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.
Wednesday, December 7, 2011
Saturday, November 19, 2011
Pics from the zoo and Halloween
Birthday, wounds, and TPN progress
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.
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.
Wednesday, October 12, 2011
Home!!
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!
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!
Thursday, October 6, 2011
Another bleed, but...
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!
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!
Saturday, October 1, 2011
Day 23
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.
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
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.
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.
Sunday, September 25, 2011
Day 17 since surgery
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!
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!
Thursday, September 15, 2011
Still in the ICU but improving
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.
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.
Sunday, September 11, 2011
Still intubated
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.
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.
Friday, September 9, 2011
Fistula surgery
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.
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.
Sunday, August 14, 2011
Peter's recent trips to Boston
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.
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.
Thursday, July 7, 2011
Reciting the alphabet - forwards and backwards
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!
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!
Monday, June 27, 2011
Fistula increases again
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?!
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?!
Tuesday, May 31, 2011
The survey says... chyle
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.
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.
Wednesday, May 4, 2011
A chyle leak
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!
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!
Monday, April 18, 2011
The fistula, the fistula
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.
Monday, April 4, 2011
Reconnected!!
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!"
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!"
Wednesday, February 23, 2011
Cheeseburger!
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.
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
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!
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!
Tuesday, February 8, 2011
Liver concerns and another cancelled surgery
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.
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.
Tuesday, January 11, 2011
Back on Protonix again
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.
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.
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