Haemophilia A in the highlands: the investigation and management of two families in Tari.
Dyke T., Flew S., Bramwell S., Murray F., Horby P.
A 12-year-old boy from Tari in the Southern Highlands of Papua New Guinea presented with prolonged bleeding from a minor injury to the lip. He had a history of profuse bleeding and joint swelling following minor trauma. He has two younger brothers with a similar history. It was demonstrated that they had a coagulation profile compatible with factor VIII deficiency and a family tree suggestive of haemophilia A. A further case was investigated some months later. Despite the neighbouring places of residence of the two families no familial connection could be established by involved discussions between family members. This was confirmed by reviewing the data held on the demographic surveillance system of the Tari Unit of the Papua New Guinea Institute of Medical Research. These families are considered against a background of the diagnosis and management of this condition in a rural part of Papua New Guinea. The long-term support of these patients and other similarly affected individuals presents difficult clinical and ethical problems for rural health services.