Date of Completion

January 1980


Anthropology, Cultural




This is a study of thalassemia on the island of Cyprus where 27 percent of the population carries one of the thalassemia genes. Thalassemia is an incurable genetic disease of childhood in which death occurs during the first year if untreated. With modern costly treatment, including monthly blood transfusions and related symptomatic therapy, affected persons commonly live into their teens.^ Thalassemia is an inherited defect in the synthesis of the adult hemoglobin molecule. Evolutionary theory suggests that its high incidence in human populations was the result of a balanced polymorphism where thalassemia heterozygotes had a reproductive advantage due to natural protection from the malaria parasite. With the eradication of the presumed selection factor (malaria) after World War II, the human adaptation to environmental stress no longer applies.^ The primary aim of the study is to describe and assess the quality of life of thalassemic children in Cyprus. From this ethnographic and statistical analysis it is possible to identify those factors of home life, social relations, and medical care which contribute to more positive coping behaviors (of the children and their parents) in the face of terminal illness.^ In this study, it was hypothesized that children from more cohesive families, with higher socioeconomic status, casual adherence to the Greek Orthodox religious tenets would exhibit more positive adjustment as would those with medical management approximating a more comprehensive treatment program, with little manifest deformity. Field work to test these hypotheses was carried out in Cyprus over a seven-month period in 1976-1977.^ Extensive formal interviews, psychological testing, direct observation, home visits, school visits and teacher ratings provided the data for testing the hypothesis.^ Considerable variation in patient adjustment was documented. One-third of the patients were identified as experiencing significant coping problems.^ Among the variables tested for association with patient adjustment, family cohesiveness is most important. In addition, components of other variables were related to several aspects of patient functioning.^ Results of the study indicate that patient psychosocial well being depends not only on optimal medical management. Maintaining family functioning is critical to good patient adjustment. Future program plans recommended include, in addition to continued optimal medical management, patient education services, family counselling services, and disease prevention. ^