The official definition of ‘rare disability’
In its first two articles, the arrêté JORF n° 186 du 2 août 2000 defines rare disability:
“Rare disability corresponds to a rare configuration of deficiencies or associated disorders, often including an intellectual deficiency, whose incidence rate cannot be higher than one case per 10,000 inhabitants. Handling rare disability requires the implementation of particular protocols involving more than the simple addition of techniques and means employed to counterbalance each of the impairments considered individually.”
According to the first article, persons considered as having a rare disability are those presenting impairments which fall under one of the following categories:
- The combination of a serious auditory impairment with a serious visual impairment
- The combination of a serious visual impairment with one or many other impairments
- The combination of a serious auditory impairment with one or many other impairments
- Severe dysphasia, whether related or not to another impairment
- The combination of one or many serious impairments and a serious, chronic or progressive health condition, such as:
- A mitochondrial condition
- A metabolic condition
- A progressive nervous disease
- Severe epilepsy
The current definition
Even before this definition from the arrêté JORF n° 186 du 2 août 2000, ‘rare disability’ had appeared as a notion during fieldwork in the 1990s, particularly as a means of referring to cases not fully corresponding to the definitions of multiple disability, concomitant disability, or indeed, in situation of high dependency.
Nowadays, and following a gradual legal process, a number of key texts have come to form definitions of these situations, notably the Code de l’action sociale et des familles (Social action and family code) which reuses the categories defined by the aforementioned decree. All these key texts highlight the combination of three types of rareness:
- the rarity of the demographic: a prevalence of 1 case per 10,000 people – 3,000 children and adults monitored by the Centre de ressources handicaps rares
- the rareness of the combinations of impairments: the complexity of consequences on the activities day-to-day life and on participation in social activities
- the rareness and complexity of technical support arrangements: a number of areas of expertise are required during diagnosis, functional assessment and the elaboration of adapted support projects for these people
Complex support mechanisms throughout the course of a life project
Beyond the definition criteria, it’s the extent of their consequences on the activities of day-to-day life and on participation in social activities which is a source of concern for people with rare disabilities and their relations, and which raises, for professionals, the question of challenges regarding the medico-social support mechanism to be used.
Moreover, since each case is unique in expression, care-giving methods require a systematic coordination of very specialised and personalised interventions, both in human and technical terms, to respond to the specific needs of each care recipient over the course of their life story.