September 2017 Update

Well, I would say it is time for a Johnstone update! We last left off in mid August and Sarah and I were headed back to work as the school year was getting underway. In August we were traveling to Boston every other week for clinic visits for Porter and visiting UVM for his blood work on the off weeks. At Porter's last visit to Boston in August, they lengthened our clinic visits to 4-6 weeks! We had one clinic visit this month, and we will return on November 6. Porter also does not need blood labs every week! All of these updates should tell you how well Porter is doing.

Noodles are the best!

Every day is a bit different, but Porter is now eating a significant portion of food by mouth. We still supplement his daily intake with some liquid formula and pureed foods. But, we are starting to back off a bit on the purees as he eats more and more on his own.

The physicians also reduced Porter's medications a bit. He was taking nine medications since discharge from the surgery in June. He now only takes six medications including one that stimulates his appetite a bit. We think it is working and will continue until he shows us he can detect and satisfy his own hunger on a consistent basis.

Porter's morning regimen post transplant.

Porter's liver function tests are normal and he is maintaining a good body weight. Our only issue lately has been keeping his kidneys happy. Even with a reduction in medications, his blood tests show that his kidneys are still working hard to process all the meds. The secret to reducing stress on his kidneys is hydration. While Porter is eating a lot more, he is more reluctant to drink. So, we are trying our hardest to provide more than a liter of fluid via his G-tube every day. That is a bit tricky! A liter is a lot of fluid for a little person!

During our visit to Boston last week they said that they wouldn't need any blood lab work for a month. We realized that two weeks is the longest Porter has ever gone without blood work, let alone a month! Another sign that Porter's transplant has made his life more stable. We are also grateful that with the exception of his little infection back in July, we haven't been to the emergency department in months! With at least one ED admission per month essentially since birth, it's more than a bit surreal.

Also at our last clinic visit, Porter's blood test for Epstein Barr Virus (EBV) was elevated. We knew EBV was in his system; his liver donor tested positive for EBV, as do many adults. Porter's EBV has risen since our August clinic visit, so we were told to lower his immunosuppression medication (Prograf) in hopes of giving his immune system a small boost so it can effectively lower the EBV level.

We had a nice conclusion to our summer and the leaves are starting to change around here, telling us Fall is well underway. Our next challenge will be cold and flu season this winter. Stocking up on hand sanitizer!