We met with the lung transplant surgeon bright and early at 7:30 am. When our CF doctor first presented Gessner's case to the transplant committee, the initial reaction was to transplant Gessner's lungs first and then, after he recovers, the liver. But apparently the lung transplant surgeon now thinks that it would be better to do both the lungs and liver at the same time. Apparently Gessner has pretty good liver functions and that if his liver disease stayed as is, he would never get a liver transplant. But, the lung transplant will put a lot of stress on his liver and the surgeon is concerned that his liver wouldn't survive the procedure and he would go into liver failure before they were able to get him a new one. This opinion is based on the level of cirrhosis in Gessner's liver and the fact that he has varicies. His opinion is that Gess's liver doesn't have enough "functional reserve" to sustain the lung transplant.
Transplanting the liver at the same times as the lungs is an even riskier procedure and does not have as good of outcomes as just a lung transplant. The procedures are difficult to do together because the systems are so different and the treatment immediately post-op is different.
The other issue that we addressed was pain medication. Gessner has had a lot of trouble with pain (in his lungs). He can't take ibuprofen (because of his bleeding issues) and can only take a bit of tylenol (because of his liver disease). He is also allergic to nearly all narcotic pain medications. Because of this, he takes IV demoral. He has needed it a lot more lately. He has worked with the pain specialist to try to find an alternative to control the pain, but nothing has worked so far. The issue is tolerance. With the long term use, Gess has built up some tolerance to the drug. The concern is that post-transplant they would not be able to control the pain and then Gess wouldn't be able to recover well. So, he needs to try to eliminate the IV demoral or at least dramatically reduce the amount he takes in order to "reset" his tolerance point. I'm not sure how this is going to affect the timing of listing or the rest of the evaluation at this point.
From the surgeon's standpoint, the things that Gess can do are 1) exercise, 2) gain weight, and 3) work with the other docs on the demoral issue.
We didn't really talk about the lung transplant surgery itself, so perhaps we will have to have another appointment with him once everything else is settled. I'm not sure about that.
After meeting with the lung transplant surgeon, I went to work and Gess went to CF clinic. He talked about the pain management issue and the CF doctor is on the same page as the transplant surgeon. They talked about the difference between palliative care and gearing up for transplant. Right now Gess's lungs seem to be okay infection-wise, so not antibiotics. Next clinic appointment is in a month.
This morning we met with two of the liver transplant surgeons. This appointment was supposed to be later in the week, but after Gess's last hospitalization the transplant coordinators moved the appointment up. The liver transplant doctors are not sure about the liver transplant and whether Gess needs one. They said that his liver functions are actually pretty good. What they would like to do is look into some other options to see if there is an alternative to transplant. The regular liver doctors have considered these things as well, so I am not convinced that anything will come of this, but it is worth the doctors at least having a discussion. The idea is that there may be a way to fix or at least reduce his bleeding issues, which could reduce the hemoptysis (bleeding from the lungs) and thereby delay the need for transplant.
The options that the liver transplant surgeons talked about are removing or reducing the size of his spleen, trying some different medications, and a TIPS procedure (transjugular intrahepatic portosystemic shunt). Again, we aren't sure if any of these will be viable options for Gessner, but it is something that the transplant teams will discuss.
Second appointment of the day was with the diabetes clinic. Gess's sugars have been running a bit high, so the doc thinks that getting them under better control will help Gess gain weight. So, we came up with a new plan for his insulin.
Third appointment was dentist to make sure that his mouth is infection-free. Good to go on that one.
After all of that we both got massages and then went to a movie, which was a decent end to a hectic day.
On Friday we see the lung transplant pulmonologist, Gess does his 6-minute walk test, and we meet with the pain specialist. Those are all of the transplant appointments that we have scheduled right now, so hopefully we will find out the next steps soon.