Existential concerns for people with motor neurone disease: Who is listening to their needs,priorities and preferences?

Introduction

Motor neurone disease (MND) refers to a group of age-related neurodegenerative disorders of unknown aetiology. Motor neurons transmit electrical signals from the brain (upper motor neurons) and the spine (lower motor neurons) to muscle groups in the body in order to coordinate movement. In MND, motor neurons progressively degenerate and eventually die. At the time of diagnosis a person learns that MND is a life-threatening disease with no known cure (Leigh et al., 2003), and they are usually offered the drug Riluzole, which has been shown to increase survival time by up to 3–4 months (McDermot and Shaw, 2008). A person may learn at the time of their diagnosis that they have one of four types of MND: Amyotrophic Lateral Sclerosis (ALS), ¹ Progressive Bulbar Palsy (PBP), Progressive Muscular Atrophy (PMA), or Primary Lateral Sclerosis (PLS) (Motor Neurone Disease Association (MNDA), 2013). The prevalence, areas affected, signs and symptoms, and prognosis for each of type of MND vary significantly. ALS is the most common form of MND and a person typically has a life expectancy of 2–5 years; in contrast, the life expectancy for PBP may be only 6 months (Forbes et al., 2004). Life and death concerns may also vary (Bolmsjo, 2001; Harris, 2015), and palliative care is recommended from the time of diagnosis (Oliver, 2002), although United Kingdom (UK) practice guidelines do not recommend palliative care until the disease progresses or a person experiences a loss of speech or respiratory insufficiency (MNDA, 2015).

Motor Neurone Disease Association (2015) practice guidelines

A review of the MNDA (2015) practice guidelines emphasises how issues concerning losses with associated grief and bereavement, anxiety, depression and cognitive changes, should be managed by the local mental health or psychological support team (MNDA, 2015), while muscle weakness, dysarthria, dysphasia, saliva management, nutrition and enteral feeding, acute dyspnoea, respiratory insufficiency, assisted ventilation and pain should be managed by a multidisciplinary team; thus providing a psychological, functional/medical, approach to care. Brown (2003) highlights this point, as all members of a multidisciplinary team described their caring role as functional, while people living with MND have holistic needs. While it is recognised that a functional and medical approach to care management of MND is necessary (Hughes et al., 2005), existential issues may occur in conjunction with functional/medical issues, and need early recognition and management.

The author of this paper found that people living with MND are experiencing ‘existential loss’ and have existential concerns for life and death, ² but are not referred into the palliative care services until the second year of their illness trajectory (Harris, 2015). It could be argued that loss, grief and existential issues are discussed with occupational therapists and other members of a multidisciplinary team while addressing function, and therefore referral to palliative care services is not always needed until later in the illness trajectory. The Motor Neurone Disease Association guidance recommends referral into the palliative care service with caution, acknowledging that palliative care has stigma attachments (MNDA, 2015). However, not referring a person into the palliative care services until the second year of a person’s illness trajectory may have significant implications for a person and health care professionals. The Mental Capacity Act (2005) was introduced to protect those people at risk of losing their ability to make and communicate informed decisions.

The Mental Capacity Act (2005)

The ‘best interests’ principles that underpin the Mental Capacity Act (MCA) (2005) are also central to delivering good palliative and end-of-life care (National Council for Palliative Care, 2008). The MCA must be adhered to by health and social care professionals in England and Wales working with people for whom impaired mental capacity may be an issue (Department for Constitutional Affairs (DCA), 2007). People living with MND may experience cognitive changes, thus rendering the MCA highly applicable to these individuals. A review of the NCPC (2008: 9) practice guidelines further emphasises how the determination of capacity is extremely difficult if a person has lost their ability to communicate by any means. Impaired capacity is considered if: ‘a person is unable to make a decision, if he/she cannot understand relevant information, retain that information, use or weigh that information as part of making a decision, or is unable to communicate his decision by any means’. The important point being made here is that a person living with MND is at risk of losing their ability to communicate. A multidisciplinary team of health and social care professionals have a legal obligation to discuss and document a person’s needs, priorities and preferences for end-of-life care before they lose the ability to communicate and sign the appropriate documentation.

Communicating needs, priorities and preferences

Preston et al. (2012) called for an early discussion on the needs, priorities, preferences and end-of-life care decisions. In sharp contrast, Munroe et al. (2007) argued that patients diagnosed with ALS learn that their life expectancy is years rather than months, and consequently health and social care professionals should not discuss end-of-life care decisions so early in the illness trajectory. These two studies both emphasise appropriate timing for conducting end-of-life discussions. The Court of Protection, created under the MCA, rules on life and death medical decisions, and in 2012 a case was brought by a man living with MND who expressed his wish to halt life-sustaining treatments, through the blinking of his eye (Doughty, 2012). This unique case highlights how people need to be informed regarding how their MND will progress early into their illness trajectory, and to be given the opportunity to discuss and document their needs, priorities and preferences for their end-of-life.

Who is listening?

Listening to existential concerns and holding discussions about death and dying are difficult. Ray et al. (2012) suggest that people living with MND may wish to be cared for at home until close to death, and like Bolmsjo (2001), they understood that a discussion on dying for those involved in their studies remained a taboo. The MCA and advance care planning discussion might include a person’s understanding about their illness and prognosis, their concerns, and their preferences for types of care or treatment, and the availability of these (NCPC, 2008). Having the freedom to discuss and document existential concerns and preferences early into the illness trajectory of MND is the key message of this paper. It takes a multidisciplinary team approach to manage people living with MND in the community, along with the support of family. Observing people living with MND near their end-of-life has been described as ‘existential suffering’ (O’Toole, 2011). Joint advance care planning is recommended in order to understand (interpret) the meaning of life for another. However, this raises a number of questions: Given that an occupational therapist may be the first health care professional to assess a person newly diagnosed with MND, how equipped are occupational therapists to facilitate these conversations early in the illness trajectory? How would they take the outcomes forward while acknowledging the potential impact on the wider team?

Conclusions

It is clearly understood that people diagnosed with MND have been diagnosed with a life-limiting illness and may have existential concerns for life and death, with some individuals wanting to talk about their concerns early in their illness trajectory and while they can still communicate (Harris, 2015). Occupational therapists and other health and social care professionals need to listen to a person’s existential concerns for life and death early in their illness trajectory; however, if they do not feel that they have the necessary skills or tools to have these discussions, then they need to make a referral to the palliative care team. A person remaining in control of their future reduces the pressure on those caring for people living with MND at their end-of-life, and also reduces the risk of them having decisions made by a Court of Protection.