Motor neurone disease (MND) can cause weakness in the chest muscles involved in breathing. This leads to shortness of breath and symptoms including disturbed sleep and headaches. Ventilation support allows a person to breathe more efficiently and can also extend survival.
The MND Association has funded research into respiratory management and ventilation support for people living with MND.
A study looking at withdrawing ventilation support at the request of a patient with MND has recently been published in the journal BMJ Supportive and Palliative Care. It was led by Professor Christina Faull, from LOROS – the Leicestershire and Rutland Hospice – in conjunction with the University Hospital of Leicester, and has been part-funded by the Association.
Healthcare studies are an important part of medical research. Their purpose is to try and solve issues surrounding treatment and management, to improve the delivery of care to patients, and improve support for their families. This type of research also helps provide evidence that a treatment is helpful and beneficial for the clinical commissioners, who, in turn, provide funding for the treatment and support.
The main treatment to help people living with MND breathe is non-invasive ventilation (NIV), though it is not suitable for everyone.
NIV involves a machine that supports breathing by increasing the amount of air that can be breathed in. This is done through a mask over the nose (or nose and mouth).
NIV is often first used during the night, in response to difficulties in sleeping associated with breathing. However, as MND progresses, patients can become more reliant on NIV and may also use it during the daytime.
Patients may also receive ventilation support via invasive ventilation tracheostomy – this is where a tube is inserted into the windpipe through the neck, which is attached to a machine that can help with breathing.
More information on ventilation support can be found in our Care Information Sheet 8B.
Withdrawal of ventilation
Someone currently using ventilation support can request to withdraw it even though life may be shortened once this treatment is removed. There may come a time when a person with MND feels that breathing support is no longer helping or it has become a burden.
People who have MND and are receiving ventilation support should know that withdrawal is an option available to them. When discussing this request with their doctor they should get the help and support they need to make their decision.
Withdrawing ventilation support has been a difficult topic for both health-care professionals and people with MND to discuss, over fears that withdrawing treatment may be seen as assisting death. It is important to stress that withdrawing ventilation support is not legally classified as assisted dying.
Interviews with 24 doctors with a range of specialities shared their experiences of withdrawing ventilation support at the request of a patient with MND.
The interviews revealed that doctors had many concerns about withdrawing ventilation support from their patients. These were divided into concerns about their role as a doctor and duty of care, about why their patient was asking for treatment to be stopped, and, if they did so, whether this would be classified as assisting the patient to die and therefore breaking the law.
Doctors had also experienced difficulties in talking about this topic with their colleagues, with patients with MND and with families, especially if the person with MND had difficulties with communicating their wishes.
Professor Faull, lead researcher on this study said “Many doctors had experienced negative reactions from other healthcare professionals when these colleagues were unclear of the distinction between palliation of symptoms, withdrawal if treatment and assisted death.
“The research we carried out found that guidance is needed for professionals who support a patient with MND who wishes to withdraw from ventilation. Open discussion of the ethical challenges is needed as well as education and support for professionals.”
Professor Faull is currently involved in developing guidance for healthcare professionals on the withdrawal of assisted ventilation at the request of a patient with MND. This is in conjunction with the Association for Palliative Medicine, with support from the MND Association and is expected to be published in autumn 2015.
Research paper: Phelps, K et al. BMJ Supportive and Palliative Care Published Online First, Open Access (11 Sept 2015) doi: 10.1136/bmjspcare-2014-000826
Care information sheets 8A Support for breathing problems, 8B Ventilation for motor neurone disease, and 8C NICE guidelines for non-invasive ventilation (NIV)
Association for Palliative Medicine Position Statement: Withdrawal of ventilatory support at the request of an adult patient with neurological or neuro-muscular disease.
Information sheet for professionals P6: Evaluation and management of respiratory symptoms in motor neurone disease.
This blog has also been posted on the MND Association’s research blog.