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J Pediatr Genet 2016; 05(03): 141-149
DOI: 10.1055/s-0036-1584357
Original Article
Georg Thieme Verlag KG Stuttgart · New York

The Psychosocial Impact of Fabry Disease on Pediatric Patients

Nicolle Bugescu
1   Department of Clinical Psychology, Palo Alto University, Palo Alto, California, United States
,
Paige E. Naylor
1   Department of Clinical Psychology, Palo Alto University, Palo Alto, California, United States
,
Kyr Hudson
1   Department of Clinical Psychology, Palo Alto University, Palo Alto, California, United States
,
Christa D. Aoki
1   Department of Clinical Psychology, Palo Alto University, Palo Alto, California, United States
,
Matthew J. Cordova
1   Department of Clinical Psychology, Palo Alto University, Palo Alto, California, United States
,
Wendy Packman
1   Department of Clinical Psychology, Palo Alto University, Palo Alto, California, United States
› Author Affiliations
Further Information

Publication History

12 August 2015

25 April 2016

Publication Date:
13 June 2016 (online)

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Abstract

Fabry disease (FD) is a multisystemic disease that has previously been reported to result in poorer quality of life and psychosocial functioning in impacted adults. However, prior to the current study, limited data were available on the impact of FD in children and adolescents. Therefore, the present study examined the differences of quality of life, psychosocial functioning, and depression in children with FD as compared with a healthy sample. Results indicated that children with FD were experiencing poorer quality of life than their healthy counterparts. Notably, results consistently identified adolescents with FD as more heavily impacted than younger children, although not to the same degree as adults with FD as reported in previous studies. Therefore, adolescence may be a critical point in the development of individuals with FD during which effective multidisciplinary interventions could be utilized to prevent poor quality of life and psychosocial functioning in adulthood.

Keywords

Fabry disease - pediatric - psychosocial functioning - inborn error of metabolism
 

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