Would recommend strongly AGAINST considering liver transplant unless there
are some unusual circumstances; althought the tranplant would be
potentially curative, at least 20-30 per cent will die because of the
transplant, and about 20-30 per cent have problems, such as graft versus
host, which can require lots of problems and hospital stays, and only 1/2
do extremely well, and fade away with a normal life. Liver transplantation
requires lots of attention, pain, and hospital care early on.

Would strongly urge that attention be paid to the chronic acidosis, as well
as to carnitine, and special diet, since many children have progressive
renal disease and loss of nephrons starting in the teenage years, and I
think that attention to acidosis may help forestall this complication and
the need for a renal transplant.

pbenke
Paul J. Benke, M.D., Ph.D.
Box 016820, Mailman Ctr.
University of Miami Sch. Medicine
Miami, FL 33101