This information is for health and social care professionals. You may also want to see our information for people living with or affected by MND on:
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 and ventilatory failure is the most common cause of death.
Respiratory function significantly predicts both survival and quality of life in people with MND. Monitoring respiratory function therefore helps the health professional and the person with MND to reach decisions about management in a timely way.
Effective management of the respiratory symptoms of MND requires some understanding of the person’s wishes for care, including end of life care.
Initial and regular opportunities should be given to the person with MND (and, where appropriate, their carer/family) to discuss, in a sensitive way, decisions for end of life.
This should take place whether or not someone decides to have assisted ventilation.
If someone is considering assisted ventilation, discussions need to take place in relation to continuing or withdrawing this intervention. The progressive nature of MND means the person will continue to deteriorate physically, despite ventilatory support. The treatment may be started to help with night-time symptoms but this can, over time, lead to 24-hour dependence. The person with MND may have strong views on this.
Discussions should include:
- planning end-of-life care
- considering whether to record an Advance Decision to Refuse Treatment (ADRT) in certain circumstances in the future (see below)
- considering what to do if NIV fails because of either an acute, but potentially reversible, deterioration in health, or irreversible disease progression
- strategies to withdraw assisted ventilation if the person with MND wishes.
People may find early discussions about these issues difficult. It is the health professional’s role to show willingness to discuss these topics, provide guidance and make referrals to the appropriate services.
Key decisions reached with the person with MND (and, where appropriate, their carer/family) should be relayed to all other relevant health and social care professionals.
Our End of life guide may support you in these discussions.
Also, see our information on withdrawal of assisted ventilation.
Monitoring respiratory function will help to ensure that placement of a gastrostomy tube, if needed, is performed in a timely manner.
Respiratory evaluation is necessary prior to any surgical procedure requiring sedation, such as percutaneous endoscopic gastrostomy (PEG). Earlier placement is recommended while the individual is well enough and their respiratory function is adequate to undergo this procedure.
Current research is looking into optimal timing of all types of gastrostomy, including radiologically inserted gastrostomy (RIG) and per-oral image-guided gastrostomy (PIG).
Some people with MND will experience mild to severe cognitive change, and a proportion will have frontotemporal dementia. For these people, and others with different factors that limit their cognitive function or mental capacity, careful consideration needs to be given to the appropriate management of any respiratory impairment.
The individual’s ability to weigh up the various treatment options and give informed consent will be important.
An ADRT allows people to make decisions to refuse treatments or have treatment withdrawn, including those treatments that sustain life.
When appropriately recorded as part of advance care planning, a decision by someone with MND to discontinue the use of ventilation is legally binding, and these wishes should be followed.