A busy last two weeks...

The trip to Boston went well. Peter is a great traveller in the van! A little over a year ago he was needing to be air ambulanced to Boston. Now he is enjoyably riding in the van for 7 to 9 hours, and this time with the G tube clamped the whole time AND enjoying his ice-cold Elecare along the way. No gagging! Our biggest challenge in the trip was on the way up with the duodenostomy leaking twice. There we are in the parking lot of the gas station with Peter lying on the backseat changing the ostomy bag, mad at me because the sun was in his eyes, but we were unable to change that situation b/c the bag was already off before we realized the sun was the problem versus the cramped quarters and wetness. The second duodenostomy leak was corrected by removing the ostomy bag and applying an ABD pad and wrapping a diaper over that and then tackling the application of a new bag once we got to our hotel room a few hours later.

Our visit with the doctors and nursed in Boston went well. We were outpatient the whole time. Dr. Puder thought Peter looked very good - better than he ever has. We agree. Peter charmed many with his improved walking and impressive progress in vocabulary since his last visit two months ago. Peter has gained some weight and height, whereas at his last checkup he had plateaued. We are now able to stop his calcitriol (vitamin D), as it appears by labs that he is no longer having ongoing bone loss, but rather is now having good skeletal growth. Hooray!! We also are being encouraged to begin baby foods on Peter. I wasn't expecting that news at all. The idea at this point is mainly to help Peter get used to eating, versus gaining real nutrition from the food. He had his first baby food tonight, Gerber lamb, and he did quite well with it... just a few tiny spoonfuls, but seemed to enjoy it and tolerate it. Meanwhile we will also continue to go up in his Elecare feeds. When we get to 2 1/2 oz per feed then we will plan to test his outputs to see how well he is digesting what he consumes. We are at 50 mL per feed now, so we are 2/3 of the way towards that goal. If he can prove he is using what he eats, then we will discuss lowering the amount of TPN he receives each week. Also, we are getting to run the TPN over 12 hours now. It is so nice that he can run around that much longer free of something attached to his PICC line.

The upper GI went well. We didn't have any crazy ostomy bag repairs afterwards, which has happened in the past, but this time we were able to avoid. Maybe that is because I was prepared with every ostomy need I could think of and a side room available if we needed it, kindly arranged ahead of time by one of the nurse practitioners. The results show that things do flow in a linear fashion from the stomach to the ileostomy, without too much leakage from the other ostomy sites along the way. We will need to keep an eye on the duodenostomy that it doesn't begin to pouch or back up as we increase feeds. Peter does not seem to have developed any new fistulas and the stricture was unremarkable, which is also positive news. Dr. Jennings did point out that we still will need to know how Peter is circulating around the portal vein clot before any surgery could be considered, and to know more about the liver and bowel function. The gut still appears shortened even with use, so perhaps there is scarring causing the shorter gut versus just disuse. The bottom line seems to still be to keep feeding him and continue slow advances in the feeds, and we will continue these thoughts in two months when we are up again.

The leaky G tube still leaks... that is another story for later.

Then 36 hours after getting home the PICC line cracked!!! I don't know why, but it did. There was no trauma that I know of, but maybe it broke from the wear and tear of a toddler who occasionally tugs at it and who puts a whole lot of tension on it when he begins to walk away from his IV faster than one of us can catch up with the IV bag. We were able to get another PICC placed at CHOP two days later, and so far there is no sign of infection. Whew!!

I am hoping to post a photo or two from Boston soon. We finally made it to Boston Commons!!