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The wait begins

I finally heard from the University of Kansas Medical Center pulmonary lab, and I’m having my pulmonary function tests on July 7th. Pulmonary function tests, as medical tests go, aren’t bad. All you have to do is breathe into a big tube. The hardest part of the test is getting your lips to fit around the mouth piece and keeping the nose clip on. But, these are the tests that cause me the most anxiety.

They are the biggest predictor of how my lungs are doing, and how quickly they may be going downhill. This time I’m not sure if it’s possible to get really good news.

While my FVC was still in the normal range a year ago (very good news), several of the secondary scores weren’t so hot. These are early predictors of pulmonary fibrosis. The rate of the development of fibrosis varies greatly in Hermansky-Pudlak Syndrome. Some people seem to have a very rapid development, while for others the process is very slow and happens over many years. You never know.

I’m very much on the edge for being eligible for our clinical trial at the National Institutes of Health. As a result, I’m not sure what to hope for next Friday.

If my numbers fall, it means I’ve got a shot at getting in the trial, and thus a 2/3 shot at getting a drug that clearly slows the development of the fibrosis. But, if I’m doing slightly better, or even the same, then I’m not eligible for the trial. That would be good news because it would mean my disease hasn’t progressed. At the same time, I’m at a prime age to start to see some advancement. It could be another five years before this drug is on the market. I could go from perfectly fine, to seeking a transplant, very easily in five years. Or, I could be completely fine for five years. It seems like there’s no perfect solution.

I know that God has a plan, and I just need to show up, puff, and accept whatever the result is as good news. But, my brain gets in the way of such perfection.

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