The remote self-assessment of vital capacity in patients with MND

Patients with amyotrophic lateral sclerosis (ALS)/ motor neuron disease (MND)
are burdened by frequent clinical visits, due to the complexity and severity of
their disease. During these clinical visits respiratory function is assessed
with pulmonary function tests (e.g. vital capacity, maximal inspiratory
pressure) to determine the need for non-invasive ventilation (NIV). NIV
facilitates ventilation by providing pressured air during inhalation. In
current healthcare practice, patients with ALS/MND who suffer from respiratory
impairment get referred to a pulmonary specialist by their rehabilitation
physician or physical therapist. This often happens when patients* respiratory
function has already decreased severely, to the extent that these patients need
to initiate NIV within a matter of a few weeks. This has a major impact on the
life of patients and their caregivers.
The late detection of reduced respiratory function is partly due to a lack of
monitoring in between clinic visits. It would be valuable to have patients
regularly self-assess their respiratory function from home in between clinic
visits, as this will facilitate the timely detection of reduced respiratory
function and possibly improve the referral to the pulmonary specialist. In
addition to that, it will reduce the need for patients to visit the clinic for
respiratory assessments, which reduces travel and clinical burden.

Primary
- To determine the validity of home-based self-monitoring of VC in patients
with MND.

Secondary
- To determine the reliability of home-based self-monitoring of VC in patients
with MND.
- To evaluate the feasibility of 4-weekly home-based self-monitoring of VC in
patients with MND.

Observational study. Patients will independently perform a pulmonary function
test at home, without intervention from the researchers.

During a home visit, the patient will perform a lung function test under the
supervision of a researcher and then independently perform a lung function
test. During the follow-up period, patients will perform a lung function test
at home at 4 different times, namely at 1 day, 4 weeks, 8 weeks and 12 weeks
after the home visit. Lung function tests will be performed 3 times per
session. This is a non-invasive and low-burden measurement that will not take
more than 5 minutes per session. Patients will also complete two questionnaires
at baseline and at follow-up 2, 3 and 4, and fill in an extra (3rd)
questionnaire at the last follow-up (12 weeks). All questionnaires take no more
than 5 minutes to complete.

The burden is therefore very low and there are no risks involved. Besides that,
the patients are used to perform a lung function test and filling out
questionnaires for healthcare purposes.