A Cystic Fibrosis Pilot Survey in Three New England States

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Abstract
A pilot survey of known cases of cystic fibrosis was made to develop experience for a larger undertaking. A total of 1060 medical sources (pediatricians, a sample of other physicians, and clinics) were queried by mail in Dec. 1959. There was a 90% response. Of 621 reported patients under medical supervision during the period 1952-59, 26% were diagnosed prior to 1952; in each succeeding year the number of cases diagnosed was greater than the number of deaths, thereby increasing the total number of cases known each year. Between 1952 and 1959, the number of patients doubled, newly diagnosed cases increased by about half, and deaths remained about the same. Among the 621 patients were 169 fatalities. About the same number of deaths occurred in the period 1952-55 as in the period 1956-59; but in the earlier period 30% were infants and only 7% children 10 years and older, whereas in the later period 19% were infants and 28% children 10 years and older. The age at which patients were first detected as cases was about the same in each 4-year period. The prolonged life of fibrocystic children in recent years apppeared to contribute more to the higher percentage of deaths found in the oldest age group than did the late detection of cases. A preliminary rough estimate of the incidence of diagnosed cases of cystic fibrosis is 42 per 100,000 live births, or 1 case per 2300 live births. This is a lower bound estimate because it excludes diagnosed cases in newborns who died before leaving the hospital and it excludes patients who were never under medical supervision of a pediatrician or a clinic. While the findings cannot be generalized and applied to the nation they do point to important problems and possibilities.

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