Keywords
Quality of life
Activities of daily living
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Abstract
Objective: To assess the quality of life (QoL) of individuals with mucopolysaccharidosis (MPS) using the 5Q-5D instrument. Methods: A descriptive, prospective, and analytical observational study was conducted to evaluate QoL in MPS patients at a University Hospital in Campina Grande, Paraíba, Brazil, using the simplified EQ-5D-3L instrument for adults and EQ-5D-Y for children and adolescents. Results: The sample consisted of individuals with MPS I, IV, and VI, with a mean age of 20.6 ± 8.5 years, with an equal distribution of genders, and a mean age at diagnosis of 4.9 ± 6.5 years. The QoL instrument revealed that the most affected areas included mobility and self-care, followed by pain and/or discomfort and daily activities. On the self-rated health scale, 5Q-VAS, the mean score was 79.5 ± 20.3. Conclusion: It was evident that patients with MPS I, IVA and VI perceive their health better than indicated by the tests, despite severe restrictions in daily activities requiring caregiver assistance for various tasks.
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