Life-table for Cystic Fibrosis

Abstract

A survey has been made of 499 children attending the Hospital for Sick Children who were diagnosed as suffering from cystic fibrosis ; 399 cases presented without neonatal intestinal obstruction and the remainder with meconium ileus. The difficulties of diagnosis in the former group are discussed. The series of life-tables have been constructed for each of the two groups of presentations of the disease: one entering the affected child at diagnosis, the other from birth. The true life expectancy for the disease will probably lie between the two estimates provided by the different methods of entry to the life-tables. The life-table for cystic fibrosis not presenting as meconium ileus with patients entering at diagnosis shows that by the end of the first year of life over two-thirds of the children had died. At the end of the fifth year over four-fifths were dead. By the end of the tenth year nearly nine-tenths were dead. The life-table for cystic fibrosis not presenting as meconium ileus with patients entered from births shows that by the end of the first year of life approximately one-seventh of the children had died. By the end of the fifth year about one third were dead. At the end of the tenth year rather over a half, and by the end of the fifteenth year nearly three-quarters were dead. The life-tables for meconium ileus show that by the end of the first week of life a quarter of the children had died. At the end of the second week over a third were dead. By the end of the first month over a half, and at the end of the third month three-quarters, were dead. © 1966, British Medical Journal Publishing Group. All rights reserved.