Tuesday, December 02, 2008

My Hero

I’ve waited to blog about this until after it happened – and I’ve been delayed by all this horrible hacking – but it’s been on my mind for weeks now.

My brother volunteered to undergo a lung lavage at the National Institutes of Health to help the researchers there working on Hermansky-Pudlak Syndrome. I’m so proud of him! I didn’t want to blog about it beforehand, or even mention it on the listserv, because I didn’t want Ryan to feel any additional pressure to do it should he have second thoughts.

The lung lavages have been very useful to the scientists working to find a cure for the pulmonary fibrosis of Hermansky-Pudlak Syndrome. Because of the bleeding disorder associated with HPS, they cannot take samples from our lungs for fear of complications – so the lung lavages offer the closest look possible at the disease process in the lungs as it unfolds.

Lung lavages aren’t horrible, but they aren’t exactly fun either. The doctors put a tube with a camera on the end down your throat, pass it through the vocal cords and into the upper regions of the lung. They then shoot a saline solution into the lungs to make you cough. And, best of all, you have to be awake for the whole thing. You have to be able to cough on demand. Cells from the lungs are then sluffed off with all that coughing, sucked up, and then collected.

The good news is they give you wonderful drugs so you have no memory or awareness of this horrible process – it’s as if you were asleep the whole time.

Several HPS’ers have undergone a lavage, myself included – although I can no longer offer myself up for this because I’m in the drug trial.

Perhaps the only thing worse than having HPS is knowing that one of the people you love most in the world – your sibling – has HPS. I was the one that had to tell Ryan he had HPS because we obviously shared this albinism gene. I’ll never forget that horrible conversation. I was on my mobile phone sitting on the floor late at night in a hospital hallway. Ryan wasn’t too happy with me. I was shattering his world and things were all going in the right direction for Ryan. I felt so guilty, but he had to know.

Having a sibling that has HPS is an extra set of emotions to cope with. My biggest fear is that someday one of us will have to watch the other die from a disease that we both share. It’s one of the reasons I’m so fanatical about my work with the HPS Network. I have to know I’m doing everything I can to keep that from happening. And yet every time there’s a loss in HPSland this fear, this thought, runs through my mind.

I’ve watched many siblings lose a loved one to this disease. In one instant I can feel overwhelmingly sad, and in another overwhelmingly angry. Why don’t more people hear us? Why does this scenario continue to unfold, seemingly unseen by the greater world?

Ryan has been lucky. So far he’s avoided the bowel and pulmonary issues I have. Who knows why. It just happens that way sometimes. Ryan has never said anything, but I’m sure he has similar thoughts. He has many friends in the HPS community. He has HPS himself. But I know there was a part of him that agreed to undergo this procedure for me.

Ryan and I have always been like that – even when we were children beating each other up. There wasn’t anything we wouldn’t do for each other.

It might have taken a while for Ryan to get over being mad at me after I told him he had HPS, but since then he’s been one of my chief supporters. In the wake of my recent employment developments, he’s been one of my chief defenders and protectors.

I’m so lucky to have such an incredibly sweet and caring brother like Ryan. He’s my hero.

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