Psychobehavioral factors and family functioning in mucopolysaccharidosis

Abstract

Abstract Background: We aimed to evaluate the impact of mucopolysaccharidoses (MPS) on family functioning and related factors. Methods and Results: Twenty-five families of MPS patients were included. The communication domain in adaptive behavior exhibited mean scores below the cut-off points in MPS II (35.6±24.3) and III (35.0±22.6). The total difficulty score was associated with the communication domain score in adaptive behavior (p=0.012). Parents of patients with MPS III and VI reported lower (47.1±2.7) and higher (77.4±21.6) quality-of-life scores, respectively; however, there was no significant among-group difference. The quality-of-life scores were positively associated with cultural/intellectual domain (p=0.003) and adaptive behavior (p<0.001) scores. Regarding family functioning, the greatest effects were observed in the areas of independence, intellectual/cultural, activity/recreation, and expressiveness. The domain scores did not differ according to MPS type, treatment, or neurological involvement. The organization domain significantly differed (p=0.015) between families diagnosed for <2 years and >2 years. Family functioning in the cultural-intellectual domain was negatively associated with caregiver stress. Conclusion: The impact of MPS on family functioning extends beyond physical aspects and encompasses social and emotional dimensions. By promoting coping strategies, facilitating support networks, and recognizing the positive aspects of caregiving, the overall impact of MPS on families can be mitigated.