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The diagnostic experience for people with MND and their caregivers in the U.K.

Open AccessPublished:October 28, 2022DOI:https://doi.org/10.1016/j.jns.2022.120483

      Highlights

      • The SPIKES criteria were generally not met at diagnosis appointment.
      • There is need to raise awareness of NICE MND Guidelines and ensure adequate training, time and funding to ensure communication.
      • Care provided at specialist MND Care Centres was of a higher standard than in routine neurology clinics.
      • There is evidence of improvement in comparison with earlier studies in the provision of privacy and ensuring there were no interruptions at diagnosis.

      Abstract

      Objective

      How an MND diagnosis is communicated has implications for how individuals adapt to their illness. The consultation process with the neurologist, diagnosis delivery, and adherence to UK guidelines, were explored from the perspectives of people diagnosed with MND and family caregivers.

      Methods

      A cross-sectional approach with people with MND and their caregivers in UK. An anonymous online survey, based on the SPIKES protocol for delivering bad news and containing questions focusing on the UK NICE MND guideline, was distributed in 2018–19.

      Results

      69 people with MND and 39 caregivers responded. People with MND were more likely than caregivers to rate highly their neurologist's skills and ability and their satisfaction with delivery of the diagnosis. The amount of time spent with the neurologist at diagnosis had an impact on the level of satisfaction and rating of the neurologist's skills and abilities. The SPIKES criteria were generally not met. Many of the NICE MND guideline recommendations were not met, though adherence was greater in MND Centres.

      Conclusions

      While there is evidence of satisfaction with the delivery of the diagnosis amongst people with MND and caregivers, there is room for improvement. There is a need for greater awareness of the requirements of people with MND and caregivers. There is also a need to raise awareness of the NICE MND guideline and ensure adequate training, time and funding to ensure communication at this difficult time is acceptable and effective. Where possible it would be preferable for referrals to be made to MND centres.

      Keywords

      1. Introduction

      Motor neurone disease (MND) is a progressive neurological disease, characterised predominantly by motor neurone damage leading to progressive muscle weakness. The average prognosis is two to three years although 25% of people diagnosed with MND live 5 years and 10% survive 10 years [
      • Shaw C.
      • Quinn A.
      • Daniel E.
      Amyotrophic lateral sclerosis / motor neurone disease.
      ]. However, there are often delays in diagnosis, with an average delay of just over 14 months [
      • Matharan M.
      • Mathis S.
      • Bonabaud S.
      • Carla L.
      • Soulages A.
      • Le Masson G.
      Minimizing the Diagnostic Delay in Amyotrophic Lateral Sclerosis: The Role of Nonneurologist Practitioners.
      ]. Many people with MND (pwMND) have a short prognosis and may have appreciable disability at the time of diagnosis. Cognitive change is common, with frontal lobe dementia in about 15% and other cognitive change in up to 35% of cases [
      • Bersano E.
      • Sarnelli M.F.
      • Solara V.
      • et al.
      Decline of cognitive and behavioral functions in amyotrophic lateral sclerosis.
      ].
      Delays in arriving at a diagnosis contribute to feelings of uncertainty and anxiety amongst patients and their families [
      • Remm S.
      • Halcomb E.
      • Stephens M.
      Experiences of being diagnosed with MND “I just want to know”.
      ,
      • Aoun S.M.
      • Breen L.J.
      • O’Brien M.
      • O’Connor M.
      “The shock of diagnosis”: qualitative accounts of people with Motor Neurone Disease reflecting the need for more person-centred care.
      ]. The time of the diagnosis of MND is challenging for both the person receiving the diagnosis and the clinician delivering it [
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ,
      • Aoun S.M.
      • Breen L.J.
      • Edis R.
      • Henderson R.D.
      • Oliver D.
      • Harris R.
      • Howting D.
      • O’Connor M.
      • Birks C.
      Breaking the news of a diagnosis of motor neurone disease: a national survey of neurologists’ perspectives.
      ]. Studies vary on the level of satisfaction with delivery of the diagnosis with figures ranging between 36% and 50% of pwMND being dissatisfied [
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ,
      • Aoun S.
      • Breen L.J.
      • Oliver D.
      • Henderson R.D.
      • Edis R.
      • O’Connor M.
      • Howting D.
      • Harris R.
      • Birks C.
      Family carers’ experiences of receiving the news of a diagnosis of Motor Neurone Disease: a national survey.
      ,
      • McCluskey L.
      • Casarett D.
      • Siderowf A.
      Breaking the news: a survey of ALS patients and their caregivers.
      ]. Whilst recognising and appreciating the difficulties experienced by neurologists, pwMND and their families have expressed concern at communication of the diagnosis being abrupt, insensitive and lacking empathy [
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ,
      • Aoun S.
      • Breen L.J.
      • Oliver D.
      • Henderson R.D.
      • Edis R.
      • O’Connor M.
      • Howting D.
      • Harris R.
      • Birks C.
      Family carers’ experiences of receiving the news of a diagnosis of Motor Neurone Disease: a national survey.
      ]. A Japanese study reported increased anxiety amongst patients and their families at the time of the diagnosis with many feeling that insufficient time was allocated for communication and inappropriate information about ventilatory support was provided [
      • Hirayama T.
      • Izumi Y.
      • Nakayama Y.
      • et al.
      Communicating the diagnosis: a survey of patients with amyotrophic lateral sclerosis and their families in Japan.
      ]. How the diagnosis is communicated has implications not only for future patient-professional relationships, but also for the way individuals adapt to their illness [
      • Chio A.
      • Borasio G.D.
      Breaking the news in amyotrophic lateral sclerosis.
      ].
      Studies have shown that neurologists do find the telling of the diagnosis stressful. An Australian study, which influenced the current study, sought to ascertain the nature and extent of challenging experiences at the time of the diagnosis from the perspective of pwMND, their families and also their neurologists [
      • Aoun S.M.
      • Breen L.J.
      • O’Brien M.
      • O’Connor M.
      “The shock of diagnosis”: qualitative accounts of people with Motor Neurone Disease reflecting the need for more person-centred care.
      ,
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ,
      • Aoun S.M.
      • Breen L.J.
      • Edis R.
      • Henderson R.D.
      • Oliver D.
      • Harris R.
      • Howting D.
      • O’Connor M.
      • Birks C.
      Breaking the news of a diagnosis of motor neurone disease: a national survey of neurologists’ perspectives.
      ,
      • Aoun S.
      • Breen L.J.
      • Oliver D.
      • Henderson R.D.
      • Edis R.
      • O’Connor M.
      • Howting D.
      • Harris R.
      • Birks C.
      Family carers’ experiences of receiving the news of a diagnosis of Motor Neurone Disease: a national survey.
      ]. Neurologists reported experiencing stress and lacking confidence in communicating the diagnosis. They expressed an interest in further education regarding diagnosis delivery [
      • Aoun S.M.
      • Breen L.J.
      • Edis R.
      • Henderson R.D.
      • Oliver D.
      • Harris R.
      • Howting D.
      • O’Connor M.
      • Birks C.
      Breaking the news of a diagnosis of motor neurone disease: a national survey of neurologists’ perspectives.
      ]. Similarly, in the UK, a study of 49 neurologists showed that nearly half reported moderate levels of stress when giving bad news, even though they attempted to meet the standards of good practice [
      • Anestis E.
      • Eccles F.J.R.
      • Fletcher I.
      • Simpson J.
      Neurologists’ current practice and perspectives on communicating the diagnosis of a motor neurodegenerative condition: a UK survey.
      ]. Over 75% of the neurologists did not follow any specific guidelines or protocols [
      • Anestis E.
      • Eccles F.J.R.
      • Fletcher I.
      • Simpson J.
      Neurologists’ current practice and perspectives on communicating the diagnosis of a motor neurodegenerative condition: a UK survey.
      ].
      The use of protocols to help in the telling of the diagnosis has been widely recommended. The European Association for Palliative Care and European Academy of Neurology Consensus document on palliative care for progressive neurological disease recommended that communication with patients and families should be structured following validated models [
      • Oliver D.J.
      • Borasio G.D.
      • Caraceni A.
      • de Visser M.
      • Grisold W.
      • Lorenzl S.
      • Veronese S.
      • Voltz R.
      A consensus review on the development of palliative care for patients with chronic and progressive neurological disease.
      ]. An example of a validated protocol is SPIKES [
      • Baile W.F.
      • Buckman R.
      • Lenzi R.
      • Glober G.
      • Beale E.A.
      Kuelka AP.SPIKES- a six-step protocol for delivering bad news: application to the patient with cancer.
      ] which follows a six step process (see Table 1).
      Table 1Six domains of the SPIKES protocol (Baile et al., 2000).
      Step 1: SETTING UP – establishing the appropriate setting
      Step 2: PERCEPTION – finding out what the patient knows and understands about their condition
      Step 3: INVITATION – finding out how much the patient wants to know; being invited to give bad news
      Step 4: KNOWLEDGE – providing knowledge and information to the patient
      Step 5: EMOTIONS – exploring the patient's feelings
      Step 6: STRATEGY – creating a strategy with the patient to take forward
      The UK National Institute of Health and Care Excellence (NICE) has developed guidance for the assessment and care of people with MND [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ] which complements the international guidance on delivery of the diagnosis [
      • Borasio G.D.
      • Sloan R.
      • Pongratz D.E.
      Breaking the news in amyotrophic lateral sclerosis.
      ,
      • Miller R.G.
      • Jackson C.E.
      • Kasarkis E.J.
      • England J.D.
      • Forshew D.
      • Johnston W.
      • et al.
      Practice Parameter update. The care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/ behavioural impairment (an evidence-based review).
      ,
      • Anderson P.M.
      • Abrahams S.
      • Borasio G.D.
      • de Carvalho M.
      • Chio A.
      • Van Damme P.
      • Hardiman O.
      • the members of the EFNS Task Force
      EFNS guidelines on the clinical management of amyotrophic lateral sclerosis: revised report of an EFNS task force.
      ]. The NICE guideline (NG42) [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ] includes a number of recommendations regarding support at diagnosis (see Table 2). There are a number of specialist MND care centres and networks across England, Wales and Northern Ireland, funded by the MND Association, staffed by professionals with specific and extensive expertise in MND. However, not everyone diagnosed with MND will be referred to one of these specialist centres but will receive follow up from general neurology services.
      Table 2Recommendations for support at MND diagnosis (NICE guideline NG42 2016).
      • The diagnosis should be given by a neurologist with up-to-date knowledge of the disease and experience of treating people with MND
      • Patients should be asked about how much information and support they wish to receive about MND, and about their preferences for involving their family. They should be provided with information at diagnosis or when they ask for it.
      • People diagnosed with MND should be provided with a single point of contact and information about how to address concerns between appointments and in an emergency.
      • People with MND should be offered a face-to-face, follow-up appointment with a healthcare professional from the multidisciplinary team within 4 weeks of diagnosis
      • When MND is suspected or confirmed, the person's GP (family physician) must be informed without delay
      • Sufficient time should be set aside to discuss the person's concerns and questions
      • Referral for social care assessment should be made if the person has any social care needs
      • Carers should be advised that they have a legal right to have a Carer's Assessment of their own needs

      1.1 Aims

      The aims were to:
      -explore the experience of receiving a diagnosis of MND from the perspectives of pwMND and their caregivers.
      -investigate how current UK practice was adhering to the recommendations set out in the NICE MND guideline as this had not previously been investigated [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ].

      2. Materials & methods

      The same approach and data collection tools adopted in the earlier Australian study [
      • Aoun S.M.
      • Breen L.J.
      • O’Brien M.
      • O’Connor M.
      “The shock of diagnosis”: qualitative accounts of people with Motor Neurone Disease reflecting the need for more person-centred care.
      ,
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ,
      • Aoun S.M.
      • Breen L.J.
      • Edis R.
      • Henderson R.D.
      • Oliver D.
      • Harris R.
      • Howting D.
      • O’Connor M.
      • Birks C.
      Breaking the news of a diagnosis of motor neurone disease: a national survey of neurologists’ perspectives.
      ,
      • Aoun S.
      • Breen L.J.
      • Oliver D.
      • Henderson R.D.
      • Edis R.
      • O’Connor M.
      • Howting D.
      • Harris R.
      • Birks C.
      Family carers’ experiences of receiving the news of a diagnosis of Motor Neurone Disease: a national survey.
      ] were utilised, involving a cross-sectional approach with an anonymous national survey of pwMND and their caregivers. The questionnaires were developed from the international literature, including published MND guidelines, and extensive consultation with appropriate stakeholders including MND associations and clinicians. The SPIKES protocol [
      • Baile W.F.
      • Buckman R.
      • Lenzi R.
      • Glober G.
      • Beale E.A.
      Kuelka AP.SPIKES- a six-step protocol for delivering bad news: application to the patient with cancer.
      ] was used to measure features of effective communication of bad news. To build on the original study we added questions directly relating to the NICE guideline NG42 (NICE 2016) [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ] which were published after the Australian study was conducted.
      Links to the participant information sheets and surveys were made available through various social media platforms, websites and newsletters, including those of the MND Association (England, Wales and N. Ireland) and MND Scotland as well as other MND-related charities and appropriate organisations. Staff from these organisations also shared study information with potential participants. Paper copies of the participant information sheet and survey were also available should they be preferred by participants, with pre-paid envelopes provided for their return to the research team.
      The survey for pwMND consisted of questions concerning demographic information, symptom onset, date of diagnosis and time spent by the neurologist when delivering the diagnosis. The ability/skills of neurologists in delivering the diagnosis, as perceived by pwMND, were assessed using a 5-point Likert scale ranging from poor to excellent. Directed questions requiring either ‘yes’, ‘no’ or ‘do not recall’ responses, or directed statements with responses on a five-point Likert scale from strongly agree to strongly disagree were used to assess each of the six SPIKES [
      • Baile W.F.
      • Buckman R.
      • Lenzi R.
      • Glober G.
      • Beale E.A.
      Kuelka AP.SPIKES- a six-step protocol for delivering bad news: application to the patient with cancer.
      ] protocol domains (Table 1). Open ended questions were included for each domain to capture more in-depth information from respondents.
      The family caregiver survey replicated the pwMND survey with the addition of a question on their relationship to person with MND.

      2.1 Analysis

      Descriptive statistics are reported for categorical variables with mean, standard deviation and range being calculated and reported for continuous and discrete variables. As in the original study, further statistical analysis was performed with the use of non-parametric tests to explore the relationship between variables. Responses were split into two groups based upon responses to rating questions; those rated ‘poor, below average or average’ were assigned to one group (average or below = low rating), while those rated ‘good or excellent’ were assigned to a second group (above average = high rating). Indicative responses to the open ended questions were selected to illustrate the above and below average experiences within each domain.
      Thematic Analysis [
      • Braun & Clarke
      Reflecting on reflexive thematic analysis.
      ] was used to identify patterns of meaning in the open text responses across the dataset, following a rigorous process of data familiarisation, coding, and theme development and revision. Measures to assure the rigour of the data were incorporated, with members of the research team independently undertaking aspects of analysis before coming together to compare and agree coding [
      • Creswell J.
      • Poth C.
      Qualitative Inquiry and Research Design Choosing Among Five Approaches.
      ].

      2.2 Ethics

      Potential participants were provided with a link to an online Participant Information Sheet explaining the purpose of the study and containing contact details for the research team should they have any outstanding questions. The surveys were submitted anonymously but if any identifying features were included, these were removed prior to analysis. Details of organisations to contact should participants be in need of support as a result of taking part in the study were provided. Ethical approval (Ref: SC38) was obtained from the University Faculty of Health & Social Care Research Ethics Committee.

      3. Results

      3.1 Participants

      109 pwMND accessed the survey and 69 submitted completed responses (59 completed by the person themselves, 5 with help of a family caregiver and 5 help not known). Thirty-nine family caregivers completed to the survey. See Table 3 for demographic details.
      Table 3Participant characteristics.
      People with MNDCaregivers
      Number6939
      Mean Age61 (32–89) SD 10.2255 (29–80) SD 12.83
      Male59% (41)22% (8)
      Female39% (27)78% (28)
      Married / partner75% (51)79% (30)
      Education University38% (26)53% (20)
       No formal education3% (2)0% (0)
      In work23% (15)42% (16)
       Retired59% (41)38% (14)
      Time since diagnosis to survey completion (in days)Mean 1378 days (3.78 yrs)

      SD1697 (4.65 yrs)

      Range 8–7097 days

      (8 days to 19.44 yrs)

      Median 849 days (2.3 yrs)
      Mean 714 days (1.95 yrs)

      SD 796 days (2.18 yrs)

      Range 36–3187 days (36 days to 8.73 yrs)

      Median 468 days (1.9 yrs)

      3.2 MND centres

      Twenty (29%) pwMND who responded to the survey received their diagnosis at an MND Centre while 42% (n = 16) of caregivers who responded to the survey reported that their family member was diagnosed at an MND Centre.

      3.3 Ratings of neurologists' abilities/skills and satisfaction with delivery of diagnosis

      The skills and ability of the neurologist was assigned a high rating (above average) or a low rating (average or below). People with MND were more likely than caregivers to provide a high rating for their neurologist's abilities and skills at delivering the diagnosis. Those pwMND seen at an MND Centre were substantially more likely to provide a high rating than those not seen in MND Centres (See Table 4).
      Table 4Satisfaction with skills of neurologist and delivery of the diagnosis.
      pwMND (All)pwMND

      MND Centres
      pwMND

      Not MND Centre
      Caregivers

      (All)
      n6920
      6 respondents were unable to recall if they were seen at an MND centre. They have been assigned to non MND centre group.
      49
      6 respondents were unable to recall if they were seen at an MND centre. They have been assigned to non MND centre group.
      39
      Rating of Skills & Abilities of Neurologist
      Non Response5.80% (4)5% (1)6.12% (3)10.26% (4)
      Above average62.32% (43)85% (17)53.06% (26)43.59% (17)
      Average or below31.88% (22)10% (2)40.81% (20)46.16% (18)
      Satisfaction with Delivery of Diagnosis
      Non Response4.34% (3)6.12% (3)10.26% (4)
      High rating52.17% (36)70% (14)44.9% (22)41.02% (16)
      Low rating43.4% (30)30% (6)48.98% (24)48.72% (19)
      Satisfaction with information Provided at Diagnosis
      No Response5.13% (2)
      Low Rating53.62% (37)40% (8)59.19% (29)48.72% (19)
      High Rating46.38% (32)60% (12)40.82% (20)46.15% (18)
      low asterisk 6 respondents were unable to recall if they were seen at an MND centre. They have been assigned to non MND centre group.
      Satisfaction with the delivery of the diagnosis was given a high rating (above average) or low rating (average or below). People with MND were more likely than caregivers to highly rate their satisfaction with the delivery of the diagnosis. As previously, pwMND seen at an MND Centre were more likely to provide a high rating for satisfaction than those pwMND not seen at an MND Centre (see Table 4).
      There was a correlation between caregivers' rating of skills and ability of the neurologist and caregiver satisfaction with the telling of the diagnosis rs (33) = 0.830, p ≤ 0.005. There was also a correlation between pwMND's rating of the skills and ability of the neurologist and their satisfaction with how the diagnosis was delivered rs (63) = 0.797, p ≤ 0.005.
      Open text responses indicate aspects of the delivery of the diagnosis impacting on level of satisfaction (see Table 5); quotes are labelled with identification codes indicating FC for family caregiver and pwMND for person with MND.
      Table 5Open text responses on aspects of the diagnosis impacting on satisfaction.
      Negative commentsPositive comments
      Anything would have been better, than just a diagnosis, and a see you later we will be in touch” (FC32)‘The MND consultant was gentle and kind when delivering the diagnosis. I felt very supported by him’. (pwMND36)
      “Neurologist just came over as completely uncaring” (FC37).‘[Diagnosis] Delivered in a very professional way but in a very caring and understanding manner’ (pwMND60)
      ‘[MND]Diagnosis is never going to be easy, but this experience set us back psychologically and has made it all much harder to deal with. We felt, and still feel one year on, quite traumatised and angry about how we feel we were treated (FC17).‘He was compassionate and answered our questions to the best of his ability’ (FC15)

      3.4 Timing of appointments

      People with MND stated (n = 57) that the time between the initial onset of symptoms and the first appointment with the General Practitioner varied greatly, with a median of 126 days, (range 12 to 1461 days). The median time from first symptom to being told the diagnosis stated by pwMND (n = 63) was 398 days, (range 35 to 3348).
      Most pwMND (93% n = 64) reported that the diagnosis was given by a neurologist. Thirty-seven (54%) pwMND reported being offered an appointment for further discussion with a team member; this was almost always undertaken by neurologists at MND centres (80%, n = 16) but was only offered to 41% (n = 20) of pwMND where the neurologist was not at an MND centre. The average time before a further appointment with a neurologist was reported by pwMND to be 65.4 days, with a range of 4 to 182 days (median 56). Caregivers provided further insight into the diagnostic process. Many pwMND had been to several appointments before the diagnosis was given; 34% (n = 13) reported one previous appointment, 24% (n = 9) had two appointments, 16% (n = 6) had three appointments and 24% (n = 9) more than three appointments.
      PwMND who rated the skills of the neurologist highly had longer diagnosis appointments (Mean 36.83, SD16.115, n = 41) compared with those pwMND who gave a low rating (21.58, SD10.145, n = 19) (p ≤0.005). PwMND and caregiver ratings of the neurologists' abilities/skills increased as the duration of the diagnostic consultation increased (see Fig. 1) as did their satisfaction with the delivery of the diagnosis (see Fig. 2).
      Fig. 1
      Fig. 1PwMND and carer ratings of the neurologists' abilities/skills against the duration of consultation.
      Fig. 2
      Fig. 2PwMND and carer satisfaction with the delivery of diagnosis against the duration of consultation.

      3.5 SPIKES protocol

      The responses of the pwMND and caregivers when asked about the six aspects of the SPIKES protocol are shown in Table 6. The elements of the protocol reported to be most highly adhered to were in the ‘Settings’ domain. With 95% (n = 65) of pwMND and 87% (n = 33) of caregivers reporting having privacy during diagnosis consultation. However, the domains focused on what they knew (Perception) and what they wanted to know (Invitation) were equally poor for both groups. Only 29% (n = 20) of pwMND and 24% (n = 9) of caregivers were asked how much they knew about MND. With 32% (n = 22) of pwMND and 24% (n = 9) of caregivers being asked how much they wanted to know. Under half of pwMND (46%, n = 32) and caregivers (47%, n = 18) were satisfied with the information they were given at diagnosis (Knowledge).
      Table 6Comparison of SPIKES between pwMND and caregiver. Most questions had a yes/no format and unless otherwise stated in the table the figures refer to a response of ‘yes.’
      pwMNDCaregivers
      Setting
      Privacy94% (65)87% (33)
      No interruptions88% (61)87% (33)
      Perception
      Person asked how much they knew about MND29% (20)24% (9)
      Person wanted details about their condition‘A lot’ 38% (26)

      ‘just enough’ 46% (32)

      ‘a little’ 16% (11)
      ‘A lot’ 24% (9)

      ‘just enough’ 45% (17)

      ‘a little’ 24% (9)
      Invitation
      Asked what they wanted to know about the condition32% (22)24% (9)
      Knowledge
      Satisfied with details provided46% (32)47% (18)
      Emotion
      Diagnosis given with warmth and empathy62% (43)58% (22)
      Strategy
      Referral to MND team54% (37)45% (17)
      Details of MND Association36% (25)32% (12)
      Open text responses provide greater depth to understand respondents' experiences of communication in relation to the SPIKES protocol during the delivery of the diagnosis (see Table 7).
      Table 7Open text responses relating to the SPIKES protocol domains.
      DomainOpen text response
      Setting“I could not believe such devastating news could be given over the phone(FC29)

      “The diagnosis was given, from behind a desk. We were then told to join a queue for a blood test. I had to ask a nurse for a private space as were distraught” (FC17)

      “We needed more time to get used to the news. A smaller room, cup of tea and a nurse would have been good. Was no time to take in the news. MND pack and phone number would have been good. I felt the way they handled it was really bad.” (pwMND32)
      PerceptionHe didn't ask if we understood anything he was explaining and felt uncomfortable when we asked questions.(FC3)

      “Although I knew a lot about MND it was still a shock to get the diagnosis. I was told far too much (i.e. about feeding and breathing tubes, house adaptations) when I was first diagnosed” (pwMND6)

      “Caring, wanting me looked after by a specialist and understanding that I didn't want to know everything about MND straight away” (pwMND22)
      InvitationNo build up to the devastating news” (FC16)

      “The neurologist allowed us to drive the questions and how much we wanted to know. He kept checking whether we were happy to hear stuff. (FC25)

      “The neurologist checked with me throughout to assess how much I was able to take in (I was writing notes as well)” (pwMND39)
      KnowledgeHe wasn't an expert and told us this many times(FC15)

      “The lack of knowledge by both neurologists about MND was apparent. Neither referred me to an MND specialist. (pwMND88)

      “Knowledgeable consultant” (pwMND1)
      EmpathyHe was unemotional in telling us the diagnosis, and he did not show any empathy when I was upset” (FC3)

      “The abruptness! Lack of empathy! Knowing that I was alone the neurologist SHOULD have called his CNS to support me - even for a cup of tea! (pwMND45)

      “The neurologist was very warm and delivered the bad news as kindly as possible. He was honest about the prognosis but made us feel we would be given support (FC39)

      “Very compassionate and not hurried” (FC24)
      StrategyThe worst time of our lives were made worse by not being able to sit down and talk to someone and understand what was happening and what would come next” (FC29)

      “I felt I was left to fend for myself.” (pwMND31)

      “Firm and clear, with reasons. Ongoing care model agreed” (pwMND23)

      3.6 NICE recommendations

      Most pwMND (94%; n = 65) and caregivers (92% n = 35) felt that the neurologist giving the diagnosis was knowledgeable about MND. Few respondents had been asked how much information they wanted to receive about their condition (pwMND 32%, n = 22; caregivers 24% n = 9) but just over half (pwMND 57% n = 39; caregivers 53% n = 17) felt they were given enough detailed information about MND at diagnosis and 46% (n = 32) pwMND and 47% (n = 18) of caregivers were satisfied or very satisfied with the information provided at that time. The average length of the consultation when the diagnosis was given was 32 min (range 10 to 60 min). Almost two thirds of respondents (pwMND 58% n = 39; caregivers 57% n = 17) felt they were allowed enough time to express their emotions and 54% (n = 36) pwMND and 57% (n = 17) caregivers felt they were allowed enough time to have their emotions responded to. More than half of the respondents (pwMND 58% n = 37; caregivers 54% n = 15) reported being offered a follow up appointment with a member of the MDT.
      Although there was usually discussion about the diagnosis itself in neurology clinics the provision of basic information occurred for less than half of the respondents – including discussion of symptoms, treatment and local services and local support. For those seen in the MND Centres there was higher, but not universal, compliance with most of the recommendations (see Table 8). Discussion about legal rights was rarely held and discussion of the need to contact the Driving and Vehicle Licensing Authority (DVLA) was uncommon, except in MND Care Centres, even though MND is a notifiable condition. Advance care planning was also very rare (see Table 8).
      Table 8Compliance with NICE guideline NG42 comparison with MND centres and non-MND centres.
      People with MND

      Dx NOT at MND centre
      People with MND

      Dx at MND Centres
      Caregivers Dx NOT at MND centreCaregivers Dx at MND centre
      Not sure/ do not recall = 4Not sure/ do not recall = 2
      N = 43N = 20N = 20N = 16
      What is MND
      How diagnosis was reached

      Certainty of diagnosis
      70% (26)

      65% (28)
      80% (16)

      70% (14)
      60% (12)

      55% (11)
      81% (13)

      56% (9)
      Types / causes of MND
      Prognosis42% (18)60% (12)40% (8)56% (9)
      Symptoms31% (11)40% (8)25% (5)31% (5)
      Disease Progression43% (15)60% (12)40% (8)38% (6)
      Treatment46% (16)60% (12)30% (6)38% (6)
      Place of appointment56% (21)75% (15)55% (11)38% (6)
      HCPs involved in care51% (17)75% (15)40% (8)19% (3)
      Local services36% (9)65% (13)25% (5)31% (5)
      Local support21% (5)40% (8)20% (4)25% (4)
      Legal rights4% (1)10% (2)3% (1)0% (0)
      DVLA33% (9)60% (12)20% (4)13% (2)
      ACP7%20% (4)11% (2)6% (1)

      4. Discussion

      The telling of the diagnosis to someone with MND is known to be difficult not only for the pwMND and their family but also for the neurologist [
      • Aoun S.M.
      • Breen L.J.
      • O’Brien M.
      • O’Connor M.
      “The shock of diagnosis”: qualitative accounts of people with Motor Neurone Disease reflecting the need for more person-centred care.
      ,
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ,
      • Aoun S.M.
      • Breen L.J.
      • Edis R.
      • Henderson R.D.
      • Oliver D.
      • Harris R.
      • Howting D.
      • O’Connor M.
      • Birks C.
      Breaking the news of a diagnosis of motor neurone disease: a national survey of neurologists’ perspectives.
      ,
      • O’Connor M.
      • Aoun S.
      • Breen L.
      Australian family carer responses to the experience of a loved one receiving a diagnosis of Motor Neurone Disease - ‘our life has changed forever’.
      ]. There is evidence of improvement in delivery of the diagnosis over the last 30 years, but many patients had not understood what they had been told or knew what to expect in the future and more than half were alone when they received their diagnosis [
      • Johnston B.
      • Earll L.
      • Mitchell E.
      • et al.
      Communicating the diagnosis of motor neurone disease.
      ]. One study found that in some instances patients and families had bad experiences at the time of diagnosis, such as being told in a large group of professionals, during a ward round, a lack of time afterwards and poor communication skills [
      • O’Brien M.R.
      • Whitehead B.
      • Jack B.A.
      • Mitchell J.D.
      From symptom onset to a diagnosis of amyotrophic lateral sclerosis/ motor neuro disease (ALS/MND): experiences of people with ALS/MND and family carers – a qualitative study.
      ]. There have been several publications and guidelines suggesting ways of improving communication during diagnosis delivery [
      • Borasio G.D.
      • Sloan R.
      • Pongratz D.E.
      Breaking the news in amyotrophic lateral sclerosis.
      ,
      • Miller R.G.
      • Jackson C.E.
      • Kasarkis E.J.
      • England J.D.
      • Forshew D.
      • Johnston W.
      • et al.
      Practice Parameter update. The care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/ behavioural impairment (an evidence-based review).
      ].
      This study did show that there are still many issues that need to be addressed. There are certainly contrasts to the results in Australia [
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ,
      • Aoun S.
      • Breen L.J.
      • Oliver D.
      • Henderson R.D.
      • Edis R.
      • O’Connor M.
      • Howting D.
      • Harris R.
      • Birks C.
      Family carers’ experiences of receiving the news of a diagnosis of Motor Neurone Disease: a national survey.
      ], with substantially fewer caregivers satisfied with the delivery of the diagnosis in our study. Although it is possible that our findings contain some pwMND/carer dyads, as a result of the anonymous nature of the survey we cannot be certain if the caregivers are linked to the pwMND who responded or if they were less satisfied because they are representing a different cohort of pwMND who had a worse experience. The gender difference in the study between the pwMND and caregivers was large (see Table 3). There were more female caregivers in this study (78%) and more male pwMND (59%). This may have influenced the differences observed in the satisfaction rates between pwMND and their caregivers as studies have shown the important difference gender makes in perceptions of the quality of care [
      • Schnitzer S.
      • Kuhlmey A.
      • Adolph H. Holzhausen
      • Schenk J.
      • L.
      Complaints as indicators of health care shortcomings: which groups of patients are affected?.
      ,
      • Teunissen T.A.M.
      • Rotink M.E.
      • Lagro-Janssen A.L.M.
      Gender differences in quality of care experiences during hospital stay: a contribution to patient-centered healthcare for both men and women.
      ].
      However, there were similarities with just over two-thirds of pwMND in both studies rating neurologists' abilities and skills at delivering the diagnosis as above average [
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ]. Our satisfaction results are also lower than in Germany [
      • Abdulla S.
      • Veielhaber S.
      • Machts J.
      • et al.
      Information needs and information-seeking preferences of ALS patients and their carers.
      ] and Italy [
      • Palese F.
      • Gigli G.L.
      • Manganotti P.
      • Passadore P.
      • Rana M.
      • Verriello L.
      The diagnostic, therapeutic and assistance pathway for amyotrophic lateral sclerosis in a north-eastern Italian region: satisfaction of patients and their caregivers.
      ]. However, in our study there was an increased level of satisfaction when pwMND were seen at an MND Centre.
      The SPIKES criteria were generally not met. However, there was a positive response rate for the provision of privacy and ensuring there were no interruptions, which does show improvement on earlier studies [
      • O’Connor M.
      • Aoun S.
      • Breen L.
      Australian family carer responses to the experience of a loved one receiving a diagnosis of Motor Neurone Disease - ‘our life has changed forever’.
      ]. The Perception aspect was not well addressed, while the Emotion aspects were more positively reported. The Strategy aspect, in particular information about the MND Association, was lower than the situation in Italy [
      • Palese F.
      • Gigli G.L.
      • Manganotti P.
      • Passadore P.
      • Rana M.
      • Verriello L.
      The diagnostic, therapeutic and assistance pathway for amyotrophic lateral sclerosis in a north-eastern Italian region: satisfaction of patients and their caregivers.
      ], and in Australia [
      • Aoun S.M.
      • Breen L.J.
      • Howting D.
      • Edis R.
      • Oliver D.
      • Henderson R.
      • O’Connor M.
      • Harris R.
      • Birks C.
      Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better?.
      ] where more pwMND were referred to an MND Association at diagnosis.
      Many of the recommendations from the NICE guideline NG42 were generally not met but compliance was higher in the MND Care Centres. The survey was undertaken three years after the NICE guideline [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ] had been issued and it is disappointing that compliance with the recommendations was limited. The respondents may have been receiving the diagnosis when the NICE guideline [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ] was still newly issued and the recommendations may now be more widely known and adhered to though a recent UK study highlighted that 75% of neurologists were not following any specific guidelines or protocols at diagnosis [
      • Anestis E.
      • Eccles F.J.R.
      • Fletcher I.
      • Simpson J.
      Neurologists’ current practice and perspectives on communicating the diagnosis of a motor neurodegenerative condition: a UK survey.
      ]. Without appropriate strategies to alter behaviour, changes are likely to be slow and there has been no additional funding allowing extra time for clinic appointments or extra staff to facilitate these new requirements.
      It has been suggested that there is the need to develop a programme to facilitate implementation to ensure that guidelines are understood and then used [
      • Grol R.
      • Buchan H.
      Clinical guidelines: what can we do to increase their use?.
      ]. Guidelines are based, where possible, on randomised clinical trials and concerns have been expressed that these trials, and thus the guidelines, may be seen as less relevant in day to day practice [
      • Chong C.C.N.
      Pros and cons of clinical practice based on guidelines.
      ]. Thus, it would seem that there is a need to prioritise the implementation of the recommendations of the NICE guideline NG42, alongside increased information and funding. Furthermore, this needs to be accompanied by training in a person-centred approach to delivering bad news. This holistic approach ensures that delivery of the diagnosis acknowledges the individual's psychosocial, spiritual and emotional needs in addition to their medical and practical concerns [
      • Breen L.
      • Aoun S.
      A person-centered approach to breaking bad news.
      ]. Empathy training is crucial for any success in implementing best practice guidelines, more so than funding or additional personnel [
      • Breen L.
      • Aoun S.
      A person-centered approach to breaking bad news.
      ]. Regular audit would also allow deficiencies to be identified.
      The study shows that the care provided at specialist MND Care Centres was of a higher standard than in routine neurology clinics. There is increasing evidence that the multi-disciplinary team (MDT) approach improves the care of pwMND, can be beneficial in supporting them to help cope with the effects of the illness, and may even increase survival [
      • O’Brien M.R.
      • Whitehead B.
      • Jack B.A.
      • Mitchell J.D.
      Multidisciplinary team working in motor neurone disease: the views of patients and family carers.
      ,
      • Aridegbe T.
      • Kandler R.
      • Walters S.J.
      • et al.
      The natural history of motor neuron disease: assessing the impact of specialist care.
      ,
      • Rooney J.
      • Byrne S.
      • Heverin M.
      • et al.
      A multidisciplinary clinic approach improves survival in ALS: a comparative study of ALS in Ireland and Northern Ireland.
      ]. The exact nature of this effect is unclear but would seem to be due to the close collaboration and communication within the MDT and was a major recommendation within the NICE guideline [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ]. Although not all pwMND are able to attend an MND Centre throughout their disease progression, the initial assessment, diagnosis and telling of the diagnosis is a very important time for a specialist team to be involved.

      5. Strengths and limitations

      There are limitations to this study. The number of respondents was small and may reflect a biased group of people, who were willing to respond to the survey, as they may have experienced poor care and this had stimulated their response.
      Whilst we do not claim the findings to be generalisable, the study was open to pwMND and their caregivers throughout the UK and participant characteristics broadly reflect the wider MND population and responses include the views of people from a wide range of geographical locations. The qualitative study of the free text responses also give strength to, and support, the quantitative results.

      6. Conclusion

      The study has shown that there is still a need for neurologists and neurology services to be more aware of the needs of pwMND and their caregivers when they are told the diagnosis. The fears of the disease and the possibility of a distressing death can be reduced by clear and honest communication, which will also build up trust, allowing open communication throughout the disease progression. The NICE guideline [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ] clearly recommends how the discussion should be managed and is based both on the evidence and the personal experiences of a wide range of professionals, pwMND and their caregivers. There is a need to increase awareness of the NICE guideline [
      • National Institute for Health and Care Excellence
      Clinical Guideline NG42. Motor neurone disease: assessment and management.
      ], and ensure that there is adequate training, time, personnel and funding to allow the conversations to be most effective and acceptable for pwMND and their caregivers. Regular audit of the recommendations may be a stimulus for change. The study has shown that the experiences of pwMND and their caregivers is better at MND centres and where possible people that have suspected MND should be referred to an MND centre to ensure correct diagnosis, communication and follow up.

      Acknowledgements

      The authors would like to acknowledge the MND Association, MND Scotland and the UK MND Clinical studies Group for their assistance in raising awareness of the study.

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