Psychosocial Functioning in Youth with Barth Syndrome.
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.
Storch
Eric A
EA
Departments of Pediatrics and Psychiatry, University of South Florida, St. Petersburg, FL.
Keeley
Mary
M
Merlo
Lisa J
LJ
St Amant
Jay B
JB
Jacob
Marni
M
Storch
Jill F
JF
Spencer
Carolyn
C
Byrne
Barry J
BJ
Dev Psychopathol. 1997 Winter;9(1):59-73
9089124
J Behav Med. 2004 Jun;27(3):297-318
15259457
Health Qual Life Outcomes. 2007 Jul 16;5:43
17634123
Diabetes Care. 2003 Mar;26(3):631-7
12610013
Child Dev. 1985 Apr;56(2):448-61
3987418
J Pediatr Psychol. 2001 Apr-May;26(3):155-62
11259517
J Dev Behav Pediatr. 2007 Feb;28(1):22-30
17353728
Health Psychol. 1995 Jul;14(4):333-40
7556037
Psychol Med. 1983 Aug;13(3):595-605
6622612
Child Care Health Dev. 2006 Jan;32(1):19-31
16398788
Pediatrics. 2005 Jun;115(6):e626-36
15930188
Med Care. 1999 Feb;37(2):126-39
10024117
Pediatr Nurs. 2004 Sep-Oct;30(5):373-80, 401
15587530
J Intellect Disabil Res. 2007 Feb;51(Pt 2):109-24
17217475
J Child Psychol Psychiatry. 2007 Aug;48(8):785-92
17683450
J Consult Clin Psychol. 1985 Aug;53(4):500-5
4031205
J Am Acad Child Adolesc Psychiatry. 1992 Nov;31(6):1112-9
1429414
JAMA. 2005 Jan 5;293(1):70-6
15632338
Ambul Pediatr. 2003 Nov-Dec;3(6):329-41
14616041
J Am Acad Child Adolesc Psychiatry. 2004 Feb;43(2):221-30
14726730
J Adv Nurs. 2006 Feb;53(4):392-402
16448482