Withdrawal of ventilation support at the request of a patient: the ethical, moral and legal issues

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.

Ventilation 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.

Study findings

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.

Prof Christina Faull, LOROS

Prof Christina Faull, LOROS

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.

More information:

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)

End of Life Guide

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.

Allied Professionals Forum – abstract deadline fast approaching!

Alliance Logo artMy name is Rachel Patterson, and I am the General Manager of the International Alliance of ALS/MND Associations.

I’m writing this ReCCoB post to make you all aware that the deadline for abstract submissions for the Allied Professionals Forum is this Monday (15 June 2015)! Continue reading

ENCALS 2015 report on TDP: RNA metabolism and disease

ReCCoB reporter, Dr Jakub Scaber

ReCCoB reporter, Dr Jakub Scaber

The fifth session of the ENCALS meeting focussed on the role of TDP-43 in ALS.

The first talk during this session was given by myself (Jakub Scaber, MRC/MND Association Lady Edith Wolfson Fellow, University of Oxford). I was given the opportunity to present neuropathological data from the Oxford Brain Bank.

Our study reaffirmed previous findings of the central role of TDP-43 pathology in C9orf72 cases as a common pathway between FTD and ALS cases. We were able to show that TDP-43 pathology at post-mortem correlated very well with the disease phenotype during life, more so than GA dipeptides and sense foci, which are direct pathological consequences of the hexanucleotide repeat. There was some increased GA dipeptide burden in disease-relevant areas of patient brains who had FTD during their lifetime, and sense foci were ubiquitously present in all cells. How the C9orf72 pathology and the later TDP-43 pathology are linked remains a key question in the investigation of this mutation. Continue reading

The wait for ProGas is over… at least for now.

Friday saw the highly anticipated publication of results from the ProGas study, which was part-funded by the MND Association.

I manage the publication of evidence-based information resources for health and social care professionals at the MND Association. I can’t recall a time when I’ve felt so much pressure to delay publishing a resource due to emerging findings. Continue reading

ENCALS Thursday 21st May 2015 – Oral session 1 – Genes and Genomics

The ENCALS meeting this year has been held at Trinity College in Dublin. This suggestive place hosted more than 250 participants and several international guests as the eminent researcher Prof. John E. Landers,Professor of Neurology at University of Massachusetts, who opened the “Genes and Genomics” session with a talk entitled “Using Next-Generation Sequencing to Dissect Amyotrophic lateral sclerosis (ALS) Pathogenesis”. Prof. Landers pointed out how the investigation of rare variants are important in the discovery of pathways underlying the neurodegenerative mechanism of rare diseases and how exome sequencing became an important strategy for identifying human disease genes by spotting rare variants.

Continue reading

ENCALS 2015 Meeting, 21 – 23 May, Dublin: Highlights and insights of cognition session

This year Dublin was not only home to the annual ENCALS meeting 2015, but also to a referendum on gay marriage. One week after the meeting the buzz of the sessions is still with me.  This was an excellent event and the varied scientific sessions and posters helped to build my knowledge and understanding of research into the causes and consequences of ALS/MND. Time spent in Dublin also gave me many exciting opportunities to network with colleagues across Europe. Here is an outline of the ENCALS 2015 cognition session.

The session was chaired by two inspiring ladies and world’s leading MND researchers, Prof Sharon Abrahams from University of Edinburgh and Dr Marwa Elamin from Trinity College Dublin. Continue reading

ENCALS Friday 22nd May 2015 – Oral Session 3 – C9orf72 and novel therapeutics

Leonard Petruccelli commenced the session discussing mechanisms of toxicity and therapeutic approaches for C9FTD/ALS, beginning by highlighting the enormous advancement in the understanding of the genetics and neuropathology of FTD and ALS since 2006. The three proposed mechanism of C9orf72 mutations were presented; 1) a gain of toxicity as a result of RNA foci in cell nucleus, 2) haploinsufficiency and 3) dipeptide repeat (DPR) protein toxicity. Around 20 diseases contain RNA repeat expansions however repeat associated non-ATG translation (RANT) of the C9orf72 hexanucleotide repeat expansion was seen to be specific to the brain with anti-C9 RANT immunoreactivity specific to C9FTD/ALS patients. Continue reading