My dear nephew of 6 years old and liver transplantation

[a_undre]

Hello everyone

My lovely nephew who's 6, has a rare genetic disorder. He unfortunately makes too much LDL cholesterol, which can lead to heart and cerebral issues. At the current rate he could have a significant life event before he's 10.

My sister is distraught and having difficulty deciding on the correct pathway. He currently has a weekly session of apherisis, which is like dialysis however it removes the LDL in the blood. The first results of which look promising bringing his levels down to near normal.

Now the prospects for his future are : either continue the apheresis until a new drug treatment becomes available. That could be a decade, few decades, never... it is a risk. The other option being liver transplant (the liver produces around 80% LDLs), hence transplantation would effectively cure it.

My sister is at a crossroad and the decision seems very hard. What are the long term survival rates? what are the complications long term? what meds are needed and for how many years?

I would like any long term transplant patient to give their experiences and how the whole situation unfolds.

My kindest regards

[Ktleesond]

Hi my son has a rare liver disease, biliary atresia. He hasn't yet had a transplant but it's likely that he will need one at some point in the future. I know that his doctors will exhaust every other option up until that point and that a liver transplant is the final course of action. Liver transplants are quite successful nowadays but it comes with its own pitfalls- risk of rejection, immunosuppressants, affect of medication on kidneys etc. Having said that, I'm in contact with mums whose children have had transplants and their children are doing amazingly well and have been given a second chance at life. One young woman just celebrated her 35th birthday, 34 years after having a transplant!! For my son, a transplant may be his only chance at life but I can understand what a big decision it would be if it's elective. I'm sorry I couldn't be of more help. There is lots of practical information about transplants on the cldf website x

[a_undre in reply to Ktleesond]

Thanks for the reply. I appreciate all the advice and am grateful for your help. The issue at the moment is weighing up if the aphaeresis with medications will be sufficient enough, and if liver transplantation is much more harder as he child grows older?