Patient story


by Jan Windz

When we met Josiah he was living in a group home for medically fragile children. At that time he was 4 years old, very tiny, non-verbal and had just started to walk. He was wholly g-tube fed and on oxygen 24/7. He would become cyanotic when you took off his oxygen to pull his shirt over his head. He was considered to be terminally ill and non-adoptable. His prognosis was that he would be dead within a year without a heart lung transplant. However, he was not considered a candidate for a transplant because he was in the foster care system. He carried diagnoses of primary pulmonary hypertension, complex congenital heart disease, severe infantile scoliosis, and Noonan's syndrome. He was also, at that time, thought to be autistic.

We had been approached about adopting Josiah because we already had some experience with transplantation. On August 31, 2001 I was blest to be our adopted son, Felix's kidney donor!

As soon as we brought Josiah home I got him in to the pediatric cardiologist at UC Davis Medical Center in order to have them prepare the packet for transplant evaluation at Stanford. About a month after we sent the package to Stanford I received a call from Dr. Jeff Feinstein, at the Wall Center for PH, Lucille Packard Children's Hospital. He said "before we jump to transplant, why don't we actually try treating the PH!"

Since Josiah has begun being treated by Dr. Feinstein he has become a new child. He has now been on Tracleer (Bosentan) for almost three years. For us this has been an absolute miracle drug. He has never had any side effects. The child who became cyanotic in moments of being off of his oxygen now needs to be reminded to wear it. He can go outside without it and ride his bike for 30 minutes at a time! He still gets most of his nutrition from his g-tube feedings, but he is taking food orally as well. He hasn't stopped talking in the past two years (except when he is asleep)! He is learning, loves school and has only had one, very brief hospitalization since he has been with us. A year ago Sildenifil was added to his regimen. While the affect hasn't been as dramatic as with the Tracleer, it does seem to be helping as well. We are currently anticipating surgery in December for his scoliosis. Dr. Feinstein will work with the surgeon, also at Stanford, to be sure that Josiah will tolerate the surgery.

We don't know what the future will bring. Josiah still has severe pulmonary hypertension. However, he is currently doing incredibly well. And every day brings new advances in medicine.

We are truly blessed.


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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.