Disease related stress in children with Neurofibromatosis type 1 in Germany from their own and their parents´ perspective

Abstract

Introduction and Objectives: Neurofibromatosis type 1 (NF1) is a rare disease characterized by benign tumors of the peripheral nerves and typical skin disfigurements. There is evidence that children with NF1 experience psychosocial problems (Noll 2007). The aim of this study: obtaining data about NF1 children´s stress from their own and their parents´ perspective. Materials and Methods: In a cross sectional study, a total of 109 children with NF1 and one parent of each child separately filled in a questionnaire on the child´s clinical status, quality of life (QoL), disease related stress, body image, support options and diverse health care parameters. The parents´ statements were compared to the children´s views by paired t-tests. Results: The children were aged between 10 and 17 (44.3% female). 27.5% had at least one relative with NF1. The children´s stress was rated higher by the parents then by the children themselves (3.1±2.8 vs. 3.9±3.1, p<0.01, scale from 0=no stress to 10=extremely stressed). The parents evaluated their own stress considerably higher than their child´s (5.1±3.0 vs. 3.1±2.8, p<0.001). Additionally those parents and children with other affected family members considered themselves more strained and the children rated their quality of life lower (67.9±20.2 vs. 78.4±15.4, p<0.05, scale from 0=minimum to 100=maximum QoL). Conclusions: Children with NF1 seem to have a more optimistic view on their burden then their parents. The finding that parents rate their own stress levels even higher than their child´s indicates a broader family concernment. It seems that those with affected family members do not find it easier to cope with NF1. Therefore psychosocial support of the family should be an essential part of health care of children with NF1. Literatur: Noll RB, Reiter-Purtill J, Moore BD, Schorry EK, Lovell AM, Vannatta K, Gerhardt CA. (2007). Social, emotional, and behavioral functioning of children with NF1. Am J Med Genet Part A 143A:2261–2273.