Boston is not in favor of giving them to a child like Peter. However, several months ago we did have Peter on cowmilk yogurt. Once we decided to try using Peter's gut again though, Dr. Kamin felt I should stop the exposure to cow milk due to an increased likelihood of allergy with Peter's currently immature gut.

After looking into things last week... wondering why the ileo looked cloudy now vs digested as it had prior to the cipro, and speaking with some other people, and researching online, it seemed logical to at least try the kefir, but with goatmilk vs cow. For those of you who wonder what kefir is, it is similar to yogurt, but drinkable. There are several probiotics in kefir, similar to good yogurt. We have some lovely looking ileo output if I do say so. And a less-stinky fistula and ileo too. One of Peter's home nurses even felt that his breath was better.

Of note, Peter does not sweat either... odd issue with him, but I have only seen him sweat twice, and it was only on his head under his helmet. He will have an increase in body temperature if he is hot, up to 100 degrees, but no sweat. If we mist him with water it acts like sweat and he cools off. There is a person we know who had GI issues as a child and no sweat either, and as an adult she started eating yogurt with acidophilus and she now produces sweat. Could be coincidence in her case, or maybe there is some link with bowel health and sweat. Hearing that was the final push we needed to at least try the kefir though.

We are hoping Dr. Kamin remains in favor or neutral to the kefir. I think if allery is an issue down the line, goatmilk will be easier to deal with than cow milk.

outside videos

2008-07-20T22:51:00.005-04:00

Omegaven News Article

2008-07-10T11:50:00.008-04:00

Omegaven is in the headlines again! Thanks Chelle for posting this link on Carter's blog.

http://health.usnews.com/articles/health/best-childrens-hospitals/2008/05/29/thanks-to-fish-oil-shes-a-golden-girl-no-longer.html?msg=1

Be sure to read the comments left about it and feel free to add your own. Those of you who saw Peter at CHOP and who see him now know that he would have died without Omegaven or a transplant. The more personal stories that people can see, the better chance of Omegaven saving more children. It was a news article that told us about Omegaven, not the doctors who were caring for Peter at the time. Several parents have found out about Omegaven from blogs and internet searches. It is important that parents know it is out there, what it has done for others, and that they have an opportunity to get it for their child.

Setbacks and progress

2008-07-07T14:43:00.002-04:00

Last week we had to stop feeds altogether after experiencing bleeding from the ileostomy, fistula, and even backing out the G tube. (It was "coffee grounds" in appearance for those of you familiar with GI bleeding). So the question of course was, "why now?" We had actually decreased the concentration of formula because his ileo looked milky in color a couple of days before, and we had not gone up in volume. What was different though was that he was on enteral cipro given through the G tube for bacterial overgrowth in the small intestine. He has routinely been treated off and on for bacterial overgrowth when the ileo gets a very strong odor. This time though, he had been vomiting after the morning dose, which is unusual. After seeing the bleeding, and looking for a reason why, I googled cipro and bleeding. Apparently cipro can cause GI and intestinal bleeding. Now whether this is really the culprit we likely won't know, but we stopped the cipro and stopped the feeds to give the bowel a rest, and 24 hours later the bleeding stopped and it has remained stopped. That was Wednesday. Today is Monday and we are beginning feeds again, cautiously again. So far, so good.

A close setback was the drastic change in the amount of lactated ringers (another IV solution that he gets in addition to TPN) that he needed. We went from 400 mL a day while up in Boston, which was down from 430 the week before, to 275 to where we are now which is still fluctuating between 150 and 200. This is all in a period of 3 weeks. A year ago his lactated ringers was around 1300 mL a day because he would lose so much from his upper GI tract. Now he loses so much less that we can run that much less lactated ringers solution. We think this is all good, but if we don't keep close count it is easy to have Peter get so much fluid that he begins to gain fluid weight and his abdomen begins to get big again with ascites... which then can affect the bowel. He had gained a pound in a week before we got to a point where we were able to figure out his lactated ringers needs again. With all that being said, it could be that the fluid in the abdomen made him more prone to bleeding and that is the source of the bleeding described above. Or it still could be that he still has enough portal hypertension that he will remain prone to bleeds period, fluid in the abdomen or not.

Progresses are several. First, his last albumin was 3.0. We haven't seen his own produced albumin in the 3 range for year and a half, I think. That is a good sign of liver function... the last one was 2.7 though, so we will need to see more 3's to believe it. Second, he is crawling off on his own now, to the point we have had to install a lock on the basement door and are making plans on how to install a gate on our odd staircase which has no banister. Third, I left for the weekend to attend my brother's wedding, and Peter and my wonderful husband made it through the weekend beautifully. I haven't left Peter since May 2006, when he was on TPN, but without all of the dressings and potential complications that he now has. Peter doesn't necessarily NEED ME, but the fact that he has been well enough to even contemplate leaving is the big progress. Even with the bleed mentioned above, he has been doing well enough to feel it was OK to leave. A few months ago we had to deal with bright red fistula bleeds which needed intervention to stop the bleeding and often needed an entire dressing change to go along with the intervention. He also had the elevated CRP that no one could figure out that we were waiting to explode into sepsis, which fortunately never happened but had huge potential keeping us jumping every time he felt warm or began to get fussy. How nice it will be if Peter continues on this plane of wellness.

Funny, if you tell someone the number of ostomy bags your child is wearing and that he is fed via IV and that he has nursing help six days a week, how ill that child sounds. But, if you look at how things were and where we are now, he well he sounds. I guess we will see how well he actually is sometime in August when we go back up to Boston for more tests and imaging of his bowel.

Peter can ride a scooter-bike now!!

2008-06-25T14:17:00.007-04:00

Peter has been riding this little scooter-bike since the second week in June. He has a little game he plays where he rides over to his ball, bends down to pick it up, throws it, and then rides over to the ball to begin all over again. He'll be popping wheelies soon.

To the mom who said hello

2008-06-25T14:10:00.002-04:00

While we were at Children's, one of the mothers who was there with her child said that she recognized Peter from his blog. I didn't get a chance to talk further with her b/c Peter was ready to keep walking, and when I would get free time later, her child's room was dark, suggesting that the child was sleeping. If you read this, I wanted to let you know that I appreciate your saying hello and encouraging Peter when you saw him. If you want to email, you can reach me at the email in the "about me section." Or you can post something on the blog, and if you don't want it posted for everyone to see, just say so and I can read it but not post it. I hope all is going well with your little one. - Kathryn

Boston visit June 2008

2008-06-25T13:37:00.003-04:00

We were up at Children's Boston Sunday through Friday. It was a good visit. Peter is now up to 25mL of Elecare five times a day... by mouth, no need for a feeding pump!! I think he would take more if we would let him, but it is not time to advance him in amount yet. We are however trying to increase the caloric density of the feeds. He has gone from 20 calories to 26 calories per ounce. The goal now is to get to 30 calories per ounce and hold.

We decided to try to free Peter from as many tubes as possible during the day, so his LR and TPN will both run over 16 hours, rather than the LR going 24 hours a day. And we took off the foley bags that collect the Gtube drainage and fistula drainage. We will hook them back up at night, but in the day he is free of those as well. When we feel he is stable with the feeds, we will take off the foley bag on the ileostomy in the daytime too. He will continue to still have the ostomy bags on his body, but without the long foley drains hanging off those bags, it is hard to tell he has them at all when his clothes are on.

We met with Dr. Jennings for a third consult on Peter. He is not a transplant doctor, but has an excellent reputation and tremendous talent as a surgeon. Interestingly, he was a resident with Dr. Flake (the surgeon from CHOP). I am coming to realize that we have three exceedingly talented surgeons weighing in on Peter, with reputations of excellence a mile long. What a blessing! Dr. Jennings took me downstairs to look over Peter's MRIs and Catscans and then we met with the head pathologist at CHB and looked at Peter's liver biopsies from Dec 2006 and May 2007. Then Dr. Jennings was called to surgery. Dr. Jennings and I finished up our consult via email. I am posting his comments here:
"To be blunt, I think this kid would have died if not for Omegaven, and now we have a child no one has seen before. These kids all died up to a few years ago. Now we have some time, who knows how much time, to get him to a physiologic position that he can either heal his fistula(s) or be ready to close them. And we have used some of that time to drop the portal pressures and control the bleeding. We need to get him to use his intestines. I think the fistulas are only one problem, the other is his liver function and ability to recover or tolerate surgery. I am not sure I know where we stand. the liver biopsies were not end stage, and they are a year old. He has improved in the interim. This is uncharted territory, and dangerous, but I am not without hope for Peter. I am encouraged that he is tolerating a little feeds, and I would very gently keep pushing."

So far it seems that everyone is in agreement that the best thing for Peter now is to feed the gut and continue to give Peter time to lead us in the direction he needs to go. Several times I heard the comment that "Peter is writing his own book. He is doing something that has not been done before."

When we go back up again in August, we will reimage the bowel to see what connections he has in there. All of the doctors will try to be in the room while the imaging is happening live. If the connections seem simple and he seems to have a bowel that is functioning as we begin to feed him more, then perhaps we can do a "simple" surgery to fix what we can. If there is a very complex bowel that can only be surgically handled by complex surgery, then he may not be a surgical candidate any time soon for fear of creating more bowel fistulas. The thought is that Peter may be changing internally and that even the images he had in February may be much different now. We keep hoping!!

Nutritionally, Peter has plateaued on the growth and weight curves for his age. We are really hoping the bowel works from a nutritional standpoint too, in that we can't increase the TPN any further without increasing the number of hours he is on TPN. Interestingly, we have had to increase the vit C again, and he will now get 180mg IV added to the vitamins in standard TPN, up from an additional 100mg. Since fall 2006 we have had to add nutrients to Peter's TPN above what is standard for his age. Vit C, zinc, vit K, protein are all in excess of standard dosages. The protein and vit K extra amounts were added in Boston. Sodium for Peter seems best given in small amounts as he holds on to it strongly. We can decrease the calcitriol (vit D) now; since he is doing so much weight bearing exercise his bones are getting stronger.

Peter did a great job showing himself off this time in Boston. He must have walked almost an entire mile in the hospital - seriously. Over and over I heard, "I can't believe that is Peter!!" Or, "Have you seen Peter? Can you believe how good he looks?" Something seems to be going in Peter's favor. The prayers continue in thanksgiving and in request for his continued healing and happiness.

Starting up the bowel

2008-06-11T20:27:00.003-04:00

We have restarted trophic feeds again. It is the first attempt again at feeding since March, when Peter started getting ill (from flagyl?). We started by clamping the G-tube a little bit each day until we hit our goal of being clamped for 12 hours a day. And we started feeds (elecare) with 1 tsp three times a day. Now we are at 2 tsp three times a day. The goal is 3 tsp three times a day, and then we are headed up to Boston for another check-up and a more rigorous testing of the bowel under controlled conditions.

I am excited to go up and resolve the question of whether the bowel works or not, as well as to see our third and final surgeon about whether Peter's bowel can be reconnected or whether we need to go on to transplant. The docs have been optimistic as much as they can... hard to know what to tell a parent when a child is doing things that have not been done before... as least as far as the liver goes. Peter should be getting chronically worse with his cirrhosis and his bleeding should be increasing and energy decreasing and he should be getting sick more and more. We don't see that... and we are thanking God every day!! To say that he is overcoming all of his health issues is hard to say. Maybe he is resolving some of them, or maybe he is just in a good place of equilibrium for now.

We will be testing his bowel function while we are in Boston by feeding him more aggressively and testing what comes out the different ostomies to see how well things are digested. We will be testing the liver function with a methionine breath test, which is still experimental but less invasive than a liver biopsy. We will also be looking at Peter's nutrition and determining how well his TPN is meeting his needs and what should be adjusted.

Peter's anemia is improving more and more since he stopped bleeding and since we put him on IV iron. His last hemoglobin was 9.9!! Platelets were only 68 though... they still are not normal. Labs generally improving over the months, but I can't say truly normal. The portal clot likely is still our biggest culprit of all of Peter's problems, as it continues to produce high pressure in the liver/portal system, affecting the spleen, possibly the intestine, and likely the liver too. Nonetheless, we are now at lab draws just every other week. CRP abruptly returned to normal three weeks ago and is holding at normal.

We are looking forward to showing off Peter's other progresses such as his walking and talking. A year ago we were still being transported to Boston with a medical flight crew and ground and air ambulance. Now we are driving up, Peter enjoys the ride, and we are planning whether or not to bring his walking toy or just have him hold my hand while PETER walks around the floor! Amazing!!

Goodnight Peter!

2008-05-27T10:01:00.003-04:00

Peter has been getting up at night the past few weeks to ask for water and then to do the toddler-thing and refuse to go back to sleep. "Let me have the cup Peter." "NO." "Well, you need to lie down, Peter." "NO." "Yes, you do. Your diaper is wet and I need to change it." "NO." He protests very loudly at this point and I remind him that everyone is sleeping. "The trees are sleeping. Don't wake them up." "Flowers?" "Yes. The flowers are sleeping." "Kisty?" "Yes, Kristy is sleeping." "Pat?" "Yes, Pat is sleeping." And we go through the names of his nurses and therapists. The fact that he has woken up any family members doesn't faze him and we go on listing other people/things that are sleeping until he decides he can in fact let everyone/everything continue to sleep by being quiet and going back to sleep himself.

Last night was a particularly long night of protests. To try to emphasize my point that it was time to sleep and he needed to settle down, I looked at him face to face and then went on further to talk about the animals he was keeping up. "The birds are sleeping, Peter. And the dogs." "Bees?" Surprised he made the leap to animals with me, I said, "Yes, the bees too." "Turtles?" "Yes, the turtles too." Now I am just amazed that he is going on this tangent with me and listing just animals. "Giraffes?" "Yes, giraffes are sleeping." Peter growls. "Yes, the lions are sleeping too." After he gives a lower growl I tell him, "Yes, the bears are asleep too. Now you go to sleep Peter." "NO." Ahhh, the great discoveries and trials of toddlerhood!

Mystery CRP, walking and talking

2008-05-22T10:33:00.005-04:00

Peter's CRP has been elevated for the last month. It began to go up the last time Peter was ill - when we discovered what we think is a connection between enteral flagyl and his last 3 bouts of illness. No one yet seems to know what to make of it, but it is not going down. Peter has not grown anything from culture, doesn't act in the least bit ill, and temps have been good. The rest of his labs have been stable too. In fact, the last set of bilis went down again. Unfortunately, the CRP elevation still leaves us unable to test his bowel with feeds, as it could be an irritated bowel causing the CRP elevation and we don't want to stress things further. The bouts of illness also coincided with the increased clamping times of his G-tube as well as with the enteral flagyl, which we stopped at the same time we took away the enteral flagyl. We don't really know which caused Peter to become ill each time. I am voting for flagyl since that would be a simpler answer. But since we don't know and CRP is still up, no clamping, no formula feeds, no real testing of the bowel's function. We may have to wait for more hospital tests to be performed when Peter goes to Boston again before we can move on testing the bowel function.

Meanwhile, our little guy is learning to walk solo. He is just doing solo steps in spurts, but he is doing it. He gets scared to hold onto nothing though. It takes a good amount of coaxing until he practices several times going back and forth between a person and the couch. When he walks around the house though, he is barely holding on to one finger of one hand of the person walking with him. He really doesn't need the support much at all. The little car walker is really only for outdoors now, as he has gotten so stable walking indoors holding a finger.

AND our champ is beginning to talk! Speech came to see him after a month, and she was thrilled with how much he is speaking now. We had been trying to use a combination of sign language, picture communication book, and speech for the last few months. This last couple of weeks Peter has been repeating what he is asked to say (with some toddler variations in the way the words come out of course). He is FINALLY saying the word "NO," and I have never been so excited to hear a toddler say no. "Peter, we need to change your diaper." "NO." There are pros and cons of course to a toddler who can communicate that he does not agree with your plan, but better he say no than just yell about it. The hard part is not smiling when he so cutely says "NO." I don't want to encourage that too much!

So what did Dr. Flake say?

2008-04-30T20:52:00.004-04:00

While we were at CHOP two weeks ago inpatient, the GI doctor there felt that the best way for us to get Peter's bowel up and going is to surgically put it back together. That would give it the best shot at showing us how functional it is. Dr. Kamin has said the same. Sounds simple. Dr. Kim has told us that Peter's bowel may never be able to be surgically closed as of our last trip to Boston in February. However, Dr. Kim encouraged us to get another opinion, as someone else may see an option that he doesn't. So while we were at CHOP, Dr. Flake did come to see Peter and me. The bottom line is that he also feels Peter is a tremendous surgical risk and that his option is really only transplant at this time. Risks include: portal hypertension (bleeding risk during and after surgery, and possible poor healing from altered cirulation), increased coagulation times (bleeding risks again), ascites (poor healing and fragile bowel tissue), prior surgical scarring of the bowel (adhesions in the bowel that would be stronger than the bowel itself, lending the bowel to tearing when trying to separate the adhesions), as well as the facts that we don't know if the bowel actually works, and that we still may end up needing a liver tranpslant down the road if the liver truly isn't much better than it was. Finally, Peter has to heal from what Dr. Flake felt would be multiple risky operations. He said he has people with healthy bowel develop fistulas after bowel surgery and worries that Peter would only develop more fistulas with surgery. A transplant would give him a whole new bowel, new liver, and remove the portal hypertension because his portal vein and clot would leave with his liver and be replaced with the new liver and its circulatory veins and arteries.

Not the answers we were hoping for, and Dr. Flake apologized that he couldn't give us the non-transplant answer that we wanted. So we are planning to seek the advice of a third surgeon who works with Dr. Puder who is the Omegaven doctor at CHB. We would plan to see him the next time we are up in Boston. That will give us time for the TPN nutrition changes to kick in and maybe even to begin testing the bowel a bit more. If he also says there is no other option, then that is our answer. But he may see things differently, as he has for two other short-gut children that we are aware of. Dr. Kamin reminds us that Peter is doing things now that have not been done - kids as sick as Peter have either gone on to transplant or died, not acted better and better, and not improved by labs and examination. So he may still surprise us with the improved TPN nutrition and drop the ascites, may continue to lower the coagulation numbers (last INR was 1.3), and may have a healthier bowel with the improved nutrition. Portal hypertension may still be of concern with operating, or maybe there is something we can do to assist with that risk as well.

Everything has some big risks - surgery to put the bowel together, transplant and post-transplant living, staying on TPN, having portal hypertension, having a central line (the PICC line). Once we get our third opinion, then I guess we weigh the risks and try to choose to keep trying to rehab the bowel or to transplant. Or we may not have a choice... but we feel that we would be fine with that too. We have been praying for direction and patience while we wait to see which way his bowel and liver and surgical opinions go. We are always grateful for Peter's smiles and playfulness... which have been more abundant than ever. Maybe that is God's way of giving us patience - having Peter show us that he is content and patient, and we should be too.

Keeping us guessing

2008-04-30T20:11:00.005-04:00

Peter had ANOTHER mystery illness with low-grade fever, nausea, abdominal tenderness, fluid gain, increased respiratations and heartrate, and grunting respirations. And again it was the weekend... at night. And then the idea struck that perhaps these mystery illnesses have something in common. Peter has not been ill in so long, and then three times in less than two months?! Looking back through notes there are two things that seem in common as possible causes - enteral flagyl or the more lengthy time spent with the G-tube clamped off. So, we stopped both, and Peter pulled right on out of the illness within 24 hours after stopping both things. On Monday's routine lab draw, his numbers all look good, except for the C-reactive protein (CRP) which went really high and is a marker of inflammation or infection, but is non-specific. We got cultures drawn from the PICC and urine (done at home, to our great relief), but he is still acting well since our Friday illness, so the CRP may mean he picked up a virus, or that the possible allergy also caused inflammation, or somthing else... but if he is acting well nothing to worry about, unless it keeps rising in subsequent labs.

The enteral flagyl was to treat bacterial overgrowth in his gut which is a common issue in kids that aren't eating. We will switch back to cipro again, every 3 weeks for a week via G-tube. He has tolerated that well in the past.

We will also hold off on clamping the G-tube again for another week or two until we feel certain Peter is not ill by labs and behavior and time. So, still no enteral feeds and now no clamping. Not making much progress in the enteral department this way, but it would be hard to decide if he is having intolerance to feeds or to clamping if we threw that into the picture and he started acting ill again.

He seems to be liking the extra protein and calories in the TPN. Weight is still down, but his energy is definitely up. He keeps wanting to walk with his "car" walker from room to room, pausing to look in the refrigerator, kitchen cabinets, and to smell almost every spice that we have... several times a day. And, he pauses to look at his reflection as he walks past the stove and the dishwasher, with a gigantic grin of well-deserved satisfaction.

Peter's walker, "car"

In the kitchen

In and out of CHOP again

2008-04-18T20:23:00.003-04:00

We had another unexpected admission to CHOP last week. Peter was fussy, temp on/off, and then began vomiting. If you get a fever with a central line, it pretty much automatically means that you need to obtain blood cultures and begin antibiotics for at least 48 hours. If nothing grows out of the culture, antibiotics can be stopped. Last time Peter continued antibiotics without anything growing because it was possible he had a pocket of infected fluid. This time though, he had been exposed to a cold, strep throat, and a GI bug with vomiting. After nothing grew out of the cultures, he was taken off antibiotics and sent home, looking pretty good for a kid who three days before was vomiting and two days before slept almost the whole day and had started retaining fluid. Peter almost always retains fluid when he gets hit with a virus or bacterial infection.

The good that came out of the admission was that we had a very astute GI attending (the director of the GI dept) who saw beyond the current illness and began discussions with our Boston team about Peter's overall illness and progress. This has helped us in the reevaluation of his TPN nutrition, which always seems to be such a delicate balancing act. Peter's liver numbers are good enough that there has been some question if he is actually getting enough protein, even though by the books he is receiving a huge amount. We are going to go ahead and try making some big changes, increasing his protein and increasing his calories by quite a bit, which hopefully will boost his energy and reduce the ascities. We have our fingers crossed. It seems worth a try, and if Peter can't handle it, then we can always go back down on the protein. Dr. Kamin is in complete agreement, and I think is glad to have another GI who also feels that Peter may need more protein, whether "the books" say he is getting enough or not.

We also met with Peter's CHOP surgeon, Dr. Flake, and discussed Peter's surgical issues with him. That is for another post, but the meeting was sincere and was actually suggested by Dr. Kim the last time we were in Boston. So, I must say, that as much as we always dislike an unexpected hospitalization, especially if Peter is acting ill, the admission gave us the opportunity to discover and address some needed tweaking in Peter's care.

Insurance fun

2008-04-09T21:33:00.002-04:00

So, the next time your insurance company cheerfully tells you that they have approved a service for you, find out if they are applying it to your out-of-network lifetime maximum. It has come to my attention recently that several of Peter's services have been approved this way. Things that should be covered in-network should not be penalized as out-of-network if there was no one available to provide the service in-network. But that is not how the insurance company has billed it, nor is it how it was presented to me. I was just told the services were approved... and so I relaxed and we cruised along getting Peter the services needed. So now our lifetime out-of-network max is halfway met now, and the one justifiable out-of-network provider that we need every last out-of-network dollar for is Children's Hospital Boston.

"Well, you can still use unlimited in-network providers."

"Can you tell me the breakdown of just where all of these out-of-network costs are coming from?"

"Well, if you access the website and pull up your claim..."

"There are over 250 claims! AND there is NO indication of which ones you have paid in-network vs out-of-network. AND everytime I try to ask someone if this place or that provider are in or out of network I get a different answer! ... If my son needs a transplant at the out-of-network provider, is it still covered if we exhaust out-of-network benefits?"

"I can't tell you that it is covered, but I can tell you that since there is no one in network who does this type of transplant that you would be approved to have it done out-of-network."

"So it IS covered?"

"Well, I can't say that it is, but let's just say that since there isn't anyone in-network then you would have to go out-of-network, and transplant is covered. Do you catch what I am trying to say to you? In other words, I can't tell you it is definitely covered, but it is approved. And follow up services would be approved too since it is transplant and no one in network does that type of transplant."

So, is it me, or does this insurance-talk make no sense at all? I still can't figure out if Peter is covered for transplant if we blow through all of the out-of-network monies or not. And that was the case manager's explanation!! I'm not pushing for socialized medicine, just want straight, consistent answers and fair processing of Peter's bills. It is amazing how so many people in big companies can forget about being human beings and just treat you like a number. What if I were your sister or your mother or your daughter and asked you that same question? Well, enough ranting for now. There are more calls to make tomorrow.

Ultrasound results

2008-04-09T20:25:00.003-04:00

Dr. Kamin reviewed the ultrasounds from CHOP and the good news is that there is NOT air in the liver as was initially feared. Yea!!

That being said, to be extra careful, we are going to wait at least one more week before restarting feeds, as to not blur a possibility of an unfound lingering infection rearing its head now that he is off antibiotics. Peter has had no feeds since he went into CHOP on March 14. As of yesterday though, we are allowed to give him fruit in a mesh bag again. He has been having water all along.

Peter's CRP is back down to normal. He ended antibiotics 8 days ago and is still afebrile and labs support that he is as good as he was before the emergency hospitalization. We are thinking now that maybe he did have a bowel obstruction... maybe. A piece of the wafer from the fistula appliance was found in the ileostomy appliance on day two of our admission. Possibly it balled up inside and caused a brief blockage inside his gut which is still quite unaccustomed to anything more than liquid at this point. The stricture towards the end of the ileostomy would be a likely place for something to back up. The other thing it may be though is a sort of intestinal angina, where the intestine cramps up as blood flow tries to increase for digestion but can't. The result is similar to heart angina, where a diseased heart tries to increase blood flow for an increased activity level but physically can't. I don't think we'll have an answer to this though until we retry the bowel with formula again.

We are thinking more about transplant these days... preparing for the possiblity that this bowel just doesn't work and/or this liver just can't go enough of the distance to avoid transplant. Peter still has a portal vein clot that will never go away, and only a portion of liver left from the initial surgeries, that is damaged, but spared enough by Omegaven to get our son as strong as he is now and to keep him home for over a year now. The ascites is still ever present, the spleen remains large... mainly thought to be from the portal hypertension due to the portal vein clot, vs from TPN cholestasis. The chances of Peter's bowel working well enough to get off TPN are a very long shot. This is not to say that we are done trying to use this gut yet... if the bowel works, we will praise the miracle that it will be. But if we still are getting nowhere in the next few weeks to months, then we can still say that we tried all that we could, and the new chapters of Peter's life will then contain the words "transplant" instead of "short gut."

In and out of hospitals

2008-03-19T21:34:00.007-04:00

So the visit to Boston at the end of Feb. was a well-check. We reimaged the upper and lower bowel and ultrasounded the abdomen. Results are that the gut appears to be truly short at this time, but whether this is due to scarring or disuse is still to evolve. Peter still seems to have his own unique pathway from the stomach to the ileostomy, so it is possible for formula to pass the whole way through from stomach to ileostomy, although we do know that some will exit out the duodenostomy and maybe the jejunal fistula as well. The liver appears homogenous in the ultrasound, which I am told is a good sign. Dr. Kim, Peter's surgeon for transplant or reconstruction, was not very optimistic about Peter's abdominal ultrasound, concerned about the amount of ascites still present and the shortness of the gut we now see, as well as the usual concerns about bleeding times and portal hypertension. However, he and the GI and Omegaven doctors all felt that we should begin to try feeds again, and we will talk about the surgical prospects (or lack of them) after we have seen how the bowel actually is working and digesting food now. So we agreed to start feeding 1 Tbsp and clamping the G tube (which is usually to drainage) for 2 hours afterwards. After 4 days go to doing this twice a day. After 4 days go to doing this three times a day.

He seemed to be doing great until this past Friday, March 14. Out of the blue Peter began to fuss and act quite agitated, very uncharacteristic of him. I changed all of his ostomy bags, G tube dressing, examined all I could for irritation and could not pinpoint what the problem was. About 5 hours later his temp went up to 100 and we called Dr. Kamin for advice. He told us to go to the ER to check for bacteremia and for peritonitis, ASAP. We wanted to go up to Boston, but he felt that something acute could be going on and wanted us triaged up there if need be, but not to drive such a distance not knowing if Peter was in trouble. So we were admitted, cultured, started antibiotics, and had another ultrasound as well as abdominal and chest x-rays. Peter and I stayed in CHOP through Tuesday, and we still don't know what the problem was or is, but he is home again, on IV antibiotics and is to hold on feeds for now. It is possible it is just a cold. It is possible he has an infection of one of the pockets of ascites in the abdomen. It is possible that his gut can't even handle 3 tablespoons of formula a day. It is even possible, although not much more than a "hmmm" was accredited to this: that the little piece of fistula ostomy appliance that I found in his ileotomy bag caused a temporary blockage and a lot of pain and inflammation until it passed though the gut. As always with Peter, nothing is ever very clear as to what the problem exactly is or what the cause was. In CHOP he put on a lot of fluid and his albumin dropped down, both of which he does when he gets ill with virus or bacteria, but now he is back down in weight and edema. The good news of it all is that many people at CHOP saw how well Peter has done on Omegaven and I got the chance to talk about it to several nurses and residents and two of the attendings. Various CHOP personnel say it is "good stuff," and that they are working on getting it. But they have been "working on getting it" for a year, and that just doesn't make sense when so many other hospitals have been able to get it already. Peter isn't the best candidate to make a grand impression for Omegaven because of his portal clot and severe intestinal condition. But his liver numbers are impressive and the people who remember Peter also remember his liver numbers, so maybe that is statement enough for the benefits of Omegaven. Well, more to post later.

Taking a walk in the house Feb 22

2008-02-22T22:33:00.011-05:00

I still need to figure out the lighting issue so videos don't come out so dark, but here is Mr. Peter walking in the house. PT wants us to have him hold hands to walk now... he was holding onto the shopping cart for support, but as you see in this video his sister found it useful for transporting his 3 drainage bags. Peter's daddy has the IV bag on his own back and Peter is holding his hands. We can't get over how quickly he is walking now! He can take some steps holding just one hand, but they are much slower steps and fairly wobbly. Judging by how quickly he has progressed in his walking, we think he'll be solo within a month. Not sure how we'll juggle the IVs and drainge bags then!?!

Happy Anniversary!

2008-02-21T20:58:00.004-05:00

Tomorrow is February 22. It is Peter's one year anniversary home from Children's Boston... after being hospitalized for nine straight months. Way to go Peter!!

We go up to Boston again shortly. (I mentioned in the last post that we were making plans; they were for this upcoming visit.) We had been hoping that his direct bilirubin would have hit 0.4 by now, but he had a slight bump in the bili mid December and it just is not budging back down again. He was 1.5 again on Monday, with the total at 2.8. The lowest Peter had gotten was 1.2 direct and 1.9 total. On the flip side, his albumin has remained at a better number, with 2.5 this past week and 2.9 two weeks ago. These numbers are up from 1.4 back around late June, and I am thinking we were even down around 1.2 or less in August. AND, his liver function tests have improved overall. The bleeding times (INR, PT, PTT) are still off though.

So, we will see what the good doctors in Boston have to say while we are there. Peter has come so far in this last year, especially considering that for the first half of that year home his health went up and down several times to the point we even had him actvely listed for transplant. We are praying that Peter remains in good form while we are there and really does an impressive job showing off. His homecare nurses have been phenomenal in helping him work on his PT and OT goals, and he has much to demonstrate. Last time we were in Boston he just took a nap... everyone ooohed and aaahed over his lack of jaundice and his nice weight gain. When he was awake, he pretended not to know any of his colors or shapes or animals while others were in the room, regaining full knowledge once it was just him and me again. Come on Peter, we're counting on a real show this time!

Ups and downs

2008-01-31T20:53:00.001-05:00

The good news:
Peter went for two whole months this time before he needed a blood transfusion!! Last time I think it was five weeks, which even then had been an improvement.

Peter now has a simple G tube, not a G/J, as the J portion (which actually was in his duodenum) clogged. The doctors in Boston felt that Peter would be able to go to just a G tube since he was tolerating the clogged J portion so well. His G tube is still to drainage, but to go from 4 drainage bags with tubing down to 3 really does make things easier. I can hardly imagine how excited we will be when we get down to zero drainage tubes hanging!

Peter is walking more and more now. He has learned how to cruise the couch and he will take walks around the house holding onto his shopping cart which is in turn holding his drainage bags. The nurse or one of us goes behind him and carries the IV bag and makes sure the cart doesn't go faster than he does.

Peter is happy! That is always a good sign.

The bad news:
The G tube is still putting out yellow fluid as well as clear fluid. This indicates that the flow of bile is not always going downstream as it should, but also is getting up into the stomach.

Peter's direct and total bilirubins have been inching upwards. We are not sure why. As such, we are seeing if we can lower it by taking away all enteral liquids except for water. He had been getting tiny amounts of milk, plain yogurt, popsicle, and apple in a mesh bag... for months, as to his desire. So far he doesn't seem to miss these treats and is content with water and brushing his teeth twice a day. The bilis stopped dropping in December and then began to go up. Last liver labs from January 21 were:

direct bili 1.4
total bili 2.9
but, albumin at a much nicer level of 2.7
and the other liver function tests were at better levels too,
however the INR (bleeding time) was up to 1.7 from a low of 1.4 a month or so ago.

The local GI doctor who helps us with our transfusions thought we should do some iron studies to see if Peter needs iron added to his home TPN. The iron could help out with the anemia. In the past Peter's iron levels have been good, but maybe now he needs supplementation. I don't know if anemia from low iron levels would affect his bili numbers and INR. It will be a question to ask if the iron levels come back low.

Neither good nor bad news:
We are making plans to go up to Boston again. Plans include an ultrasound of the abdomen and an upper GI with small-bowel-follow-through, as well as a visit in TPN clinic. Well, more on all of that later... I have just been alerted that my darling son is peeling up tegaderm around his ileostomy and that will be a huge mess if he succeeds in getting the ileo bag off!

New year, new PICC

2008-01-03T20:47:00.000-05:00

The replacement PICC procedure went well. Dr. Cahill at CHOP was able to rewire the same size and type of PICC through the same opening, and it was able to be done without intubation, just sedation. There was some initial hold up in getting into IR because there has been some type of recall on the PICC that Peter was to receive, but after scouring the hospital, one was located that was the right size and type and not of the recall lot.

Overall the procedure went well and I was finally feeling relaxed. As we were getting ready to go though, I asked for a copy of Peter's labs so I could pass them on to the doctors and the company who prepares his TPN. This is a routine request I have wherever we go and labs are drawn. It is never a problem in getting the labs. I carry a folder of Peter's labs with me and in fact had gone into the folder to show one of the nurses in IR his last set of labs. To get a copy of labs this time though was QUITE the ordeal "due to HIPAA regulations," I was told. It didn't seem to matter that just two days ago that CHOP's ER was able to just hand lab results to me. This dept refused to give me a printout, BUT I was allowed to look at them and write them down off the computer screen. Then I said I really needed a printed copy too since they also were refusing to fax the results to anyone, so a phone call to medical records and a signed form later I got the printed copy. But even then the nurse wouldn't fax the labs over to Apria who was in need of them to update TPN right away if needed... instead the nurse showed me the unit's fax machine and had me dial the number. The absurdity of the matter didn't seem to faze her or the attending doctor (not Dr. Cahill)in the recovery area. So it goes so often at CHOP though... a frequent lack of logic when it comes to dealing with patients. Fortunately that does NOT seem to be a problem we incur at Children's Boston, which is another reason we continue to go there.

In our seven hours there, we ran into 8 people who remembered Peter from our seven month stay in CHOP. And we had two hellos passed on to us from people who heard Peter was in CHOP. Those who knew him before were all impressed with how far he has come along now. I told everyone I could about Omegaven. When we were in the ER three days before when the old PICC became dislodged, we saw two of Peter's residents who had cared for him during our seven month stay. They also were impressed with his growth, coloring, and general improvements. I hope that someone from CHOP can take the evidence that is seen with Peter and conclude that Omegaven is indeed worth using at CHOP too. It would be a logical (there's that word again) thing for CHOP to offer to parents for their children given all of the evidence out there, including the evidence of one of their former patients - Peter.

One last 2007 punch

2007-12-31T21:39:00.000-05:00

Peter's PICC line has unfortunately dislodged on us... Sunday afternoon... two days before New Year's Day.

Peter was getting his usual PICC dressing change on his usual day, Sunday. But he decided that he had had enough of the dressing change faster than we could stop him from moving, and the PICC line moved out of its insertion site about an inch. The look on my face told the other children that things were not good and then I burst into tears. I called Dr. Kamin in Boston to see if he thought it had moved too far. He said we should get an x-ray to determine if the PICC had moved too far out. The local hospital said they didn't want to x-ray Peter because they didn't have a radiologist in house... Sunday afternoon... and they don't usually do children.

Long story made short, we ended up going to CHOP's ER. We got an x-ray taken about two hours after we got there and were told the PICC had indeed moved too far to use for TPN... could end up throwing a clot. Lots of phone calls to Dr. Kamin in Boston(what a dedicated doctor!) and talks with the ER and ER calling Interventional Radiology... go to Boston's IR? ,go inpatient at CHOP?, need a transfusion?, will anyone get worked up about Peter's current anemia (normal for him, but still abnormal), etc. So we drew a CBC just to see where that was and behold he was 8.2, up from the week before, so no transfusion! Boston was expecting 8 inches of snow, so that answered the question of driving up there from PA. And IR was double-booked for the entire next day and closed for New Year's Day, so it was pointless to stay in hospital... and actually we were glad to hear that, so it made the decision easy to come home and wait for an available IR appointment.

Now Peter is home on a make-shift concoction of electrolytes and sugar in IV form until he gets a new PICC placed this Wednesday at CHOP. Fortunately it should be able to be done outpatient and he should be in and out within about three to four hours from arrival to departure. The procedure should quick once he is in there, with light sedation, no intubation expected.

Peter has had this PICC line since October 2006, so I guess we can't complain too much. The timing stunk, but at least it didn't happen on Christmas!

Still improving

2007-12-19T15:59:00.001-05:00

Peter still gets weekly labs, but his PICC line makes it painless for him. I always spend the rest of the day fretting about what the numbers will be. Usually hemoglobin is the main worry, as he continues to have bleeds from the fistula off and on. When his hemoglobin gets down to 7.0 or so he needs to get another blood transfusion.

This week the worry was more about what the other numbers would be, since last week's hemoglobin was 9.0 and he usually doesn't drop down to 7 within a week. Last week he showed us that he was holding onto fluid and that threw his albumin and sodium off, and he had some fluid building up in his legs. It seems that his fistula is not putting out as much fluid lately... which is a good sign, but in return we ended up needing to decrease the amount of replacement fluid we've been giving him.

The results of this week's labs are back. This week we seem more balanced in the fluid department and albumin is back up to 2.4 (still low, but better than 2.0) and sodium is normal. Direct bili stayed at 1.2 this week, as it did last week, and the total bili went up to 2.1 from 1.9. The discussion is whether this is due to addition of plain yogurt or not. We have been giving small amounts of milk as desired for months and thought the yogurt would also be alright, but we are going to cut it back from 3 to 5 tsp daily to just 1 tsp daily. Hopefully the bili's will continue to go downward consistently as they had been. Dr. Puder reminds us to be patient and not change things enterally until the direct bili gets to around 0.4.

The hemoglobin this week is 8.4, so if we can keep bleeds at bay, maybe we can avoid transfusing during Christmas week too. The fistula bleeds so much due to irritation and due to clotting ability still not being normal. That should improve as the liver improves. We are also experimenting with different ostomy products with the help of Children's Boston wound care nurse and some of our own trial and error. The fact that Peter is able to keep any ostomy appliance on his abdomen at all is quite remarkable - a good advertisement for the Convatec company indeed. He just folds himself right in half when he wants to get onto his tummy, and that is exactly where the fistula and that one ostomy appliance are. (He has two other ostomy bags too, but they don't get quite as taxed as they are more off to the sides.)

Peter knows his entire alphabet at age two and one month. He still doesn't talk, but you can ask him any letter and he'll find it. He learned the letters in about 3 months. Dr. Puder says the Omegaven babies seem to be very bright. I don't know if it is the Omegaven or the one-on-one care that he gets with home nursing. Regardless, knowing the alphabet at an early two years of age is definitely worth bragging about, even if Peter isn't walking or talking yet!

And though I don't write about it in every post, I never forget that the true source of all of Peter's progress, and our strength, is our good Lord. God is good and we are ever grateful. Merry Christmas to all of you who take the time to keep up with Peter's news.

Inactivated on transplant list!!

2007-12-06T21:54:00.000-05:00

We went up to Children's Hospital Boston for our very first outpatient visit! And we drove for the first time too! We live seven hours away from Boston, so it is quite a drive. Peter did amazingly well. We have previously had to go up via Medflight due to Peter's difficulty in travelling in a car without getting beside himself - never knowing if it was something wrong or a simple tantrum or what. Up until August he didn't ride in a carseat, but rather a make-shift car bed. So, seven hours in a carseat with only one long stop, and very few tears or complaints. Who could ask for more?

Dr. Kim, Dr. Kamin, Dr. Puder, and Dr. Lo all came in to see Peter. Everyone commented on how good he looks. And we have decided to make him inactive on the transplant list... the urgency for transplant seems to be gone. The liver injury appears to be halted, possibly reversing. With the liver cooperating now, we don't expect that he will suddenly nose-dive into illness as he would do if he were still declining in health of the liver. This is really amazing. There have been about 70 children pull out of liver injury on Omegaven at Children's Boston. We expect that Peter will follow in their tracks and continue in his improvement. He is apparently the first child to pull out of/halt the liver injury who has been as sick as he was for as long as he was.

We still have the bowel issues to deal with. We don't know if the bowel will work well enough to eventually come off TPN, but with the inflammation to the liver gone, we can soon begin to test the bowel. Since we don't know that the bowel will work, and we don't know if surgery to reconnect the bowel will be successful, and because Peter's liver was so sick for so long, we are for now just inactivating him. I have officially unpacked my bags that have been packed since August for when the transplant call came. We are a little nervous about our decision, but deep inside it seems right. Peter acts like he feels the best he has ever felt. He looks good and labs are good. We continue to pray and we thank God every day for his happy little self.

Comments Enabled

2007-11-08T08:37:00.001-05:00

Still new to blogging. I think now I have the settings set for comments if anyone wants to leave a message.

So we are saying no to transplant for now

2007-11-07T20:25:00.000-05:00

Peter's bilirubin has dropped 21 points since we listed him in August! His CRP (C-Reactive Protein - an inflammatory marker) is normal for a few weeks now. Going by how the other Omegaven children have improved, we should see Peter's bilirubin hit normal within just a few more weeks. With a normal CRP and a normal bilirubin, we should be able to begin refeeding Peter and then see what his bowel can really handle since the liver inflammation should be gone. This is still all theory to some extent because none of the other children have pulled out of liver injury after this long. They have all gone on to liver/multivisceral transplant or, sadly, have died by this point. Peter is not just hanging on by a thread either. He has gotten remarkably stronger since the listing and has even started standing.

The transfusions still are frequent - every 3 to 4 weeks. The portal hypertension is probably still at play, as is the fragility of the enterocutaneous fistula and its proneness to bleed from the stoma site itself. The INR remains elevated, that is a bleeding number that would be better to be lowering at this point. The elevation of the INR and the low (but stable) albumin number may likely be due to his intestinal losses from the fistula, as opposed to the liver still being so sick. It is all hard to tell right now.

Peter is due to go for another check up in Boston in a few more weeks. We are actually going to drive him up vs Medflight, and we are going to be seen outpatient vs inpatient. Very exciting news.

The multivisceral/liver transplant call was one we would have accepted just two months ago when Peter seemed like he was not going to pull out. I hope we have made the right choice in saying no to transplant for now and at least seeing whether the bowel will work once the liver finishes the recovery it seems to be making.

In case anyone is wondering what changed after we listed him, we increased the dose of Omegaven by 11% since we hadn't adjusted the dose when Peter had gained weight. But probably more importantly, we pulled the feeding tube out. He was getting all he needed with TPN but we had trophic feeds going at 2mL an hour nonstop running through a sick bowel. Dr. Puder has seen with his studies that when the sick bowel is allowed to rest or to work less the bilirubin then begins to to down and the CRP goes down. The Omegaven allows the patient to have time for all of this to happen without the liver getting worse on TPN, and also seems to play a role in healing the liver with the omega 3s. Once those numbers are normal then the bowel can begin to be refed more aggressively in attempts to rehab the bowel. The liver injury does not seem to return once it has cleared. Peter's portal vein clot and his ever-draining bowel add additional complications into the picture making it unclear if Peter's course could follow that of so many of the other Omegaven children. We will see.

We have the most wonderful doctors at Children's Hospital Boston - Dr. Kamin, Dr. Kim, Dr. Puder. We are ever so grateful for their ongoing support. They never just "shelf" Peter as this continues on and on. They are respectful of our decisions and indecisions. They are genuinely nice people. What a true blessing!

On top of the world

2007-09-03T21:32:00.001-04:00

So Peter is acting like he just feels wonderful. He has accomplished the task of rolling onto his tummy and back onto his back over the last week. He is working on tummy-time play for twenty minutes at a time. He has been sitting in the highchair contentedly for an hour to an hour-and-a-half, either reading, gnawing on a piece of fruit (in a mesh bag of course, as to not clog up his G/J tube), or just watching the rest of us at the table.

His coloring is good this whole last week. He gets labs drawn tomorrow. We are prepared for a transfusion if need be, but if his behavior and coloring are actually telling us accurate information, then he should not need one again this week. Peter has fooled us all before though... looking just fine, but with labs that tell a different story.

I feel like I am the eternal optimist here, a characteristic that I would not necessarily have claimed myself to have prior to Peter's situation, but the increase in Omegaven and the removal of the enteral feeding tube quite possibly could have helped out his liver immensely. The enteral feeding tube may have been irritating to the very fragile bowel Peter has, and the feeds, though slow, were continuous and perhaps were also too much for him to handle. (Dr. Puder has discussed bowel stress as an added strain to an injured liver several times as we have discussed lab results.) The Omegaven dose for Peter went up by 11 percent about four weeks ago, which I think is a substantial amount when we are talking about a med that is given at 10mL/kg. He went from 100mL to 111.6 mL.

So tomorrow we will look at the lab results and see if Peter is doing as well as he looks, or if he is looking and acting well despite his illness. I do hear and read that with liver/intestine illness combined, the person can seem more well than someone who needs a liver alone, and then suddenly become quite ill. The tranplant listing process seems to accomodate for that by adding an additional 23 points to the listing score for the liver/intestine/multivisceral patients, as compared to a person waiting for a liver alone.

I'll keep you posted on Peter's labs.

**** UPDATE on Kawasaki and coronary arteries from August: Cardiology reviewed Peter's records and images from CHOP and CHB and it seems that Peter has large coronary arteries, but that they have not suddenly enlarged. With his INR elevated already he does not need any kind of anticoagulation. Whenver the INR returns to normal we will revisit the coronary arteries and see if we feel the need to anticoagulate with low-dose aspirin therapy or not. There is a risk of clots with large coronary arteries. We will reimage the coronary arteries at another time when he is put under anesthesia for another procedure, but for now the concerns are minimal and at bay.