The next phase – headed for lung transplant

I’ve put off writing this blog for days. I’ve had so much to do, but that is only part of it. I’ve been on an emotional roller coaster that would be enough to turn anyone’s stomach. I just couldn’t do it.

But, like so many things in my life, I need to do it, whether I want to or not.

I need to do it for me because writing is therapeutic for me. I need to do it because I’ve been so blessed by the outpouring of support from friends around the world. It simply isn’t possible for me to return all of the messages, texts, phone calls etc. I want you all to know what is happening in hopes you’ll continue to offer that support and prayer, whether I can talk to you just now or not. Lastly, I need to do it because I am committed to putting a face on Hermansky-Pudlak Syndrome (HPS). I don’t know how my words on this blog might be used one day, but I hope that they are somehow. I hope people beyond the HPS community begin to understand us, our journey and our need for their help to find a cure.

I knew I wasn’t going to get good news at NIH, but I honestly didn’t think it would be such bad news. The cough that has troubled me for two years now was worse. It got so bad that, if my bladder wasn’t empty, I’d wet my pants from coughing. If I coughed after eating, I’d throw up the food. Bruises started appearing on my stomach, I think from the force of the coughing. I thought it seemed more like asthma than pulmonary fibrosis. Some days I’d find it hard to walk up and down the stairs to do laundry, while the next day it would seem effortless. The cough would get worse, then better, then worse. It just seemed like things varied too much to be the steady progression of pulmonary fibrosis.

Well, I was half right.

My asthma was indeed out of control. I’d been to the doc about it in Feb. but no changes were made in my medication. I think it was chalked up to progressing pulmonary fibrosis, which frustrated me. In March I saw Dr. Seward. He can’t run tests like my home doctors can since I come from so far away, but he had given me some Codine that helped the cough a lot. I was afraid to take too much of it though, so tried to only take it at night so I could get some sleep.

The violent coughs continued and I grew anxious about my NIH trip. I didn’t see how this violent coughing could not be doing some kind of damage to my lungs. I had a feeling the CT would show advancement.

By the time I arrived at NIH I was a basket case. I was so nervous about what they would find, and what implications it might have for my life that finally seemed settled and together and really happy. I was such a nervous wreck I totally botched the first round of pulmonary function tests.

By Tuesday Kevin was adjusting my asthma medications and it seemed like things were getting better. I think we all thought that if there were advancement, it would be slight. The problem was the asthma. But, when the CT scan came back, it showed a lot of progression for a year’s time. Everyone seemed surprised by the rate of progression, which scares the pants off me. I think most of it probably occurred in the last six months when the cough got really bad, but of course I’ll never know that for sure.

We repeated the pulmonary function tests and while they were better than the ones from the first day, they were not great. While my FVC remains relatively okay (slightly below normal) my DLCO is low at 50. Mind you, it has been 50 for some time now, so I’m puzzled as to why I need oxygen now. Yet, that’s the case.

Oxygen was the second shock. When I’m sitting my oxygen saturation is okay, but as soon as I start to move, it falls. I’ve been walking the dog two to three miles a day. Yes, I felt winded, but thought it was because I was so out of shape. I did suspect something as all that walking never got easier over time. Usually, when you start exercising more, your endurance grows. For me, it never did.

The whole thing seems like a bad dream. This may sound silly, but all these years that I’ve expected this to happen, and all of these years that it didn’t, finally had me somewhat relaxed about making long term plans. For years I was afraid to think of anything beyond six months or a year seriously for fear things would change, my life would change, and I’d have to mourn the loss of expectations and dreams. It was easier not to get too attached to them. It was as if I thought that if I kept an eye on the freight train coming down the track to hit me, it would somehow move slower. I had finally started to let go of that kind of thinking. I made some plans and commitments recently that were long term for the first time in a long time, and I was happy.

Now, my life is turning upside down. I’m trying to move near a transplant center as fast as I can. My insurance won’t cover this, so I have to figure out new insurance, a new social services structure, find a new place to live, and get rid of almost every possession I have just to hold onto the hope that a transplant is possible for me. It might not be, and even if it is, I might not make it to get one.

I have cried, sobbed, been numb etc. but mostly I feel overwhelmed.