La présente recherche a été menée à la demande d’une association de parents (Vaincre les maladies lysosomales [VML]) dont les enfants polyhandicapés sont atteints de maladies génétiques rares. Plusieurs parents évoquaient alors des épisodes de « dépression » chez leurs enfants conduisant parfois à la prescription d’antidépresseurs. Comme la question de la souffrance psychique chez les enfants polyhandicapés a été peu étudiée, l’objectif de cette étude était de constituer un premier état des lieux.

Au moyen d’entretiens semi-directifs, nous avons interrogé les savoirs et savoir-faire de quatre familles et quatre professionnels qui accompagnaient treize enfants polyhandicapés atteints de maladies lysosomales.

Plusieurs facteurs (principalement : épilepsie, douleur, solitude et pertes des compétences), signes (passifs et actifs) et modes de souffrance (angoisse, peur, déprime, frustration, colère) ont été identifiés ainsi que plusieurs types de réponses (réconfort, traitements médicamenteux, socialisation).

Le protocole de recherche a présenté deux limites : d’une part, l’intrication complexe entre la souffrance psychique et d’autres troubles somatiques tels que la douleur et, d’autre part, le recours aux seules interprétations de l’entourage pour accéder au vécu subjectif des enfants.

Bien que le faible effectif de familles rencontrées ne permette pas de généraliser les résultats obtenus, ces derniers conduisent néanmoins à saisir plus finement comment les proches repèrent et donnent sens à la souffrance psychique des enfants polyhandicapés ; ils invitent également à développer des méthodologies permettant à l’enfant d’exprimer ce qu’il vit et ressent.

In this paper, we present the results of research conducted on the psychological distress of lysosomal-disease-affected children. Lysosomal diseases are rare genetic diseases most often leading to severe disabilities, both psychological and physiological. As frequently reported by their relatives, affected children experience nervous breakdowns, which are sometimes treated with antidepressant prescriptions. However, mental impairment as well physical disabilities can prevent children from making their pain noticed and identified by their relatives. This raises a new research question: when disabilities are severe, how should the psychological distress of affected children be identified?

Recent studies on the care of children with multiple disabilities (San Salavadour 2000; Scelles 2003; Camelio 2006; Pautrel, 2009) have used the children's family and caregivers to access their feelings, considered to be translators of children's feelings because they understand their nonverbal language (Camelio, 2006). Using this methodology, four parents from the French not-for-profit association called “VML” (Vaincre les maladies lysosomales) and four professionals were involved in semi-structured interviews. The goal of these interviews was to identify signs of possible psychological suffering, the context in which those signs were expressed, the meaning and the value attributed to it by the family and caregivers, and the reaction as well as an evaluation of that reaction. Thirteen children were involved, 12 of whom were described as having shown signs of psychological distress. Six lysosomal diseases were represented.

Two types of signs were reported: active signs (e.g., agitation, screaming, crying) and passive signs (e.g., no communication, withdrawal, lack of facial expression). Most of the time, passive signs were interpreted by the family and caregivers as evidence of deep psychological distress. The meanings of both types of sign were the following: fear, anxiety, distress, sadness, depression, stress, anger, and frustration. The family and caregivers responded by socializing with the child, providing him with comfort, assisting him in meeting his basic needs, administering medication, etc. Loneliness, disease-progression-related loss of abilities, physical pain, and epilepsy were the main factors of psychological distress. Three children were prescribed an antidepressant, two with an anticonvulsive effect.

The results of this research depend on the difficulty making a distinction between physical and psychological pain and the interpretation of the child's relatives remains an imperfect translation of what the child feels. Further research to overcome these shortcomings is currently under consideration. Moreover, quantitative analysis is needed to make this research more robust.