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 get increasingly fibrotic, the tissue gets harder, can’t expand and contract, and eventually atrophies, or shrinks up. This is making my airways pulled apart in places. It was a new one for me in terms of pulmonary fibrosis, but it sort of makes sense I guess. It is something I’ve heard associated more with COPD, or Cystic Fibrosis. I’ve probably had it developing for some time, but it is now very visible on the CT. Or, at least it was the first time it was notable enough that it showed up in the report.
It also explains why my cough (which I have had for almost a decade now) is now so much worse. They are trying a treatment regimen to help, and so far I think it is helping. Some of you have asked what it is, and I’m happy to share, but with this disclaimer. I’m always a bit hesitant to share details of my treatment regimen when it is not in the context of a direct conversation. The reason is we are all different. Our PF can even be different. I don’t want HPSers reading this, and then going to their pulmonogists and saying, HPSers do this…as it might not be effective or necessary for everyone. That being said, they have prescribed abuderol nebulizer treatments several times a day, and a drug called gabapentin. This drug, like all of our drugs, is off label for us. Some have been using it with some success to treat the cough of IPF, and so, given how debilitating this cough has become, my doctor and I decided to give it a shot. The drug is on label to treat seizures and neurological pain. I’m not sure why it would help with the cough, but I think it may have to do with the spasms that happen when I have a big coughing fit. Since starting the drug, I am coughing a bit less, but still coughing. It is easier to bring up the mucus I am coughing up now. When I do have a big coughing fit, it doesn’t last as long. They had gotten to run about 30 minutes,, which is incredibly disruptive and exhausting, especially when they are several times a day! I have also noticed my O2 sats a bit better. Don’t get me wrong. I still need a LOT of oxygen, but for example, I can get up and walk to my bathroom without my oxygen on and am still okay. My apartment is super small, so that’s maybe 11 to 20 steps. For the last month, however, just that would make my 02 go into the basement, so this is a minor improvement. It hasn’t made any difference in the 02 I need for exercise or walking around, but every little bit helps.
The gabapentin is a challenge for me to adjust to, for whatever reason. It has given me hand tremors, made me nauseous and made me so sleepy I have literally done nose plants on my computer screen trying to work. It makes my affect very bla. Sometimes I am talking and the words are clear in my head, but I can’t get them out my mouth. This is improving though, so I hope as I adjust to it, these continue to lessen. My ab muscles are appreciating a bit of a break from chronic severe coughing. Really, with all the coughing I do, I should have abs of steel!
After I do the abuderol nebulizer, I am doing three sets of ten with a device called an acapella. It makes your airways vibrate so it is easier to get the mucus out. Getting this crap out is important as it is a perfect breeding ground for bacteria, and thus, respiratory infections. As I said in my last post, getting this sort of infection is a big deal problem for me these days. It isn’t just a matter of getting some antibiotics and getting through a cold. It could cause an acceleration of my fibrosis. That might up my lung allocation score, but there’s no garuntee that just because my score rises, that lungs will be available. You just don’t know. I think of all my friends who have passed waiting for lungs, and I know that as positive as I am, as much as I say when I get my lungs (not if) that it may very well not happen.
So, I just keep working to give myself the best odds.
The other big worry to me at the transplant appointment is that I couldn’t complete the six minute walk on eight liters of oxygen. I had done so at NIH just a few weeks before, and done pretty well actually. At Inova, however, they used the pendant cannula I use on a day-to-day basis. This is a conserving device to try to help the tanks last a bit longer. They go so fast when you are using a lot of oxygen and it can be very life limiting. But, I know from rehab, that it isn’t ideal for full on exercise. It just can’t keep up as well. At NIH, they didn’t have a high flow cannula so they used a regular oxygen mask. I asked my respiratory therapists at rehab about it because it really worried me, but they said the different was likely due to the mask vs. pendant cannula and not me. Also, when I do that six minute walk, I walk faster than I do in every day life.
The other reason I couldn’t finish the six minute walk is that they changed their protocol for how they do them. It used to be they would bump the oxygen up if you went too low. Now, if you go under 80 they just make you stop and they conclude the test. If they had bumped it for the last minute of the test, I would have covered as much, if not more, ground than I had at NIH. So much really depends on the speed you are walking, and the type of oxygen delivery device you are using. I was very worried when they wouldn’t let me finish the test because years ago an HPS friend who was needing so much oxygen she had to be in the hospital, had to be able to complete the six minute walk to stay on the transplant list. I could have finished the walk if I walked slower, or used more liters of oxygen. But, I guess that isn’t the rule here as I am still listed.
I’m still not entirely sure if it is better to walk as fast as you can without running, even if it means your oxygen goes too low, or if it is better to walk at a slower pace and finish the test. The rules of the game, sort of speak, seem a bit murky.
The last thing that came out of the transplant appointment is that they want to do another echo. I had one just a few months ago. They are keeping an eye on whether I am developing pulmonary hypertension. I am at high risk for it. If I had it, it would give me a big bump on the lung allocation score. Treating it might also mean I might use less oxygen. So far, however, I haven’t developed it. Knock on wood! Long term strain on your lungs can start to impact your heart. So far, that hasn’t happened to me. But, if this goes on long enough, it probably will.
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