Epilepsy is a condition where the brain does not function properly and causes seizures. These makes the body move randomly and fast. In some cases, it can lead to breathing difficulties, loss of consciousness and even death. Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) are rare types of epilepsy that start during early childhood and can cause severe negative consequences, including developmental problems and premature death in some cases. Their carers are also impacted by poor quality of life and work restrictions. DS and LGS are often misdiagnosed leading to those affected receiving the wrong treatment. This feasibility study intends to find a method to identify with DS and LGS patients using their healthcare records from the Clinical Practice Research DataLink linked to the Hospital Episodes Statistics. This would require that patients with epilepsies are screened for the types of seizures that are related to DS and LGS, including when they started and ended, and their responses (including unresponsiveness) to treatments that were prescribed. The feasibility study will also assess what possible data are available from the general practitioners’ and the hospitals’ records on complications, referral to neurological and social care as well as causes of hospitalisations and death. This information will be used to make decisions to conduct future research on DS and LGS to assist healthcare practitioners and service providers understand how many patients suffer from DS and LGS and to assess their response to treatment with a view to improve their care.
Dravet Syndrome (DS) and Lennox–Gastaut syndrome (LGS) are rare epileptic encephalopathies which start in early childhood and continue into adulthood. Both syndromes are characterised as drug-resistant and are associated with cognitive dysfunction and poor quality of life. Carers of DS/LGS patients can also experience poor quality of life too and work restrictions. Misdiagnoses are not uncommon and can delay the correct treatment. The codes utilised within the Clinical Practice Research DataLink (CPRD) linked to the Hospital Episodes Statistics (HES) to record DS/LGS are inadequate to identify them, especially as patients can be misdiagnosed. Additional criteria, e.g. type of seizures (e.g. myoclonic), resistance to treatment, will also be used to identify DS/LGS. This feasibility study will aim to identify which criteria are required to enable their identification and will screen the CPRD and HES records (Inpatient, Outpatient and Accident & Emergency datasets) of epilepsy patients for the relevant fields that contains these criteria, which will then be used to construct separate algorithms to be used for future research. The feasibility study will also assess what data are available from the general practitioners’ and the hospitals’ records on complications (inc. on Mother-Baby Link), referral to neurological and social care as well as causes of hospitalisations and death (from the ONS death registry). Data from this feasibility study will enable to the identification of DS and LGS patients (especially the latter which has heterogenous aetiology) for the conduct of future research will describe the epidemiology, health resource utilisation and cost burden of these epilepsies on the healthcare system in England as well as their impact on the sufferers’ quality of life. This data will be published in the public domain for healthcare practitioners and service providers to utilise in order to improve healthcare provision and treatment outcomes for patients with DS and LGS.
Seizures types, frequency and duration
Mortality (cause-specific, overall; number, and proportions)
Survival (feasibility of defining and estimating disease-free or progression-free, overall) Hospitalisation, outpatient visits and general practice consultation (cause-specific and overall)
Comorbidities, including cognitive dysfunction
Specialty caring for DS/LGS
Referal to neurological and social care
Basic summary statistics (including counts, proportions, mean and, standard deviations, medians and interquartile ranges, etc) will be obtained. Rates in person-time will be calculated wherever appropriate.
Indraraj Umesh Doobaree - Chief Investigator - Takeda UK Limited
Indraraj Umesh Doobaree - Corresponding Applicant - Takeda UK Limited
Ananya Roy Chowdhury - Collaborator - Takeda Development Center Americas, Inc.
Ning (Julia) Zhu - Collaborator - Takeda Development Center Americas, Inc.
SUDHAKAR MANNE - Collaborator - Takeda Development Center Americas, Inc.
CPRD Aurum Pregnancy Register;CPRD GOLD Pregnancy Register;CPRD GOLD Mother-Baby Link;HES Accident and Emergency;HES Admitted Patient Care;HES Outpatient;ONS Death Registration Data;Patient Level Index of Multiple Deprivation;CPRD Aurum Mother-Baby Link