Caregiver-reported quality of life in individuals with developmental and epileptic encephalopathy and other severe neurodevelopmental encephalopathies

TL;DR

This study explores factors affecting quality of life in individuals with severe neurodevelopmental conditions, finding that cognition and touchscreen use are key predictors.

Contribution

The study uses regression tree analysis to identify novel predictors of quality of life in individuals with severe neurodevelopmental encephalopathy.

Findings

Lower cognition scores correlate with significantly lower quality of life scores.

Higher cognition and touchscreen engagement are associated with improved quality of life.

Small functional gains in alertness and hand skills may meaningfully enhance quality of life.

Abstract

Information on factors contributing to quality of life (QOL) informs meaningful patient-centred care. We evaluated factors influencing QOL in individuals with developmental and epileptic encephalopathy (DEE) and other severe neurodevelopmental encephalopathy conditions using hypothesis-free regression tree analysis.

A questionnaire was completed by 242 caregivers of individuals two years or older. QOL was measured using the Quality of Life Inventory-Disability (QI-Disability). Independent variables described health, functional abilities and daily activities. The R package rpart was used to build the regression trees to explore the most influential factors associated with QOL.

Median age was 8.8y (interquartile range 4.6–14.9 y). Mean total QI-Disability score was 60.2 ± 14.1 out of a total possible score of 100. The subgroup with the lowest QOL scores comprised individuals with low (raw score < 4) cognition scores measured with the Developmental Profile-4 (n = 52, mean score 46.4) whereas higher QOL scores were achieved by individuals with higher cognition scores and capacity to engage actively when using a touchscreen (n = 123, mean score 67.5).

Regression tree analysis suggests that cognition and use of touchscreens were important factors for QOL. Findings suggest small neurodevelopmental and functional gains may meaningfully improve quality of life for individuals with severe neurodevelopmental encephalopathy.

People with severe neurodevelopmental conditions experience challenges that affect their everyday lives, including difficulties with health, functional abilities and independence in activities of daily living. There is limited understanding of what impacts quality of life in this population. In this study, caregivers of 242 individuals with severe neurodevelopmental conditions completed a questionnaire to capture the child’s quality of life, health, everyday functioning and daily activities. The analysis searched for the factors from other measures administered that were important in predicting quality of life. The most important domain predicting the total quality of life score was cognition. Individuals with lower cognition scores had lower quality of life scores while those with higher cognition scores, particularly those with ability to participate in touchscreen activities, had higher quality of life scores. Findings suggest the value of monitoring and supporting even small functional gains in alertness and hand function skills to enable more engagement with people and objects may meaningfully improve quality of life.

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