There are a few things that make the experience of those with Hermansky-Pudlak Syndrome (the types that cause lung disease) different than the experiences of many others with pulmonary fibrosis. We get to see it coming.
For the most part the topics of discussion on the HPS adults listserv are pretty benign. They’re just every day topics – some perhaps unique to us in respect to skin care or low vision issues – but generally nothing too serious.
Right before Baby got sick, however, the topic turned to something I know we all think about, but often rarely vocalize; knowing that we will one day, sooner or later, develop pulmonary fibrosis.
Most people with pulmonary fibrosis have idiopathic pulmonary fibrosis (pulmonary fibrosis of no known cause). They don’t learn about PF until it’s upon them. We, however, get to see it coming. We watch our friends, and sometimes our cousins or siblings, develop PF. We watch as they get sicker and love them and support them knowing the entire time this is what is in store for us.
It’s an experience I think more and more people will share as we learn more about genetics, and what our genes might be able to tell us about our futures.
We all cope with this in different ways. Some of us are better than others at compartmentalizing life. Some chose to ignore the issue all together until the day arrives when it can’t be ignored any longer. Some of us have more chronic issues and couldn’t ignore the issue if we wanted to do it. Some people feel they can live a more full life if they have nothing to do with HPS or the HPS community until they have to – and others of us cope by diving in with both feet, legs, hands and whatever else.
I don’t judge. We all do what we have to do, and what we can do, and that’s different for everyone.
I do sometimes wish, however, that outsiders could understand what it’s like to “live with the enemy.” We go on with our marriages, our children, our careers etc. Outsiders might not realize this inner thing we live with every day, no matter how we choose to handle it. We might not know the timing, but short of being hit by a bus, we know what’s in store for us. It’s a glimpse into the genetic crystal ball most never have to experience – at least not yet.