Health and educational outcomes of children with sickle cell disease in England

Research question: While complications in sickle cell disease (SCD) have been described in the literature, population-based studies on health, healthcare use, and school experiences of children with SCD are lacking. What is the overall scope and prevalence of co-morbidities for children with SCD nationally?

How would SCD complications affect healthcare usage and educational progress among children with SCD in England?

Background: SCD is one of the most common genetic diseases in the UK, with approximately 17,500 affected individuals and almost 300 affected babies born annually.

SCD is associated with recurrent episodes of acute pain, complications such as stroke and progressive organ damage, leading to frequent hospitalisations.

SCD-related pain, fatigue, and neurological complications may limit children s participation in schools and impact their academic attainment. SCD patients in England are of African-Caribbean descent.

They are vulnerable to disparities in healthcare due to stereotyping, structural racism, and inadequate awareness of SCD in the NHS. Children with SCD are underrepresented in research participation as well.

Aims and Objectives: The study aims to provide comprehensive insights into health and educational outcomes among children with SCD in England.

Specific objectives include (1) prevalence and risk factors associated with mortality and SCD complications in child and young people patients, (2) use of hospital care and specialist care in these patients, and (3) educational outcomes in school-age children with SCD compared with unaffected children.

Methods: We will conduct a population-based retrospective cohort study by linking databases in hospital care, specialist care, and education.

Paediatric SCD cases in England will be extracted from the National Haemoglobinopathy Registry (NHR), a national database of sickle cell patients.

The SCD cohort will be validated using clinical coding from Hospital Episode Statistics (HES), a national database of hospital care in England. A control cohort of children unaffected by SCD will be derived from HES birth records.

The ECHILD project has linked HES records to the National Pupil Database (NPD), a database of education outcomes for all state-schooled children in England.

We will collaborate with the charity Crescent Kids to conduct a pre-study survey and run focus groups before and during the project across major cities in England.

Responses from the survey and focus groups will help us identify parents concerns and understand parents experience of caring for children with SCD.

Timelines for delivery: Before 11/2024: Complete the pre-study survey, obtain access to ECHILD linked data and the NHR, apply for ethical approvals, and organise the first-round focus group meeting. By 31/05/2025: Publish study protocol, complete data extraction within ECHILD.

By 31/10/2025: Complete linkage of NHR to HES, analyse data, organise the second-round focus group meeting, disseminate and publish results.

Anticipated Impact and Dissemination: Study results will help healthcare professionals to make evidence-informed decisions about prioritisation of risk factors in managing paediatric SCD cases. Findings will be communicated to parents ,healthcare professionals and policy makers.

Research papers will be presented at academic and practitioner-led conferences to raise awareness of the consequences of SCD and deficiencies in healthcare and education services.