Monday, September 09, 2013


We all have certain expectations in life, and with many expectations comes trepidation. For those of us living with HPS types 1, 2  and 4, pulmonary fibrosis is an expectation in our lives. We know it is coming, most likely before we’ve reached a ripe old age. Pulmonary Fibrosis is an ultimately fatal disease. The age of onset varies widely, even among siblings.

We live never knowing when this will present its self, or if anything we are doing might be a contributing factor.
It’s what makes HPS pulmonary fibrosis different from other types. It’s not just a genetic pre-disposition. It’s a fact – sooner or later.

Recently the HPS Network conducted a survey of people with HPS as part of a grant application. Researchers wanted patient feedback. Besides the obvious, the cure, what do those with HPS want researchers to focus on most? What most greatly impacts their quality of life?

Perhaps unique to HPSers with pulmonary fibrosis, it is the certainty of one day developing a fatal lung disease for which there is no treatment or cure. It’s a lot of anxiety to live with! But, bringing to the surface this reality for us was very helpful! It might seem obvious, but in the world of pulmonary fibrosis, it’s a little different. We want to know why it is that some people develop the lung disease at 20, while others at 40 and still others at 60? What happens? What contributes? What can we do to, at the very least, slow it down?

Such answers would be valuable for our physical health, but also our mental health.

I still believe early diagnosis and knowing your gene type are one of the best defenses we have. I believe it because, in my role with the Network, I’ve seen what happens when people don’t have this information. But, it is information that comes with a price. Knowing may make us more careful and our doctors more vigilant, but it also means living with this fuzzy crystal ball about the future. 

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