I was recently asked by a relatively newly diagnosed person with HPS why they should bother to quit smoking – after all, they theorized, they’re going to develop lung disease anyway. What difference does it make if it comes from smoking, or some rare genetic malady? I wanted to blog about the subject, not to beat smokers over the head about their smoking, but to address both why it’s an especially bad idea to smoke if you have HPS and why this sort of fatalism that some of us HPS’ers have is misplaced. (I may not smoke, but I haven’t been immune to similar lines of thought from time to time when I’ve been discouraged.)
First the smoking. If you’re old enough to understand speech, then you already know that smoking is bad for you. But for someone with Hermansky-Pudlak Syndrome, smoking is doubly dangerous. In fact, some of our doctors even advise us to not even go to restaurants or bars where other people are smoking. There’s a lot our doctors don’t know about why or how fibrosis begins to develop in our lungs – but as a general rule, fibrosis or scarring develops in response to something. In some people it’s exposure to dangerous toxins or materials such as asbestos. In some people, it’s in response to some injury to the lung. And in some people it’s the body trying to heal it’s self in response to some disease insult. People with HPS types 1 and 4 (that we know of) are already genetically pre-disposed to develop fibrosis. Adding yet another insult to the genetic one that’s already present could potentially bring on pulmonary fibrosis earlier than it might have otherwise developed.
Another reason smoking is an especially bad idea for people with HPS is that right now there are no FDA approved treatments for the pulmonary fibrosis of HPS. That means that should you develop lung problems in the future, you’re best shot at a treatment is a clinical trial. Being a smoker will prevent you from being eligible for many clinical trials. The current trial for Pirfenidone, for example, won’t take anyone unless they’ve been smoke free for three or six months (I can’t remember which.) That’s precious time that you can’t get back if you have to first kick the habit before being able to seek treatment.
So, what if you’re already a smoker? Well, the truth is right now you can’t repair existing damage to your lungs. But, that’s not a reason to keep smoking. I know of one person with HPS type 1 who was a smoker in her early 20s. She’s now in her 40s and has excellent lung function and no sign of fibrosis. I doubt very much that she’d be in such great condition if she hadn’t given up smoking. The body is an amazing thing. If you take care of it, it generally responds well.
The second thing I wanted to address is the general idea that it doesn’t matter what we do to our bodies – we’re doomed anyway. NOT TRUE! There’s no denying that HPS brings with it serious complications, but the age of onset for those serious complications varies widely! There is a lot of progress being made in the research. Who knows what might be learned in a year, or two years, or five years? I know of several people who were told they shouldn’t expect to be around very long, and now, years later, they’re walking around doing well.
I empathize with HPS’ers that have to kick the habit. It can’t be easy, even if you know what the stakes can be. I know I need to lose weight, but there are times when the call to chocolate is over powering, so I can’t even imagine how tough it would be to kick nicotine. But, in this war against HPS we fight many personal battles – and this is a battle that has to be won in order to win the overall war.
First the smoking. If you’re old enough to understand speech, then you already know that smoking is bad for you. But for someone with Hermansky-Pudlak Syndrome, smoking is doubly dangerous. In fact, some of our doctors even advise us to not even go to restaurants or bars where other people are smoking. There’s a lot our doctors don’t know about why or how fibrosis begins to develop in our lungs – but as a general rule, fibrosis or scarring develops in response to something. In some people it’s exposure to dangerous toxins or materials such as asbestos. In some people, it’s in response to some injury to the lung. And in some people it’s the body trying to heal it’s self in response to some disease insult. People with HPS types 1 and 4 (that we know of) are already genetically pre-disposed to develop fibrosis. Adding yet another insult to the genetic one that’s already present could potentially bring on pulmonary fibrosis earlier than it might have otherwise developed.
Another reason smoking is an especially bad idea for people with HPS is that right now there are no FDA approved treatments for the pulmonary fibrosis of HPS. That means that should you develop lung problems in the future, you’re best shot at a treatment is a clinical trial. Being a smoker will prevent you from being eligible for many clinical trials. The current trial for Pirfenidone, for example, won’t take anyone unless they’ve been smoke free for three or six months (I can’t remember which.) That’s precious time that you can’t get back if you have to first kick the habit before being able to seek treatment.
So, what if you’re already a smoker? Well, the truth is right now you can’t repair existing damage to your lungs. But, that’s not a reason to keep smoking. I know of one person with HPS type 1 who was a smoker in her early 20s. She’s now in her 40s and has excellent lung function and no sign of fibrosis. I doubt very much that she’d be in such great condition if she hadn’t given up smoking. The body is an amazing thing. If you take care of it, it generally responds well.
The second thing I wanted to address is the general idea that it doesn’t matter what we do to our bodies – we’re doomed anyway. NOT TRUE! There’s no denying that HPS brings with it serious complications, but the age of onset for those serious complications varies widely! There is a lot of progress being made in the research. Who knows what might be learned in a year, or two years, or five years? I know of several people who were told they shouldn’t expect to be around very long, and now, years later, they’re walking around doing well.
I empathize with HPS’ers that have to kick the habit. It can’t be easy, even if you know what the stakes can be. I know I need to lose weight, but there are times when the call to chocolate is over powering, so I can’t even imagine how tough it would be to kick nicotine. But, in this war against HPS we fight many personal battles – and this is a battle that has to be won in order to win the overall war.
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