Warning Sweat

An anxious mother carried her two-year-old daughter into Boston's Children's Medical Center on a blistering afternoon last week. Dehydrated and suffering from severe shock, the child was near death. Only five years ago, considering the season, most physicians might have made a spot diagnosis of heat prostration. But the center's Dr. Harry Shwachman, developing an idea conceived by Manhattan's Dr. Paul di Sant'-Agnese, had a new and simple test to apply in such cases. An aide took the little girl's foot (with an older child he would have used the hand) and pressed it onto a sheet of agar in a flat glass dish. The agar was laced with silver nitrate and potassium chromate. A normally moist hand or foot would have left only a faint imprint. But this child's sweat contained up to ten times the normal amount of salt, and it etched a sharp print on the agar. With 99% certainty the test indicated a diagnosis of cystic fibrosis. Other more complicated tests confirmed it.

Cystic fibrosis (TIME, March 1, 1954) is so named because victims have a fibrous, diseased pancreas that fails to secrete enough of the enzymes (notably trypsin) needed for digestion; it has also been called mucoviscidosis because the lungs become clogged with a thick, gummy mucus. Unrecognized as a separate disease until 1938, it is now being identified more and more often, occurs (by some estimates) in one child out of 600. At that rate, with its high mortality, cystic fibrosis ranks as one of the most deadly diseases of children in the U.S.

Eat More, Gain Less. Symptoms may appear almost immediately after birth or may be deferred a few years. They fall into two main patterns: voracious appetite with failure to gain weight, and persistent cough that fails to expel the mucus, so that breathing eventually becomes rapid, shallow and ineffective, and the child turns blue. Victims are especially subject to lung infection.

A hereditary disease transmitted, by a recessive gene which both parents must have, cystic fibrosis affects glands throughout the body. Why its symptoms are concentrated in the pancreas and the lungs is not known. Such problems have been subjected to intensive research since 1955, when the National Cystic Fibrosis Research Foundation was organized. It now has 31 chapters, has passed out $140,000 in research grants. One of the foundation's mainsprings is handsome Dr. Wynne Sharples, 33, mother of two victims of the disease, and now married to Dr. Robert Denton, developer of a breathing apparatus that helps some fibrocystic children.

Everything in the Book. Until the antibiotic age the outlook for 90% of cystic fibrosis patients was hopeless--doctors are amazed today to find adults who have survived the disease in mild form. For 80% of those who have it, the outlook is still grim, says Dr. Sharples. But while there is no specific cure, there are helpful treatments. Diet must be rich in protein, low in fat, high in calories (bananas are good), supplemented with vitamins A and D, and with preparations of animal pancreas to supply the missing enzymes. Every antibiotic in the book is used at some stage in most cases; erythromycin and the three tetracyclines are given daily by mouth, and doctors change drugs often because the steady use of one or two might encourage resistant bacteria. Penicillin and streptomycin are usually mixed with detergents in inhalators (of either mask or "croup kettle" type) to sterilize the lungs at the same time as mucus deposits are loosened to be coughed up.

For a limited number of cases Dr. Denton's pressure breathing apparatus is an additional safeguard. An oxygen cylinder, hooked up to an aerosol bottle and a mask, forces air into the child's lungs under pressure so that it reaches corners hitherto closed, and increases breathing efficiency. The trouble is that even selected patients should have the treatment at least twice a day, which means laborious visits to clinics, or training a parent in the operation of tricky (and expensive) apparatus. Says Dr. Denton: "It may be just as dangerous as it is valuable."

Parents of fibrocystic children usually face a hard financial squeeze. Bills for drugs alone run from $70 to $100 a month; special diet and other treatment may double the cost. But now, for the first time, doctors are helping victims to survive into their teens; as the years pass, they hope to see them reach adulthood not much more severely handicapped than the insulin-taking diabetic. Meantime, research goes on to find first causes and, eventually, a cure.

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