The Impacts of Caregiving for Patients with X-Linked Retinitis Pigmentosa (XLRP): Findings from the EXPLORE XLRP-2 Study

Abstract

Introduction

Informal caregivers play an important part in the healthcare of patients with chronic diseases, including those leading to visual impairment. X-linked retinitis pigmentosa (XLRP) is considered one of the most severe forms of retinitis pigmentosa and causes declines in vision starting in childhood, ultimately progressing to legal blindness in adulthood. Caregivers are expected to play an increasing role in patient care, but real-world impacts of XLRP on caregivers are poorly evaluated.

Methods

EXPLORE XLRP-2 was an exploratory, multicentre, non-interventional study. Cross-sectional surveys were used to gather experiences directly from caregivers across Europe and Israel by both validated and newly developed caregiver-reported outcome surveys.

Results

Seventy caregivers of patients with XLRP associated with retinitis pigmentosa GTPase regulator (RPGR) mutations were enrolled, of whom 68 were included in analyses; 87.7% of caregivers were female and mean (standard deviation [SD]) age was 49.4 (11.7) years. They were most commonly either spouses (50.8%) or parents (41.5%) of patients. Caregivers spent a mean (SD) 28.7 (34.5) hours per week caring for patients. Of the 72.3% of caregivers who were employed, 34.8% worked part time; of the 27.7% of caregivers who were unemployed, 33.3% cited caregiving responsibilities as a cause for unemployment; 23.1% and 46.2% of caregivers reported any level of depression and anxiety, respectively, with few additional impacts captured by the surveys.

Conclusions

Some caregivers reported employment and mental health impacts in this study. However, despite many hours spent per week caring for patients with XLRP, the surveys did not reflect the expected burden experienced by caregivers, highlighting the need for further research in this field.