Perceptions of people with motor neurone disease, families and HSCPs: a literature review

Motor neurone disease (MND) is a result of degeneration of the upper and lower motor neurones in the brain and spinal cord (Ilardi et al, 2013). Worldwide, MND is also known as amyotrophic lateral sclerosis (ALS), or Lou Gehrig disease. Throughout this literature review, the term MND has been used, as it is the most commonly used term to describe this disease in the UK, where this review was conducted. The rapid deterioration of the motor neurones causes impairment of muscles controlling mobility, swallowing, speech and breathing (Mitchell and Borasio, 2007; Lillo et al, 2010; Kiernan et al, 2011; Caga et al, 2015a;b). This resulting impairment of the muscles leads to swallowing difficulties (dysphagia), poor formation of words (dysarthria) or inability to form words (anarthria), paralysis of upper and lower limbs, mild frontal lobe impairment, cognitive impairment and breathing difficulties (Wijesekera and Leigh, 2009: Phukan et al, 2012).

The prevalence of MND is 6 to 8 cases per 100 000 people at any one time. Care of people with MND varies across the UK (Howard and Orrell, 2002; Wilson, 2016). MND multidisciplinary teams (MDT), centres, clinics and networks provide coordinated multidisciplinary care in various ways (O'Brien et al, 2011; Skelton, 2013; Hogden et al, 2017). To define teams in this review, terms such as MDT, multidisciplinary centres (MDCs) and integrated care were used. The extensive care and support, including MDT care needs, generated by MND places an enormous burden on families, friends and loved ones who provide unpaid care (Ray and Street, 2006; Ozanne et al, 2011; Locock et al, 2012; Oyebode et al, 2013: Foley et al, 2014; Vibert, 2017). However, the experience of loss of control by the plwMND (plwMND) described by Foley et al (2012) can result in rendering control to service providers and causes plwMND to search for control over healthcare services. The substantial burden (Aoun et al, 2012) on family caregivers include increased emotional and total burden over time (Chiò et al, 2005; Gauthier et al, 2007; McNamara and Rosenwax, 2010). Identified care needs, including service gaps in palliative care, assistance for caregivers with several aspects of patient care needs, symptom management, financial and respite care, were indicated in several Australian studies (McConigley et al, 2014). The effects of MND are multifaceted, leading to many adjustments in everyday life and in the long term; the condition can compromise many personal and social aspects of people's lives (Hughes et al, 2005).

The burden of care for this debilitating disease, which ultimately affects most activities of daily living for plwMND, is significant (Ushikubo and Suzuki, 2016;). There are implications for health and social care service provision because the disease progresses rapidly (McConigley et al, 2014). Health and social care services must be more proactive to meet the needs of plwMND and their families or significant others (McConigley et al, 2014).

To date, reviews on MND care have focused on multidisciplinary care, palliative care and the roles of healthcare professionals in delivering care (Aoun et al, 2012; 2016; Riemenschneider et al, 2013; Sakellariou et al, 2013; Hardiman, 2015; Hogden et al, 2017). However, such work does not illuminate the wider complexity of processes that underpin service delivery. Currently, there is limited knowledge on how plwMND experience life with the disease (van Teijlingen et al, 2001; O'Brien, 2004a; Hughes et al, 2005). It is important that the experience of living with MND is explored, so that health and social care professionals (HSCPs), plwMND and their carers understand each other's perspectives when making decisions about care.

Furthermore, a Cochrane review on multidisciplinary care (Ng et al, 2009) for people with MND demonstrated a divergence of perspectives between HSCPs, family carers and plwMND. The intention of this literature review is to highlight key components and challenges for plwMND, their families and HSCPs discussing service provision, from the diagnostic stage to the end of life. For those working in community care, information relating to the care of plwMND in the home setting is now more significant than ever before, following the COVID-19 pandemic, where emerging anecdotal data and an ongoing mixed method study (HB et al, unpublished) is beginning to show perspectives by plwMND and their families on the impact of at-home care in comparison to virtual service delivery. Papers by Glasmacher et al (2021), the European Network for Cure of ALS (ENCALS) (2020) and Oliver et al (2021) also reflect the impact of the COVID-19 pandemic on the care of plwMND.

Method

The critical appraisal process was informed by Hawker et al (2002). The aim of the review was to find out what the perceptions of plwMND, their families and HSCPs are relating to service delivery to plwMND and their carers. The review asked the following questions:

• What is known about the perceptions of plwMND, their families and HSCPs on service delivery to plwMND?
• What are the facilitators and barriers to service delivery from the perspectives of plwMND, their families and carers, and HSCPs?
• How does the structure or mode of delivery of services impact on the perceptions and experiences of plwMND, their families, and HSCPs?

Methodological strategies relating to the strength and rigor of the studies were evaluated. The search history covered the period 2010 to 1 June 2021, and focused on qualitative, quantitative and mixed method studies, scoping for appropriate materials. The review aimed to explore a wide range of published literature on the topic and included available relevant literature regarding the COVID-19 pandemic.

The review used a coding tree (Figure 1) to demonstrate the themes from the review, using root and child codes (explained further under the data analysis section). This review was conducted in accordance with the Preferred Reporting Item for Systematic Reviews and Meta-Analysis (PRISMA). The PRISMA flow diagram (Liberati et al, 2009) was used to demonstrate how articles were excluded. The flow diagram allowed for the stream of information through the different phases of review. It mapped out the number of records identified, included and excluded, and the reasons for exclusions. The PRISMA flow diagram used for the review is not included in this paper to allow for flow of the narrative. However, records identified through database searching can be found in Table 1.

First, abstracts were reviewed for relevance to the study questions and achievement of the the inclusion criteria, based on their title and subject descriptors. These were obtained for data synthesis; full copies of the articles were then evaluated to see if they were appropriate or relevant to the literature review question. Articles identified through reference list and bibliographic searches were also considered for data collection based on their title (citation searching). The first author (HB) independently selected articles against the inclusion criteria. Discrepancies in paper selection were resolved by looking at articles a second time with the three co-authors from the research team (NJ, NT and KB), carefully reviewing to select relevant articles for review before selected articles were retrieved.

Search strategy

The search strategy was designed to access both published and unpublished materials or grey literature. The literature was searched using EBSCO Discovery Service, with support from the University of Portsmouth School of Health and Care Professionals Faculty Librarian. Free text and Medical Subject Headings (MESH) were used. The Cumulative Index to Nursing and Allied Health Literature (CINAHL), the British Nursing Index (BNI), the United States National Library of Medicine (MEDLINE), PubMed and the Health Management Information Consortium (HMIC) were searched (Box 1). These databases are cited as leading databases for support of practice, education and research for health providers. Reference lists and bibliographies of the articles collected were also searched.

Box 1.Keywords and MESH terms

• Family member/significant other/friend and motor neuron/e disease
• Amyotrophic lateral sclerosis/significant other/friend/carer
• Lou Gehrig Syndrome/significant other/friend/carer
• Multidisciplinary team/collaboration/MDT/integration
• Healthcare professionals/social care professionals
• Palliative care/home care/hospital care/community care/acute care

To enable an efficient search, a limit was set to remove duplication, and Boolean logic using the terms ‘AND’/’OR’ was employed. The use of ‘and’ was to encourage a more specific and focused search, while ‘or’ was used to connect concepts and broaden the search to ensure that relevant references are obtained. Inclusion criteria (Table 1) were set to refine the search further.

Data analysis

Data analysis involved the application of Braun and Clarke's (2006) six phases, including familiarisation with the data in phase 1, generating initial codes in phase 2, searching for themes in phase 3, reviewing the themes in phase 4, defining the themes in phase 5 and writing the report in phase 6. In Figure 1, the main themes are identified as root codes and followed by subordinate child codes. Data was analysed manually by hand by the first author (HB) so that immersion and familiarisation with the data was achieved, as per phase 1 of Braun and Clarke's (2006) thematic analysis. All root codes were generated using content analysis methodology (Elo et al, 2014). Themes represented a level of patterned response or meaning from the data that was related to the literature review question. Themes in the review were inductive in a ‘bottom up’ (Frith and Gleeson, 2004; Braun and Clarke, 2006) approach and were strongly linked to the data (Patton, 1990). The subordinate codes elaborated descriptions for each root code. All root codes were generated using content analysis methodology (Elo et al, 2014).

Table 1 demonstrates the inclusion criteria and justification for excluding or including articles in the LR.

Selection of papers

Papers were excluded if they did not explore patients' perceptions about MND care. The papers selected were judged on the validity of the results and the appropriateness of the techniques used in relation to the topic of this paper. Papers that were mainly related to the cure or medical treatment of MND were excluded as, although they were empirical, they did not meet the criteria for this review. This literature review was focused on plwMND, their families and HSCPs' perspectives on care received and delivered. Papers that were mainly methodological were judged by the usefulness of the proposed methodology in relation to this literature review.

Results

Findings

The initial search strategy using MESH and free text with hits using truncation yielded 2837 articles with search terms including ALS and MND. Grey literature using the same search terms, ‘ALS’ and ‘MND’, yielded 50 articles, after duplicates from both search and grey literature were removed, which accounted for 2700 papers. Abstract screening included 500 manuscripts and 44 manuscripts from reference citation. After abstract screening, 114 manuscripts were moved to full-text screening. After full-text screening, 53 manuscripts were excluded because they did not meet the study criteria, and 61 articles were accepted for quality appraisal, data extraction, and coding.

Critical appraisal

Studies were assessed independently for methodological validity by the first author (HB), before inclusion in the review. The quality assessment tool used for the studies was drawn directly from Hawker et al (2002). This was deemed appropriate for this literature review and is user-friendly. The tool (Hawker et al, 2002) contains nine questions, each of which can be answered ‘good’, ‘fair’, ‘poor’ or ‘very poor’. Any disagreements between the authors were resolved through discussion, and with the assistance of a third reviewer (NT) where required. This protocol was found to be an adequate way of understanding the data and achieving rigour.

All papers included in the literature review were assessed for quality: 20% of papers were rated of medium quality, whereas 80% of papers reviewed were high-quality. The reasons behind papers being scored as medium quality was that there was no elaboration on how ethical approval was obtained or if it was obtained at all to undertake the studies; there was also methodological blurring present (Dillon and Wals, 2006). There were some omissions in how transferability could be achieved in a few papers, and how findings will be transferred to, or have applicability in other settings. Transferability in qualitative research is akin to generalisability in quantitative research (Noble and Smith, 2015); however, from a qualitative perspective, transferability is primarily the responsibility of the one doing the generalising. Lincoln and Guba (1985) proposed four criteria for judging the soundness of qualitative research, including credibility, dependability, and conformability, and explicitly offered transferability as one of the prerequisites for reliability in a qualitative study. Transferability could be achieved if the results or findings from the review is transferred to other contexts or settings (Shenton, 2004).

Some 80% of the studies reviewed in this literature review conformed to Lincoln and Guba's (1985) principles for soundness in qualitative research. The review revealed four main themes: delay in service provision for plwMND; communication challenges between plwMND and healthcare professionals; delayed diagnosis; and the need for improved education for HSCPs caring for plwMND and their families or carers. These main themes are discussed in the following paragraphs.

Delay in service provision

The perceptions of plwMND and their families revealed there were often delays in service delivery at end of life (EoL), which would typically cause anxiety, as plwMND and their families were not prepared for EoL and have not had a conversation about palliative care with a healthcare professional (McVeigh et al, 2019). Whitehead et al (2011) found that increased discussion with professionals could relieve people's anxieties regarding EoL care and reported that there was a need to introduce palliative care services early; however, for some, EoL care was provided at a very late stage of the MND trajectory. Participants in Foley et al's (2014) study felt that there was lack of control over decisions made by healthcare professionals and that there was a disconnect between themselves and their attendant healthcare professionals, resulting in apprehension about many aspects of care. However, when participants felt connected at a later stage, they reported a sense of team effort and the ability to discuss EoL decisions with HSCPs, which typically resulted this a regained sense of control (Foley et al, 2014).

Families found that the needs of plwMND and their carers are not adequately met at EoL. Hughes et al (2005) noted a lengthy procedure around referral processes in England. Ang et al (2015) agreed with this conclusion, citing that the diagnostic procedure is often extensive as there is ‘no one test to confirm the diagnosis’. There is also a lack of precise prognostic predictors, leading to difficulties in determining when a person with MND is at the terminal stage. This usually leads to anxiety and uncertainty among plwMND and their family members. There is also a prolonged period of frailty and disability before death compared to other conditions, such as cancer, where there is often a long period of high functioning followed by a short period of rapid decline toward death (Mistry and Simpson, 2013; Ang et al, 2015). In O'Brien et al (2012), one participant suggested that there was very little information regarding the condition's progression, including how the disease can affect life in the future, which was not usually discussed. Access to services, including adult social care, was difficult, especially soon after diagnosis (Hughes et al, 2005). This often meant that several telephone calls to adult social care were needed before plwMND can get an appointment (Hughes et al, 2005).

Some professionals believe that services should be restructured to reduce demarcation between providers to enable follow up of caseload between hospitals and community services, to improve coordination of services and provide consistent care. Hogden et al (2020) suggest that the care of plwMND should be patient-centred, with this approach undertaken in both practice and policy throughout each level of healthcare, with clinicians, healthcare managers, executives, patients and families all playing a role in practising and promoting it. Seamless delivery of patient-centred care (PCC) could be enhanced through education and training for service providers in optimal delivery of PCC for plwMND. Patients, families, care providers, services and organisations may benefit from policies and guidelines that integrate PCC across the range of health sectors accessed by people living with this complex disease. Service providers, therefore, need to consider the individual needs of the plwMND and significant others. In essence, the needs of plwMND should be considered according to how symptoms present, so symptom management should be a significant aspect of care planning.

Although guidelines for MND care have traditionally focused on symptom management (EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis et al, 2012), more recent guidelines take a PCC approach to support frontline care—in particular, the National Institute for Health and Care Excellence (NICE) guidance (NG42) and quality standard (QS126) that promote PCC throughout the care journey (NICE, 2016). For example, a review by O'Brien et al (2019) highlights that non-invasive ventilation optimisation requires an individualised approach to respiratory management, tailored to the differing needs of each patient. Ultimately, this should lead to improved survival and quality of life. The review expands on recommendations in current international guidelines for the use of non-invasive ventilation in plwMND and identifies areas for future research. In short, four principles (Collins, 2014) apply in PCC: affording people dignity, respect and compassion; offering coordinated care; offering personalised care; and enabling people to develop their unique range of capabilities to participate in their own care.

Other implications in service delivery include how plwMND access services. O'Brien et al (2012) identified that understanding how to access services, with critical information on signposting, information about local services and available resources are not made available to carers. In the O'Brien et al (2012) study, it was highlighted that signposting could be useful, but plwMND and their families were not sure who provides the information between the MND nurse or the GP. It is suggested that a list of people who could help is made available to plwMND and their families. However, respite care, for example, was appreciated when it was available (O'Brien et al, 2012), but availability varied across locations. Participants in O'Brien et al's (2012) study reported ‘being weary’ but found relief when they had support from the hospice. However, there were reports of problems with respite care in O'Brien at al's (2012) study by some participants, as a result of uncertainties—for instance, participants were not sure when services could be provided, as they were not offered definite dates and times of available respite services, including availability of beds.

Advanced care plans (ACPs) are a professional communication process, to be conducted throughout the whole course of the condition's trajectory (Seeber et al, 2019), aiming to involve plwMND and their families in decision making, especially before the ability to communicate verbally deteriorates. Seeber et al (2019) argue that, with the support of recent literature, a structured approach using ACPs could enhance the quality of life of plwMND and their families. ACPs can help plwMND to achieve a sense of control, facilitating important family discussions; however, the timing and style of introduction needs sensitivity, with the tools and strategies used evaluated for efficacy and ease of implementation (Murray et al, 2016). Connolly et al (2015) comment that, although ACPs can preserve autonomy, their legal validity and usage varies between countries.

Clinical management of EoL should aim to maximise quality of life of both the patient and caregiver and, when possible, incorporate appropriate palliation of distressing physical, psychosocial, and existential distress. Training of HSCPs should include the development of communication skills that help in sensitively managaging the inevitability of death. The emotional burden for healthcare professionals supporting individuals with a terminal neurological condition should be recognised, with structures and procedures developed to address compassion fatigue and the moral and ethical challenges related to providing EoL care. This is significant as Ando et al (2019) suggest that, because of the absence of curative medication for MND (Connolly et al, 2015), palliative care and understanding quality of life in MND is a clinical priority.

Themes in this section identified delays in service provision are common, from the perspective of plwMND and their carers. However, preparation for EoL and discussion around what to expect could allay anxieties for plwMND and their families. Services such as counselling were crucial for MND centres, as generalist clinicians have limited experience of caring for plwMND. This makes access to the right care at the right time difficult, leading to significant distress (van Teijlingen et al, 2001; Whitehead et al, 2012). Other models of care have included the use of telemedicine. Hobson et al (2019) found that telemedicine is feasible, but uptake can be challenging, with implications for systems and healthcare resources, including hardware and software costs, capability of systems and data storage costs. The use of PCC has also been recognised to empower plwMND to participate in decisions about their care.

Communication between patients and professionals

During the LR, it was found that there were usually communication difficulties between plwMND, their families and HSCPs, especially during the rapid changes in the MND trajectory and the delivery of the diagnosis (O'Connor et al, 2017). Participants tended to guard their private lives and were protective of their family members, which usually hindered HSCPs in making enquiries to family members about symptoms and care needs (Lerum et al, 2017). McConigley et al (2014) suggest that care providers had to be able to predict and communicate changes in care needs, rather than wait until a crisis had occurred before making and communicating changes. If changes were not predicted or the status of the person living with MND changed rapidly, a swift response was then required. Being able to know what is happening with plwMND helps in planning for the next stage in the disease trajectory (McConigley et al, 2014). McConigley et al (2014) and Connolly et al (2015) felt that care providers needed to communicate carefully and empathetically. However, some care providers find communicating empathetically challenging (Galvin et al, 2015). Communication was required to help families to accept changes in palliative care. Studies in the Foley and Hynes (2018) systematic review suggest that families and caregivers prefer to have some additional support, although there is also a significant desire to maintain some control and sense of normality in their lives. This need for control by family members usually meant that they did not engage immediately with services when support was offered, suggesting that plwMND typically depend initially on family members to support their needs (Foley et al, 2014).

The obligation to care for the patient meant that family caregivers will often defer care from support services on occasions (Foley and Hynes, 2018); this impacted connection with service providers, resulting in communication breakdown. Responsiveness to plwMND and their family's needs may enhance collaboration and promote tailored primary care and support for plwMND and their families (Lerum et al, 2017). Nevertheless, few systematic reviews have concentrated on the relationship between the terminally ill and their families in the decision-making process (Foley and Hynes, 2018). There is a need to improve team communication to enhance continuity of care (McConigley et al, 2014). However, it is difficult to know exactly what is happening within a team, as there can be a reduced number of people involved in the care process, which could impact continuity of communication and care for plwMND.

Delayed diagnosis

Most studies reviewed document a delay of referral to an MDC of 12-15 months from first symptom to diagnosis, and many patients experience further delays before referral to specialist services (Hughes et al, 2005; Galvin et al, 2015; Hogden et al, 2017). Participants reported that they were often referred to various specialists before seeing a neurologist (Galvin et al, 2015). There is also a need to introduce early palliative care services, as the prognosis with MND is predominately poor, with an outcome which usually leads to EoL (Whitehead et al, 2011). It has been suggested that people diagnosed with MND should be cared for in palliative care settings from the time of initial diagnosis (McConigley et al, 2014).

In England and Wales, the mean time from onset of symptoms to diagnosis was 16.2 months, while the median time was 15.6 months from first symptoms to diagnosis in Northern Ireland (Galvin et al, 2015). A review of diagnosis timelines for plwMND over a 20-year period at one treatment centre in the UK noted uncertainties in symptom identification, and treatment and management pathways, from the first symptoms experienced to a definite diagnosis (Galvin et al, 2015). There were inefficient referral pathways, with patients not being referred to specialist physicians or a neurologist (Galvin et al, 2015). Data suggest that use of unnecessary investigations and the lack of knowledge about MND demonstrated by non-specialists in making a definitive diagnosis may also delay referral to MDCs (Galvin et al, 2015). Hughes et al (2005) suggest that there is often a lengthy process around referral. Whitehead et al (2011) maintain that leaving care support decisions to a late stage in the condition's progression led to delay in service provision and care staff struggling to meet the needs of plwMND. There were various anxieties expressed by plwMND, including relating to the length of prognosis, not having their wishes considered and the prospect of what will happen to their family (Whitehead et al, 2011). Fear of death and what could happen in the final stages were also relayed (Foley et al, 2014). Nevertheless, there were also expressions of wanting to maintain independence. However, the realisation that it was not possible to do so was recognised, with plwMND realising that the progression of MND was fast and consequently making legal and financial arrangements, including in relation to wills and power of attorney, and making decisions to stay at home with regional hospice home care (Foley et al, 2014).

Educational needs of care professionals

PlwMND and their families value experienced and competent healthcare professionals (Lerum et al, 2017), but O'Brien et al (2012) identified dissatisfaction with the standard of care given by healthcare professionals as a result of a lack of knowledge and training. Foley et al (2007) asserted that there was lack of information about MND among healthcare professionals in Ireland. Foley et al's (2007) participants trusted healthcare professionals and agreed that services contributed to their wellbeing and the support they received, and they believed that services assisted in coping with the progressive illness. Nevertheless, there was a gap in knowledge and a need to develop approaches to understand how professionals address problems. This assertion by Foley et al (2007) was also supported by Lerum et al's study (2017), where participants highlighted that there was a need for assistive devices and medical equipment, and practitioners delivering care should display the skills and confidence to demonstrate and model the best methods of usage.

PlwMND often need respiratory care and assistance, especially with suctioning mucous, cough assistance and bi-level positive airway pressure. However, practitioners felt they were not well equipped to deliver such care. McConigley et al (2014) suggest that keeping up-to-date with current knowledge and services related to MND was difficult for professionals who provided care infrequently. The need for qualified healthcare professionals and unqualified paid care providers, such as nursing assistants, to be trained on how to care for plwMND and their families is critical (McConigley et al, 2014). Awareness was demonstrated of the need to understand MND's disease trajectory, the difficulties in predicting the course of the condition and how to manage specific symptoms (McConigley et al, 2014).

The impact of COVID-19 on care

The COVID-19 pandemic has caused significant delays in NHS pathways; most GP appointments now occur online, with subsequent delays in secondary care assessment (Burchill et al, 2020). Given the rapid progression of MND, plwMND may be disproportionately affected by this, resulting in late-stage new presentations. A pre-COVID model reproduced known features of the MND diagnostic pathway, with a median time to diagnosis of 399 days and a prediction of 5.2% of plwMND presenting as undiagnosed emergencies (Burchill et al, 2020). The impact of COVID-19 on services resulted in diagnostic delays from 558 days, when only primary care was delayed by 25%, to 915 days, when both primary and secondary care were delayed by 75% (Burchill et al, 2020). The model predicted an increase in emergency presentations, ranging from 15.4-44.5%, and suggests that the ongoing COVID-19 pandemic will result in more later-stage diagnosis and emergency presentations of undiagnosed MND. Late-stage presentations may require rapid escalation to multidisciplinary care. However, GPs and healthcare professionals encounter as few as one or two presentations of early MND onset symptoms throughout their career (Jellinger, 2010), which may result in delayed diagnosis or referral.

Social and nursing EoL home care services are also likely to experience increased demand if predictions are correct, and have already faced increased challenges, demand and restrictions because of the pandemic. Findings (Burchill et al, 2020) imply there will be increased pressure and demand for palliative care hospital services, which will experience increased admissions of newly diagnosed MND patients who are close to the EoL. This increase of admissions is also likely to apply to hospices and community palliative care services.

In an online survey, conducted from September-December 2020 in UK to understand the involvement of specialist palliative care services for plwMND, 97% of the respondents were involved in the care of plwMND; it was found that services were greatly disrupted for plwMND as a result of the pandemic (Oliver et al, 2021). There was disruption in clinic attendance, resulting in mostly virtual contact; home visits were also restricted, and general support for plwMND was reduced (Oliver et al, 2021). Glasmacher et al (2021) also investigated the impact of the COVID-19 pandemic on care delivery. Nine consultants and seven nurses completed the survey representing Scottish health boards. The majority reported that provision of gastrostomy, non-invasive ventilation and respiratory function testing were adversely affected, but prescription of riluzole, palliative care and EoL care were comparatively unaffected (Glasmacher et al, 2021). NCALS (2020) recognised the more severe impact of COVID-19 on the respiratory system of plwMND as a result of the weakness of the respiratory muscles triggered by the condition, but highlighted that ENCALS' centres were working together to optimise support for plwMND.

Delays in service provision will limit the quality of life for plwMND and will undoubtedly result in a diagnosis occurring at a later stage of condition progression. This contrasts with what NICE recommend as current best clinical practice, which is that all patients with suspected MND are given a neurology appointment within 4 weeks of referral from the GP/primary care provider, in order to receive a confirmed diagnosis (NICE, 2016).

Discussion

Summary of the findings

Themes identified in this literature review revealed a lack of knowledge among HSCPs caring for plwMND and their families, with implications for service provision and quality of care, including service fragmentation, lack of coordination, carer strain resulting in burnout and breakdown, and failed provision (Lerum et al, 2017). Lack of knowledge would sometimes result in delayed diagnosis and poor standard of care, and could also cause families to be wary of MND care provision (Hughes et al, 2005). Competencies with advanced work that incorporates the survival of the person with MND, such as the use of assistive devices, gastrostomy feeding and respiratory support, are required, but are not usually assured. HSCPs in the study by Lerum et al (2017) reported five main challenges:

• Building relationships with those giving and receiving care in the home
• Preventing caregiver burnout and breakdown; providing tailored care
• Ensuring good working conditions in patients' homes
• Recruiting and retaining qualified nursing assistants.

However, some plwMND agreed that professionals who were qualified or trained will deliver high standards of care, compared to unqualified professionals (Lerum et al, 2017). HSCPs found that plwMND and their families were often reluctant to accept support from services as they wanted to protect their privacy and secure their independence (Lerum et al, 2017), which presented a challenge to the delivery of high standards of care. Typically, services were accepted when the plwMND is dying, which impacted the standard of care delivered because of the delay in acceptance preventing timely preparation for EoL. Lerum et al (2017) highlighted advances in medical technology, suggesting that primary care work had become increasingly complex. All healthcare professionals and healthcare assistants caring for plwMND at home may require specific training to safely deal with the condition's complexities (McConigley et al, 2014). Education should be offered on an as-needed basis. However, McConigley et al (2014) acknowledge that, because MND is a rare condition, practitioners may not come across plwMND frequently and, therefore, may not necessarily utilise the skills necessary to manage this condition in the rest of their practice.

Detailed planning (Lerum et al, 2017) included working conditions that allowed the tailoring of approaches to personal, emotional and existential care preferences for plwMND. In Lerum et al's (2017) study, healthcare professionals argued that caring for plwMND placed a severe strain on nursing assistants, and it was found that there was limited knowledge among healthcare assistants about the condition.

Some other challenges to service delivery, as perceived by plwMND and their families, included communication and relationships with HSCPs. In turn, HSCPs faced difficulties with team location and the need to travel long distances to provide care, which impacted their ability to conduct face-to-face meetings with plwMND and team members (Lerum et al, 2017). Lerum et al (2017) also suggested that HSCPs sometimes felt that plwMND and their families want to reduce contact with them as a way of coping with the condition, while HSCPs would generally prefer to establish a connection and prepare in advance for the various stages of MND before they manifest. Bespoke communication was identified as a requirement to tailor and personalise care messages effectively with professionals involved in care of plwMND; specialised communication skills were required for interaction with plwMND because of the difficulties resulting from anarthria or dysarthria, with communication aids often needed (McConigley et al, 2014). Ensuring that all members of the multidisciplinary team were kept informed about and involved in care was critical.

Integrated care teams or MDCs can support care delivery for plwMND, providing access to professionals who can address the symptoms (Hogden et al, 2017). However, timely referral to services, including integrated care teams or MDCs, is affected at present, and plwMND can sometimes find it difficult to travel to clinics or centres as a result of progression of the condition and mobility issues. HSCPs have advocated regular MDT healthcare intervention for plwMND when clinically appropriate, because of the effectiveness of collaboration between professionals within MDTs and between teams. There is also a call for early initiation of referral to specialist services in MDTs. This review found that people with MND engaged with healthcare services in line with their own life transitions and trajectories and narrated their experience of healthcare services at both family and societal levels.

Implication for practice

To address the holistic care of plwMND, it can be argued that service providers must pay close attention to life trajectories and the relationships of people with MND, enquiring about their roles within the family (Foley et al, 2017). The familial roles that plwMND maintain (eg parent, spouse, partner) shapes their response to MND and their decisions about care and where care is delivered. Traditionally, professionals and researchers in the MND field have conceptualised family and family care burden without enough attention to the roles people with MND have adopted within their families. However, consideration of these roles are critical, even as plwMND become physically dependent on health or adult social care services and roles change as the condition progresses. The role of social services within MND care is not well defined, despite their significant contribution to the care process. In this review, family members' and plwMND's views can be incorporated into the MDT decision-making process with a consideration on choice of place for care. These findings are relevant for future research relating to the impact of services, including integrated care teams, social services and care in the acute and third sector. There is a dearth of literature on how health and care services can meet the needs of plwMND and their families; further studies are needed to inform practice development in the care of plwMND.

To analyse MDT working, the authors in this review evaluated perspectives from papers published in peer reviewed journals. These papers considered the severity of the condition and the impact of MND on patients, family caregivers and unpaid carers, and justify the need for collaborative working among HSCPs in delivering cohesive care to this patient group and their families. It is clear that the expectation of care and the support available to HSCPs to deliver evidence-based care to plwMND, and the roles of patient carers and ALS support associations within the MDT team, has not always been considered. A greater understanding of MDTs, care practices and the decision-making processes of plwMND and their families can generate more effective models of care. The European Federation of Neurological Societies guidelines recommend that more frequent review for plwMND at MDCs following diagnosis or during the later stages of the condition may be needed, with less frequent reviews if the condition is progressing slowly (Hogden et al, 2017). Foley et al (2017) agrees that guidelines for best practice in ALS can emphasise the role of the patient and the family in decision-making processes.

MDT working was described as coordinated working among various relevant disciplines, including the patient's own GP and practice team, clinical nurse specialists, occupational therapists (OTs), speech and language therapists (SLTs), social workers, dieticians and palliative care teams (Howard and Orrell, 2002; Skelton, 2013). However, O'Brien et al (2011) recognised the limited evidence for the efficacy of MDT working in the wider literature. Nevertheless, the study by O'Brien et al (2011), which was qualitative narrative-style research that included plwMND and their family/carers, acknowledged that patients and families affected by MND require support from a wide range of HSCPs. In O'Brien et al's (2011) study, the MDT involved specialist MDT professionals from a care centre who worked closely with generalist professionals from a community care team, including OTs. There was also recognition that a psychological team within the MDT is of value because of the emotional and psychological impact of the condition on families and patients. However, psychological services were not often available within the MDT, and there was frequently inequity of services, including palliative care (O'Brien et al, 2011).

O'Brien et al (2011) also found that carers were appreciative of the support they received from MDT members, but identified that there was dissatisfaction with the level of support received from health and social care services. In view of this, it appears that carers demand more input into developing HSCPs so that they can better assist family carers to manage the challenges they face. There was recognition that paid social care support was significant for maintaining wellbeing in family caregivers of plwMND. In the absence of social service support, family caregivers rely on HSCPs.

There was a contention that GPs will not be able to meet the needs of plwMND at home if they are not familiar with the condition progression, as they are not trained in MND care. Carers suggest that there is a knowledge and skills gap, and this should be addressed through development and preparation for HSCPs when they are caring for MND patients in their homes (O'Brien et al, 2011).

Hogden et al (2017) outlines a comprehensive definition of what MDT care should consist of, which includes medical practitioners in genealogy, psychology, neurology, gastroenterology and respiratory medicine. Healthcare professionals in Hogden et al's (2017) study included physiotherapists, OTs, pathologists, nutritionists, SLTs and social workers. Healthcare professionals in genetic counselling, neuropsychology amd counselling were also included in the MDT. The value of inter-service communication was recognised (Hogden et al, 2017). O'Brien et al (2011) concurred with this in their study on the delivery of high standards of care for patients. The review by Hogden et al (2017) also acknowledged the value of patients and families as significant members in the communication process. Families and patients were fundamental stakeholders in the decision-making process when planning care. The different models discussed in this literature review recognise and acknowledge the value of the patient centredness within MDTs.

There was a collective agreement among the papers reviewed about what MDT working provides. This literature review identified that there was a development in the inclusion of the MDT members as time has passed. The MDT has grown from that described by Skelton (2005) to the MDT outlined by Hogden et al (2017). Multidisciplinary team working was described as coordinated working among various relevant disciplines including the patient's own GP, and practice team, clinical nurse specialists, OTs, SALT, social workers, dieticians, and palliative care team (Howard and Orrell, 2002; Skelton, 2013). In the MDT of Skelton (2005), and Hogden et al (2017), the GP was supposed to be included; however, this literature review argues that GPs will seldom encounter plwMND (O'Brien, 2004b). Nevertheless, there was acknowledgement that the comprehensive description of the MDT by Hogden et al (2017) may not be applicable or achievable across all MDTs. Further consideration is needed into what should constitute the best kind of MDT. Moreover, further studies are required to evaluate the preparedness of MDTs and the effectiveness of these teams in meeting the needs of plwMND and their families.

There is an argument that generalist HSCPs need to have some understanding of MND if they are caring for plwMND in palliative and EoL care. O'Brien (2004a) found that most GPs and community nurses who are likely to come across patients with MND are not well informed or knowledgeable about the condition. This is significant as expertise about the condition is likely to support and aid early diagnosis, with consequent adequate care planning in palliative and in EoL care (Holmes, 2005). The extensive care and support demanded to manage MND, including MDT care needs, also places an enormous burden on families, friends and loved ones who provide unpaid care (Foley et al, 2014). Better education and training of HSCPs on the impact of MND could help to alleviate this.

The effects of MND on plwMND, paid carers, unpaid carers or families are multifaceted, leading to many adjustments in everyday life and in the long term. MMD can compromise both personal and social aspects of people's lives (Hughes et al, 2005). The degree of preparedness on how to care for plwMND during palliative care and in EoL by all HSCPs is significant, but generalist HSCPs should pay particular attention to ensuring preparedness and knowledge as a result of their limited experience with plwMND.

The implication of the COVID-19 pandemic on plwMND is currently being evaluated in HB's ongoing doctoral research.

Strengths and limitations

The literature review included data from peer reviewed papers. A strength is that peer reviewed papers were evaluated for quality and reliability by assessing study methods and methodology. However, the literature review only presented papers in the English language and papers from certain countries, including the UK, Ireland, Scandinavia, Singapore, Canada, Japan, Australia, Norway, India and the USA. This may have excluded important information from other countries and regions, potentially leading to cultural and demographic bias. Papers may have also been missed because there was only one author with limited capacity screening for papers in databases, websites, journals and reference lists and performing database searches, paper selection and quality appraisals.

The authors believe that incorporating papers from qualitative, quantitative, and mixed method perspectives revealed relevant standards in areas of service provision for plwMND. One of the critical limitations in conducting this literature review was that it required a great range of evidence from diverse databases with peer-reviewed material, and gaining entry into various databases was problematic and required database permissions. The authors mitigated this by using university resources and databases with peer-reviewed material and, more specifically, primary studies.

A measure taken to mitigate some of these limitations was the provision of librarian training and support for the database searches, and the methodology for selection, data extraction, quality judgements and thematic arrangements of the review was a collaborative process agreed with the co-authors during doctoral supervision.

Conclusions

There are significant gaps in service delivery to plwMND, their families or carers. HSCPs should be aware of these gaps so they can put strategies into place to meet needs of plwMND and their families. The perception of plwMND and their carers is that the lack of knowledge among HSCPs impacts service delivery, including timely referrals for treatment and diagnosis.

Other challenges in service delivery include communication between plwMND, and HSCPs. This seems to impact ownership of care, as plwMND and their families or carers become protective of their lives, experiences and condition process, possibly limiting acceptance of service provision in a timely manner. Plans should be put in place to mitigate this. There needs to be collaborative decision-making between plwMND, their families and HSCPs at an early stage to allay anxieties and build a trusting relationship.

The literature review offered the opportunity of evaluating barriers encountered by plwMND, and HSCPs. Key findings and processes that explain difficulties in service delivery for plwMND were identified through common main themes and sub-themes, which were critically analysed. The experiences of plwMND and their families regarding service delivery could contribute to policymaking. Standard operating procedures on how services can be delivered could also be devised.

Standard operating procedures could include procedure for communication between HSCPs and plwMND and their families especially relating to virtual communication, and to consider dysarthria and anarthria. Standard operating procedures could also relate to EoL care, palliative care including pathways for hospice care. Education for unqualified HSCPs and qualified HSCPs should be considered, including the management of complexities such as managing symptoms and equipment. Awareness and follow up of NICE guidance [NG42] (2016) could be adapted when HSCPs are caring for plwMND.

Key points

• There are still delays in service provision for people living with motor neurone disease, including challenges in communication between people living with motor neurone disease and healthcare professionals and delayed diagnosis.
• There is the need for improved education for health and social care professionals caring for people living with motor neurone disease and their families/carers.
• There is a need for standard operating procedures when health and social care professionals are caring for people living with motor neurone disease.
• Health and social care professionals must raise the profile of National Institute of Health and Care Excellence guidelines for the care of people living with motor neurone disease.

CPD reflective questions

• What is known about the perceptions of people living with motor neurone disease, their families and health and social care professionals on service delivery to people living with motor neurone disease ?
• What are the perceived facilitators and barriers to service delivery from the perspectives of people living with motor neurone disease, their families/carers and health and social care professionals ?
• How does the structure or mode of delivery of services impact on the perceptions and experiences of people living with motor neurone disease, their families and health and social care professionals ?
• What are the experiences of people living with motor neurone disease, their families and health and social care professionals during the pandemic?