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Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London

From the Department of Paediatric Haematology and Oncology, Royal London Hospital, London (PT, JE, DR, SC, OW); Infection Control Office, The Windeyer Institute of Medical Science, University College, University of London (PC); Sickle and Thalassaemia Centre, London (BS); Department of Haematology, Homerton Hospital, Homerton Row, London (RA, AS); Neurosciences Unit, Wolfson Centre, London, UK (FK)

Correspondence: Paul Telfer, Department of Haematology Royal London Hospital, Whitechapel Road, London E1 1BB, UK. E-mail paul.telfer{at}bartsandthelondon.nhs.uk

Background and Objectives: We investigated outcomes in a UK neonatal cohort as a benchmark for care of children with sickle cell disease (SCD).

Design and Methods: Two-hundred and fifty-two children (180 with hemoglobin [Hb] SS, 64 with HbSC, and 8 with HbS/ß thalassemia), identified during 1983–2005 by universal birth screening in East London, were followed in a hospital and community-based program which included penicillin V prophylaxis from 3 months of age, 23-valent pneumococcal polysaccharide vaccine from 1993, conjugate pneumococcal vaccine from 2002 and transcranial Doppler screening from 1991.

Results: At the end of 2005, there were 2158 patient years of observation. The median age of the patients was 7.8 (interquartile range 3.3–13.0) years, and 2.8% of those enrolled had been lost to follow-up. The estimated survival of children with HbSS at 16 years was 99.0% (95% confidence interval, CI, 93.2 to 99.9%) and pneumococcal sepsis rate was 0.3 (95% CI 0.1–0.8) episodes per 100 patient-years. The risk of overt stroke was 4.3% (95%CI 1.5 to 11.4%) and could be further reduced by transcranial Doppler screening from infancy and transfusing all children with high-risk scans. No deaths, strokes or episodes of pneumococcal sepsis were observed in children with HbSC or HbS/ß thalassemia. The mortality rates from HbSS were significantly lower than those in other reported cohorts.

Interpretation and Conclusions: Mortality in childhood SCD can virtually be eliminated in a well-resourced health service setting linking community-based care with a specialized, hospital-based center. SCD continues to cause substantial morbidity from acute complications and chronic organ damage. We recommend setting up of clinical networks to optimize the management of SCD.

Key words: neonatal screening, sickle cell, mortality, infection, cerebrovascular disorders.

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