Josiah's Journey with PH+

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 blessed 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.

Our Journeys: Kevin Doty

There were only ten steps up to my apartment, but it seemed like there were 10,000. I dreaded going home every day from work because I knew I would have to walk the short distance from the parking lot to my apartment, and then scale those ten dreaded steps each day. I was always so out of breath by the time I got into my apartment, and I had to take ten or fifteen minutes just to catch my breath, and slow my heart rate down. When I started getting those jabbing chest pains with the breathlessness, I began to worry, and went to see my primary care doctor. My doctor ran an EKG test on me, and immediately referred me to a pulmonary specialist. In March, 2006, after several agonizing months not knowing what was going on, my doctor performed a carotid artery catheterization, and discovered that the mean pressure in my right lung was 46, and a diagnosis of pulmonary arterial hypertension was proclaimed. I was immediately prescribed with oxygen therapy, as well as Tracleer and Revatio.

Years earlier, in the summer of 1986, I was diagnosed with a rare disorder called Pulmonary Histiocytosis X (the X part of the name meant that at the time, doctors were still pretty puzzled as to why my white blood cells (histiocytes) were attacking the cells in my lungs and leaving scar tissue. The doctors weren't quite sure what to do for me, so I became a bit of a white lab rat for them, and they started trying different medications to see if any helped me. I was given a huge prescription of prednisone every day, and when that didn't work so well, chemo-therapy was added to the regimen. The chemo seemed to help, and my disease went into a kind of "remission", and I was able to perform normal things, and lead a normal life.

Fast forward now to the end of 2005 - I had moved up to the Seattle, WA area, and had lived

there for about a year when those pesky symptoms began to return. This time, they were far worse than they had ever been previously - it took very minimal effort to get me out of breath while it took considerably more effort in the past to get me breathless. I began to worry when I started having sharp chest pains to go along with being out of breath on minimal effort. I had several tests run on me during this time, including all the heart checkups imaginable, and when all those tests showed no signs of disease or trouble, I was referred to a pulmonary specialist. Upon telling Dr. O'Brien my symptoms, he immediately ordered a carotid artery catheterization, and discovered the pressure in my right heart was at 46. I was immediately put on oxygen therapy, as well as Tracleer and Revatio, and although these drugs didn't help me at first, I think they have helped me regain a little bit of my stamina and ability to breathe easier on exertion. For about a year, I was completely home bound, except to go see the doctor, or to go to the hospital for one thing or another.

Currently, I am able to get out and do my physical therapy, and have even started playing golf again. I look forward to hopefully going back to work in the near future, and being able to rely on myself again, rather than leaning on family members so heavily for everything. Without my family however, I would never have made it this far - I am so grateful for all they do and have done for me during this trying time in my life.

I am also grateful to the PH Association, and all they do to support those of us who have this terrible disease. I found their website by chance one day as I was looking for more info on pulmonary hypertension shortly after I was diagnosed. There is so much information on their website, and I have even joined one of the support groups here in my area that enables those patients and patients' families to interact with each other, and discuss new medications/treatments, tricks to surviving with PH, and just being able to talk to someone who knows exactly what I'm going through with the symptoms, and the different obstacles we must face on a daily basis. These support groups also give us access to many doctors who specialize in pulmonary disorders, and pulmonary hypertension specifically. These doctors take time out of their busy schedule to come talk to our groups to let us know about new medications or procedures being done for PH patients, and graciously answer all of the questions asked by nervous patients, and their family members. The support group meetings have been a real blessing for me since I was diagnosed - just knowing I'm not alone in this fight, and that there are really talented doctors and scientists out there that are working extremely hard to help find a cure for this malicious disease, gives me hope. It's nice to have something positive like hope bouncing around in my brain rather than the despair I felt early in my diagnosis.

I look forward to the day when I can walk up a flight of stairs, and not have to catch my breath for twenty minutes before doing anything else. I hope for the big breakthrough in science and medicine that will eliminate this disease, I hope the drugs on the market now, and those coming out in the future will prolong our lives until we see that glorious day … I hope I can celebrate my 50th birthday, and my 60th, and 70th.

I hope ...