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.

Pics from July Boston trip

This is Peter in pre-op, looking at the smiley face Dr. Jennings drew on his index finger and his big toe. Those smiley faces produced three days of amusement for Peter!


This is the finished dressing with nursing pad and tegaderm covering the dressing pictured below.


This is the old dressing with bottle nipple over allevyn, over aquacel, over zinc paste.

When you consider that this dressing got changed one to three times a day b/c the leaking was that bad, you can see why we wanted to get that gastrostomy site fixed! Peter and we are all happy with the new, no-dressing-needed site!

Boston updates... from July!

Peter went up to Boston at the end of July for SURGERY! These were not the intestinal closing surgeries that we hope for... one day. But they were surgeries nonetheless. The big deal about "surgery" for Peter is that we had been told in the past the he is a terrible surgical candidate due to his portal hypertension (great bleeding risk), elevated INR (another bleeding risk), and history of poor healing from earlier surgeries on the abdomen. Dr. Jennings has discussed Peter's surgical risks and possibilities with us before (see old posts). As such, we all felt it was a good idea to do these surgeries to help make Peter more comfortable as well as to see if he heals better then he has in the past and to see if the bleeding turned out to be terrible or not.

The surgeries that Peter had done were to move the gastrostomy to a new site, close the old gastrostomy, and close the bilateral hydroceles. This all came about because we were going to be putting Peter to sleep for an upper endoscopy (EGD)to evaluate his esophageal varices (which are due to portal hypertension). I will try to post pictures of his last gastrostomy site and how we had to dress it to keep it from pouring out. The old dressing consisted of aquacel, allevyn, zinc paste, part of a nipple from a baby bottle to stabilize the G-tube, a nursing pad over all of that, surrounded by tegaderm all along the borders. This would be changed one to three times a day, depending on leakage amounts. Our new beautiful G-tube only needs tape to keep it secure, no dressing, just a little skin prep or zinc to protect from scant G-tube ooze. The old site healed remarkably well with some stitches to close the stomach opening and wet-to-dry dressings to close the rest of the hole. It is just a tiny scar now. The hydroceles healed well too. There are two perfectly-healed thin scars from that procedure. Dr. Jennings and Dr. Kamin said that the amount of bleeding was not much, especially considering that he has portal hypertension. Also, the EGD revealed that there has been no increase in the size of the esophageal varices since the evaluation two years ago.

We have started nighttime pump feeds with Peter...very slow rate to begin with. (Elevating the head of a toddler bed-- to keep him from lying flat and getting formula in his lungs while he is tube-fed-- is not as easy as one would think, as the waterproof mattress is slippery and the sheet then moves quite a bit.) We are hoping then to not have to chase him around the house every hour to drink his formula, and to hopefully leave him more physiologically hungry during the day so he will want to work more on eating food. We are still only giving about two ounces of baby food daily, but aspire to increase that over the next few weeks.

Along with pump feeds overnight we are changing from Peptamen Jr to Vital Jr. They are similar products but the flavor of Vital Jr is better than Peptamen Jr and there is a prebiotic in Vital Jr as well.

We have increased his IV iron to see if he can pull out of his anemia. We went from 15mg iron dextran to 25mg. He had pulled out of the anemia before once we put him on IV iron, but he has gained weight and possibly just needs a bigger dose. I am greatly reluctant to put him on enteral iron b/c of the GI side effects that can come from enteral iron.

Back to Boston later this month. That will be just to check in with the Intestinal Rehab Clinic. We are thinking we will get another reduction in TPN as Peter is gaining weight well. He sure seems to feel good!

We have also started vitamin D drops. Endocrinology wants his vitamin D levels up at 30 or above given his istory of osteopenia. Despite going outdoors daily for one to two hours all Spring and Summer, his vitamin D level is only 22.