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To help, or to hurt

When I was at NIH being screened for the drug trial, I had the privilege of spending nearly two hours talking to one of the researchers investigating the cause of the pulmonary fibrosis of Hermansky-Pudlak Syndrome. The science was fascinating as he tried to sell me on the idea of why they need more young people with Hermansky-Pudlak Syndrome to volunteer for lung lavages. (As if he had to sell me on it. Since we had never met before, I don’t think he realized he was preaching to the choir.)

One other aspect of the conversation sticks in my mind however. We seemed to be building a good rapport, so he confided some of his concern for the HPS patients. “I sometimes worry,” he began, then he fumbled around for the right words to convey his meaning.

“Denial isn’t always such a bad coping mechanism,” he started again. He observed that some of the HPS patients didn’t seem to fully appreciate the entirety of their disease process. “I worry,” he started again, “I worry that by bringing some of the people with HPS back to the NIH time and time again that we’re making their lives more difficult. We’re putting the reality of their disease process front and center. I worry that we’re robbing people of their coping mechanism, of allowing them to lead more normal lives. It seems like so many of the patients with HPS show signs of depression.”

I was really surprised to hear this coming from one of our researchers. He’s fairly new, and I was impressed that he’d picked up on the subtly of our emotional well being so well. His concern was touching, but I was a bit worried that he perceived the work of NIH in this way.

“Well, get over it,” I bluntly told him. He looked a little surprised.

“We need to be here, and we need you,” I explained. I pointed out that the HPS’ers truly in denial don’t come to the NIH to participate in research. They don’t believe there’s a problem, or they don’t want to know there is a problem, and thus they have no motivation to take time out of their lives for poking and prodding while they still feel healthy.

The people who volunteer to come to NIH, on the other hand, at least typically have a general understanding of HPS and what they may be at risk for. Pretending an elephant isn’t in the living room doesn’t make the elephant disappear. We can choose to respond to our HPS in two ways – we can ignore it and just wait for the inevitable to happen one day, or we can be pro-active about it and work to guarantee that tomorrow is better than today.

“Coming here gives me hope,” I explained, “If we didn’t come here, there would be nothing to do to fight this disease.”

As for depression, yes, I’d agree. There is a high rate of depression and anxiety about the future among the HPS’ers. We are seldom as open about it publicly as I wish we were, usually because we are all so close. When something isn’t going well, sometimes the HPS’ers don’t confide it to the group as a whole for fear of upsetting everyone else. But, they do often confide it to a few close friends. Many a night I’ve spent hours on the phone talking with another HPS friend about our deepest fears for the future. These fears are real, and it isn’t reasonable to expect us to not think about them.

“You can’t expect to have someone come here, tell them they’ve got pulmonary fibrosis, and then expect them not to be teary or depressed,” I explained. “What you can offer is the knowledge that someone cares and is doing something to help us fight it.”

“I don’t want people to come and volunteer for all of these tests because they’re afraid they’re dying,” he said.

“That’s just not realistic,” I argued. “For those of us that have been a part of the HPS community for any length of time, learning to deal with loss is just part of the process.” I told him about some of the friends I’ve lost over the years such as Benny or Victor. “How can you reasonably expect me to watch these things and not be afraid the same fate might be in store for me?”

But, the problem is that while dealing with the darker side of HPS is a reality for us, we cannot dwell there. We each must create coping strategies of our own to regain our normal lives as much as possible given our particular health status. We have to know that our futures are not written yet – that research continues. We have to know that every one of our days has value.

There’s a line in a song by Chris Rice that I love. It says something to the effect of no matter how rich or poor you are, everyone only gets 24 hours in a day. What are you going to do with those hours? That’s up to us.

I explained to this researcher that the docs at NIH could help most by helping people understand how to maximize their lives – what can we do to keep the research moving forward and what can we do to take the best care possible of ourselves? Information is power and the researchers studying HPS at NIH and elsewhere have the ability to give us that power. That doesn’t mean that we won’t have tough days. But, with the help of researchers and our support systems (and for some of us our faith in God) we can have many, many happy ones as well.

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