Children and young people with intellectual disabilities have an increased risk of death, which may be avoidable via the provision of good quality health care. Epilepsy is among the most commonly attributed causes of death in children and young people with intellectual disabilities. However, many clinicians and researchers argue the increase in the risk of death in children and young people with both epilepsy and intellectual disabilities is driven by non-epilepsy-related causes, such as respiratory disorders. This project will look at the risk of death among children and young people aged 0-24 who have epilepsy, with the aim of comparing epilepsy patients with versus without co-existing intellectual disabilities. The study will also include children and young people with intellectual disabilities without epilepsy, and compare all three groups to a control group of children and young people in the general population. We will also look and see whether there are differences in the risk of epilepsy-related deaths between these first two groups. This information will be used in clinical guidelines and be used by services to reduce the risk of death in children and young people with intellectual disabilities.
Children and young people with intellectual disabilities have a standardised mortality ratio of 11.6. Epilepsy is among the most common contributing causes of death in children and young people with intellectual disabilities. Available evidence suggests that around 90% of epilepsy deaths occurs in children and young people who have co-existing intellectual disabilities. However, the quality of the studies reporting this high proportion of overall deaths in children and young people with epilepsy lacks rigour to inform policy and service developments. This study will use systematic methods to identify a large cohort of children and young people with epilepsy and a large cohort of children and young people with intellectual disabilities. A representative control group of children and young people in the general population will also be included.
We will use CPRD primary care data between 2006 up to 2018. Linkage of the cohorts to the Office of National Statistics Death registration up to 2018 will be used to compare the proportion of deaths of children with epilepsy that are among children with intellectual disabilities, the proportion of deaths that are caused by epilepsy, as well as to compare the differences in the mortality rates and causes of death. Three study groups will be defined to answer these research questions:
Study groups:
1. Population aged 0-24 with intellectual disabilities
2. Population aged 0-24 with epilepsy:
a) epilepsy and co-existing intellectual disabilities
b) epilepsy without intellectual disabilities
3. Representative sample of the general population aged 0-24, with a sampling frame of 7:1 to study group 1
Descriptive data will be analysed using t-tests, Mann-Whitney and Χ2 tests. Cox proportional hazards models will be used to analyse whether there are significant risk differences in all-cause mortality, epilepsy-related mortality and avoidable mortality between the three study groups.
Primary outcomes:
The primary outcomes will be:
• The proportion of deaths occurring in those with epilepsy which occur in those with co-occurring intellectual disabilities
• The proportion of deaths which are epilepsy-related and non-epilepsy-related in children who have epilepsy with and without co-occurring intellectual disabilities
• The proportion of deaths which are avoidable using the Office of National Statistics UK definition for children and young people
• All-cause and cause-specific hazard ratios including for avoidable deaths
Secondary outcomes:
The secondary outcome will be:
• Proportion of deaths which are related to life-limiting conditions (LLC)
Laura Hughes-McCormack - Chief Investigator - University of Glasgow
Gillian Smith - Corresponding Applicant - University of Glasgow
Bhautesh Jani - Collaborator - University of Glasgow
Craig Meville - Collaborator - University of Glasgow
Iain Carey - Collaborator - St George's, University of London
Jill Pell - Collaborator - University of Glasgow
Michael Fleming - Collaborator - University of Glasgow
Sarita Soni - Collaborator - University of Glasgow
ONS Death Registration Data;Practice Level Index of Multiple Deprivation