The impact of Pelizaeus-Merzbacher disease on the family.
The impact of Pelizaeus-Merzbacher disease on families and caregivers of affected children has not been well-studied. Parents, relatives, and caregivers from 18 families with 20 affected children with Pelizaeus-Merzbacher disease were asked to complete the Children's Health Questionnaire-Parent Form 50, an instrument used to assess health-related quality of life in children and family impact of illness. Mean and standard deviation (S.D.) of subscale scores were calculated and compared with previously published norms. Agreement between mothers and fathers was calculated using Cronbach's test. Mean scale scores in this cohort were lower than previously published norms for the following categories: physical function (25.9 vs 96.1, >2 S.D.); family activity (55.6 vs 89.7, >1 S.D.); and parental time impact (66.2 vs 87.8, >1 S.D.). However, family cohesion (73.3 vs 72.3, <1 S.D.), self-esteem (71.1 vs 79.8, <1 S.D.), behavior (78.9 vs 75.6, <1 S.D.), and mental health scale scores (74.2 vs 78.5, <1 S.D.) were similar to previously published norms. Parental agreement was poor, with 5 of 8 parent pairs (63%) differing in their responses (<0.7) Although impact on caregiver time and limitation of physical function and family activities is high, parents and caregivers in the cohort appear to remain cohesive and view their children's psychosocial health as similar to normal children.
McGuire
Lisa M
LM
Division of Pediatric Neurology, Department of Neurology, Indiana University School of Medicine, and the Roudebush Veterans' Affairs Medical Center Health Services Research and Development Service, Indianapolis, Indiana 46202, USA.
Williams
Linda S
LS
Walsh
Laurence E
LE
DeMyer
William E
WE
Golomb
Meredith R
MR
D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D017028:Caregivers / Q000523:psychology; D002648:Child; D002675:Child, Preschool; D017281:Cost of Illness; D005190:Family / Q000523:psychology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D020371:Pelizaeus-Merzbacher Disease / Q000503:physiopathology* / Q000523:psychology*; D011788:Quality of Life; D011795:Surveys and Questionnaires