Like a lung transplant or any other organ transplant, a liver transplant does not give a normal life expectancy. And, in our case, a liver transplant can only happen if he can also get a small bowel due to his meconium illeus, his small intestines is too short to hook in a new liver without a new small bowel as well.
So, as I said, no I have very little hope that these new drugs will do much for *my* child's life. But also again, the bulk of his challenges are from what CF has done to his liver and not his lungs. Ironic actually. We focus so heavily on the lung issues, and certainly we are totally aggressive with his lungs here. But, for him, its the liver that will likely determine his life expectancy as well as quality of life.
In the end, its all CF though. CF claimed a massive portion of his bowels before he was even born and claimed his liver before he was 3 months old. The rest of his life is about managing the aftermath of this disease. And I don't see any drug in the pipeline that would prevent what happened to him, which started before he was even born and anyone knew he had CF.