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

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Abstract

Objective

To evaluate England’s NHS newborn sickle cell screening programme performance in children up to the age of 5 years.

Design

Cohort of resident infants with sickle cell disease (SCD) born between 1 September 2010 and 31 August 2015 and followed until August 2016.

Participants

1317 infants with SCD were notified to the study from all centres in England and 1313 (99%) were followed up.

Interventions

Early enrolment in clinical follow-up, parental education and routine penicillin prophylaxis.

Main outcome measures

Age seen by a specialist clinician, age at prescription of penicillin prophylaxis and mortality.

Results

All 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.

Conclusion

The 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.

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