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Motor neurone disease (MND) is a progressive and terminal disease that attacks the motor neurones, or nerves, in the brain and spinal cord. Respiratory muscle weakness occurs eventually in everyone with MND.
Effective management of respiratory function is likely to have a positive impact on quality of life for the person with MND.
This is a method of providing ventilator support to a person with MND using a close-fitting mask that may cover the nose and/or mouth.
The ventilator detects when the person with MND tries to take a breath and delivers an extra flow of air to increase the volume inhaled. The machine can also be set on different modes, for example, to give extra breaths.
A trial of NIV can be offered if the person’s symptoms and the results of respiratory function tests indicate they are likely to benefit from the treatment. It is important to discuss both the benefits and limitations of the intervention with the person with MND (and, where appropriate, their carer/family).
For someone who has severe bulbar impairment, NIV may have limited benefits. People with cognitive problems may benefit, if these symptoms are the result of respiratory impairment.
Before starting NIV, the multidisciplinary team should prepare a comprehensive care plan, after discussion with the person with MND (and, where appropriate, their carer/family). This should cover the long-term support offered, with arrangements in place for device maintenance and 24-hour emergency clinical and technical support.
It will be necessary to provide training for the person with MND and any carers, and advice around secretion management and cough management (if needed).
Experimentation to find the most appropriate interface (mask) is often needed based on the person’s needs and lifestyle factors. When starting NIV, ideally there should be gradual acclimatisation during the day when the person is awake, regular treatment at night will then usually begin. The time of using the machine will build up as necessary.
Very occasionally, a person with MND will be fully ventilated via a tracheostomy due to an acute episode of respiratory insufficiency. This can happen as an initial presentation of the disease with the diagnosis of MND made in intensive care. There can be difficulties weaning the person from the ventilator long-term.
Some units will be reluctant to offer a tracheostomy with full ventilation electively. This is because of the impact on the family and the need to provide specialist care in the future, which may not be possible to deliver at home.
Great thought must be put into supported discussions with the person with MND and, where appropriate, their carer/family.