This information is for health professionals and service providers.
Due to the complex and rapidly progressive nature of MND, referral to a specialist palliative care service should be initiated early in the course of the disease with subsequent periodic review.
Palliative care involvement can assist the multidisciplinary team with the initiation and timing of end of life discussions.
People with MND should be supported to plan ahead and talk through their needs and wishes for end of life care.
Providing the opportunity for these difficult conversations early on, before the person with MND has problems with communication or cognition and before the need is urgent, will help them to feel more in control and more able to focus on getting on with living.
Palliative and end of life care are often sensitive topics for people and need to be introduced carefully and preferably when initiated by the person with MND and/or their family. Introducing the concept of a palliative approach to care early on may help people to better understand the difference between palliative care and end of life care. Establishing links with a specialist palliative care service, if available, may also help establish relationships to support choice and control and optimal end of life care.
The person with MND is likely to have fears and questions about death and about how they will die and be cared for in the very final phase of the disease. The person with MND and their family may also have questions about when to go to hospital or a hospice, or whether they can stay at home for as long as possible, or be supported to die at home.
Addressing these concerns through ongoing discussion, assistance with completing an advance care plan, appropriate support and the provision of evidence based and quality information will help allay fear and support people to plan ahead. It is very important to be attuned to the patient’s readiness to have these discussions as well as their style of decision-making.
The MND Australia publication "End of Life Care: A guide for people living with MND" is available to help make these sensitive and important conversations easier. Health professionals and providers may find the guide a useful resource to share when the topic of end of life care and MND comes up in conversation. Having the guide on hand and ready to be shared in a discrete and organic way, rather than directly, can help to better support someone learning about end of life care on their terms, and when they feel ready.
There are five parts in the guide. Each part has individual sections that provide specific information and advice on a common theme. For example, Part 1: What Do I Need To Know? introduces some of the most important concepts, like the benefits of being organised, the need to have plans in place for medical, financial, legal and family needs, and when is a good time to start talking about end of life care. Later in the guide, Part 3: What my family, friends and others close to me need to know explores ways to release tension and work through emotions through conversations with family and children.
The five parts are:
1: What do I need to know?
2: Things I’m likely to experience
3: What my family, friends and others close to me need to know
4: What else might I need to consider?
5: How do I get more help?
Call the MND Info Line (Freecall 1800 777 175) to speak to the MND Association in your state or territory about the guide, ordering copies and what may help when sharing it with patients.
Initiating discussions about end of life is often also very difficult for health professionals and providers. It is therefore important for the team to meet regularly to coordinate care, discuss how and when to introduce discussions regarding end of life care and advance care planning, and to provide peer support. The availability of professional supervision is also very important for health professionals and service providers involved in the care of people living with MND.
As MND has no curative treatment and has a prognosis for many of only 2–3 years, end-of-life issues may need to be considered early in the disease progression, particularly as there may be delays in diagnosis so that the disease has progressed by the time the diagnosis has been made. There is evidence that patient and families do appreciate the discussion of end-of-life issues and the NICE guideline recommended that end of life should be discussed if the patient or family ask and when considering and commencing new interventions, such as gastrostomy and NIV.
However, there is often reluctance to discuss the issues of dying and death, although many patients and families have great concerns about the future. There are many myths that dying of MND is ‘distressing’ and ‘horrendous’ and is ‘due to choking’……
…….These myths need to be confronted and evidence provided that death can be peaceful (Oliver, 2019).
End of life conversations can be a source of great anxiety and discomfort for the patient, their families and health care professionals, and as a result such discussions may be avoided. The issue of advance directives should be raised soon after the diagnosis (Jackson et al 2015).
The experience at the end of life can have a significant impact on patients and their families. Due to the complexities of management of advanced ALS it is recommended that specialist palliative care services are involved early and throughout the disease. Advanced care planning allows patients and their families to document their attitudes towards future events but enactment of these wishes may not always be straightforward and decisions should be reviewed regularly (Hobson and McDermott 2016).
Goals of treatment should be discussed regularly with the person living with MND and their carer. As MND progresses to the end of life phase, the goal of palliative care changes from maximising function to providing compassionate, person centered care according to the person's beliefs and preferences.
Good symptom management and access to psychosocial, emotional and spiritual support, based on the needs of the individual, is essential in order to achieve a peaceful and dignified death and to support the carer and family. Refer to Psychological support for people living with MND section.
As end of life approaches it is vital that the health professionals involved regularly check symptoms and ensure appropriate treatment and support. Refer to the Managing Symptoms section.
Support services in the home may be need to be increased as end of life approaches, particularly if the person with MND has expressed a wish to die at home. An urgent review of the person’s NDIA plan or aged care package and referral to community nursing service should be considered. In addition assistive technology needs should be reviewed to ensure access to appropriate aids and equipment to support comfort and carer safety. Aids and technology used to assist communication may also need to be reviewed.
Some people may express a need or desire for spiritual counselling and appropriate information and referrals should be made. It may be appropriate to explore the use of complementary therapies such as massage, music therapy, aromatherapy and other relaxation techniques.
The GP, specialist or other appropriate prescriber should consider anticipatory prescribing of a range of medications to address worsening symptoms related to breathing, pain, saliva management and anxiety, including:
Dosage and modes of administration of medications should be discussed with the palliative care physician. Carers may be trained and supported to provide and administer medications.
The terminal stage is recognised as progressive weakness and often a sudden deterioration over a few days or hours. The most common cause of death is respiratory failure, usually following upper respiratory tract infection. The terminal stage may be preceded by reduced chest expansion, a quietening of the breath sounds, use of accessory muscles (if any are left) and morning headache from CO2 retention overnight.
Signs may be noticed by the carer or a member of the care team and it is important to prepare the family for the coming days and the patient’s imminent death (often after several years of disease). Preparing the family and providing additional support may help prevent the shock of an ‘unexpected’ death (with the risk of more severe bereavement) or the family inadvertently ringing an ambulance after the patient has died at home.
Note: It is important to reassure patients and carers that death from choking is rare.
The end-of-life phase in MND is variable from one patient to another. This phase is marked by any combination of significant functional decline, respiratory failure, dysphagia, marked weight loss, recurrent infection and cognitive decline. Symptoms such as dyspnoea can be managed effectively at the end-of-life phase with appropriate pharmacotherapy, such as subcutaneous morphine (Lau et al 2018).
The value of laycarers in helping palliative care patients to remain at home cannot be underestimated. Laycarers provide psychosocial and physical care, but it needs to be remembered that they have real needs of their own. Palliative care service providers need to support laycarers so that they can be successful and robust in their caring role. This study demonstrates that skilling laycarers to provide subcutaneous injections gives them confidence and that this confidence increases significantly with experience. Embedding laycarers as part of the multidisciplinary community palliative care team, by teaching them to prepare and administer medications for breakthrough symptoms and monitor symptom relief, is likely to further empower them in their caring role (Healy et al 2018).
Anticipatory prescribing of medications to treat symptoms at the end of life may enable informal carers to administer medication whilst subcutaneous infusions may be required for on-going symptoms (Table 3). The Motor Neurone Disease Association has developed a Just-in-case box in which medication for carers or clinicians (ambulance service or nurses) to use to treat terminal symptoms can be stored and recorded (Hobson and McDermott 2016).
Dyspnea, despite liberal use of NIV, can be treated with morphine starting at 2.5–5 mg every 4 hours and increased in dose and frequency as needed. Anxiety and restlessness can be treated with lorazepam 0.5–2 mg every 4 hours as needed. (88 Rocha) Opiates and anxiolytics doses should be increased if they are not providing satisfactory control (Jackson et al 2015).
MND causes progressive loss of independence and an increased need for help with activities of everyday life. Subsequently carers will progressively increase the time they devote to caring. Identifying and addressing the physical, emotional, social and psychosocial needs of the carer is therefore integral to end of life care in MND.
The progression of MND is unpredictable and individual. Death may occur more quickly than anticipated and it is therefore very important that carers and family members are prepared and provided with ongoing practical and emotional support. It is important that care plans and information is shared by all members of the care team, and adequate nursing cover needs to be maintained at the home, hospice, residential aged care facility or hospital. An out of hours contact number for the carer and information on what to do in an emergency is vital. Regular reassurance that all will be done to manage symptoms as they arise to ensure a peaceful death is imperative.
The carer should be encouraged to seek and ask for additional support. The health professionals and service providers involved in providing support and services in the home should review needs regularly and make referrals to increase services or access assistive technology as, when and if available. Loss of speech and the impact on the person's ability to communicate with loved ones may be particularly distressing for carers. Regular assessment and review of AAC devices to help maintain effective communication as the person nears end of life will help to minimise carers social and emotional isolation.
For some people maintaining care at home may become too difficult and admission to a hospice or palliative care facility, hospital or a residential aged care facility may need to be organised. It is important that the carer and family members are supported through the decision making process and reassured that their loved one will be well cared for whatever the setting.
Our study highlighted a lack of death preparedness amongst bereaved carers of patients with MND. Death preparedness can be defined as the readiness for death experienced by the patient and their carer. A contributing factor to being prepared for death is also an awareness of dying, however carers portrayed that they were unaware the patient with MND was going to die. This finding confers with previous research demonstrating a lack of death preparedness amongst patients and carers with other forms of non-malignant conditions, such as non-malignant respiratory disease. The present study also aligned with the theory of closed awareness which refers to the patient, and potentially the carer, being unaware of the patient’s poor prognosis however the healthcare professionals involved in their care are aware and have not shared this information. This demonstrates the importance of effective information sharing between healthcare professionals, patients with MNDs and their carers. Additionally, this particular finding may provide further insight into why less than half of patients in the present study had completed an ADRT (McVeigh et al 2019).
With most of the care and support occurring at home, family carers of PwMND endure adverse effects on their physical, emotional and social wellbeing and they often describe their caring experiences as unrelenting due to the progressive nature of the disease and the relative hopelessness with respect to recovery.
To ameliorate some of the negative effects of caregiving, family carers need support in their dual role: support to enable them to care for the patient (their ‘coworker’ role) and also direct support for their own health and wellbeing (their ‘client’ role) . As Miles and Asbridge  described family carers, they are vital partners in increasing the person-centeredness of health and social care systems (Aoun 2018).
Common fears of patients faced with imminent death are choking and respiratory distress. Having early discussions with the patient and their carers regarding what is to be expected at various phases of the disease, particularly the terminal phase, can allay these fears. Throughout the illness, the role of the carers is pivotal. A significant role for the polyclinic is the physical and emotional support of carers (Lau et al 2018).
Results: the needs of patients and carers are not being adequately met in the final stages of MND and there appears a need for increased, co-ordinated support from palliative care services. The use of advance care planning tools is regarded as beneficial for patients and carers, but health professionals demonstrate a limited understanding of them. Anxiety and distress in patients, carers and bereaved carers is heightened during this period. Carer burden is excessive and may exacerbate patient distress and desire for hastening death (Whitehead et al 2012).
Carers, family and friends will likely experience feelings of grief and loss throughout the disease trajectory. The progressive nature of the condition also means that the carer and family will have provided increasing care and support during the course of the disease. This can take its toll on physical and psychological health and wellbeing particularly for the primary carer.
Provision of person centred services and support, for both the person with MND as well as their carer and family, is vital in helping them to cope with grief and loss and to maintain their own health and wellbeing. Refer to Wellbeing and support section.
Bereavement can be very distressing and isolating. Optimal care and support from health professionals, and all those involved in providing care, during the end of life period will help to prepare the carer and family for the death of their loved one. Feeling prepared and well supported is important in helping to ameliorate the impact of bereavement.
For many carers there will have been weeks and maybe even months of intense caring and support from a range of providers, family and friends. Following death this may suddenly stop. It is therefore very important that follow up is embedded into practice, based on the needs and wishes of the individual. Bereavement counselling and support should be offered to all carers.
Some people living with MND may wish to create a digital legacy, written stories or messages for their loved ones to read and reflect on following their death. These may provide comfort for the family, particularly children. Health professionals and providers may like to provide information and an opportunity to discuss this option with the person with MND and their carer early on in the disease trajectory. If interest is expressed then a health professional, service provider or volunteer could be identified to support the person to develop their digital legacy.
Creating, or using, a digital legacy by people affected by motor neurone disease generates not only challenges and barriers but also benefits. It provides a way for people living with the disease to capture their identity and biography enabling a continuation of existence after their death, in a digital form. Similarly, bereaved young people gain a sense of comfort from using a digital legacy, to see, hear and learn about the deceased (Clabburn et al 2019).
The last cornerstone of a palliative approach is bereavement support. Nevertheless, one of the modern public health paradoxes is the neglect of the bereaved (though bereavement is a universal experience). Palliative care standards propose that bereavement support should be matched to risk and need. However, studies in many countries demonstrate that palliative care services continue to adopt a generic approach in offering support to bereaved families and therefore support is not tailored to their needs. In an Australian survey, only half of the bereaved reported they had a follow up contact from the palliative care service at 3-6 weeks and a quarter had a follow-up at 6 months (Aoun 2018).
Shoesmith et al 2021, Canadian best practice recommendations for the management of amyotrophic lateral sclerosis - See table 1: palliative care and caregivers recommendations
National Institute for Health and Clinical Excellence (2016) NICE Guideline: Motor Neurone Disease Assessment and Management – see recommendation:
1.7 Planning for end of life
Andersen et al 2012, EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) – revised report of an EFNS task force - Palliative and end of life care in ALS recommendations:
Andersen et al 2012, EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) – revised report of an EFNS task force - ALS caregivers and burden of care recommendations:
Prognostic and end-of-life communication is a vital skill for health care professionals caring for patients with progressive life-limiting illnesses, and their families. The Australian 'Clinical practice guidelines for communicating prognosis and end-of-life issues with adults in the advanced stages of a life-limiting illness, and their caregivers' developed by Clayton et al in 2007 help to address these issues. The key recommendations of these guidelines are for health professionals to consider the recommendations conveyed by the acronym PREPARED:
Prepare for the discussion, where possible
Relate to the person
Elicit patient and caregiver preferences
Provide information, tailored to the individual needs of both patients and their families
Acknowledge emotions and concerns
(Foster) Realistic hope
Encourage questions and further discussions
MND Aware e-training program MND Hub – online training course for health professionals and service providers developed by MND NSW – Session 7: End of life Care
Caring at home project - symptom management for palliative care patients: [email protected] aims to improve the quality of palliative care service delivery across Australia by developing resources that will support people to be cared for and to die at home, if that is their choice. Resources have been produced for community service providers, health care professionals and carers to support carers to help manage breakthrough symptoms safely using subcutaneous medicines. The resources are applicable in all jurisdictions across Australia.
International Alliance of ALS/MND Association, 17th Allied Professionals Forum 2019, Perth:
Annual Meeting of the International Alliance of ALS/MND Association, 2019, Perth:
MND NSW, Special Interest Group (SIG) Workshop 2019:
International Alliance of ALS/MND Association, 16th Allied Professionals Forum 2018:
International Alliance of ALS/MND Association, 14th Annual Allied Professionals Forum, 2016
MND Australia, National MND Conference 2014:
MND Australia 2021, End of life of life care: a guide for people living with MND
MND Australia Fact Sheet series for people living with MND:
MND Australia blog: How to make planning ahead easier with our guide to end of life care
Record me now - legacy app
CarerHelp - a website to support carers caring for a loved one who is dying
MND NSW, Ask the Experts 2019:
MND Australia, 4th MND Connect 2018:
Health Talk - healthtalk.org - stories collected by academic researchers who interview people in their own homes, using their own words: