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Nonsmoker Searches for Cause of  Emphysema

Dear Dr. Roach: I am a 46-year-old man, nonsmoker, who has worked in factories on and off for 25 years. I am on Advair and Proair for emphysema. I also take metoprolol for high blood pressure. My dad’s second cousin passed away from emphysema. My liver and heart appear good.

At my age, is it more likely that I have hereditary low alpha-1 antitrypsin, or did I get my emphysema from working in factories? What should I eat? Can I exercise my lungs to help me breathe easier? — J

Answer: Emphysema is a chronic lung disease characterized by progressive loss of lung tissue, leading to larger and larger holes in the lung and reducing lung function. Alpha-1 antitrypsin is a protein that prevents digestive enzymes from damaging the body’s own organs, especially the lungs and liver. People who lack this enzyme, even nonsmokers, can get both emphysema and liver cirrhosis.

In medical school, my professor quipped that for every hundred people with emphysema, 90 of them are smokers, one has alpha-1 antitrypsin deficiency, and nine of them are lying. My clinical experience since then has shown me that nonsmokers can indeed get emphysema — through secondhand tobacco smoke, occupational exposure and, in other countries, home-cooking-fire smoke. The way to find out is to test your alpha-1 trypsin level with a simple blood test your physician can order.

As far as diet goes, there are usually no restrictions, although I have seen people with very severe emphysema improve a bit by adding a few tablespoons of peanut butter daily. Peanut butter — high in healthy fat and protein — reduces the amount of carbon dioxide your body makes per calorie taken in. People with severe emphysema also tend to get thin, so a few extra calories are often helpful.

Lung exercises can be very helpful, and usually are supervised by a team of health care providers, including physical and occupational therapists, respiratory therapists, nurses and your doctor. Finally, metoprolol is a good medicine for most people with high blood pressure, but it can cause spasm of the airways, so I would ask your doctor to think about changing that to something else.

Dear Dr. Roach: My husband is 50 years old; 6 feet, 6 inches tall; and weighs 185 pounds. He exercises regularly. Last weekend he woke up with some pressure in his chest. He has a history of heart disease in his family, and we went to the emergency room. One of the first things the doctor asked was, “Do you have Marfan’s syndrome?” We’d never heard of Marfan’s syndrome, so we said “no.” When the cardiologist examined my husband, again one of the first things mentioned was Marfan’s syndrome. We looked it up, and it was a huge shock to read that my husband has almost all the characteristics. He has had yearly checkups, which include echocardiograms. He played sports in high school and college, which required physicals and EKGs. My question is, Why wouldn’t any of the doctors in 50 years of regular visits know about or mention the possibility of his having this syndrome? — A.

Answer: Marfan’s syndrome is a rare disease of collagen — a protein that provides most of the structure for the soft tissues in our body. People with Marfan’s syndrome tend to be tall and thin, with disproportionately long arms and fingers. In addition to the cardiac complications of mitral and/or aortic valve prolapse and dilated aorta, other associated findings of Marfan’s include an abnormally high, arched palate; a hollow or funnel chest; and abnormalities of the eyes, including nearsightedness and abnormal position of the lens. If all the signs are present, it is usually pretty easy to make the diagnosis — once you think about it.

However, Marfan’s is variable, and its symptoms can overlap in appearance with just regular tall, skinny people. As a tall, skinny person, I was asked repeatedly if I had Marfan’s syndrome — which I don’t.

It used to be said that the life expectancy of Marfan’s patients was 35 years, but with the realization that some people have a more benign presentation, along with advances in medical treatment, the life expectancy is much improved.

Dr. Roach is unable to answer individual letters, but will incorporate them in the column whenever possible. Email questions to ToYour GoodHealth@med.cornell.edu.