[Self-assessed use and provision of assistive devices in individuals with hereditary ataxia at the interface between development in childhood and adolescence and symptom progression].

PURPOSE: Assistive devices are essential in neuropediatrics, but the database on the self-assessed use by and supply of medical aids to those affected with hereditary ataxia is small. The present work aims to examine the individual perspective of those affected by the use and supply of aids and to discuss them in the field of tension between physiological and psychosocial development and symptom progression.

METHODS

The research project represents a cooperation with the German Heredo-Ataxia-Society following the "patients as partners in research" approach. The data was collected via online questionnaires and focus group surveys and analyzed quantitatively and qualitatively using a mixed methodological approach.

RESULTS

Based on the present sample of affected adolescents and adults (n=47 online questionnaires, n=6 focus group surveys), a fundamentally positive assessment of the use of medical aids was evident. In contrast, for the individual evaluation of access and supply of assistive devices, four main problems were identified, some of which can be extrapolated to other diagnoses but amplified in children and adolescents due to the low prevalence of hereditary ataxia. Especially in these age groups, the lengthy process leading up to the diagnosis and the associated gap in the indication for assistive devices poses a serious problem. Moreover, there is lack of evidence for justifying prescription of age-group-specific assistive devices.

CONCLUSION

In addition to the necessity of improvement in the care process, there are significant gaps in the knowledge about an association between self-confidence and coping with the disease, on the one hand, and the acceptance of aids, on the other, in children and adolescents with rare diseases characterized by symptom progression. In addition, there is an urgent need to improve the evidence base for specific tools, especially their importance for promoting independence according to age and developmental phase.