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Abstract
Objective To describe the clinical presentation, risk factors, serotype distribution and outcomes of invasive pneumococcal disease (IPD) in children with sickle cell disease (SCD) following the introduction of the 13-valent pneumococcal conjugate vaccine (PCV13) in the UK.
Design Prospective national newborn screening for SCD and enhanced national IPD surveillance.
Participants Children with SCD born in England between 1 September 2010 and 31 August 2014 who developed laboratory-confirmed IPD by 31 December 2015.
Main outcomes and measures Risk of IPD in children with SCD compared with children without SCD during the surveillance period.
Results Eleven children homozygote for haemoglobin S (HbSS) and one double heterozygote for haemoglobin S and C (HbSC) developed IPD. Septicaemia (n=7) and lower respiratory tract infection (n=4) were the main clinical presentations, and serogroup 15 (not present in PCV13) was responsible for 73% (8/11) of cases. Three children with HbSS (27%) died compared with <5% nationally. Children with HbSS had a 49-fold (95% CI 27 to 89, P<0.001) higher risk of IPD compared with their peers without SCD.
Conclusions Children with SCD remain at increased risk of IPD despite national newborn screening, early penicillin prophylaxis and high pneumococcal vaccine uptake. They are also more likely to die of their infection compared with their peers without SCD. Most IPD cases are now due to serotypes not covered by PCV13. Healthcare professionals need to work more closely with families with SCD and local communities to emphasise the importance of penicillin prophylaxis, explore barriers, allay misguided beliefs and facilitate rapid access to healthcare.
- invasive pneumococcal disease
- pneumococcal conjugate vaccine
- sickle cell disease
- risk factors
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Footnotes
Contributors MD conceptualised and designed the study, involved in the interpretation of the data and writing the report, approved the final manuscript as submitted. AS conceptualised and designed the study, co-ordinated and supervised the data collection at one of the two site, involved in the interpretation of the data and writing the report; approved the final manuscript as submitted. GO reviewed the literature, analysed the data, wrote the first draft and co-ordinated the production of the manuscript, involved in the interpretation of the data and writing the report, approved the final manuscript as submitted. SC carried out the initial analyses, involved in the interpretation of the data and writing the report, had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis, approved the final manuscript as submitted. CS reviewed and revised the manuscript, involved in the interpretation of the data and writing the report approved the final manuscript as submitted. NF reviewed and revised the manuscript, involved in the interpretation of the data and writing the report approved the final manuscript as submitted. SL reviewed the literature, analysed the data, co-ordinated the production of the manuscript, had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis, involved in the interpretation of the data and writing the report; approved the final manuscript as submitted. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
Presented at These data were presented as plenary in the just concluded RCPCH conference and the abstract has been published in BMJ as cited below. GO, SC, AS, MD, SL. P08 Risk of invasive pneumococcal disease in children with sickle cell disease in England: National observational cohort study, 2010 – 2015. Arch Dis Child 2017;102(Suppl 1):A4-A4 [10.1136/archdischild-2017-313087.8]: 10.1136/archdischild-2017-313087.8.