December is here and almost gone??!!

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

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

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

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

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

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

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

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

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

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

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

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