The impact of informal care for patients with Pompe disease: an application of the CarerQol instrument.

BACKGROUND: Patients with Pompe disease, a rare progressive neuromuscular disorder, receive a considerable amount of informal care. In this study, we examined the impact of providing informal care to patients with Pompe disease. METHODS: Caregivers were administered various instruments, which measured the (impact of) informal care in the context of Pompe disease. Patients' quality of life and use of a wheelchair and respiratory support were used to investigate the impact of disease severity on the burden and well-being of caregivers. RESULTS: Of all Dutch patients with Pompe disease, 88 indicated to receive informal care, of which 67 (76%; 67 caregivers) participated in this study. On average, caregivers provided 17.7 hours of informal care per week. Higher disease burden was associated with more hours of informal care. Caregivers experienced burden due to caregiving. Half of the informal caregivers reported mental health problems and problems with daily activities due to providing informal care. Physical health problems occurred in 40% of informal caregivers. Caregiver burden was higher for patients with a lower quality of life and for wheelchair dependent patients. Burden was not associated with respiratory support. Caregivers reported deriving personal fulfillment from caregiving and, on average, would become unhappier if someone else were to take over their care activities. CONCLUSIONS: The provision of informal care causes burden to caregivers. However, caregivers also value caring for their loved ones themselves. The study may help physicians and policy makers to design measures to support informal caregivers.