Dying Teen Is Being Denied A Heart Transplant Because He’s Had Trouble With The Law

[Ms. Toad]

110. Since late middle school

They are discovering that it is likely multiple conditions with similar presentations. The number of pediatric diagnoses is growing, and they seem to follow a different pattern (more severe rapid progression, more auto-immune disorders, and vancomycin tends to put PSC in remission - as well as sometimes putting accompanying IBD in remission). I know a number of kids who were diagnosed in the 4-10 range, and even one who was diagnosed ~ age 2.

Even though my daughter clearly had PSC as a child (she had the really intense liver itching by 9th grade - although we didn't know that is what it was), she seems to be following the more adult pattern. She was one of the people in the first adult trial of vancomycin. Despite 100% success among kids who were not yet cirrhotic, the adult response has been mixed - and my daughter had a mild response, but not sufficient to make it worth all of the public health risks which go along with taking vancomycin as a maintenance med.

But even though the pediatric population is growing, PSC is still not well known among pediatric doctors. I had to lead her doctor by the nose to the diagnosis when she was 19 because she was not a 40 year old male. (Almost literally - I had to ask her college doctors to run a liver panel, and when it came back high then had to push her GI doc to run a GGT rather than watching and waiting another 6 months.)