Medical news

I.P.F., Not Aging, Could Be Causing Breathlessness
By JANE E. BRODY JANUARY 11, 2016 5:45 AM January 11, 2016 5:45 am

Daniel Castner of Napa, Calif., speaks from experience when he advises people to pay attention to their bodies and have anything unusual thoroughly checked out without delay. It’s just possible there’s a treatment that can nip a problem in the bud if treatment is started soon enough.

Mr. Castner learned at age 63 that he has a progressive, incurable lung disease called idiopathic pulmonary fibrosis, or I.P.F., that more often than not seems to come out of nowhere (idiopathic means of no known cause). Mr. Castner, now 67, was no slouch at the time. He had been an avid backpacker, cyclist, hiker, skier and gardener most of his adult life. But in 2012, while hiking with his family at Lake Tahoe at nearly 7,000 feet above sea level, he experienced “pronounced shortness of breath,” his wife, Susan, told me.

Two months later, a pulmonologist diagnosed I.P.F., a disease that reduces the lungs’ ability to extract oxygen from the air and distribute it to the rest of the body. At the time, there were no effective treatments in the United States. But there was a drug in use in Europe called Esbriet that had been shown to slow the loss of lung function and significantly reduce deaths from the disease.

In October 2014, after seeing positive results from clinical trials, the Food and Drug Administration approved Esbriet as well as a second drug for I.P.F., called Ofev. Two months earlier, under a special permit, doctors at the University of California, San Francisco, started Mr. Castner on Esbriet. He said he could still get along without supplemental oxygen, even when working in the garden with his wife.

“I can get breathy if I have to lift things, so Susan steps in to help,” he said. “I have no problem with her doing some of the heavy work. I like hanging with her.”

He wonders, though, how much better off he might be now if he had been able to start one of the new drugs at the time of his diagnosis, when his loss of lung function was only at 20 percent, rather than when he was finally able to get the drug, when his loss of function was at 60 percent.

“I’ve experienced no change in my condition, no major deterioration, since I started on Esbriet,” Mr. Castner told me, adding that he has been free of the drug’s possible side effects. Now he’s hellbent on getting the word out to the more than 100,000 people in this country known to have idiopathic pulmonary fibrosis, along with many others who have yet to learn this is why they are often breathless.

Dr. Talmadge E. King, Jr., the dean of the medical school at the University of California, San Francisco, who directed one of the definitive studies of Esbriet, said that the drug “reduced by about 48 percent the proportion of patients who declined, showing that the drug worked to slow the rate at which patients got worse. The mortality rate dropped more than we expected.”

Dr. King said, “When I started in pulmonology 30 years ago, everyone with this disease died. Now we can say to patients that you’re going to get worse, but at a slower rate, and you’re going to be alive.”

As Mr. Castner put it: “When I was first diagnosed, I started thinking about the last things I’d get to see before I died. Now I don’t think about death. I have this disease — it’s a fact of life — and even though I can’t do all the physical stuff I used to do, I can still cook and garden with my wife, go out to dinner, walk on flat surfaces and enjoy a glass of wine with friends.” All of which has inspired his effort to “get the word out to other patients that there is now hope.”

More than 14,000 patients in this country have begun treatment with Esbriet, so clearly there are many more who might benefit from it or Ofev, a much newer drug with a somewhat different mechanism of action. Studies are planned to see if the two drugs together — or with other drugs now in development — might be more beneficial than either alone.

Though categorized as a rare disease, “I.P.F. is much more common than is usually realized,” said Dr. Steven Nathan, a pulmonologist at Inova Fairfax Hospital in Falls Church, Va. “And for whatever reason, its prevalence is increasing.” He said the disease was believed to occur mainly in genetically susceptible people who are exposed to irritants like cigarette smoke or occupational dusts or fumes.

On average, it takes patients with I.P.F. two years to be diagnosed correctly. People often attribute their breathlessness to lack of fitness or advancing age because I.P.F. is typically a disease of older people. And doctors often mistake it for asthma, heart disease or chronic obstructive pulmonary disease or confuse it with one of the dozens of other interstitial lung diseases. In addition to shortness of breath, sometimes even during normal daily activities, a persistent cough that does not produce phlegm is a common symptom.

Yet with a high index of suspicion and the right tests, diagnosing I.P.F. is not that challenging. Listening through a stethoscope when patients inhale, doctors can hear a crackling sound that resembles pieces of Velcro being pulled apart. Pulmonary hypertension, or elevated blood pressure in the lungs’ vessels, is common. Its presence can be suggested by an echocardiogram and confirmed by catheterizing the right side of the heart. And while the signs of I.P.F. are usually missed with a traditional chest X-ray, a high-resolution CT scan of the chest can show a pattern of lung scarring characteristic of the disease.

A patient’s lung function is assessed with a six-minute walk test to show how much the oxygen level in the blood drops during the exercise. Another test uses carbon monoxide to measure how much air the lungs can inhale and exhale.

Esbriet, known generically as pirfenidone and marketed by Roche, is thought to counter lung fibrosis by decreasing the production of collagen and factors that promote growth and inflammation. It is not recommended for people with liver or kidney problems, and smoking can diminish its effectiveness. Ofev, known generically as nintedanib, is marketed by Boehringer Ingelheim; it too inhibits fibrosis by targeting various growth factors.

Mr. Castner, who receives Esbriet free through Medicare, said that without insurance, the drug would cost $94,000 a year. Ofev is slightly more expensive, $96,000 a year.

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