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Mortality and causes of death in children with sickle cell disease in the Netherlands, before the introduction of neonatal screening
Author(s) -
van der Plas Evelyn M.,
van den Tweel Xandra W.,
Geskus Ronald B.,
Heijboer Harriët,
Biemond Bart J.,
Peters Marjolein,
Fijnvandraat Karin
Publication year - 2011
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2011.08806.x
Subject(s) - medicine , pediatrics , sepsis , disease , mortality rate , newborn screening , confidence interval , cause of death , meningitis , vaso occlusive crisis , stroke (engine) , sickle cell anemia , mechanical engineering , engineering
Summary This study analyzed the mortality and causes of death in sickle cell disease patients in the Netherlands, to provide a baseline for monitoring the effect of the recently introduced neonatal screening programme and to indicate areas of improvement in the care for these patients. All children (<18 years) diagnosed with sickle cell disease in a tertiary hospital from 1985 to 2007 were included. Vital status was determined up to March 2008. A total of 298 children were included: 189 (63%) patients had HbSS, 17 (6%) HbSβ 0 thalassaemia, 72 (24%) HbSC and 20 (7%) HbSβ + thalassaemia. Twelve patients (4%) died during a total follow‐up of 3896 patient years. All known deaths were sickle cell disease‐related. Meningitis/sepsis ( n = 4; 33%), stroke ( n = 3; 25%) and death during a visit to the country of origin ( n = 3; 25%) were the most common causes of death. The overall mortality rate was 0·27 deaths/100 patient years [95% confidence interval (CI): 0·15–0·43]. The estimated survival at the age of 18 years was 97·3% (95% CI: 95–99%). This report confirms that the burden of mortality in sickle cell disease is increasingly shifting to adults. It is recommended that compliance to antibiotic prophylaxis, thorough counselling and support for patients travelling abroad and specialized peri‐operative care should receive continuous attention.