Objective:

The objective of this study was to develop and refine a survey for individuals with amyotrophic lateral sclerosis and their care partners that assesses for barriers and facilitators to continuing non-invasive ventilation.

Background:

Amyotrophic lateral sclerosis is a rapidly progressive motor neuron disease that is typically fatal within 2-5 years, most commonly due to respiratory failure. Non-invasive ventilation can prolong survival and improve quality of life by treating respiratory insufficiency, although tolerability can interfere with adherence. There is a lack of research evaluating these facilitators and barriers to tolerance, particularly in a systematic way.

Design/Methods:

A questionnaire, based upon qualitative findings in a COM-B model framework, was pre-tested among survey experts, neuromuscular and sleep specialists, and individuals with amyotrophic lateral sclerosis, including their care-partners. Questionnaire development and validation included item development, revision based upon pre-testing, and assessment of face validity.

Results:

There were ten rounds of pre-testing iterations to revise the questionnaire to its final form, an 84-item questionnaire. These included language clarification in question stems and answer selections, avoidance of double-barreled questions, simplification of terminology, more specific terminology in branching questions when referencing question stems, acronym clarification, formatting of branching logic questions, re-ordering of items for clarity, optimized answer choice selection, as well as additional questions to better capture the experience of non-invasive ventilation. Face validity was reached. The final questionnaire includes sections that mirror the COM-B model: physical capability, psychological capability, physical opportunity, social opportunity, reflective motivation, and autonomic motivation.

Conclusions:

The development and validation of a questionnaire to evaluate the barriers and facilitators of continuing non-invasive ventilation in individuals with amyotrophic lateral sclerosis will allow for better assessment of risk factors and modifiable barriers to target, allowing increased tolerability and adherence, leading to improved survival and quality of life in these individuals.