Two nights off TPN per week!

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

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

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

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

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

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