Active toddler challenges

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

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

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

Feeds and bleeds

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

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

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

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

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

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

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

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

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

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

Kefir

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

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

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

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

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

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

outside videos

Omegaven News Article

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

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

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

Setbacks and progress

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

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

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

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