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The private thoughts of an HPS’er

One of the things I do at the Hermansky-Pudlak Syndrome Network is a lot of listening. It’s often listening late at night, well after little ears are in bed. It’s one of the reasons I get a late start on my workday. You’d get up at 9:00 am too if you were regularly up past 1:00 am! Grin!






Sometimes themes develop during these very private, heart-to-heart chats. I feel like I can blog about this because it was shared by more than one person – several in fact.






One friend summed it up perfectly.






“You know what makes HPS different than so many other health problems?” she asked me, “It’s that even if you’re healthy, if you have a type of HPS that you know causes pulmonary fibrosis, you know it’s coming. Even if you’re lucky and it doesn’t show up until your 60s or 70s, you know it’s coming.”






I could so well identify with her.






This is an issue I suspect will arise in medicine more and more as we learn so much about human genetics. Someday it will be common place to know what your genes might have in store – and hopefully this will allow you to take advantage of a cure.






Right now, for us, there is no cure – just anticipation.






We react to it differently.






Some people avoid it entirely, not even wanting to be tested when they know their chances of having HPS are high. Some get tested, but once they determine their health is good, they put everything HPS-related aside to live life.






Others are able to compartmentalize life enough that they can both be involved in the HPS community and still maintain a full and productive life in other spheres. Many take advantage of what they know to stay as healthy as possible and help in the work to find a cure in any way they can.






Still others are impacted by their HPS in such a chronic way that there’s no hiding from it. It’s always there, like your shadow. You’re reminded of it every time your stomach is upset, every time a wound bleeds or every time you have a coughing fit.






I don’t judge. Everyone has to do what is best for them. If denial makes it possible to lead life and get up in the morning, then maybe it’s better than swimming in HPSland for that person. For me, involvement is therapeutic. It’s what makes it possible for me to get up in the morning.






But my friend is right. Either way it’s there. I know it. My loved ones know it. My friends know it.










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