December is here and almost gone??!!

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

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

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

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

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

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

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

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

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...

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

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

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

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

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

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

Omegaven News Article

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

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!!

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

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

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

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!

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

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?

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

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

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

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

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

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

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!

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

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

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.