Cystic Fibrosis of the Pancreas in Patients Over Ten Years of Age

Abstract

Summary A study of 27 patients with cystic fibrosis of the pancreas who lived to be more than 10 years of age presents a wide range of clinicd states. Four of these children on whom observations were made after they had reached the age of 10 years have died at various ages up to nearly 20 years, all with the picture of progressive purulent bronchopneumonia. One of the fatal cases, coming to autopsy after a life span of 13½ years, showed cardiac changes consistent with cor pulmonale. Among the 23 living patients all have cough and clubbing of the fingers as continuing evidence of broncho‐pulmonary infection. The healthiest show no other signs of impaired respiratory function, either by roentgenogram or following a work tolerance test; likewise, some of these healthier patients have attained a body weight and height which do not differ significantly from mean values for the appropriate age and sex. For the group as a whole, however, a trend toward significant retardation of growth in height and of gain in weight is evident, the degree of retardation being roughly correlated with the severity of the pulmonary infection. Pubertal changes may be delayed. A number of the most seriously involved patients are in effect respiratory invalids, having a markedly reduced exercise tolerance. Regardless of the severity of their somatic functional status, however, almost all of them sustain a cheerful outlook for the future and build ambitious plans in the manner of all normal children. Although their nutritional state depends primarily on the severity and activity of their broncho‐pulmonary infection, as a group these children respond fairly uniformly to changes in diet. Almost without exception they prefer to avoid fat foods, having learned that ingestion of appreciable quantities of fat is followed by abdominal pain or by a change for the worse in the number and character of their stools. Many, having taken pancreatin for several years, now appear to do equally well without it. It would appear that adequate compensation for lost pancreatic digestive function in cystic fibrosis is quite readily attainable, but that the bronchopulmonary infection, once entrenched, is difficult to eradicate. Perhaps the best hope for the future lies in early recognition of the pancreatic lesion and in the institution of an effective prophylaxis of the infection.