This decision tool is to help you learn more about your options for assisted ventilation. Assisted ventilation is not suitable for everyone with MND. It is important to discuss breathing and ventilation support with your Respiratory physician and MND health professionals, who can advise you on the options that best suit your needs.
You may not feel ready to read all the information contained in this tool. If so, read the information you wish to know, at the best time for you. If you would like more detailed information, links to other resources are provided in the relevant sections, and in the resources at the end of this tool.
MND is a general term applying to progressive, degenerative disorders affecting the motor neurones. Motor neurones carry signals from the brain to the muscles. In people with MND, the motor neurones deteriorate and can no longer carry these signals. This progressively weakens the respiratory muscles of the chest and diaphragm that control breathing, leading to respiratory failure - the main cause of death in MND. When respiratory muscles become weak, breathing is less efficient, as less oxygen is taken into the lungs and bloodstream, and it becomes harder to expel carbon dioxide from the body. This makes everyday activities, including breathing at night, much more difficult to perform. Sometimes people experiencing weakness of the respiratory muscles use other muscles to help them breathe, such as the neck and shoulder muscles. Read more from MND Australia
Weakened respiratory muscles can result in disrupted sleep, and make activities such as walking, talking, eating, coughing and even resting more difficult. This is known as dyspnoea, while breathlessness that occurs when lying flat is called orthopnoea. Other symptoms of respiratory weakness can include fatigue, daytime sleepiness, morning headaches, poor concentration, confusion and poor appetite. More information about breathing difficulties can be found at MND Australia’s website
Assisted ventilation is the use of equipment to support respiration, or to support life.
Ventilation equipment cannot prevent respiratory muscles from getting weaker, nor
improve the strength of these muscles. Use of the equipment will not cure or halt the
progress of MND. Assisted ventilation can support your body to breathe more efficiently so that your lungs can take in and expel the air they need. A respiratory assessment from your MND health professionals, including a Respiratory Physician and a Respiratory Therapist, is needed before they can advise you on which equipment is most suitable for you.
Non-Invasive Ventilation (NIV) is the preferred and most commonly used form of assisted ventilation in MND care. Use of NIV may temporarily prolong life for several months, and may improve the quality of life of people with MND by supporting them to participate in activities they enjoy.
NIV involves the use of a face mask, or a nasal mask, attached to a machine to support breathing. The face mask covers the nose and mouth to allow the correct pressure for the lungs to breathe in and out. The nasal mask sits over the nose only and a soft strap is used to help keep the lips sealed to prevent air leaking from the mouth. A variation on a nasal mask is the nasal pillow mask which has two soft silicone prongs worn inside the nostrils to deliver air through the nose. The different types of masks are shown in Diagrams 1 to 4
(Source: Westmead Hospital Respiratory and Sleep Disorders Clinic)
This type of ventilation is known as Bi-level Positive Airway pressure (BiPAP), or Variable Positive Airway Pressure (VPAP). NIV is used according to your needs, and as your needs change, the prescription of pressures or the amount of time the device is used each day may also change. This can mean changing from using the device only at night, to using it for short periods during the day, to using NIV continuously.
NIV is not suitable for everyone experiencing breathing difficulties in MND. Some people find they are unable to tolerate wearing any type of mask, or are unable to sleep with the mask on. Other people find that due to changes to the muscles of their mouth and throat caused by MND, NIV is ineffective for them.
Early use of NIV can help people with MND adjust to using the ventilator, and may offer some health and quality of life benefits. The best timing for beginning NIV needs to be discussed with your MND team, who can monitor your breathing. Nevertheless, it is never too early to talk to your MND team about your wishes for NIV, and your plans for your future care. Regular assessment of your respiratory function by your MND team is important. Some tests that you might undergo include: measuring forced vital capacity (FVC) by blowing into a spirometer; measuring your oxygen and carbon dioxide levels using a blood test; and testing respiratory muscle strength including sniff nasal inspiratory pressure (SNIP), that measures the strength of your inward breath when sniffing through your nose.More in-depth testing, such as overnight monitoring of your breathing, may be done at a specialised Sleep Laboratory.
More information on NIV can be found from MND Australia fact sheets
Invasive ventilationInvasive ventilation, also known as mechanical ventilation, is surgical insertion (by tracheostomy) of a breathing tube directly into the airway. It offers life support to people who cannot breathe for themselves and is only discussed briefly here.
Many people are familiar with invasive ventilation from the late Professor Stephen Hawking. While enabling breathing, and temporarily extending life, invasive ventilation comes at the cost of being able to speak naturally or be independent from ventilation equipment. Attitudes toward invasive ventilation differ between cultures, nations and individuals. In those countries where invasive ventilation for MND is rare (or not supported), many people feel that being connected to a ventilator offers a very poor quality of life. People living in a physically limited, or ‘locked in’ state, are unable to move their body and are fully dependent on 24-hour, specialised care from family members or health care providers. There is also a considerable physical and emotional impact on family members, who will require training to manage equipment and regularly clear secretions from the airway.
For some, the trade-off between full dependence and prolonging life is worthwhile. Eye movement is usually unaffected by MND, so people using invasive ventilation are able to communicate with their family and friends, in person and remotely, by use of specialised equipment.
It is unknown how long each person’s life might be extended by invasive ventilation, as it does not stop MND from progressing. Withdrawing from invasive ventilation means that the lungs will be unable to work, ending life. This information is only a brief overview of the many complex ethical, legal, medical, social and financial issues managing that invasive ventilation may involve. To learn more about the options and services available to you, please discuss with your Respiratory Physician and MND team. If you would like to read more, please see the reference articles at the end of this decision tool.
Your body needs adequate breath support to function at its best. Your MND symptoms will continue to progress, and once your breathing muscles are affected by MND, everyday activities may become more challenging without respiratory support. People who are no longer able to breathe well on their own are able to receive treatment from Palliative Care services to actively address their symptoms and remain comfortable. Treatments may include medication to relieve symptoms, and exercises to assist your breathing, posture and cough. Please discuss this with your Palliative Care and MND professionals if you would like more information on palliative symptom management. More information can be found from the Motor Neurone Disease Association (England, Wales and Northern Ireland). Please note: the information on this website is written for people with MND living in the UK.
Some people may change their mind about using, or withdrawing, from NIV. However, timing is important. Earlier use of NIV is likely to give you more benefits for breathing and quality of life, and allow more time to become accustomed to wearing a mask. Leaving it too late may mean that you are unable to benefit from NIV. Talk with your MND team to decide the best timing for you.
Some people decide to stop using NIV if it is no longer helping them, or has become a burden. If you are withdrawing from continuous NIV, you and your family will require support from your MND and Palliative Care health professionals.
Potential health benefits are the temporary improvement symptoms that were caused by weakened respiration in MND. These may include:
NIV may not prolong your life.
NIV will not cure or halt MND symptoms.
People with MND may be reliant on assistance from family members and health professionals to obtain and maintain ventilation equipment.
There may be costs associated with obtaining ventilation equipment.
There may be some discomfort associated with using NIV, including wearing the mask, nasal irritation and abdominal bloating.
Not everyone will achieve a good or comfortable fit with the face mask.
Speaking while wearing the mask can be effortful.
1. I want to maintain breath support for all my activities
2. I want to have better sleep
3. NIV could improve my quality of life
4. NIV could improve my length of life
1. NIV will not cure MND
2. NIV will not improve my quality of life
3. I prefer to let MND run its course
4. I do not want to depend on equipment for breathing
1. Please indicate
below how important you feel NIV is for your care overall
2. Indicate if NIV is an important choice for you right now
1) Using NIV may prolong my life
2) Having NIV will cure my MND
3) Having breathing difficulties may put my health at risk
4) NIV isn’t suitable for everyone with MND
1) Do you know the benefits and harms of each option?
2) Are you clear about which benefits and harms matter most to you?
3) Do you have enough support and advice to make a choice?
4) Do you feel sure about the best choice for you?
If you are certain that you wish to proceed with NIV, please inform your MND health professionals about your decision as soon as possible. They can then arrange referrals and inform you about your local procedures.
If you are certain that you do not want NIV, please inform your MND healthprofessionals, so they are aware of your wishes.
If you are uncertain about what to do or need more information, please read the additional information on the next page, or make time with your MND health professionals to discuss your options further.
MND Australia https://www.mndaust.asn.au/Get-informed/I-have-MND/Living-better.aspx
MND Australia factsheets https://www.mndaust.asn.au/Get-informed/Information-resources.aspx
MND Association (England, Wales and Northern Ireland): https://www.mndassociation.org/wp-content/uploads/2015/07/08b-ventilation-for-motor-neurone-disease.pdf
NICE Guidance for respiratory impairment in MND https://www.nice.org.uk/guidance/NG42/chapter/recommendations#box-1-symptoms-and-signs-of-potential-respiratory-impairment
Berlowitz DJ, Howard ME, Fiore Jr JF, Vander Hoorn S, O'Donoghue FJ, Westlake J, Smith A, Beer F, Mathers S, Talman P. (2016) Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort. Journal of Neurology, Neurosurgery & Psychiatry, 87(3):280-6. https://jnnp.bmj.com/content/87/3/280
Radunovic A, Annane D, Rafiq MK, Brassington R, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 2017, Issue 10. Art. No.: CD004427. DOI: 10.1002/14651858.CD004427.pub4. http://cochranelibrary-wiley.com/doi/10.1002/14651858.CD004427.pub4/full
A Hogden, J Labra
P Wu, K Falland, J Chien, L Hughes (Department of Respiratory & Sleep Medicine, Westmead Hospital)
Format adapted from the Ottawa Personal Decision Guide © 2000, A O’Connor, D Stacey, University of Ottawa, Canada