Billet publié sur Twitter/X le 04/03/2022 05:03

Résumé

Identifying and reducing distress in non- or minimally verbal children and adults with the most severe disabilities is important and challenging. The most likely causes of the observable signs of distress are physical pain or a strong emotional state. Parents and carers often readily recognize distress because they are attuned to behavioural signatures and change against the backdrop of atypicality. The challenge for parents, carers, and practitioners is to recognize distress, identify causes, and intervene effectively. This is a central theme for the 2022 British Association of Childhood Disability Annual Scientific Meeting (bacdis.org.uk/events/ASM-2022). The importance of identifying both physical and emotional causes of distress is self-evident for quality of life but there are other reasons. Distress can indicate untreated painful physical conditions, such as gastro-oesophageal reflux that can both directly cause self-injury but also increase the likelihood of Barrett's oesophagus in the future. Direct pathways to self-injury are either pain gating or a reflex response to pain. An indirect pathway is an interaction with learned self-injury, with pain increasing motivation for the behaviour. Emotional distress is less well recognized in non- or minimally verbal children and adults with the most severe disabilities.1 There is good evidence that anxiety, for example, is common in this group and can influence behaviours such as self-injury and aggression. Another form of emotional distress is often identified as temper in the published literature, with the term ‘meltdowns’ often preferred by parents. There is increasing evidence that these strong transient emotional states, such as the ‘temper outbursts’ in Prader–Willi syndrome, can result from fundamental differences in autonomic arousal.2 One challenge to thinking of these episodes as a form of distress is value-laden perception. For example, if the trigger for an emotional outburst is a change in routine or delay to a desired outcome, then the person might be described as stubborn or impatient respectively. This can negate recognition of a different lived experience of the triggers, strength, and valence of the emotion. It is very striking that people with Prader–Willi syndrome express significant remorse after emotional outbursts, suggesting the emotional state was both unpleasant and uncontrollable, and hence different in some ways from the experience of typically developing people. Both physical and emotional causes of distress warrant attention. For non- or minimally verbal children and adults with the most severe disabilities, the core of the problem of recognizing and responding to distress is how people experience and communicate distress. For either physical pain or emotional states, the person needs to identify that the pain or state exists, know the labels for different sensations, label the sensation correctly, understand that telling others has merit, and then communicate the right information effectively. For many people this is difficult, if not impossible, and consequently they are partially or wholly reliant on others to identify their distress and its nature. One significant problem that then emerges is diagnostic overshadowing, best exemplified in this email from a parent: ‘… (There was) a complete change in personality, ripping lumps of hair out … We then (went) to our community consultant who found that acid reflux had burned her severely … The hospital telling us that she had nothing wrong and it was behavioural or neurological …’. The problem of misattribution of the cause of distress and hence behaviour, has been exacerbated more generally by the use of imprecise terms such as ‘challenging behaviour’ with an inherent assumption that these behaviours are learned.3 When this term combines behaviours under one blanket term, this can conflate causes and outcomes or eclipse third variables that underpin behaviour. In the example above, both the change in personality and the self-injury would be subsumed under the single term ‘challenging behaviour’. Whereas identifying the combination of different behaviours alongside recognition of change might indicate pain as the common cause of each behaviour. Researchers have left the problem of identifying distress and uncovering the causes in the ‘too hard’ pile for too long. This is unsurprising given the critical problem of validation of internal states. The criterion standard for knowing if someone is in pain remains asking them. Fortunately, cracking the problem might not be as difficult as we imagine. Parents and carers can identify behavioural signatures that in combination with assessment of other parameters of behaviour (e.g. temporal patterns) give a starting point for bespoke definitions of physical or emotional distress. The validation might lie in an empirical approach of evaluating the relationship between behavioural signatures and the outcome of a systematic clinical assessment of possible causes with intervention.4 To move ahead in this area there needs to be strong and meaningful collaboration between researchers, parents and carers, and practitioners facilitated by clearly targeted pump-priming, high-risk/high-gain research funding. The initial projects do not have to be large scale, as proof of concept is the immediate goal. Once this is achieved the required interventions are ready and waiting.