Thalassemia major in Connecticut: a 20-year study of changing age distribution and survival.

There has been a near doubling of the mean age (10.7 to 19.0 years) of Yale-New Haven Hospital patients with transfusion-dependent homozygous beta thalassemia between 1973 and 1994. This change is, in part, a result of improved transfusion procedures and effective chelation therapy with deferoxamine mesylate. A concomitant trend has been a decrease in the number of births of new cases resulting in fewer thalassemia major patients less than 10 years of age in 1985 and 1994 compared to a decade earlier. Identification of individuals at risk of having children with thalassemia major by extensive screening for heterozygous thalassemia and the availability of prenatal diagnosis may have contributed to this change. Thalassemia major has become a disease of adolescents and young adults. The increasing age has resulted in "new" issues in thalassemia major patients such as sexuality and marriage as well as economic problems involving health-care insurance and employment.