Pulmonary Fibrosis

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!

Time for some sobering talk. A number of months ago, I was diagnosed with Idiopathic Pulmonary Fibrosis (IPF), which is a scarring and thickening of the tissue around the lungs’ alveoli, cause unknown. The main symptom is shortness of breath, with cough. It occurs mainly in older people, and it only ever gets worse, not better. Some people live with it for a long time; others, not so long. It’s a disease that can kill you, and I’d never even heard of it until I got it. The only “cure” is a lung transplant.

I wasn’t too worried at first, because my shortness of breath was fairly mild, and I seemed to be stable. In the last couple of months, though, my shortness of breath has gotten a little worse, and I am now taking one of the two medications that are available to try to slow the process. They have side effects, of course. I have an excellent pulmonologist, and also an appointment with a clinic at one of the leading Boston hospitals that specializes in IPF and other interstitial lung diseases. This is all to the good, but doesn’t change the fact that I have gone from being pretty dang healthy to having a life-threatening illness. That’ll sober you up…

I am upping my exercise routine to keep my lungs and body in general in the best shape possible. For that to work, I need supplemental oxygen, and that’s supposed to be arriving tomorrow. I will soon be walking the dogs with a little tank slung over my back. Yay. In the meantime, I take breaks between sets of pushups and squats, etc., and pant and wait for the numbers to go back up on my pulse oximeter.

My secret hope for a silver lining is that once I have more O2 flowing in my brain, maybe some of the creative writing blocks that have been troubling me will give way in the face of oxygen. I can hope. Meanwhile, prayers and healing thoughts would be much appreciated. I hope this will be a long story to tell, but only the future knows for sure.