Dear Dr. Gott: Please comment on bronchiolitis obliterans. My wife died of the condition last spring, and the information I received was quite vague.
Dear Reader: This term refers to swelling and scarring of the tiniest bronchial tubes, which supply air to the lungs. Formerly considered to be an infectious disease of childhood, bronchiolitis obliterans has recently been diagnosed more frequently in adults.
This dangerous lung disorder can follow viral pneumonia and the inhalation of toxic gasses such as nitrogen dioxide. In fact, a common form of bronchiolitis obliterans, called “silo-filler’s disease,” affects people in that occupation who inadvertently inhale – even for just a few minutes – the nitrogen gas that collects in high concentrations above fresh silage.
Bronchiolitis obliterans is also a complication of rheumatoid arthritis, bone marrow transplantation (for leukemia) and an allergy to the drug penicillamine. Some cases of bronchiolitis obliterans occur without any recognizable cause.
In acute cases, such patients experience marked difficulty breathing due to fluid accumulation in the lungs. After initial improvement, these patients relapse; they develop fever, cough and breathlessness. Unless treated aggressively – usually with cortisone drugs – the bronchial swelling and scarring may lead to suffocation.
The diagnosis is made according to the pattern by which the disease progresses, usually over a period of months. Biopsy may be necessary. Unfortunately, the effects of therapy are inconsistent. Those patients who don’t die are often left with disabling pulmonary symptoms, requiring supplemental oxygen.
To give you related information, I am sending you a copy of my Health Report “Pulmonary Disease.” Other readers who would like a copy should send a long, self-addressed, stamped envelope and $2 to Newsletter, P.O. Box 167, Wickliffe, OH 44092. Be sure to mention the title.
Dear Dr. Gott: My daughter was recently diagnosed with Alpha I antitrypsin deficiency. What is this condition?
Dear Reader: Alpha 1 antitrypsin is a substance in body tissues that is necessary for normal metabolism. Some people, such as your daughter, are born with a hereditary deficiency of this enzyme, causing a disease marked by emphysema and liver damage.
Symptoms include jaundice (in children) and difficulty breathing and liver scarring (in adults). The diagnosis is made by blood tests.
Standard therapy is directed toward preventing factors that accelerate emphysema, such as a prohibition against smoking. In severe cases, liver transplants may be required.
In general, patients with this condition should be under the care of appropriate specialists, such as pulmonologists and gastroenterologists.
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