1. Respiratory Management in MND

2. Session 1: Welcome, housekeeping and introduction
Fire
Facilities
Refreshments
Programme
Evaluation

3. Objectives
Identify why respiratory function is affected in motor neurone disease.
Recognise symptoms and signs of sleep disordered breathing, respiratory muscle weakness and cough failure.
Gain a basic understanding of the main tests used to assess respiratory function in patients with MND.
Gain an understanding of how non-invasive ventilation helps improves quality, length of life and the patients perspective.
Participate in interactive workshops which consider:
Measurement of peak cough flow
Assisted cough techniques (LVR bags, breath stacking and cough assist machine)
Identify factors to consider when a patient requests withdrawal of ventilation.

4. Introduction
MND is a progressive neurodegenerative disease that attacks the upper and lower motor neurones.
Degeneration of the motor neurones leads to weakness and wasting of muscles, causing increasing loss of mobility in the limbs, and difficulties with speech, swallowing and breathing.
Most cases sporadic, approximately 5-10% familial.
Around 35% experience mild cognitive change.
A further 15% show signs of frontotemporal dementia.

5. Introduction Incidence: 1 - 2 new cases per 100,000 people per year.
Around 3% respiratory presentation.
Male to female ratio is 3:2, although this varies with age and type of MND and evens out in later life.
MND kills a third of people within a year and more than half within two years of diagnosis.

6. MND and the respiratory system
Degeneration of motor neurones supplying respiratory muscles, diaphragm, intercostal and accessory muscles leads to reduced vital capacity, ability to ventilate and cough effectively.
Initially respiratory muscle weakness can lead to:
orthopnoea
fragmented sleep
daytime somnolence
unrefreshing sleep.
Latterly more severe respiratory muscle weakness can lead to:
cough failure
morning headache
dyspnoea.

7. MND and the respiratory system
Quality of life can be severely affected by respiratory muscle weakness.
Death almost always due to respiratory failure as a consequence of
respiratory muscle weakness and/or repeated chest infections.
Optimal respiratory care in MND can improve life
expectancy and quality.
Burkhardt, C. et al 2017.