Living on the frontlines

Living with a rare and chronic illness is never easy, but try doing it in the face of an epic natural disaster – no water, no power, no fuel – forget easy access to medical care, medical equipment or medications. My family connection to Puerto Rico is distant. My great grandfather was from the northwest part of the island. He came to the mainland United States to go to college, and eventually became a professor at the University of Oklahoma. He was a linguist. While I inherited an HPS gene from him, sadly I didn’t get the language gene. Living in Europe, I learned a lot about the history and culture of the other aspects of my heritage, but I never knew much about Puerto Rico. My grandmother would tell us stories about visiting the family plantation when she was a child, but that was about all I knew of the place. When I was diagnosed with HPS, one of the blessings (instead of curses) that came with the diagnosis was a sudden intimate connection to the island. Suddenly I knew tons of ...

It has been a rough few weeks here, but could be much worse. I’ve had a cold for a week and a half. Thankfully, (knocking on wood) it hasn’t impacted the amount of oxygen I need, and so far, hasn’t become a respiratory infection. Yet, just having a cold these days makes me so anxious! I don’t have any reserve at this point. One bad infection, and I’m going to be in the hospital until I get lungs, or I don’t. I just don’t have too far to go before I won’t be able to get enough oxygen at home. So, you might say, I’m paranoid. I’m paranoid about germs and when I get sick, it’s so much more than just having a little cold. Thankfully, only one day of a fever etc. The trouble is I get better and I think I’m recovered, and then it seems to come back. The medicine the transplant center gave me to help with the cough is helping. I still cough a lot though. I’m so tired all the time because I rarely sleep well. But, with the medicine, my coughing fits aren’t quite as long. I haven’t coughed so h...

We added another new HPSer to the HPS patient registry. This puts us at 87 to go on the Hundred People Search - H.P.S. Keep up the outreach! 

We have added a new member to the HPS registry. This puts us at 88 to go on this year's Hundred People Search - H.P.S. 

This past Monday I was on the HPS Conference Planning Committee video conference and my phone rang. It was Inova Fairfax Hospital. Every time I see that number my heart falls into my feet. It isn’t that I don’t want a transplant. It isn’t that I wouldn’t be thrilled to get the call. But, when that call comes, I know we will be embarking on a whole new medical saga and the road can be rocky. Surgery isn’t the only scary thing. Getting a transplant isn’t a cure. It’s trading one lung disease for another in hopes that, at least for a while, you get to be healthy enough to have a somewhat normal life. But, we are not replacing auto parts here. It isn’t a sure thing. It might not work. So, getting that call is exciting, but super scary too. When you first get listed, you may know it is unlikely to get a call, but still, you are on constant alert for the ring of the phone. Nine months in, life goes on. You do your day to day things and try not to think about it too much. At any moment this c...

I had my most recent transplant clinic appointment Aug. 3rd. As always, I’m never really sure whether to say it was a good appointment or a bad appointment. It had aspects of both, depending on how you look at it. I try to look at the up side these days. Grin! My numbers were down again, but this time, they were down enough to give me more points on my lung allocation score. The higher the score, the more likely you are to get a call. So, not sure if it is bad that my numbers progressed again, or good that at least this time, I got some points for the advancement. Last time everything was down, but not down enough. I brought them the CT scan from NIH. No need to get an extra dose of radiation, even if only a small one, for no reason. Let’s save that for when it really matters! And, the CT was done two weeks before the transplant appointment, so it was very current. Again, advancement, but no surprises there. I have developed what is called Bronchiectasis. Essentially, as my lungs ge...

Last month I made another visit to the National Institutes of Health (NIH) as part of the natural history study on Hermansky-Pudlak Syndrome or HPS. It was the first time that, to be honest, I wasn’t really excited about going. Don’t get me wrong. I was happy to go, but for me, it was different this time. I’m at a point with my HPS where there is nothing left to do. I am taking all of the medications I am able to take to help. I am on the transplant list, doing my rehab, and continuing to try to eat healthy and lose more weight. The only thing I was going to learn from the trip is that my lung disease is worse. I know that. In fact, I was pretty sure I wouldn’t learn it was stable because I get seen by a pulmonogist about every month and a half. My numbers are steadly tracking downward, and my oxygen needs continue to creep upwards. So, progression wasn’t exactly a news flash. But then going to NIH isn’t about yourself. They tell you that participating in research has no benefit for ...