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A DIAGNOSIS OF PULMONARY FIBROSIS

Q: A colleague's husband has been diagnosed with pulmonary fibrosis. He has never been sick in his life, at least seriously.

He is now in his late 60s and his wife is devastated, because his doctor said something about not being able to operate on his heart and there is no cure.

What is this condition? Is it really as bad as it seems? -- F.K., Buffalo, N.Y.

A: Yes, pulmonary fibrosis really is bad, and I sympathize with your colleague and her husband. Learning that you have a disease that affects your breathing is a frightening revelation.

Pulmonary fibrosis (also called cryptogenic fibrosing alveolitis) is the most common of a group of lung diseases that interfere with the delivery of oxygen and carbon dioxide to and from the bloodstream. It is caused by scarring and depositing of fibrous tissue on the walls between the lung's air sacs.

In most cases, the specific cause is not found. However, sometimes drugs and a variety of toxic dusts are identified as the cause.

Pulmonary fibrosis, in particular, may be due to some genetic component, and could be a smoking-related disease. It's more common in men and usually shows up in the late middle age, with difficulty breathing and a cough being the first symptoms seen.

As the doctor said, there is no cure, unless a lung transplant can be considered. The condition is long-term, chronic and life threatening. Over time, it will take its toll in terms of greater and greater difficulty getting oxygen into the bloodstream. About half the people with this condition only live about five years after diagnosis.

Medical treatment consists of supplemental oxygen and maintenance doses of steroids like prednisone, when necessary. Almost half of those taking the steroids feel better, and a quarter show some improvement.

It's good that you're concerned about your friend and her husband. At a time like this, the support of family and friends is most important.

Dr. Allen Douma welcomes questions from readers. Although he cannot respond to each one individually, he will answer those of general interest in his column. Write to Dr. Douma in care of Tribune Media Services, 435 N. Michigan Avenue, Suite 1400, Chicago, IL 60611.

This column is for informational and educational purposes only. It is not intended to provide medical advice or take the place of consultation with a doctor or other health care provider.

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