A few weeks ago I wrote about my right heart catheterization experience but only briefly touched on the purpose of the findings, and the results, and what they mean for me. Here is some background info, and the latest info I have to share. It’s probably not all that interesting to most folks, so I’m mainly documenting it here for myself so I can remember the sequence of events later on.
Why I had the test
I’ve been monitoring my oxygen levels since I was pregnant with Christopher. I did great for years, really only getting short of breath after huge meals or at elevation. But after I didn’t bounce back well from a pneumonia I had in March, in early December I finally decided I’d had enough, and called for a pulmonology appointment, for the first time in nine years (oops, but like I said I’d been doing well).
My doctor didn’t like what he saw on the echocardiogram I’d had while in the hospital with pneumonia, which indicated I might have a pressure problem in my heart and pulmonary artery. He sent me for a repeat echo along with a saline bubble study. (A bubble study is an easy but unnerving test where they agitate saline bubbles, then push them into your vein via IV, and watch on the echocardiogram screen to see how the bubbles behave when they go across your heart. The concept is pretty freaky, and I could feel the bubbles traveling up my arm and taste the saline, but it’s evidently not a risky procedure.)
The tests showed my heart to be functioning well, but that I had a hole between the two atrial chambers in my heart, called a patent foramen ovale (PFO). It’s a common defect; something like 20% of the population has it and typically doesn’t even know it. However, the echo showed there to be pretty significant blood flow through the hole, which meant that un-oxygenated blood was being shunted back through my body instead of going to the lungs for oxygen first. It also meant I was at a higher risk for stroke and heart attack. This was a new and scary development for me; I’d become accustomed to being screened for pulmonary hypertension (the pressure problem I mentioned above) for the past 12 years, but I’d never known that I might have a different worrisome problem. I was afraid to go to sleep for a few nights, but Frank talked me down and the doctor echoed his reassurances.
The next step was to get a transesophageal echocardiogram, which was like the first one except that they put the scope down your esophagus while you’re sedated, and look at the heart from the back side. It’s a clearer view without the obstructions of ribs and lungs. That test went great and indicated that the PFO was small and not really shunting blood like we’d feared, and was more of an incidental finding than anything causing a problem.
The final diagnostic test was the right heart catheterization that I described in a previous post.
What the test showed
The right heart catheterization confirmed that the hole in my heart wasn’t troublesome, but it also confirmed unequivocally that I have mild pulmonary hypertension.
What pulmonary hypertension is
The short version is that pulmonary hypertension is the narrowing and stiffening of the capillaries in the lungs and in the pulmonary artery. The pulmonary artery goes from the right ventricle of the heart to the lungs, picks up oxygen while discarding carbon dixiode, then goes back to the heart to be pumped out to the body, supplying the rest of the body with fresh oxygen. The problem with pulmonary hypertension is that when the pulmonary artery narrows, it creates higher pressure in the right side of the heart, because it’s trying harder than it should to pump blood through to the lungs. As you might expect, it also makes it harder to get enough oxygen and causes shortness of breath. It’s a progressive condition that usually ends in heart failure and death in a few years, though there are new treatments and lots of research being done. Sometimes a heart-lung transplant is done when other options have run out, but we’re not sure I’m a candidate for that, and luckily we’re a long way from having to think about it.
PH has nothing to do with the blood pressure anywhere else in the body. My normal BP is about 100/65.
It’s a really rare condition, and sometimes a cause can’t be pinpointed, though there was a spike in the number of cases in the middle of the 1990s. Remember fen-phen? Fenfluramine, the “fen” part, caused severe pulmonary hypertension and heart valve damage in dozens of people, mostly women, who ultimately died from it. Outside of drug usage, it’s not a common condition, and many doctors aren’t familiar enough with it to know how to diagnose it, much less treat it.
What it means for me
No one knows.
I saw a pulmonary hypertension specialist a couple of weeks ago (loved her and her nurse!) and she started me on a medicine called Adcirca. It’s basically the lung version of the erectile dysfunction medicine Cialis – they’re identical except for dosage, and both consist of the drug Tadalafil – except that mine costs $3,000 per month. Unfreakingbelievable. The insurance will cover it, though we’re still waiting to hear what the copay will be. (Update: the insurance will cover it 100% after my deductible for the year has been met, which definitely has been already.) I don’t want to diminish the lifestyle altering benefits of Cialis for men, but it’s ridiculous that the same drug is so much more expensive for people whose survival might actually depend on it. I’m lucky that we’re in a position to afford it and that we have good coverage.
My doctor is hopeful that this medicine will keep me stable for a very long time, and that the pulmonary hypertension won’t progress. Because I’ve had just one functioning lung since birth, and restricted space in my chest cavity since I had back surgery and a spinal fusion in 1978, I’m a different case than just about anyone else. She – and several other doctors – think that I’ve probably had mild PH for a long time, which is a good indicator that even if it does progress, it will be slow. That’s definitely our hope, anyway.
The initial diagnosis didn’t devastate me at first, for those reasons. But since then I’ve had some pretty awful days where I’ve had to stop and catch my breath after just walking a block at a slight incline. It’s much worse if I eat a big meal or drink more than about a glass of wine, so I’m generally being careful. On days like that – the last 3 days were rough ones – it’s hard not to get discouraged and let the fear creep in a bit, but I’ve also had some absolutely fantastic days as well. And: even with some rough days in the mix, I do think the medicine is working. My oxygen levels are often quite healthy, and my resting heart rate has decreased. These are good signs, and I’m glad my ticker is catching a bit of a break for now. I step up to the full dose tomorrow, and I’m hoping for even better results.
I don’t like this stupid condition being in charge of my life, so I’m doing what I can to mitigate its effects. I’m exercising much more regularly – occasionally while wearing oxygen so I can go a little harder and not get into trouble – and I’m working to lose a few more pounds because that always helps me breathe better. I use my little pulse oximeter to monitor my O2 levels, and when they’re low I just chill out for a bit until they come back up. The hardest part is not feeling guilty and antsy when I’m sitting around, but I’m trying not to beat myself up too much about that. Frank keeps offering to give me piggyback rides everywhere we go. I haven’t taken him up on it yet…but I also haven’t told him to stop offering them. The bottom line is this: I can still do almost everything that I like doing, I just can’t do it fast. We still travel, I still garden, we still take walks and go out with our friends and family. I already wasn’t running marathons, so at least I don’t have to give that up. That’s a relief. And I’ll just keep my fingers crossed about the rest.
If you’ve read this far, you’re probably exhausted. Sorry! Believe me that this was the condensed version of everything that’s happened so far. Now that I’ve gotten the back story and diagnosis details out of the way, any further posts on this will be much, much shorter.