Hereditary Diseases: Aerosol for Breathing

Though cystic fibrosis has had intense attention from medical researchers in the past few years, bedside doctors still find it bafflingly difficult to deal with the disease, which involves both the lungs and the digestive tract. It is not for lack of trying: they are using a dozen or more different drugs and other treatments. Now, Pediatrician Herman W. Reas of St. Louis Children’s Hospital has found that boosting the patient’s breathing efficiency twice a day with a new aerosol drug eases his distress and promotes his general health.

The victim of cystic fibrosis, almost invariably a child because the disease is usually fatal before adulthood, has an inherited enzyme defect that damages the oxygen-exchange cells in his lungs and reduces the elasticity of the lung walls. He does not breathe enough air in, nor let enough out. His windpipe and lungs become clogged with thick viscid mucus. The trick is to loosen and thin this mucus, and get rid of it.

Dr. Reas reports in the Southern Medical Journal that he used a new mist-making drug, N-acetylcysteine (trade named Mucomyst by Mead Johnson & Co.) on 28 patients aged 7 to 22. He clapped a face mask on his patients twice a day, before meals, and got them to inhale Mucomyst aerosol supplied under gentle pressure by a small pump. After 20 minutes, each bedridden child was turned into assorted head-down positions to help him spit out the mucus. Stronger children got rid of the mucus by taking a short but brisk run, which started them coughing.

Children with lung damage already far advanced received little benefit, but those with only moderate damage showed remarkable improvement after continuous treatment for two years or more. They could be far more active physically, they slept better (a boon to worn-out parents), they gained weight, and one grew three inches in 18 months as compared with only three-quarters of an inch in the preceding 18 months.

The Mucomyst spray is no cure, but it appears to be less irritating than previous aerosols. While its effects may be most dramatic in cystic fibrosis, the University of Mississippi’s Dr. Watts R. Webb reports that the drug seems to be equally useful in other diseases in which mucus blocks breathing.