RESUMO
AIM: Thalassaemia, the commonest genetic blood disorder in Papua New Guinea (PNG) presents daunting challenges for the affected children, their parents and families, and the health system. We aimed to describe the quality of life of affected children and adolescents and the experience of and difficulties faced by their parents in the setting of a tertiary referral hospital in PNG. METHODS: A mixed-methods longitudinal study involving baseline questionnaire, then serial interviews with parents, children and adolescents living with ß-thalassaemia attending Port Moresby General Hospital. RESULTS: Twenty-one patients and their families were interviewed over a 6-month period. Most families originated outside the National Capital District and had migrated to be near the Port Moresby General Hospital and its blood bank services. Thirteen patients had at least one affected sibling and four families had experienced the death of at least one other affected child. No child was receiving chelating agents, and most had clinical evidence of iron overload. There were important impacts of thalassemia on quality of life, including very poor school attendance and some aspects of children's self-perception. Families faced significant burdens and made genuine sacrifices to care for their children. CONCLUSION: Regular blood transfusions increase the life-span of children with thalassaemia but there is a need to achieve a hyper-transfusion regimen coupled with chelation therapy. As for all chronic illness, a focused and holistic approach is needed to improve the quality of life for affected children and their families.