I cannot stress strongly enouhg how vital having an actual Heptalogist on your team with liver complications is. Micah's liver didn't show significant issues until the last two years. Even now, if you go by his liver enzymes and strict lab values, his liver should be only marginally impacted. However, he has been low on platelets for several years, and is no longer adequately producing white blood cells. More significantly, regardless of what his liver enzymes show (and by those they predict he should have another 3-4 years), his liver does not function properly. Everytime he gets even a minor illness now, his ammonia levels rise and he shows rapid and significant signs of Hepatic Encephelitis.
Micah is a poor candidate for a liver transplant, and due to his Autism we will not consider a lung transplant for him at all. The pattern for liver disease seems to be that the liver holds fine, until some point the growth of the child surpasses what the liver can sustain. His Heptalogist has said several times that Micah's liver is not failing per se, but that it cannot sustain life for him anymore because we have brought his growth beyond a point that his liver *can* keep up. It is now that we are seeing rapid functional deterioration of his liver, and as of this week STILL not seen signifcantly in his lab values.
I have never found a GI that truly understood and knew how to manage his liver disease. His Heptalogist has been marvelous at knowing what is going on and never panickingg unnecessarily. He has offered in this final stage that rather than having to go to a new Heptalogist when we move, he will continue to consult with our new CF center's GIs so we don't have to travel 3+ hours for Heptalogy. If we were looking towards transplant, then it would be absolutely necessary that we see a new Heptalogist. However, until two years ago, our visits with Heptalogy were bi-annual check ups and labs run and consults for the abnormal platelet counts, not much more.
If you are facing liver disease CF complications in your child, please see a Heptalogist. It won't require much monitoring for many years, but it's one team member worth their weight in gold for the care of your child. He's the ONLY specialist who has been able to manage Micah's weight issues, fat soluabl vitamin struggles, and his idiopathic leg pain. While most of those fall under GI, the complications Micah has been experiencing since he was six are directly liver impacted and not merely CF GI issues. That was the subtle different that the GIs were not able to connect and thus treatment has not followed the path they expected for just CF.
