A preliminary rough estimate of the incidence of diagnosed cases of cystic fibrosis in the pilot area is 42 per 100,000 live births or 1 case per 2,300 live births. This is a minimal estimate because it does not take into account diagnosed cases in newborns who died before leaving the hospital or persons with the disease who were never under the medical supervision of either a pediatrician or a clinic. This preliminary estimate is lower than estimates made by other investigators in 1946 and in 1952 but is higher than that made in 1960. It was anticipated that with a larger population base and the refinement of techniques developed in the first two studies, more conclusive estimates of the incidence and prevalence of cystic fibrosis could be derived.

CYSTIC FIBROSIS IN EASTERN UNITED STATES (PRELIMINARY DATA)

The third survey, which covered 17 Eastern States and the District of Columbia, was conducted during the period April 1962 through April 1963. About 6,200 medical sources selected from the directories of the American Medical Association, the Osteopathic Association, the American Hospital Association, and the American Osteopathic Hospital Association, were queried. Medical sources included all full- and part-time pediatricians in private practice, all hospitals with pediatric-residency training and all other hospitals having 100 or more beds; other sources included a 1-percent sample of other physicians in private practice and a 10-percent sample of hospitals having fewer than 100 beds. By the cutoff date, better than 92 percent of sources queried had responded to the questionnaire. Another group of 1,800 medical sources were also queried but this group of physicians and hospitals were obtained by referral from sources in the original sample.

Only patients who were seen or treated by physicians or in hospitals and clinics during the 10-year period 1952-61 and who had resided in the study area at some time during that period were included in the study. Duplicate reports of the same patient were eliminated as well as reports on doubtful cases based on criteria developed in the pilot study. After weighting, the estimated number of patients seen for the disease during the 10-year period totaled 5,518.

The sampling error of the estimated number of cases is about 5 percent or 250 and the chances are 67 out of 100 that the number of treated cases is within 5,518 plus and minus 250. The analysis of patient data being reported on, however, are based on an estimate of 4,918 cases; 6 patients reported only by medical sources in the 1-percent sampling stratum were excluded in order to reduce the variability of those statistics involving relatively small-size estimates. Since survy data collected are still in the analysis stage, only a few preliminary findings are being reported here.

Of the 4,918 patients under medical supervision for cystic fibrosis at some time during 1952-61, 4,260 had been diagnosed for the disease during the 10-year period; the remaining 658 were diagnosed prior to 1952 and were living at the beginning of that year. Among the 4,918 patients there were 2,361 deaths. From 1952 through 1961 the annual number of cases diagnosed each year and the annual number of deaths attributed to the disease about doubled. However, since the number of cases detected each year was in excess of the number of fatalities, there was a substantial increase in the annual number of patients under medical supervision. During the 10-year span the annual number of patients under care tripled or increased from about 900 patients in 1952 to about 2,800 in 1961 (table 1).

Within each age group, the number of patients also increased over the 10-year span. In age groups under 1, and 1 to 4 years, the numbers about doubled; for the group 5 to 9 years the number increased four times, and for patients 10 years and older, the 1961 figure was seven times larger than that of 1952 (table 2). However, the detection of new cases of the disease in older children appears to contribute only slightly to the increasing proportion of fibrocystic patients under medical supervision at older ages (table 3). A comparison of the percentage distribution of patients dying during the periods 1952-56 and 1957-61 shows an appreciable shift in the distribution of deaths from young to older age groups (table 4). Whereas more than three-fourths of all fatal cases in the earlier period were among infants and children under 5 years of age, less than twothirds of the deaths in the more recent period were among children in that age group. At ages 10 years and older, the proportion of deaths had increased from 5 percent of the 1952-56 total to about 15 percent of the 1957-61 total.

About 2 percent or 55 of the 2,652 resident patients under care for cystic fibrosis during 1960 were Negro; 5 lived in New England, 29 in the Middle Atlan

tic States, and 21 in the South Atlantic States. In contrast, about 13 percent of the study area resident population in 1960 was Negro; the New England population was less than 1 percent Negro, the Middle Atlantic was about 8 percent Negro, and the South Atlantic almost 23 percent. Because of the apparent race differential in the expression of cystic fibrosis in the population, caseload rates for 1960 were computed for the total population and for the white population only.

For the study area, the cystic fibrosis caseload rate was 4 per 100,000 population. Among infants, however, the rate was about 30 per 100,000, 15 for children at age 1 through 4 years, about 12 for those at ages 5 to 9, and 1 per 100,000 population at ages 10 years and older. Rates for white patients were proportionately higher in each age group; rates ranged from 34 for children under 1 year of age to about 14 for those at ages 5 to 9 years (table 5). In New England, the prevalence of the disease in the white population for all age groups of children was about twice that of the Middle Atlantic and South Atlantic States. The caseload rate per 100,000 white infants in New England was 55, whereas for white infants in the Middle Atlantic and the South Atlantic States, the rates were 30 and 28, respectively.

The incidence of diagnosed cases per 100,000 live births was also substantially higher in New England than in the two other geographic divisions. In New England the cystic fibrosis incidence rate was 45 per 100,000 live births or about 1 case in every 2,200 live births in cohorts of white children born during 1953 and 1954. In both the Middle Atlantic and South Atlantic States, the incidence of diagnosed cases was about 28 per 100,000 live births, or about 1 case in every 3,500 live births among white children. These are lower bound unadjusted estimates. Factors contributing to both the higher prevalence and incidence of the disease in New England need further study. A full report on the prevalence and incidence of the disease in relation to sampling and procedures used in processing the data is currently being written.

PUBLICATIONS

It is expected that the findings of the third survey and the methodology will be published as a Children's Bureau monograph during 1966 and in other publications during the current year and in 1967. Published reports on the two previous surveys on cystic fibrosis are as follows:

Kramm, E. R.; M. M. Crane; M. G. Sirken; and M. L. Brown: "A Cystic Fibrosis Survey in Three New England States," Amer. J. of Public Health, 52: 2041-57, Dec., 1962.

Kramm, E. R.; M. M. Crane; M. L. Brown; and M. G. Sirken: "Characteristics of Cystic Fibrosis Patients Discharged from Hospitals in 1957: National Estimates," Pediatrics, 28:123-38, July 1961.

Sirken, M. G.; M. M. Crane; M. L. Brown; and E. R. Kramm: "A National Hospital Survey of Cystic Fibrosis," Public Health Reports, 74: 764–770, Sept. 1959.

The growth of interest in research in cystic fibrosis is resulting in the gradual extension of the services of State crippled children's programs to include children with this condition. Much yet needs to be done to spread the knowledge of this disease and to increase the number of clinical facilities adequately staffed to provide the necessary services. For many families, the cost of care for a child with this disorder is considerable and averages between $1,000 and $2,000 per year. The State crippled children's programs are beginning to make a significant contribution toward meeting the problem of cystic fibrosis through providing the necessary specialized services and helping to meet the costs of In 1964 approximately one-half of the States reported that children with cystic fibrosis were being served under the crippled children's program.

care.

TABLE 1.-Number of fibrocystic patients under medical supervision at the beginning of each calendar year and number newly diagnosed, under care, and dying during each year, 1952–61: New England, Middle Atlantic, and South Atlantic States1

TABLE 2.-Patients under medical supervision for cystic fibrosis during each calendar year 1952–61, by age—New England, Middle Atlantic, and South Atlantic States 1

TABLE 3.-Number and percentage distribution of newly diagnosed cases of cystic fibrosis in each 5-year period, 1952-56 and 1957-61 by age at diagnosis: New England, Middle Atlantic, and South Atlantic States 1

TABLE 4.-Number and percentage distribution of fatal cases of cystic fibrosis in each 5-year period, 1952-56 and 1957-61 by age at death: New England, Middle Atlantic, and South Atlantic States 1

TABLE 5-Cystic fibrosis cases and caseload rates for white race by residence in geographic divisions and by other selected demographic characteristics: New England, Middle Atlantic, and South Atlantic States, 1960 1

TABLE 6.-Resident white population April 1960, used for computing rates shown in table 5 of this report, by selected demographic characteristics: New England, Middle Atlantic, and South Atlantic States

FRIDAY, MARCH 11, 1966.

ST. ELIZABETHS HOSPITAL

WITNESSES

DR. DALE C. CAMERON, SUPERINTENDENT

DR. DAVID W. HARRIS, FIRST ASSISTANT PHYSICIAN

MR. M. K. MADDEN, EXECUTIVE OFFICER

MR. LYLE C. VANMERE, CONSTRUCTION AND MAINTENANCE ENGINEER

MR. F. M. HALL, BUDGET OFFICER

MR. JAMES B. CARDWELL, DEPARTMENT DEPUTY COMPTROLLER