title: Psychosocial Functioning in Youth with Barth Syndrome.
abstract: This pilot study assessed the quality of life and psychosocial functioning of pediatric patients with Barth Syndrome. Thirty-four boys with Barth Syndrome and 22 healthy male controls were administered a measure of verbal ability and completed measures of quality of life, loneliness, perceived peer support, and sibling relationship quality. Parents completed measures of parental distress, parenting stress, child academic functioning, child adaptive behavior, and child emotional and behavioral functioning. Quality of life ratings were consistently lower in youth with Barth Syndrome relative to both healthy controls and a previously reported sample of youth with cardiac disease. Compared to healthy controls, children with Barth Syndrome were rated as having more internalizing and externalizing symptoms, social problems, loneliness, and lower independent functioning. Parents of boys with Barth Syndrome reported greater distress and parenting stress relative to healthy controls. In addition, parents reported a significant need for academic accommodations, given their son's illness and associated impairments. Boys with Barth Syndrome and their parents appear to be affected by the presence of the illness in numerous ways. Results suggest the need for interventions aimed at helping children and families cope with illness-related stressors to enhance quality of life and overall functioning.
authors.affiliation: Departments of Pediatrics and Psychiatry, University of South Florida, St. Petersburg, FL.
authors.forename: Eric A
authors.initials: EA
authors.lastname: Storch
authors.forename: Mary
authors.initials: M
authors.lastname: Keeley
authors.forename: Lisa J
authors.initials: LJ
authors.lastname: Merlo
authors.forename: Jay B
authors.initials: JB
authors.lastname: St Amant
authors.forename: Marni
authors.initials: M
authors.lastname: Jacob
authors.forename: Jill F
authors.initials: JF
authors.lastname: Storch
authors.forename: Carolyn
authors.initials: C
authors.lastname: Spencer
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Byrne
references.citation: Dev Psychopathol. 1997 Winter;9(1):59-73
references.pmid: 9089124
references.citation: J Behav Med. 2004 Jun;27(3):297-318
references.pmid: 15259457
references.citation: Health Qual Life Outcomes. 2007 Jul 16;5:43
references.pmid: 17634123
references.citation: Diabetes Care. 2003 Mar;26(3):631-7
references.pmid: 12610013
references.citation: Child Dev. 1985 Apr;56(2):448-61
references.pmid: 3987418
references.citation: J Pediatr Psychol. 2001 Apr-May;26(3):155-62
references.pmid: 11259517
references.citation: J Dev Behav Pediatr. 2007 Feb;28(1):22-30
references.pmid: 17353728
references.citation: Health Psychol. 1995 Jul;14(4):333-40
references.pmid: 7556037
references.citation: Psychol Med. 1983 Aug;13(3):595-605
references.pmid: 6622612
references.citation: Child Care Health Dev. 2006 Jan;32(1):19-31
references.pmid: 16398788
references.citation: Pediatrics. 2005 Jun;115(6):e626-36
references.pmid: 15930188
references.citation: Med Care. 1999 Feb;37(2):126-39
references.pmid: 10024117
references.citation: Pediatr Nurs. 2004 Sep-Oct;30(5):373-80, 401
references.pmid: 15587530
references.citation: J Intellect Disabil Res. 2007 Feb;51(Pt 2):109-24
references.pmid: 17217475
references.citation: J Child Psychol Psychiatry. 2007 Aug;48(8):785-92
references.pmid: 17683450
references.citation: J Consult Clin Psychol. 1985 Aug;53(4):500-5
references.pmid: 4031205
references.citation: J Am Acad Child Adolesc Psychiatry. 1992 Nov;31(6):1112-9
references.pmid: 1429414
references.citation: JAMA. 2005 Jan 5;293(1):70-6
references.pmid: 15632338
references.citation: Ambul Pediatr. 2003 Nov-Dec;3(6):329-41
references.pmid: 14616041
references.citation: J Am Acad Child Adolesc Psychiatry. 2004 Feb;43(2):221-30
references.pmid: 14726730
references.citation: J Adv Nurs. 2006 Feb;53(4):392-402
references.pmid: 16448482