Newborn sickle cell disease screening: the Jamaican experience (1995-2006). J Med Screen 2007;14(3):117-22
Date
10/11/2007Pubmed ID
17925083DOI
10.1258/096914107782066185Scopus ID
2-s2.0-34848853516 (requires institutional sign-in at Scopus site) 60 CitationsAbstract
OBJECTIVES: The aim of this study was to evaluate the existing newborn sickle haemoglobinopathy screening programme in Jamaica.
METHODS: A retrospective analysis of infants screened during the period 8 November 1995 to 22 July 2006 was performed. Patient data for analyses was restricted to patients with homozygous (Hb SS) sickle cell disease. Published data from the Jamaican Sickle Cell Cohort Study was used to make comparisons with the study sample.
RESULTS: The study sample consisted of 435 patients with Hb SS disease. Acute chest syndrome was the most common clinical (non-death) event accounting for approximately 50% of all events. Acute splenic sequestration, no longer a significant cause of mortality, was responsible for approximately 32% of clinical events. Seven deaths (1.8%) occurred during the study period compared with 17.6% to the same age in the Jamaican Sickle Cell Cohort Study. There was a lower proportion of hospital admissions and episodes of serious illness in the study group compared with controls.
CONCLUSIONS: Survival estimates for the study sample showed improvement compared with the Jamaican Sickle Cell Cohort Study. This study continues to demonstrate the benefits of, and as such shows support for, newborn screening and early interventions in sickle cell disease. In addition, it highlights some of the areas for continued focus and research development. Although the current system is providing an essential and beneficial service, the study emphasizes the need for newborn screening programmes to be comprehensive care systems to be fully effective.
Author List
King L, Fraser R, Forbes M, Grindley M, Ali S, Reid MAuthor
Raphael Fraser PhD Assistant Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Anemia, Sickle CellChild
Child, Preschool
Cohort Studies
Hemoglobin, Sickle
Homozygote
Humans
Infant
Infant, Newborn
Jamaica
Survival Rate