Parental reports of health-related quality of life in greek children with neurofibromatosis type 1
Article Outline
To the Editor:
We read with interest the article by Krab et al.1 We would like to add our experience on the impact of Neurofibromatosis type 1 (NF1) on the Health-Related Quality of Life (HRQoL) of Greek school-aged children. The parents of 43 nonselected children with NF1 (27 boys and 16 girls), ages 6 to 14 years (mean: 10.1 years, SD: 3 years), completed the Child Health Questionnaire–Parent Form 50 (CHQ–PF50) in a prospective observational study. The parental form of CHQ is the only quality-of-life instrument currently translated in Greek, validated, and cross-culturally adapted for use in Greek children.2 All the children included in the study met the diagnostic criteria of the National Institutes of Health and had been regularly followed from the age of the diagnosis at the Department of Pediatrics of a tertiary care university hospital.
The results of the parental reports of the NF1 group were compared with those of a group of 61 healthy aged-matched peers (Table). The comparison between the two groups showed that almost every aspect of the HRQoL of children with NF1 is, according to their parents, significantly affected by their condition. A significant positive correlation has been found between lower CHQ-PF 50 scores and disease complications, especially optic pathway gliomas (P < .05) and cognitive deficits (P < .01). Furthermore, better family relations and stronger family cohesion positively correlated with better physical and psychological adjustment of the children (P < .01).
Table. CHQ-PF50 health concepts: summary scores of the two groups
| NF1 group (N = 43) | Healthy controls (N = 61) | ||||
|---|---|---|---|---|---|
| mean | SD | mean | SD | P value | |
| Physical functioning (PF) | 94.2 | 16.7 | 94.6 | 7.3 | NS |
| Role/social emotional/behavioral limitations (REB) | 89.1 | 19.9 | 97.7 | 13.7 | < .05 |
| Role/social physical limitations (RP) | 91.1 | 22.8 | 97.0 | 15.2 | < .05 |
| Bodily pain discomfort (BP) | 84.4 | 19.5 | 94.9 | 10.3 | < .01 |
| Behavior (BE) | 71.9 | 17.9 | 84.8 | 12.8 | < .01 |
| Mental health (MH) | 77.7 | 18.0 | 83.1 | 14.1 | < .05 |
| Self-esteem (SE) | 82.8 | 15.2 | 90.7 | 12.7 | < .01 |
| General health perception (GH) | 63.7 | 19.4 | 79.8 | 13.9 | < .01 |
| Emotional impact on parent (PE) | 59.5 | 32.3 | 78.6 | 24.9 | < .01 |
| Impact on parents' personal time (PT) | 79.6 | 26.5 | 94.1 | 10.5 | < .01 |
| Limitations in family activities (FA) | 76.8 | 21.7 | 90.6 | 13.5 | < .01 |
| Family cohesion (FC) | 76.4 | 22.0 | 83.1 | 15.3 | < .05 |
| Physical summary score (PhS) | 50.2 | 9.3 | 54.0 | 5.2 | < .01 |
| Psychosocial summary score (PsS) | 48.4 | 10.6 | 55.2 | 7.1 | < .01 |
Our results are in accordance with the observations already made in adolescents and children1, 3, 4 demonstrating that NF1 adversely affects HRQoL of affected children. Disease-related and family-related variables seem to influence the outcome of HRQoL measures.
References
- . Health-related quality of life in children with neurofibromatosis type 1: contribution of demographic factors, disease-related factors and behavior. J Pediatr. 2009;154:420–425
- Paediatric Rheumatology International Trials Organisation. The Greek version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ). Clin Exp Rheumatol. 2001;19:S76–S80
- . Quality of life and psychological adjustment in children and adolescents with neurofibromatosis type 1. J Pediatr. 2006;149:348–353
- . Parental reports of health-related quality of life in young children with neurofibromatosis type I: influence of condition specific determinants. J Pediatr. 2007;151:182–186
PII: S0022-3476(09)00215-7
doi:10.1016/j.jpeds.2009.02.056
© 2009 Mosby, Inc. All rights reserved.
