Causes of death in sickle-cell disease in Jamaica.

Abstract

A review of the causes of death in 276 patients with sickle-cell disease showed that although the greatest mortality occurred in the first five years of life, roughly one-quarter were aged over 30. Commonest causes of death in the first ten years included acute splenic sequestration, septicaemia, meningitis, aplastic crises, and gastroenteritis. In older patients cerebrovascular accidents and renal failure became common. The acute chest syndrome affected all age groups about equally but appeared to result predominantly from infection in the young and embolism or thrombosis in the old. IntroductionMost genotypes of sickle-cell disease are associated with a shortened life expectancy, but there is little information on the causes of death or on the pattern of mortality at different ages. Our experience at a major medical centre in the Caribbean over the past 30 years is therefore presented in this retrospective review of the causes of death in 276 patients.

The Natural History of Sickle Cell Disease

“…Mortality patterns are undoubtedly changing because of diagnosis by newborn screening and more effective interventions, but an earlier review in Jamaica indicated that the highest mortality occurred in the second 6 mo of the first year of life due principally to acute splenic sequestration, the acute chest syndrome, and septicemias (Thomas et al 1982). At later ages, acute chest syndrome persists as a major cause of death and the pattern of septicemias may be changing with the selection of penicillin-resistant and/or nonvaccine serotypes of Streptococcus pneumoniae and the emergence of Salmonella as an important organism (Wright et al 1997).…”

Acute Chest Syndrome in Sickle Cell Disease

“…7 Infection appears to be more common in children, whereas fat embolism occurs more often in adults. [6][7][8] …”

Clinical and Laboratory Predictors of Elevated TRV In Sickle Cell Anaemia

“…Pulmonary involvement in sickle cell disease (SCD) has been shown to be a major factor affecting morbidity and mortality, being recognized also as an important determinant of survival 1,2 .This has become apparent in recent times as increased numbers of SCD patients reach adulthood due to improved survival strategies in childhood 3 . The lungs are common sites of hypoxic and ischaemic injury, emboli from marrow infarcts/fat necrosis with increased propensity to developing pneumonias 4 .…”