Dying of a disease I never knew existed

By this time next year, if the medical forecasts are correct, I will probably be dead, another casualty of a fatal illness that most people have never heard of: idiopathic pulmonary fibrosis (IPF).

This condition has been described by Michael J. Stephen in his 2021 book “Breath Taking” as the “most frustrating and disheartening of all the diseases in pulmonary medicine.” Over a frighteningly short time (the median age of survival after diagnosis is three years), patients with IPF will become increasingly short of breath as the lungs no longer perform their vital function of oxygenating the blood. The alveoli — the sac-like pockets where this process takes place — will fill with mucus and harden until, in Stephen’s disturbing phrase, the lungs “turn to stone.”

Almost as shocking as the rapid progression of the disease is its obscurity. Roughly 40,000 people die from it every year in the United States, 5,000 more than die from prostate cancer and only slightly fewer than die from breast cancer. Stephen estimates that as many as 200,000 Americans struggle annually to survive with idiopathic pulmonary fibrosis.

Yet, as he notes, there are no TV ad campaigns, road races, color-coded ribbons, or ice-bucket challenges to raise awareness and fundraise for a cure.

When I was told two years ago that I had IPF, I had never heard of it, nor had any of my friends. My main worry at the time was colon cancer. In November 2019, it took doctors five hours of surgery to remove a sizable tumor from my large intestine. The cancer had also tainted more than half a dozen lymph nodes.

Luckily, the disease had not spread to other parts of my body, so in the nomenclature of cancer I was categorized as stage 3: a seriously ill but treatable patient. I underwent 12 sessions of chemotherapy at the start of 2020, after which a colonoscopy and blood tests found no traces of cancer.

That was the good news. The bad news was that the potent drugs administered to me during those six months — drugs that are likely responsible for my good news — can have a rare but documented side effect.

I wasn’t aware of this possibility until a visit with my oncologist at the end of my regimen. During what I expected to be a congratulatory meeting at the finish line, he pointed to a CT scan of my chest and said it showed signs of scarring, or “glassy lung.” (In radiological photographs, damaged lung tissue can present as white dots or opaque space, indicating that the alveoli are blocked.) He recommended that I see a pulmonologist.

I wasn’t too concerned. The words “glassy lung” had a pleasing sound. I imagined that, like bronchitis, it would clear up after a dose of antibiotics. The notion that scarring of the lungs could be a symptom of something deadly didn’t occur to me. This wasn’t, after all, cancer.

Medical researchers have known about pulmonary fibrosis since the 19th century, but little has been done to ameliorate its symptoms, much less to pinpoint its origins. No one seems sure of its causes. My case is called idiopathic because doctors can’t be certain that chemotherapy was the trigger.

Nothing has so far been discovered that will substantially extend life. Two drugs, Ofev and Esbriet, were approved by the Food and Drug Administration in 2014 because they were found to slow the rapidity of lung deterioration. I take two Ofev capsules twice a day. A vial of 60 pills costs upwards of $10,000, a burden relieved by a medical foundation grant.

I have no idea whether the pills are effective or not. My doctors seem just as uncertain. The only guarantee is that idiopathic pulmonary fibrosis has no cure and cannot be stopped. It is, to quote my first pulmonologist, “irremediable.”

The symptoms struck me without warning. In May 2021 I was playing two sets of doubles tennis three times a week. Two months later I couldn’t swing a racket without feeling winded. I was prescribed a steroid inhaler and large doses of prednisone, and they seemed to alleviate my breathlessness for a few weeks — until they didn’t.

Our house on Long Island today is crisscrossed with long, thin plastic tubes connecting me via cannulae to respirators that can produce up to 10 liters of oxygen a minute. I am hooked up to one or the other of these two machines, set at six liters, every hour of every day. When I go out, I carry a portable respirator that is less powerful but allows me some measure of free movement. I try not to travel far. Being away from my machines for too long taxes my oxygen level. If I go to museums — part of my job as an arts critic is reviewing exhibitions — I need to be pushed in a transport chair. Propelling myself in a wheelchair is too exhausting. My sole encounter with Covid-19 was mild. A more severe one could finish me off.

I am 69, and my only hope of living into my mid-70s is a lung transplant, an operation currently prohibited for patients like me: In U.S. hospitals where the procedure is done — there are about 65 of them and counting — a patient must be cancer-free for five years to qualify. Even if by some miracle I were to live another three years, I would then have to wait a minimum of two years to receive one lung, three years for two lungs. At the end of that period, I would be 75, past the cutoff date when lung transplants are authorized. Checkmate.

I wish I could say that the realization of the brief time I have left has altered my procrastinating habits and that I have begun to behave like those characters in Russian literature who felt liberated when infected by tuberculosis. So far, though, that hasn’t happened. I have made no progress on my long-delayed book about photography and violence; documentary film projects that were slated to begin are no closer to fruition. Thinking about anything except my day-to-day health and how to improve it is a struggle.

I don’t know whether to be grimly amused or enraged by the irony that the chemotherapy drugs designed to save my life may be what will kill me.

The discouraging lack of progress in finding a cure for such an ancient disease may explain the relative silence about IPF. Journalists throng to report on any illness when science advances understanding of it, and there hasn’t been a lot of promising news to report on this front. The prospects for treating, say, lung or prostate cancer are far more optimistic. It can’t be easy for doctors to uplift the spirits of their patients knowing that so little can be done to keep them alive. Ofev will only slow the steady pace of the inevitable — the petrification of my lungs.

If idiopathic pulmonary fibrosis is, as Stephen writes, “the big mystery in all of pulmonary medicine, a disease so cryptic it seems almost impenetrable,” one would hope that more hospitals were now trying to crack the code. This is a disease that badly needs a respected public figure to advocate for more research funding. The powerful tools of the medical-industrial complex can’t be trained on solving the riddle of IPF until more people learn that it exists.

Richard B. Woodward is an arts critic in New York. This article was originally published in the Ideas section of The Boston Sunday Globe.