Living on the frontlines

I am so blessed to have such awesome friends. There is no way I’d have the resources to get through this medical drama without all the angels in my life coming alongside and helping me – sometimes even with things I don’t even realize I need help with. I had never thought about getting a wheel chair. My legs are one thing that is working quite fine. During the past three years that my lung disease has been pretty bad, I have used wheel chairs or scooters for short-term things, like attending a conference that involves a lot of walking. But why I didn’t even think of how it could improve my current quality of life, I honestly don’t know. My focus has been on walking, walking, walking – as much as I can stand and still function, and as much as the oxygen company will allow me (a blog for another day.) Lucky for me, my dear friend Donna thought of it for me. She had a family member pass away and leave behind an electric wheel chair. Other friends joined in and transported the wheel chair

The day before the American Thoracic Society meeting, I had a transplant clinic appointment. I'm not sure how to characterize it - good, bad or neutral. Basically, my lung function fell, my six-minute walk covered less distance and the doctor reminded me (as if I needed to be reminded somehow) that although the differences are small, they've all had a downward trend for the past two years.  Sometimes I keep myself calm about things by looking for the small upsides in larger bad news. For example, the upside of getting sicker is that my lung allocation score (where I am on the transplant list) goes up. This time, however, the numbers, while not positive, did not impact my lung allocation score. So, no upside to being reminded I'm getting worse.  It was a depressing appointment.  I'm so grateful that the day after ATS started, and I got to spend time with all of my HPS friends as well as keep busy.  So, still waiting, but no change in my position on the list. I gu

While working on going through everything I collected, wrote down etc. from the recent American Thoracic Society meeting, I found this abstract about potential biomarkers in Hermansky-Pudlak Syndrome Pulmonary Fibrosis. This is not a done deal yet, sort of speak, but to me, it's an exciting development.  For HPS, finding meaningful biomarkers that directly tie to lung fibrosis progression, and not just HPS in general, could help our efforts for better treatments, and someday, a cure.  Because people with HPS can know they will develop lung fibrosis way, way, before it ever happens, we offer researchers a unique opportunity to uncover early warning signs for pulmonary fibrosis. I can't think of another pulmonary fibrosis population that 100 percent knows they will get pulmonary fibrosis, sometimes decades before it happens. There may be one out there, but I'm not aware of it.  For people with HPS, or someday others at higher risk for PF due to genetics or exposures, th