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.
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.
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