Skip to main content

Health Update

Yep, still hugely behind on blogging! I feel badly as I promised all of the people helping me raise money to try for a lung transplant that I would keep them all updated here.

So, here is the update.

A few weeks ago I had PFTs again (pulmonary function tests.) The two numbers we watch pretty closely with Hermansky-Pudlak Syndrome are the FVC and DLCO.

FVC stands for Forced Vital Capacity. It is the volume of air you can force out after taking the deepest breath you can. It is measured in liters, but also in percent predicted. The percent predicted takes into account your sex, age, weight as compared to the general population to compare you with others like you. My forced vital capacity was 74 percent of normal. While not terrific, it actually went up one point (mind you, that’s within the margin of error, but I do love it when things go up.)

The other number that we pay especially close attention to is the DLCO, or diffusion capacity. It measures how well oxygen moves through the tissues of the lung into the bloodstream. Mine was 45 percent of normal. For years doctors have told me that this is the most difficult part of pulmonary function tests to get a really accurate measure on, so not to worry if it goes up and down a bit. Well, easy for them to say! Grin!

When I was at NIH in May, it was at 49 percent of normal, and then in August, it went up to 52 percent of normal. So, I wasn’t terribly happy about this score. I wasn’t expecting an improvement, mind you – but was hoping for about the same. Whether this is in the margin of error seems to depend who I ask and how they think about it. But, it was down and that’s hard for me to shake.

I was disappointed because I felt like the test had gone really well! I am usually so anxious every time I have PFTs done. For years now I’ve been afraid each time I had to do the test that this would be the one that would catch me off guard. This test would be the one that would start the ball rolling to turn my life upside down. This time, however, my life had already been turned upside down. I’ve already been told that I need a lung transplant and that I have to move. I was still walking two to four miles a day on average (with 2 to 3 liters of oxygen). Thus, I really wasn’t expecting a big change.

What scares me is how quickly we seem to adapt to the new normal. I question whether I’d really know if there was a huge drop because it isn’t the sort of thing that changes from one day to the next. Perhaps living as a legally blind person all these years and constantly having to adapt, create solutions to get things done when one didn’t already exist etc….I just do it without thinking.

Last week my mom was here helping me work on the move, and suddenly I needed more oxygen than I had been. For two days after she left I was back on at least 2 liters 24/7. I also became more aware of little things I’m doing to adjust that I wasn’t even aware I was doing until someone else was here. The past few days, however, I’m back to using about as much oxygen as I was before the PFT and about what has been normal for me since starting oxygen in May. I’m thinking maybe the stress and all the dust etc. from packing was causing a little more stress on my lungs. These days it doesn’t seem to take much.

In the end, the change was not so huge and drastic that it altered the plans for the move one way or the other.

Maybe a crappy DLCO will keep me from getting too comfortable about all the things I need to get accomplished.

So much more to say, but not enough time. I will try to elaborate on this more later.

Comments

Popular posts from this blog

The next generation with Hermansky-Pudlak Syndrome

I'm so behind on posting about the trip to Puerto Rico. Since the episode of Mystery Diagnosis on Hermansky-Pudlak Syndrome ran right after we got home, it's been a little busy. These, however, are my favorite pictures from Puerto Rico. I know, not pretty senery etc - but these little guys and gals inspire me. They are the next generation of folks with HPS, and if we keep up the hard work, they will live better lives because of it. They motivate me.


Just waiting: A continuation of my transplant story…

Photo: This is me the week before the transplant. The one with the roses was taken on Valentine's Day. My sister-in-law gave the roses Ryan had brought her to me.

To be honest, I don’t remember much about the time I spent in the hospital before my transplant. I’m not sure if that’s because it was pretty boring and routine, so there’s just not much to say – or if it is some kind of self-preservation maneuver my brain has done to shield me somehow.

After the big rush to get to the clinic early, we spent several hours in the waiting room with my luggage as if we were waiting for an airplane at the airport. It turns out the big rush was due to a hospital policy that the clinic couldn’t request a bed for me until I was actually there. The hotel Inova Fairfax Medical Center was packed to the gills, and they wanted my name on the waiting list. It was late afternoon before a bed became available. Looking back, I’m amazed at how calm we were sitting there. Perhaps we were compartmentalizing…

Family hunting

I’ve always had an interest in genealogy. I haven’t done anything about it really. Never had the time I guess. Perhaps it’s because I’ve always been a fan of studying history. Finding ancestors somehow feels like a personal connection to the past, as if the DNA populating my cells has some sort of time travel awareness of what has gone before. Of course that’s crazy.

When I was diagnosed with Hermansky-Pudlak Syndrome my interest in my roots, especially my Puerto Rican roots, intensified. Maybe it was the realness of mortality or the connection between my newfound fascination with genetics and how it connected me to my family history.

For years I’ve thought about whomever in my family tree might have had HPS. They would have lived so long ago that they wouldn’t have known what HPS was. It wasn’t really identified as such until 1959. My grandma Cockerill, whose father was from Puerto Rico, talked about relatives in Puerto Rico she heard about as a child that had died of tuberculosis. C…