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Hermansky-Pudlak Syndrome, pulmonary fibrosis and Pirfenidone – 16-month follow up

I can’t believe that in just a few days I’ll be back at NIH for my 16-month follow-up visit in the clinical trial for pirfenidone to treat the pulmonary fibrosis of HPS. I’m happy to report I think this will be a very boring trip. When it comes to HPS and pulmonary fibrosis, I’m quite happy to be a very boring patient.

Usually I start to get somewhat anxious a few weeks before time to go. The closer the trip gets, the more fixated I become on it. I start analyzing my breathing and probably become a huge hypochondriac. It’s a coping mechanism to steel myself against any bad news.

This time, however, I’m more nervous that I might be jinxing the process. I feel great. My breathing has been terrific. Even my digestive issues have been much improved since about mid October. I’m really showing up with a few minor complaints – the skin infection that won’t go away, the sore by my stoma that hasn’t completely healed in a year, and the need to have a brief chat with Dr. Merideth about our future plans.

This is going to be a very quick trip – just three days.

I feel so blessed to be a part of this trial. Of course, I don’t know if I’m getting the real pills or the placebo, but I do know that my lung function has improved since being on the medication.
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