Mental health impact on primary and secondary Prader-Willi syndrome caregivers.

INTRODUCTION: Caring for individuals with rare diseases can be challenging and represent a burden. Nevertheless, this has been scarcely explored in Prader-Willi syndrome (PWS). Therefore, we sought to explore the psychological impact of caregiving, as well as the differences between main caregivers and other family members. METHODS: Different evaluation tools and scales were used taking into consideration the impact on caregivers. The scales were administered to those relatives who are immersed in the usual dynamics of the patient, differentiating between the main caregiver and other relatives living in the family home. RESULTS: A total of 33 families of patients with genetic confirmation of PWS were included. In this survey, 32% of primary caregivers reported a high probability of anxiety, compared with 19% of non-primary caregivers (p = 0.27). Concerning depression, 40% of primary caregivers related possible or probable cases of depression compared with non-primary caregivers 13% (p = 0.04). Regarding caregiver burden evaluated using the Zarit scale, 61% of the main caregivers presented high levels of overload, compared with 29% of the other relatives (p = 0.005). Family functioning evaluated using the APGAR scale showed a total lower response from primary caregivers, but no statistically relevant results were found [25.4 ± 6.7 vs. 26.0 ± 8.2 (p = 0.72)]. CONCLUSION: In this study, we observed that caring for people with PWS can have a significant effect on the mental health, burden and quality of life of caregivers, with a greater impact among primary caregivers compared with the other living relatives.

Role of projective psychological tests in patients with Prader-Willi syndrome.

INTRODUCTION: The purpose of this study was to evaluate the usefulness and relevance of projective techniques such as house-tree-person (HTP) and family in individuals with Prader-Willi syndrome (PWS), who have a limited ability to identify and verbalize emotions and express them often using behaviors. METHODS: We included individuals with genetic confirmation of PWS immersed in a regular transdisciplinary treatment in an institution dedicated to rare diseases. All individuals were evaluated using the HTP and family projective techniques. These instruments are commonly administered to the general population and, in this case, to people with mild to moderate intellectual disabilities, including difficulties in their communication abilities. RESULTS: A total of 25 individuals with PWS between 10 and 41 years old (15 men and 10 women) were included. We identified the presence of graphic indicators corresponding to the behavioral phenotype of individuals with PWS, such as anxiety, stubbornness, emotional lability, difficulty in achieving adequate externalization and identification of emotions, impulsivity, aggressive traits, poor social skills, need for support and interaction, low self-concept, and compulsive behaviors. CONCLUSIONS: In the present study, we demonstrated the usefulness of graphic techniques to elucidate aspects of behavior, emotions, and thoughts that individuals with PWS cannot formulate due to expression and communication difficulties.