Psychosocial Functioning, Health-Related Quality of Life, and Family Impact in Galactosemia Patients

Vierling, Sabrina; Packman, Wendy; Bugescu, Nicolle; Schmidt, Michael A.; Kountz-Edwards, Samantha

doi:10.1037/e660272012-001

Cited by 3 publications

(2 citation statements)

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“…Prader-Willi syndrome (PWS) this heightened level of distress may lead to poorer family communication and more family conflicts. 14,18 When the active and remission groups were observed separately, the results demonstrated signs of resilience. While families with an active disease presentation showed increased worry and family relationship problems, a family's functioning improves substantially once the disease reaches a status of remission.…”

Section: Discussionmentioning

confidence: 97%

“…PedsQL-Family Impact Module. The Family Impact Module 18 was completed by parents in order to evaluate the degree of impact that a child's health condition has on a family. The 36-item, self-report measure includes six domain scales, covering physical functioning, emotional functioning, social functioning, cognitive functioning, communication, and worry.…”

Section: Methodsmentioning

confidence: 99%

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The family impact of caring for a child with juvenile dermatomyositis

et al. 2017

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Background Juvenile dermatomyositis (JDM), a rare autoimmune disease, accounts for more than 80% of idiopathic inflammatory myopathy childhood cases, making it the most common idiopathic inflammatory myopathy among children. The average age of onset is approximately 7 years and commonly leads a chronic course. Symptoms of JDM include cutaneous features (Gottron's rash, heliotrope rash, or nail fold capillary changes), musculoskeletal features, calcinosis and lipodystrophy (a symmetrical deficit of subcutaneous fatty tissue), and acanthosis (thickening of the skin). Despite improvement in treatment regimens and the lowering of mortality rates, some children still lose their lives to JDM. This study assessed the effects of caring for a child diagnosed with JDM on the family system. Methods Participants included 36 mothers and 3 fathers of a child diagnosed with JDM. Parents were administered self-report measures, which assessed the overall family functioning (PedsQL-Family Impact Module), and the parents' mood and level of distress (profile of mood states). Additionally, parents were administered a semi-structured interview that included background information, psychosocial information, and sources of support. Results and conclusion Families of children with JDM reported difficulties in family functioning, communication problems, and an increased number of conflicts. Parents appeared to be experiencing higher than average levels of worry, worse physical functioning, and family relationships when compared to normative populations. Parents would benefit from psychosocial support due to the many challenges associated with caring for a child with JDM.

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Section: Discussionmentioning

confidence: 97%

Section: Methodsmentioning

confidence: 99%

The family impact of caring for a child with juvenile dermatomyositis

et al. 2017

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Health‐Related Quality of Life in Patients with MPS II

Needham

Packman

Quinn

et al. 2014

Journal of Genetic Counseling

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Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a chronic and progressive X-linked lysosomal disease that mainly affects males. The National MPS Society (2013) reports that MPS II affects 1 in 100,000 to 1 in 150,000 males worldwide. Two distinct forms of the disease are based on age of onset and clinical course: attenuated and severe. MPS II affects many organ systems including the nervous, cardiovascular, gastrointestinal and respiratory systems. Clinical manifestations can include progressive hearing loss, mental impairment, and enlarged liver and spleen. This study focuses on the health-related quality of life of individuals (HRQOL) with MPS II as measured by the parent and self-report versions of the Pediatric Quality of Life Inventory (PedsQL™). Both parents of patients with MPS II as well as patients themselves reported lower scores on all domains of the PedsQL™ (physical, emotional, social and school functioning) indicating that children with MPS II have an overall lower HRQOL when compared to a healthy sample. When compared with patients with other chronic illnesses (cancer, MSUD, galactosemia,), the MPS II sample had significantly lower scores on a number of PedsQL™ scales, suggesting an overall lower HRQOL. No significant relationships were found using scores from parent or self report PedsQL™ measures and length of time on ERT.

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