Journal Article

Evaluation of newborn sickle cell screening programme in England: 2010–2016

Hounsell, Kirsty, Dick, Moira, Sisodia, Rupa, Dormandy, Elizabeth, Streetly, Allison, Latinovic, Radoslav

2018

Overview

ObjectiveTo evaluate England’s NHS newborn sickle cell screening programme performance in children up to the age of 5 years.DesignCohort of resident infants with sickle cell disease (SCD) born between 1 September 2010 and 31 August 2015 and followed until August 2016.Participants1317 infants with SCD were notified to the study from all centres in England and 1313 (99%) were followed up.InterventionsEarly enrolment in clinical follow-up, parental education and routine penicillin prophylaxis.Main outcome measuresAge seen by a specialist clinician, age at prescription of penicillin prophylaxis and mortality.ResultsAll but two resident cases of SCD were identified through screening; one baby was enrolled in care after prenatal diagnosis; one baby whose parents refused newborn screening presented symptomatically. There were 1054/1313 (80.3%, 95% CI 78% to 82.4%) SCD cases seen by a specialist by 3 months of age and 1273/1313 (97%, 95% CI 95.9% to 97.8%) by 6 months. The percentage seen by 3 months increased from 77% in 2010 to 85.4% in 2015. 1038/1292 (80.3%, 95% CI 78.1% to 82.5%) were prescribed penicillin by 3 months of age and 1257/1292 (97.3%, 95% CI 96.3% to 98.1%) by 6 months. There were three SCD deaths <5 years caused by invasive pneumococcal disease (IPD) sensitive to penicillin.ConclusionThe SCD screening programme is effective at detecting affected infants. Enrolment into specialist care is timely but below the programme standards. Mortality is reducing but adherence to antibiotic prophylaxis remains important for IPD serotypes not in the current vaccine schedule.

• Publisher: BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health,BMJ Publishing Group LTD,BMJ Publishing Group
• Subject: Age / Age Factors / Anemia, Sickle Cell - diagnosis / Anemia, Sickle Cell - epidemiology / Anti-Bacterial Agents - therapeutic use / Antibiotic Prophylaxis - statistics & numerical data / Antibiotics / Attrition (Research Studies) / Babies / Disease prevention / Early Intervention / England - epidemiology / haematology / Health Education - statistics & numerical data / health services research / Health Services Research - methods / Humans / immunisation / Immunization / Infant, Newborn / Infants / Medical screening / Medication Adherence - statistics & numerical data / Mortality / Neonatal Screening - methods / Neonatal Screening - organization & administration / Neonatal Screening - standards / Neonates / Newborn babies / Original / Original article / outcomes research / Parents / Parents - education / Pediatrics / Penicillin / Penicillins - therapeutic use / Performance evaluation / Prenatal diagnosis / Program Evaluation / screening / Screening Tests / Serotypes / Sickle cell disease / State Medicine - organization & administration / State Medicine - standards / Streptococcus infections / Test Results / Young Children