van Heyningen A M, Levenston M J, Tamminga N, Scoop-Martijn E G, Wever R M F, Verhagen A A E, van der Dijs F P L, Dijck-Brouwer D A J, Offriinga P J, Muskiet F A J
Laboratory Medicine, University Medical Centre Groningen, The Netherlands.
West Indian Med J. 2009 Sep;58(4):301-4.
To estimate the incidence of Sickle-Cell Disease (SCD) in Aruba and St. Maarten and to determine whether universal screening would be cost-effective according to United Kingdom criteria.
Consecutive cord blood samples were collected in Aruba and the Dutch part of St. Maarten during 3 and 4 months, respectively. Samples were subjected to High Performance Liquid Chromatography (HPLC) screening of haemoglobin variants.
Of the 368 samples (87.6% of all registered births) collected in Aruba, 10 (2.72%; CI 1.3, 4.9%) tested heterozygous for the Sickle-cell gene (HbAS) and 7 (1.90%; CI 0.8, 3.9%) for the haemoglobin C gene (HbAC). Of the 193 samples (83.5%) collected in St. Maarten, 14 (7.25%; CI 4.0, 11.9%) contained HbAS and 10 (5.18%; CI 2.5, 9.3%) HbAC. Hardy-Weinberg equilibrium predicted an incidence of 2.65% for HbAS and 1.86% for HbAC in Aruba and 6.80% for HbAS and 4.86% for HbAC in St. Maarten. These figures imply a newborn rate of about 2 SCD patients per 3 years in Aruba and 2 SCD patients per year in St. Maarten.
Universal screening of newborns for SCD seems cost-effective for St. Maarten.
