Abstract, originally published in Epilepsy & Behavior
Objectives: Developmental and epileptic encephalopathies (DEEs) are rare neurodevelopmental disorders characterized by early-onset seizures and numerous comorbidities. Due to the complex requirements for the care of a child with a DEE, these disorders would be expected to impact health-related quality of life (HRQL) for caregivers as well as for patients. The objective of this literature review was to describe the impact of DEEs on the HRQL, emotional wellbeing, and usual activities (social, work, relationships, etc.) of caregivers, including the wider impact on other family members such as siblings.
Methods: A literature search was conducted in May 2020 using MEDLINE® and Embase® databases. Quantitative and qualitative studies were identified using search terms related to family, disease type (including >20 specific DEEs), and quality of life/methodology. Each study was assessed for relevance and was graded using customized critical appraisal criteria. Findings from studies that were given the highest quality ratings were summarized and used to develop a conceptual model to illustrate the complex impact of DEEs on caregiver HRQL.
Results: Sixty-seven relevant studies were identified, of which 39 (27 quantitative, 12 qualitative) met the highest appraisal criteria. The studies recruited caregivers of patients with one of eight individual DEEs, or pediatric intractable or refractory epilepsy. Most studies reported negative impacts on HRQL and emotional wellbeing in caregivers. The wide-ranging impact of a DEE was highlighted by reports of negative effects on caregivers’ physical health, daily activities, relationships, social activities, leisure time, work, and productivity. Factors that influenced the perceived impact included demographic characteristics (e.g., child’s age, living arrangements, family income) and clinical factors (e.g., feeding or sleep difficulties, disease severity). Few studies evaluated the impact on siblings.
Conclusions: There is evidence that developmental and epileptic encephalopathies (DEEs) can impact health-related quality of life (HRQL) and emotional wellbeing and can limit usual activities for the primary caregiver and their wider family. However, no research was identified regarding many individual DEEs, and only limited research assessed the impact on different family members with most studies focusing on mothers. Further research is required to understand the influence of certain factors such as the age of the patient, disease severity, and seizures on caregiver burden. Furthermore, the review highlighted the lack of appropriate measurement tools to assess caregiver HRQL in this population.