The End-of-Life Experiences of People with Motor Neuron Disease: Family Carers' Perspectives

Abstract

Background:

People with motor neuron disease (MND) face barriers when accessing palliative care. There is a lack of research about how these barriers affect the end-of-life (EOL) and death experiences of people with MND.

Aim:

This study examined the perceptions of EOL experiences of family carers of people with MND in Western Australia (WA) to identify unmet needs and gaps in EOL support for people with MND and their family carers.

Design:

Semistructured interviews were conducted with 12 bereaved family carers of people who died from MND in WA. Thematic analysis was used to identify common themes.

Results:

The themes identified can be summarized into three main areas: accessing support, accessing information, and feeling prepared.

Conclusions:

The findings indicate that increased access to specialist palliative care services by people with MND is needed to improve EOL experiences.

Background and Aims

Motor neuron disease (MND) encompasses a group of life-limiting motor system disorders that results in progressive paralysis. Amyotrophic lateral sclerosis (ALS) is the most common form of MND¹ and the two terms are often used synonymously. MND is the prevalent term used in Australia and the United Kingdom whereas ALS is common in the United States. The rate of progression and clinical manifestation of MND can vary substantially among those affected; however, all will experience a high-symptom burden.^2,3 Symptoms include muscle weakness and atrophy, twitching, muscle cramps, pain, dysarthria, dyspnoea, dysphagia, and emotional lability.⁴ There is also an association between frontotemporal dementia and MND,⁵ and some level of cognitive decline is encountered in about half of the population.⁶ Decline is often rapid and 50% of people will die within three years of symptom onset.⁴ People with MND and their families experience increasing levels of distress as the illness progresses^3,7 as they adjust to continuous and irreversible losses in physical function and quality of life.⁸

Clinical guidelines published by the European Federation of Neurological Societies (EFNS) suggest that palliative care should be offered to people with MND soon after diagnosis⁹ to provide time to establish healthcare relationships and address end-of-life (EOL) issues before the terminal stage when distressing symptoms, including anxiety, pain, and dyspnoea, may progress rapidly.¹⁰ Despite these guidelines, people with MND and their families report difficulty accessing palliative care services when needed,^11–14 and often report unmet needs at the end of life.^15–17

There are several reasons why people with MND and their families face barriers accessing palliative care services. Service eligibility criteria are often strict and require a specified and limited life-expectancy duration,² but estimating prognosis in MND can be difficult and decline can be unpredictable, which presents challenges for judging life expectancy.¹⁰ One study in the United States found that only 5% of people with MND who were referred to hospice care met eligibility criteria.¹⁸ Some palliative care providers may mistake feeding and breathing support necessary for symptom control as life-sustaining measures and decline to provide care.^1,2,10 Others may be reticent to provide services to people with MND who have very high care dependency and resource allocation needs.¹⁹ Most significantly, the relative rarity of MND (annual incidence of 1.5 to 2 per 100,000 people) results in fewer opportunities for healthcare providers to develop expertise and gain experience in caring for individuals and families with MND.^2,16

When there are barriers to receiving EOL care and supports, the burden falls on family carers and exacerbates their distress at a time when they are particularly vulnerable.⁷ As death approaches, the support resources required of MND family carers may be greater than those of the patient,²⁰ and adequate supports are essential to sustain them in their caring role.²¹

There is an emerging body of research addressing the EOL and death experiences of people with MND,^1,11,12 but it remains unclear how to provide best practice EOL care. As such, the aim of this study was to examine the perceptions of family carers of people with MND in Western Australia (WA) about EOL experiences to identify unmet needs and gaps in EOL support. The findings will help to resolve ambiguity about how to provide best practice care by providing additional evidence about gaps and unmet needs.

Methods

Design

This descriptive, qualitative study used a social constructivist framework as our aims were exploratory and applied. This framework acknowledges that people's unique experiences are valid and explores the ways people view the world and construct meaning as they engage in everyday activities.^22,23

Participants

Family carers of people who died from MND who were over the age of 18 and could communicate in English were recruited. Because MND is an uncommon condition, all eligible family carers of people who died from MND 3 to 15 months previously, and who were members of the Motor Neurone Disease Association of Western Australia (MNDAWA), were invited to participate (n = 25) to reach a minimum sample size of 10 participants. Everyone diagnosed with MND in WA is referred to the services of MNDAWA, which provides information and support to its members. Potential participants were identified through the MNDAWA database.

Setting

Participants received EOL care in a variety of settings and from an assortment of providers according to eligibility, accessibility, and preference (see Table 1 “EOL Care providers”). Three participants in regional WA received home-based care from a generic neurological nursing service. In the metropolitan region, two people with MND primarily accessed hospital (non-MND clinic)-based services and one was in residential care. The remaining six participants in the metro region received a combination of home-based care from community palliative care (n = 5), a specialized neurological nurse (n = 2), and aged care support (n = 1). Only two participants were still attending an MND clinic within three months of death (several others had attended previously but stopped when their health declined). All participants received care advice and sometimes other services (such as equipment) from the MNDAWA.

Table 1.

Characteristics of Study Participants

	MND family carers (N = 12)
Gender
Male	5
Female	7
Relationship to deceased
Spouse/partner	11
Child	1
Age
45–49	2
50–59	0
60–69	8
70–79	2
EOL care providers (could have more than one)
Generic neurological nurse (regional)	3
Specialized neurological nurse	2
Community palliative care	5
Aged care services	1
Residential care	1
Hospital-based (non-MND clinic)	2
MND clinic	2
Place of death
Home	8
Hospital	2
Hospice	1
Residential facility	1
Time from diagnosis to death
Less than 1 year	4
1–2 years	3
2–3 years	4
3–4 years	—
More than 4 years	1
Time bereaved
3–6 months	7
7–12 months	3
12–15 months	2

MND, motor neuron disease.

Procedure

A recruitment letter, participant information sheet, and consent form were sent out from the MNDAWA. Those wishing to take part were asked to respond to the researcher, who contacted interested participants by telephone to organize an interview. Digitally recorded semistructured face-to-face interviews were conducted in the participant's home by the first author, an experienced interviewer with a background in counseling. Interviews focused on experiences associated with the end of life and the death of the participant's family member. Three broad areas were explored: (1) experiences and perceptions of healthcare services and supports, (2) experiences and perceptions of information available from healthcare providers to help prepare for the end of life, and (3) the death experience. Prompt questions were used to elicit details, stories, and examples (Appendix 1, Interview Questions and Prompts). Interviews were conducted until saturation was reached and no new themes were emerging.

Ethical approval

Ethics approval was granted by Curtin University (HR137/2012). A protocol for minimizing risk of emotional and psychological harm was used to minimize potential distress resulting from the interview.

Analysis

Interviews were transcribed verbatim and identifying information was removed. Familiarization began as soon as possible after each interview. The accuracy of the transcriptions was checked against the audio recordings. Transcripts were read through several times, and the research team met and discussed issues raised in the interviews to achieve familiarization with the data. Thematic analysis was used to analyze the interviews,²⁴ and initial codes were identified, reviewed, and revised. Key ideas were collapsed into manageable categories, which were grouped together to develop meaningful patterns. Mapping and interpreting patterns in the data within and across groups enabled the development of overarching themes. N-Vivo 9 software (QSR International) was used to manage, code, and sort the data, and to assist with the analytic process. To increase rigor, a clear audit trail was maintained, decision making at each stage of the analysis was recorded,²⁵ and segments of the text were analyzed by a coresearcher to challenge assumptions and reach agreement on final themes.²⁶

Results

Twelve people were recruited and interviewed (response rate 48%). Most of the deceased (66%) were cared for and died at home. All but one family carer were spouses/partners. More than half (58%) were interviewed less than six months after the death of their spouse/partner (see Table 1 for more demographic information). Analysis of the data revealed three main themes: accessing appropriate supports, accessing information, and feeling prepared.

Accessing appropriate supports

Many MND family carers reported difficulty accessing appropriate and sufficient support at the end of life. Some felt they were not seen often enough at the MND clinic.

“If I was going to make a criticism. … it would have been the fact that that because her progress through the disease was reasonably rapid, more frequent visits to the clinic would have been better.” MND 11

Others did not receive desired community palliative care services.

“I was really angry with [palliative care services]. I thought they let us both down terribly. You'd think they would have helped me knowing that [he] didn't have much longer to live.” MND 7

Some received community palliative care services for a time, and then they were taken away.

“There was a day when the [palliative care nurse] come and said to me ‘Oh I think we won't come any more.’ … I said ‘You think that's fair on me?’ …” MND 1

Some participants believed care providers lacked sufficient knowledge and training about MND.

“The palliative care people got someone to come in one night to give me a break. Then I spent the night getting up. They kept coming and waking me and saying there's something wrong and he's not settling and they can't turn him. They weren't qualified people.” MND 9

One MND family carer felt healthcare providers did not take the time required to provide adequate care to people with MND who had high-dependency needs.

“That was the number one thing issue, of people not giving the time that I think is required for an MND patient. I really do think an MND patient is unique…Right at the moment I can still walk, talk, eat, drink. I can do everything. She could do nothing.” MND 2

As a result of gaps in support, including in-home nursing support and respite support, several MND family carers described stressful caring experiences that affected their own health.

“I lost 8 kilos altogether in weight and I haven't got a lot to start with … it wasn't I wasn't eating, in fact it was stress. And stress is a terrible thing. I mean, you're on the go from half past 6 in the morning and you're still going half past 8 at night.” MND 4

“They told me I was going to have 2 hours every day respite…but I didn't get it, and it was a week before I got any respite at all. I was a mess… I didn't get any sleep, lost about 3 kilos. You just get through it, but it was hard.” MND 9

Accessing information

Most MND family carers said they had difficulty obtaining information about the end of life. Some reported that no one discussed death with them.

“[Nobody talked to me about it.] I wasn't aware of what to expect in those final stages. I did start to read more [to find out on my own].” MND 7

Others reported that their healthcare providers said they could not predict what would happen.

“But towards the end, no they hadn't given us any information as to what the disease process would be. And in fact nowhere along the line did I get any indication of what the disease process was.” MND 10

“She [health care professional] couldn't help me and give me answers, you know. I wanted answers to things and they just weren't there.” MND 2

Some MND family carers did not know who to go to for answers to their questions about the dying process, and said that dealing with a multitude of service providers added to the confusion.

“I would have thought that it would be better for the whole MND process to have a single liaison person….” MND 11

Families who felt they had a relationship with a specific provider fared better in having their information needs met.

“I didn't know what was required … [the neurological nurse] provided me with so much information.” MND 12

Feeling prepared

Most MND family carers said they were not prepared for the death of their family member, and death was often unexpected. In some instances, people with MND had seen a healthcare professional hours or days before death, but there was no indication that death could be imminent.

“We had a visit from the nurse on the Friday before [the Sunday when] she died…She didn't say to me she hasn't got long to go or we ought to be making some other arrangements, which is the information I would have liked to have.” MND 11

“Sometimes it's hard for me to accept … because the night before [after going to the doctor, he] was alright…[in the morning] I go upstairs and I find him on the floor. That's what I didn't understand.” MND 1

In one case, an MND patient was released from a hospital palliative care unit where he had been receiving respite care and died at home hours later. In this case, the family carer said she made it clear she did not feel equipped to provide care in the final hours and she was not comfortable with her husband dying at home.

“When he was discharged from the hospital in the end they were unsure whether he should be discharged or not … [but] she said to me, “There's no way we can keep [name] here … [I did not feel prepared] because we had no idea he was dying.” MND 7

Sometimes, families were in the midst of treatment decisions when death occurred.

“In the last few weeks, there was continued talk about getting a different wheel chair… There was no talk to me personally or generally … that her life was in imminent danger.” MND 3

When people with MND died, it was often sudden and traumatic for their families.

“I didn't understand that her life was in danger. Therefore, even when she was making these dreadful noises …I did not realize that this was near the end.” MND 3

Discussion and Implications

These findings demonstrate key issues encountered by family carers of people with MND that affected dying and death experiences, primarily related to the systems and supports available to people with MND and their families. MND family carers had difficulty accessing the information and support they needed at the end of life, and they felt unprepared for death. These findings mirror previous research from the United Kingdom and Australia. In a qualitative study with 24 people with MND and 28 current and former family carers, Whitehead et al.¹¹ found that the support and information needs of people with MND and their family carers were not being adequately met at the end of life that caused distress. Ray et al.¹² found half of MND carers reported negative experiences because they did not realize that the end of life was near and they were unprepared for death.

An early introduction of palliative care services for people with MND has been recommended,^10,27 but many in our study did not receive these services. Some received visits too infrequently. Some had services introduced only to have them taken away. Some received services very late in the disease trajectory, including on the day of death. This echoes previous findings that people with MND have unmet palliative care needs.^11,28

When care was provided, oftentimes care professionals did not appear adequately trained or experienced with MND care, supporting findings from previous research^15,29 where people with MND and their families expressed concerns about health professionals' lack of knowledge and skills. Previous research with MND health professionals has stressed that knowledge of the disease is essential to meet the needs of people with MND and their families.³⁰ It is noteworthy that family carers of people with MND in this study who qualified for regular visits from either a generic neurological nurse or a specialist community neurological nurse (under a care program only for people with neurodegenerative conditions who are under the age of 65)³¹ reported more positive EOL experiences than those who received nonspecialist aged care and/or palliative care services, suggesting that specialized neurological nursing care provided in the community, drawing on a palliative approach, could result in more positive outcomes at the end of life.

EOL care came from many different sources and MND family carers found it difficult to know who to turn to for information, especially about the end of life. This resonates with previous findings that MND multidisciplinary care and palliative care providers need better coordination,¹¹ as well as findings that suggest a reliable coordinator should be delegated who can provide answers to questions.^29,32

When some MND family carers sought to discover what to expect as their family member approached death, they felt they were not given concrete information. This mirrors previous findings that MND family carers desire information about prognosis^33–36 and that their information needs are not being met.^15,16 The dearth of information led to a lack of preparation and contributed to unexpected and traumatic death experiences for families.

Hogden et al.³⁷ found that the common focus of people with MND and their family carers on living in the present, and “living” rather than “dying,” creates a challenge for health providers when trying to discuss death and help prepare people with MND for the end of life. Other research with MND health providers has found that high-level communication skills are required to help families move toward acceptance of death.³⁰ These findings suggest that research on how to best engage people with MND and their families in EOL discussions is needed, along with training in effective communication.³⁸

Part of feeling prepared is accepting that death is imminent, but acceptance of death can be challenging for many MND family carers.³⁹ None of the participants in this study mentioned accessing counseling or psychological support. Previous research supports the importance of counseling for both people with MND and their family carers, but it continues to be a gap in care and clear referral pathways are needed.^7,16

Limitations

The findings of this study are restricted to the MND family carers in WA who participated in the research, which limits their generalizability. However, these findings reflect and build upon previous research from other countries. The examples provided express issues faced by the family carers of people with MND at the end of life in their own words. In this study most of the participants were spouses. Children or other carers could have different perspectives and experiences.

Conclusion

To provide best practice care, clearer information on what to expect at the end of life is required. People with MND and their families had difficulty accessing much needed support and information from their healthcare providers about death and dying. Combined with the less predictable nature of MND, these factors contribute to MND family carers feeling unprepared for death. These findings reinforce recommendations that the psychosocial needs of people with MND and their families receive greater attention, especially at the end of life. The provision of appropriate counseling would enhance quality of life and improve care at this distressing time. Increased information, along with better-coordinated specialized services, may help to provide optimal EOL care. Education on the needs of people with MND and their family carers is needed alongside effective communication strategies for healthcare professionals working with people with MND. Early referral to palliative care has been advocated for people with MND; however, it may be more appropriate that specialist neurological care using a palliative approach is adopted. Further research is needed to develop appropriate services and test interventions to evaluate their impact on the dying and death experiences of people with MND.

Footnotes

Acknowledgments

The authors wish to thank all of the people who took part in this study. We are grateful for the advice received from the members of the Project Advisory Group, from Dr. Rob Edis and the Shenton Park MND Clinic, and for the support this study received from the Motor Neurone Disease Association of Western Australia.

Author Disclosure Statement

No competing financial interests exist.

This study was supported by a grant (SG12/014) from the Cancer and Palliative Care Research and Evaluation Unit (CaPCREU) at the University of Western Australia.

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