Big news! I am now on the active waiting list for a single lung transplant! This would be at the Brigham in Boston. It means I can expect a phone call at any hour of the day or night: today, next week, next year, whenever a donated lung becomes available that’s a good match for me. The matching algorithm is quite complex, all designed to optimize outcomes for the maximum number of people. This is both exciting and scary. And the wait is likely to be nail-biting.
When the call comes, I will drop whatever I am doing and vanish from the grid for a while: a few weeks in the hospital, and then home if I am lucky or to rehab if less lucky. My support staff is gearing up: wife, daughters, son-in-law, dogs…and numerous friends have offered to pitch in with meals, etc. The total recovery time is measured in months.
I am keenly aware that my good fortune, when it comes, will be due to someone else’s misfortune. I will be getting the lung of someone who generously decided to become an organ donor upon their premature death. It’s a sobering thought. When the time comes, I hope the family of the donor will take comfort in knowing that as a side effect of their loss, someone else was given life. A hard comfort, perhaps.
I’ve been waiting for a couple of months now to see if I’ll be listed as eligible for a lung transplant. I got the news this week—I’ve been approved! Yaaaay!!
Er, almost. I’ve been approved, pending (what now?)… an allergy consultation to see how allergic I really am to certain antibiotics that over the years have given me rashes or sneezing fits or whatever. Why do they care about this, of all things? It’s because a transplant means immunosuppression to prevent rejection of the new lung. And that means they need all the tools they can get to treat any infections that might come along. So, a couple more weeks, to see what the allergist says. And then, hopefully… then we can wait to see if the insurance company signs off on it. And then be listed.
I’m home from the Brigham after six days in the hospital for lung-transplant evaluation. I survived all the tests they could throw at me, ranging from neuropsych to colonoscopy to arterial catheterization… to trying to sleep in a hospital room with lights going on and off all the time, and beep! beep! beep! As far as I can tell, I passed all the tests, with the exception of getting a decent night’s sleep. That one I failed. The only test outstanding is an esophageal swallowing test, which they had trouble scheduling. I scheduled it myself after I got home. Also, I need to meet with a surgeon. When those are done, The Committee will meet to decide my fate—i.e., whether I get listed for a lung transplant, and what ranking.
There was a contingent of docs that wanted me to stay in the hospital through it all, right up through actually getting a lung (or two). Their reasoning was that I could suddenly go downhill at any time (an exacerbation, they call it), and become acute. While acknowledging that risk, I declined to stay, on the grounds that sitting in a hospital doing nothing is a terrible way to stay healthy, and that I would be safer, healthier, and in a better mental state at home where I can exercise, do my work, and live life. And not wear out my support team before anything has even happened.
I am hopeful about getting listed and about having the operation. It’s a daunting prospect, most daunting of all (in my mind) a lifetime of immunosuppression to avoid rejection. I like my healthy immune system. But those are the breaks. I like even better the thought of breathing freely, and no more oxygen machines. I credit folks on various support groups who have enthusiastically embraced their lung-transplant experiences for turning me around on this. Thanks, guys.
Maybe. Here I sit at Brigham Hospital, waiting for an “expedited evaluation” for a lung transplant operation. This is based upon a meeting with the medical director of the lung transplant program, who suggested that the time is right for me now, before I get any worse and while I’m strong enough to get through it in good shape. (If you’re just joining us, I have pulmonary fibrosis, a kind of scarring of the lungs, and my choices are to undergo a slow decline until it kills me, or risk a lung transplant—if I qualify.)
I checked in twenty-four hours ago for a battery of tests, but unfortunately, not much has happened yet. I am sitting here, bored, typing on my tablet. Got tired of watching TV on my tablet. I should be reviewing the new recording of Panglor on my phone, but I couldn’t concentrate.
Tomorrow. Tomorrow it’s going to start happening. That’s what they tell me. I hope they’re right.
Here’s another of my promised updates on my battle with pulmonary fibrosis. Now back in Boston (see below), I just had my first CT scan in a couple of years, and the new images show that my interstitial lung disease has progressed more or less the way my pulmonologists expected. This is a lot like, Sorry to report, but we’re continuing to spiral in toward the black hole. Still, according to my pulmo, she’s seen worse. My need for supplemental oxygen has doubled in the same time frame, which is not great news, either. I have a “new” 10L oxygen concentrator here at home now, as well as the actually new one I got in Puerto Rico. This thing is loud! It might be saving my life for now, but it’s killing my hearing and threatening my daughter with migraines at the same time. Looks a little like Robbie the Robot.
The new drug that promises to stop the progression of this damnable disease has just completed dual trials, with favorable results (according to my doc, who keeps her ear to the rails). There’s hope that it could be approved for use before too long. But that’s only if the Musk/Trump wrecking ball currently demolishing our government doesn’t disrupt the FDA’s drug approval process. (Bets, anyone?) That aside, in April, when I come back from Puerto Rico for the second time, I’ll be looking to get listed as a lung transplant candidate.
That’s all kind of a downer, isn’t it? Let’s see if I can end this on a more positive note: I have a whole lot of people praying for me. And I can still do all the push-ups and squats and lunges and weight-lifts I was doing before. I just need a little extra push in the atmo department. And ear muffs. What’s that? (Cupping hand to ear.) No, I can hear you just fine! It’s just… can you stop mumbling and speak up a little?
Upon arriving home from the tropics, this was the scene that greeted me. Brrrr.
I said I’d post from time to time on how I’m doing, health-wise. I guess it’s about that time. I just had my semi-annual visit with my pulmonologist, complete with the usual PFT (pulmonary function tests), to see how well my lungs are breathing. The answer is, almost as well as this time last year. I am reasonably stable, which with pulmonary fibrosis is the best outcome you can expect with the treatments now available.
I’d be lying if I said I wasn’t a little disappointed. The Chinese herbal treatment I’m on is reported to have helped several other PF patients get measurably better, and of course that’s what I wanted, as well. But I don’t know the details of the other patients.
My doc was pleased that I’m doing as well as I am. We discussed the Chinese herbs, and when I said the main definite benefit seems to be improved energy, she assessed that as a win and suggested I keep taking them. We talked about some new drugs coming along in clinical trials; there’s one she’s excited about, which offers the hope—no, not of a cure—but of real stability and the halt of decline. I said how soon, and she said it was being fast-tracked with two simultaneous trials, and if the tests prove out, it could be available in a year or two. Now I’m excited about it.
Now, if only they had something to keep me and my family from tripping over the damn oxygen hose all the time! What we exos have to put up with, carrying our own atmospheres around with us!
For six weeks now, I’ve been gulping a pile of Chinese herbs from my potions master that look like this, dissolved in hot water, twice a day. Yum. (No, not yum. Gaghh.) So how’s it going? Is it working? I’m glad you asked. Reports from my crack team of observers say that I appear to have more energy, more focus, more life. Myself, I can’t tell! But I believe them, and I’ll take the win. Also, my years-long morning cough has largely gone away. I feel subjectively that I’m less short of breath when I exert myself. Does that mean it’s working? I hope so.
Empirical evidence is less clear. My O2 readings are about the same—dipping down when I exercise and coming back up, as always. I’m using the same amount of supplemental oxygen. I won’t have pulmonary function tests at the docs until August, and then we should know if this regimen is working in a measurable way. Fingers crossed.
Though I had high hopes for better on the O2 end of things, I am encouraged by the energy, and the other things. Keep on kiting!
In the doctor’s office, they call it a “communication” in the heart or sometimes a “shunt.” When I asked if that was another way of saying a “hole,” the doctor laughed and said, “Sure, if you want to call it a hole, it’s a hole.”
What we’re talking about is called a “patent foramen ovale (PFO)”—pronounced foh-RAY-mun oh-VAY-lee—a hole in the heart that didn’t close the way it should have after birth. It’s a small opening between the upper heart chambers, or the atria. It’s normally present during gestation in the womb but closes up during infancy—except when it doesn’t. Then it’s called a patent—or open—foramen ovale. That’s what I’ve got, and it turns out to be the cause of most of my shortness of breath when I’m exerting myself. What’s happening is that some of the blood that should be getting pumped to my lungs for fresh air is getting shunted instead to the left side of my heart and sent back out to the rest of my body. And then my saturation O2 drops.
We learned this during some recent tests: one where they did an echocardiogram while injecting micro-bubbles into a vein, and another where they ran catheters into my arteries to measure various parameters, while having me pump away on an exercise bike until I was gasping. They gathered lots of good data, I was assured. They thought I might have pulmonary hypertension, but I don’t.
What does this mean about the pulmonary fibrosis? Well, I still have it; that hasn’t changed, alas. But it’s milder than my breathing needs would indicate. The accusing finger points at my heart for that.
The good news is, this is something they can probably fix. First, though, I have to get scheduled for another test, to get clearer images of the shunt. If that goes as expected, then I’ll be, er, shunted to cardiology for the next phase.
Stay tuned. It should be exciting.
This is a railway shunt. Not really what we’re talking about.
They came and took away my oxygen concentrator, and also almost a dozen small tanks that were clustered in the other room. In their place, they left a new oxygen concentrator—one that I think puts out a flow closer to the advertised rate—and a Dalek-like machine that sits on top, which refills tanks while I sleep. Now, instead of being limited to the ten smallish tanks that I was allowed per month, I have only two, but I can use them as fast as I can fill them.
This is for going out, of course, especially for dog walks. I’ve learned that my clever Inogen portable concentrator (POC) is fine for going out to the store or whatever, or just knocking about where exertion is low. But when it gets more aerobic, like dog-walking, it just can’t keep up with my needs as well as a small tank in a backpack. I believe this is because the POC gives the O2 in little pulses when I inhale, which is less assistance than a tank on continuous flow. The duration of a tank is shorter, of course, but all I need is 30-40 minutes to walk the dogs, and for that it’s better.
The new machine has a different and no less annoying continuous drone from the old one, and since it’s near where Allysen works all day, I spent that afternoon putting together some sound baffling to try to cut the decibels. That part is still a work in progress.
The last two weeks have been all about oxygen. O2. Breathing stuff. Not that important until you’re not getting enough. Two weeks ago is when the first oxygen supplies arrived. (In case you missed it, the reason for my oxygen is spelled out in Pulmonary Fibrosis and Me.) We now have the constant drone and rhythmic ptoosh of a big oxygen concentrator, which sits centrally located in our apartment and sends supplemental oxygen through a network of green tubes laid out through the house: one to the bedroom, one through the bedroom and up through a hole in the closet ceiling to my office, overhead, and one that I just snake around behind me as I move about. I’m on the O2 most of the time, to keep my blood saturation levels up. I can go off for short periods, but if I’m active, my O2 percentage will drop right into the 80s, percentage-wise, which isn’t great. Most people are pretty stable around 97-98, as I used to be. Also, staying on the O2 seems to reduce my coughing. Here’s the big guy.
Also, I bought a secondhand portable concentrator that I can wear in a backpack when I’m out doing things like walking the dogs.
I never knew walking the dogs was such an aerobic activity! I really have to pace myself. But as promised, I have increased my daily exercising—leg lifts, crunches, etc., before getting out of bed. The highlight is forty squats and forty pushups while the coffee is brewing. That’s something to look forward to every morning! (Not really.) Stopping between sets to breathe really hard and wait for the O2 to come back up, that’s the ticket. Here’s my cool ring oximeter.
Sometimes I get sick of the cannula in my nose and I switch over to Darth Jeff mode. I sound just like the guy in the black suit.
Weird thing about in-home oxygen supplies: It apparently has never occurred to any of these companies that people might need to split their O2 feed into different rooms. They’ll give you the hoses and connectors and cannulas that you need, but when you ask about a splitter valve, they wonder what language you’re speaking. Well, okay, I thought, you can get anything on Amazon, right? Um… After much searching online, I did eventually find a link to a really overpriced two-way plastic valve that works (the gray one on top of the machine). A week later I realized I needed more, and eventually I found the brass gizmo with the yellow knobs. I had to MacGyver a connector between them, because no one seems to make an O2 connector that’s female on both ends. It’s not rocket science, people! Lots of folks are out there looking for the same solutions as I was, but you can’t get it off the shelf. Victory to those who can think outside the box. I feel like I’m controlling a submarine here.
But how am I doing? you ask. Well, some days I feel kind of discouraged. Most days I soldier on. You’re not taking me without a fight, dammit. I’ve got stuff to do!
