The mucopolysaccharidoses are a set of rare, inherited conditions that can have a catastrophic impact on those affected and their families. Because of the rarity of these disorders, little is known regarding the challenges faced by families of those affected and what coping mechanisms are commonly used. Coping is a way to manage demands that occur in one’s environment or within oneself. Medical social workers historically have facilitated this process while providing support to patients who are responding to pressures of their diagnosis and the system. A questionnaire of demographics and qualitative questions, along with the Pediatric Inventory for Parents (PIP) and Brief COPE, was sent by electronic survey to caregivers of children with MPS. The results of Brief COPE showed that problem-focused coping was more frequently used than emotion-focused (p < 0.001) or dysfunctional coping (p < 0.0001). Acceptance was the most frequently used coping strategy (p < 0.05). The results of PIP showed that emotionally distressing events were the most difficult (p < 0.001), while events related to medical care occurred at the highest frequency (p < 0.001). Psychosocial support provided by medical social workers significantly increased acceptance of caregivers (p = 0.04). Guidance on what to expect provided by any member of the care team increased denial (p = 0.02) and the difficulty of emotional distress (p = 0.04). This study identified commonly used coping strategies and measured stress among caregivers of children with MPS, as well as access to and use of psychosocial support services. Results highlight the urgency to improve the coverage and quality of psychosocial support and other support services.
|Original language||English (US)|
|Title of host publication||JIMD Reports|
|Number of pages||9|
|State||Published - 2018|
Bibliographical noteFunding Information:
Acknowledgments Dr. Li Ou is a fellow of the Lysosomal Disease Network (U54NS065768). The Lysosomal Disease Network is a part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR) and NCATS. This consortium is funded through collaboration between NCATS, the National Institute of Neurological Disorders and Stroke (NINDS), and the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The authors would like to thank Jennifer Werner for building the questionnaire within Survey Monkey.
This study was approved by the University of Minnesota Institutional Review Board (IRB, study #1702S0831) and supported by the National MPS Society.
- Parental stress
- Psychosocial support
- Rare disease