Patients’ perceptions of services and preferences for care in
amyotrophic lateral sclerosis. A review.
Foley, Geraldine, Timonen, Virpi and Hardiman, Orla – all of Trinity College Dublin
Published in Amyotrophic Lateral Sclerosis 2012
Acknowledgements:
This work was funded by the Health Research Board (HRB) of Ireland
Bios
Geraldine Foley, BScOT, MScOT, is a HRB research fellow and a doctoral student at
Trinity College Dublin, Ireland.
Virpi Timonen, BA, MPhil, DPhil, is a professor of social policy and ageing at Trinity
College Dublin, Ireland.
Orla Hardiman, BSc, MBBCh, BAO, MD, is a professor of neurology and a HRB
clinician scientist at Trinity College Dublin, Ireland, and a consultant neurologist at
Beaumont Hospital Dublin, Ireland.
1
Abstract
Background: Service providers and service users often have different perspectives
on health and social care services.
Design: We have undertaken a systematic review of empirical data between 1988 to
March 2011 relating to ALS service users’ perspectives on health and social care
services. Forty-seven texts were extracted and a narrative synthesis conducted.
Results: Few studies have explored ALS patients’ experiences in relation to their
satisfaction with services. ALS patients expect dignified care but they are often
dissatisfied with health care services and have unmet expectations of their care.
Most studies of decision-making and preferences for care have focussed on end-oflife intervention. Various factors influence preferences for care from the service user
perspective and people with ALS may adjust their use of services as they negotiate
change.
Conclusions: Further research on the timeliness of services to meet changing needs
of service users is required. The service user experience of allied health care services
prior to end-of-life care also warrants investigation. Service providers need to
support people with ALS as they negotiate feelings of acceptance and independence.
Research to identify the key parameters of the ALS patient experience of services is
required.
Key words:
Review, services, care preferences, decision- making, multidisciplinary care
2
Introduction
The goal of health care services in ALS is to provide prompt and user orientated care
based on clinical need (1). The role of health care professionals is to improve quality
of life for service users and support them throughout the course of living with ALS
(2). Models of care that are client-centred and responsive to the evolving nature of
the condition are considered best practice for people with ALS (3).
There is a general consensus that optimal care in ALS should comprise services that
support physical, psychological, social and existential needs of service users and their
carers (4-6). The ALS service user has a pivotal role in the caring process and his or
her experiences of care are central to the care approach in ALS. However, despite
research on the benefits of multidisciplinary care in ALS (7-9) few studies have
explored the delivery of service from the user’s perspective.
Care outcomes are often influenced to a greater extent by service users’ perceptions
of care and not necessarily by care providers’ intentions regarding care (10). Users
and providers of palliative services can hold different perspectives on the benefits
and outcomes of care (11). Service users’ understandings of care are idiosyncratic,
based on a combination of personal experiences and ‘common sense’ knowledge
about illness (12). Appreciation of ALS service users’ perspective about services and
attention to their preferences for care is an important component in the quest to
provide better outcomes for service users.
This review examines recent literature from the ALS service user perspective
(experiences and expectations) on health and social care services. The primary aims
are to draw attention to factors that may influence perceptions of and preferences
for care, and to outline research on how people with ALS use services as they
negotiate change in their health and lives.
Method
Search strategy
3
A review of empirical data published in full and in English between 1988 to March
2011 on the ALS service user perspective on services, was undertaken. Databases
used included; Medline, Cinahl, AMED, PsychInfo, Evidence Based Medicine Reviews,
and Web of Science. Search terms used included; ‘amyotrophic lateral sclerosis’ or
‘motor neurone disease’ and/or ‘services’, ‘healthcare’, ‘experiences’, ‘expectations’,
‘satisfaction’, ‘decision-making’, ‘perceptions’, ‘perspectives’ and ‘preferences’.
Separate electronic manual searches (including early online first articles where
available) in the following palliative care journals; American Journal of Hospice &
Palliative Medicine, European Journal of Palliative Care, Journal of Hospice &
Palliative Nursing, International Journal of Palliative Nursing, Journal of Pain &
Symptom Management, Journal of Palliative Medicine, Palliative & Supportive Care,
Journal of Palliative Care and Palliative Medicine were also undertaken covering the
same period and using the search terms ‘amyotrophic lateral sclerosis’ and ‘motor
neurone disease’.
Selection criteria
We included only studies where patients themselves reported on their care or where
patients, who did not report directly on their care, completed standardised
measures which were then used to investigate factors associated with their choice of
care. We included all of the above studies where published in full irrespective of
study design. We excluded studies which reported only the views of carers and/or
service providers on services. In addition, we excluded studies not published in the
English language. Although structured approaches exist to judge the strength and
reliability of quantitative and qualitative research to determine exclusion criteria for
analysis, no criteria for further exclusion was applied owing to the paucity of data
found on the topic.
Synthesis of data
Because of the diversity of study designs, a statistical synthesis was not appropriate
and a narrative synthesis was undertaken (13). The first author conducted a
preliminary analysis of each selected study, critically appraised each study and then
4
explored similarities and differences between studies. All authors then synthesised
and interpreted the evidence as it related to the purpose and aims of the review.
Findings
In total, using the above search terms, 47 texts from 43 studies were found which
report from the ALS service user perspective on health and social care services. For
clarity and purpose, we have categorised these studies under the following headings;
‘Overall views, experiences and expectations of health care services’, ‘Use of
assistive devices’, ‘Communicating the diagnosis’, and ‘End-of-life decisions’. Studies
are also summarised in Table no. 1.
Overall views, experiences and expectations of health care services
A number of studies found that the primary expectations of service users were
emotional support, timeliness of care and access to multidisciplinary care (14-17).
Some service users expected more information than that which was provided to
them (18). A Scottish study (19) showed that only 25% of patients felt that their
needs had been met. Problems identified in relation to care included long waiting
periods for multidisciplinary care. The majority of respondents also accessed
voluntary sector services to alleviate burden of care. These findings are consistent
with an Irish study (20) on access to community based services. They are also
consistent with a survey of home care needs of ALS patients in Ohio, USA (21) where
the majority of patients did not receive services.
A number of investigators have performed qualitative studies on the meaning of
care for ALS service users (22-26). Service users’ primary expectations centred on the
need to be valued by health care professionals and acknowledged independently of
their disease (22). Service users expressed concerns about inadequate emotional
support (23) and a lack of continuity and co-ordination within services (23,24). The
effort in developing and maintaining relationships was often perceived as the
responsibility of service users as opposed to service providers (23,25). However, an
Irish qualitative study found that service users expressed satisfaction with services
(26).
5
UK based qualitative studies have also explored expectations about care and
compared these with care received (27-29). Most service users felt that services
were deficient in providing timely and co-ordinated care. Problems identified by
service users included the absence of care co-ordinators, limited access to
specialised equipment, and a lack of knowledge about ALS among health care
professionals. Service users had concerns about professionals’ detached approach
and their lack of knowledge about ALS, which for many service users resulted in
problems accessing services and receiving poorly timed care. However, an audit on
service users’ perceptions of hospice based clinics co-ordinated through a UK based
tertiary ALS centre reported satisfaction with services (30).
Use of assistive devices
A qualitative study was performed in Scotland to investigate perceptions of
augmentative and alternative communication (ACC) among ALS service users and
their communication partners (31). Whilst service users indentified benefits of ACC,
they also expressed a strong desire to use their own speech even if it was
unintelligible to others. The majority of participants found ACC unsuccessful as it
impacted on close personal interaction in real life settings. There was also
dissatisfaction among service users with respect to the timely provision of ACC and
inadequate support and training to use ACC devices.
Studies of assistive devices at two different ALS clinics in the United States have
found that service users expressed satisfaction with wheelchair mobility and
indicated that it impacted positively on their lives by facilitating independence and
promoting well-being (32,33). Similarly, the use of telemedicine and an integrated
home based cough assist programme was associated with high levels of satisfaction
(34-37). However, despite satisfaction with telemedicine, some service users may
still value face-to-face contact when discussing psychological and emotional
concerns (37).
Communicating diagnosis
6
A survey was undertaken at a large Italian ALS specialised centre to investigate how
ALS service users seek information and rate their satisfaction with how physicians
communicate the diagnosis of ALS (38). Investigators found that even though service
users experienced negative feelings about their diagnosis, most participants sought
information about ALS and were satisfied with how physicians communicated the
diagnosis to them. These findings are consistent with an audit on service users’
experience of a fast-track system for diagnosing ALS at a specialised centre in
Preston, UK (39). However, they are contrary to a qualitative study which explored
patient care experiences during the period between symptom onset and diagnosis.
Participants expressed dissatisfaction with how information was delivered and
complained about a lack of knowledge about ALS among physicians to recognise
early symptom presentation (40). They are also contrary to findings from large scale
survey research in the United States where patients expressed dissatisfaction with
communication of the diagnosis and with the accuracy of the diagnosis received
(41,42).
End-of-life decisions
Studies of ALS service users’ preferences for care have focussed primarily on
decision-making regarding end-of-life treatment (cardio-pulmonary resuscitation,
ventilation and enteral feeding) and in patient assisted suicide (43-59).
Service users demonstrate ability to express preferences for care and follow up on
these preferences. Desire for information and participation in decision-making about
care can remain stable but preferences for life sustaining treatments may change.
Some participants who are initially in favour of cardio-pulmonary resuscitation later
opted against it and others changed from declining resuscitation to opting for it.
Changes in service users’ care preferences were independent of functional status,
economic and demographic characteristics. Preferences for care were determined in
part by service users’ attitudes to life: those who demonstrated a greater
attachment to life were more likely to form preferences in favour of life sustaining
treatment (43-49).
7
Decisions to avail of assisted ventilation may be determined by service users’ view
on the potential for assisted ventilation to improve quality of life, their ability to
control discontinuation of ventilation and the likelihood of ventilator support to
extend their life (50). Factors which were found to have discouraged the use of
mechanical ventilation include the potential adverse effects on quality of life,
resignation to the fatality of ALS, likelihood of increased burden of care and the
impact of burden of care on family and carers (50-52).
Some service users who underwent emergency long-term ventilation (tracheostomy)
without advance directives in place may not have chosen long-term ventilation had
they been aware of ensuing burden of care (53). Education about mechanical
ventilation was a key factor in determining indecision or regret with respect to
choosing mechanical ventilation in that service users who received advice and
information were more likely to have no regret than those who do not receive
sufficient information (52). ALS patients were also somewhat more ambiguous
towards assisted ventilation than patients with other neuromuscular diseases (54).
Positive attitudes were noted among service users who chose ventilation because
despite the physical limitations imposed by ventilation, service users valued the
extended lifespan it offered (44,45,50). Despite service users’ clarity about why they
chose or would choose ventilator support some participants did not actively seek
medical information about the benefits and/or contra-indications of ventilation (50).
Decisions to opt for, not use or discontinue ventilation were not based on the
expected benefits of medical intervention alone but were also influenced by
conflicting emotions, life situation and family support (55,56). Choosing long-term
ventilation to sustain life was associated with service users’ own perceptions of their
health status and appraisal of life situations (50,56). However whilst participants
valued additional life span afforded by ventilation, some also recognised the
limitations of ventilation on sustaining quality of life (45,47,56).
Feelings of hopelessness among service users predicted or influenced preferences
for patient-assisted suicide (57,58). Service users who expressed interest in patient8
assisted suicide had greater distress and a lower quality of life than those who did
not wish to hasten death. Maintaining control in making decisions about end-of-life
care was also important for ALS patients. Some patients preferred to make decisions
independently or at least favoured shared decision making with family and/or
service providers. Control preferences also remained stable over time (59,60).
In summary, a number of different methods have been used to investigate ALS
service users’ views on and preferences for care. Qualitative investigation has
explored the meaning of services for users at an individual level (14,17,2229,31,40,50,55,56). Experiences have also been captured by a combination of
national surveys on ALS and related services (19,20); by a large multiple regional
survey on services for people with progressive neurological disorders (including ALS)
(15-16); by a regional survey on home care services (21); by regional and clinic based
surveys of communication preferences (38,39,41); and by an international survey
specific to perspectives on misdiagnosis (42). A number of quantitative crosssectional (45,57,58), quantitative longitudinal, (prospective) (43,44,46,47,49,59) and
retrospective (48) studies, and mixed method studies (51,52) have focussed on the
relationship between variables that may influence and/or predict service users’
preferences for care and treatment choices in ALS.
Overall, few studies have investigated the experiences and perspectives of ALS
service users on services. Some studies include other diagnostic groups (1517,20,54,59)
and
many
studies
include
carers
(15-18,23,27-
29,31,38,40,41,44,55,57,60) or if not counted within their sample, included carers’
views in their analysis (19,21,30,34,35,47,49,51-53,56). Most studies are based in
Europe and North America. Sample sizes are small and in most cases findings are not
generalisable to the ALS population. No standardised patient questionnaires on
health and social care services have been used. Only one study (15,16) has used a
generic patient satisfaction with care measure and a small number of studies
(32,33,37,38) have measured satisfaction with specific aspects of care only. The
majority of data is derived from interviews and is descriptive in nature. Some
findings are contradictory and may be attributed to small sample sizes, purposeful
9
sampling procedures, bias within interpretation and differences between health care
systems. There are no control studies and results from qualitative investigation have
not been used to construct survey instruments for use with randomly selected
populations. The parameters of ALS service users’ experiences remain to be more
fully mapped before they can become subjects of large-scale quantitative research
focused on the key aspects of their health care experiences.
Nonetheless, despite variation in design, there are common findings across studies.
Multidisciplinary care is often subsidised by the voluntary sector to compensate for
gaps within the public service sector (19,20). Service users have expectations for
dignified care and seek multidisciplinary intervention to meet a combination of
physical, emotional and social needs (14-17,22-29,40,50). ALS service users report
satisfaction with assistive devices (32-37). However, they are often dissatisfied with
delays in diagnosis and with how the diagnosis is communicated to them (24,28,4042). Service users may seek information to make choices about care (38,43) but feel
that they do not receive sufficient information (18,27-29,40). Service users have
uncertainty in relation to accessing care, feel health care professionals lack
knowledge about ALS, view services as fragmented and perceive services inadequate
to alleviate their burden of care (15-17,19,21,27-29,40). Some service users also view
service providers disconnected from the challenges posed by ALS and inattentive to
the personal losses incurred by service users (23,28,40).
Whether service users opt for or decline services or change their preferences for
services, the decision making process of ALS service users about services is complex a multitude of factors may influence how service users make decisions about care. It
is clear that the degree of physical disability has little effect on service users’
perceptions of these services (43,46-48,50). A combination of personal values
(44,47,49,50,56), desire to maintain control (29,45,50,59,60), perceptions about
quality life (45,47,50,56,57), care burden (50-56) and psychological distress (56-58)
are likely to influence service users’ preferences for care. Though service users’
preferences for care may determine their treatment choices (46), service users may
also have conflicting feelings about care (52-55). Preferences may also change over
10
time (43,46) in order to accommodate to support systems and evolving perspectives
about living with illness.
Discussion
Overall, the literature reviewed here suggests that ALS service users experience and
decide about care in the context of both intrinsic (personal) and extrinsic (external)
support systems. The ALS service user experience of care is multi-dimensional. ALS
patients have diverse needs and their expectations of services include psychological
and social support and not physical care alone.
Expectations of and dissatisfaction with services for ALS service users are consistent
with other progressive neurological groups including Multiple Sclerosis, Parkinson’s
Disease and Huntington’s Chorea (15,16) and when service users with progressive
neurological illness seek psychological and social support in addition to physical care
(61,62). How ALS service users approach health care services is also consistent with
studies in palliative care. Service users with life-limiting conditions negotiate
between independence (control) and dependence (acceptance) in terms of when
and how they choose health care services (63). For some service users, negotiation
may involve communicating preferences for care to extend life (64). It may also
involve making choices about care with regard to quality of life but not necessarily
extension of life (11). Negotiation for service users may also occur when they seek
accurate information but do not necessarily consider the reality of their prognosis
(65) and when their own perceptions about care burden and outcomes of treatment
determine their preferences for care (66). Overall, primary expectations of care
centre on dignity and a genuine understanding on behalf of service providers (67).
Most ALS service users engage with health care services. How they view and make
choices about care is grounded primarily in how they interpret their own lives and
judge potential benefits of care. In a study to identify what people with ALS view as
meaningful in their lives, researchers found that people with ALS shift their focus
from health issues to supportive relationships. They also found that severity of
illness and degree of functional impairment may have little impact on what ALS
11
service users find most meaning in and attach importance to (68). Research also
suggests that ALS service users may shift towards self-transcendence values
(benevolence, self direction and universalism) in order to cope with progressive
illness. Conservation values (safety, harmony and security) may also influence quality
of life for people with ALS (69). This suggests that services that focus on providing
security and maintaining the status quo may improve subjective quality of life for
service users.
This review focuses only on the service user and has not reported from the view
point of the carer (60) or service provider (28) (including views from carers and/or
service providers within the studies extracted), who may influence the experience of
care for service users. This is because the objective has been to place the service
user at the centre of the enquiry, as they are the most reliable informants regarding
their own health care experiences. However, from the literature reviewed, it is
evident that service users’ perceptions about care burden may well have an impact
on how they make decisions about care (50-56).
Conclusions
The literature suggests that patients’ perceptions of care are fundamentally similar
but there are some contradictions between studies. The majority of investigations on
the ALS patient perspective on health care services have been undertaken in Europe
and North America. It would be useful to explore the impact of different health care
systems, if any, on patient satisfaction with services to enhance our understanding of
the ALS patient perspective on services.
Studies which have examined service users’ preferences for care are few. Of those
conducted, studies have predominantly focused on decision-making and preferences
for care in relation to end-of-life treatments i.e. ventilation and enteral feeding. Few
studies report directly on why or how service users decide to engage with allied
health and social care services for non end-of-life treatments. Greater focus on
service users’ preferences for and decision-making in services prior to end-of-life
12
care is required to complement our current knowledge base, and to improve the
user/service provider relationship.
ALS service users have expectations of a broad range of services, and their
expectations about life may also change. Service users adapt to progressive disability
and place progressively less emphasis on physical function and more emphasis on
psychological, social, and existential well-being. There is a dearth of studies which
focus on how services should be delivered to match service users’ shift. Further
research on timeliness and flexibility of services that fit with changing views of
service users as they progress along the disease trajectory in ALS is required.
The literature suggests that ALS service users have expectations around the need to
exert control and be autonomous in their decisions about care. This poses many
challenges for service providers as service users’ negotiation between acceptance
and independence also requires careful negotiation on behalf of service providers as
they advise and participate in the decision-making process about care. As for other
terminally ill service users, people with ALS make decisions about care as they adapt
to change. The processes of decision-making and factors that guide them warrant
further investigation.
Further research is required to pinpoint the key dimensions of the ALS patient
experience of health care services. It not yet apparent what methods best capture
patients’ experiences of services. We believe that an improved understanding of the
parameters of ALS patients’ experience of services (through qualitative research)
would unearth relevant domains of satisfaction with services. A new instrument
scale/measure for measuring ALS patient satisfaction with services should include
relevant domains of satisfaction with services and be used in larger (randomised
control) studies on ALS patient satisfaction with services.
Conflict of Interest
The authors report no conflict of interest.
13
References
1. Mitsumoto H, Rabkin J. Palliative care for patients with amyotrophic lateral
sclerosis. “Prepare for the worst and hope for the best”. JAMA. 2007; 298:
207-216
2. Oliver D, Borasio GD, Walsh D, editors. Palliative care in amyotrophic lateral
sclerosis. From diagnosis to bereavement. 2nd ed. Oxford: University Press,
2006
3. Hardiman O, Traynor BJ, Corr B, Frost E. Models of care for motor neurone
disease: setting standards. Amyotroph Lateral Scler Other Motor Neuron
Disord. 2002; 3: 182-185
4. Andersen PM, Borasio GD, Dengler R, Hardiman O, Kollewe K, Leigh PN et al.
Good practice in the management of amyotrophic lateral sclerosis: clinical
guidelines. An evidence-based review with good practice points. EALSC
Working Group. Amyotroph Lateral Scler. 2007; 8: 195-213
5. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W et al.
Practice parameter update: the care of the patient with amyotrophic lateral
sclerosis: drug, nutritional, and respiratory therapies (an evidence-based
review): report of the Quality Standards Subcommittee of the American
Academy of Neurology. Neurology, 2009a; 73: 1218-1226
6. Miller RG, Jackson CE, Kasarskis EJ, England JD, 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): report of the
Quality Standards Subcommittee of the American Academy of Neurology.
Neurology. 2009b; 73: 1227-1233
7. Traynor BJ, Alexander M, Corr B, Frost E, Hardiman O. Effect of a
multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a
population based study. J Neurol Neurosurg Psychiatry. 2003; 74: 1258-1261
8. Chio A, Bottacchi A, Buffa C, Mutani R, Mora G. Positive effects of tertiary
centres for amyotrophic lateral sclerosis on outcome and use of hospital
facilities. J Neurol Neurosurg Psychiatry. 2006; 77: 948-950
14
9. van den Berg JP, Kalmijn S, Lindeman E, Veldink JH, de Visser M, Van der Graff
MM et al. Multidisciplinary ALS care improves quality of life in patients with
ALS. Neurology. 2005; 65: 1264-1267
10. Normand C. Measuring outcomes in palliative care: limitations of QALYs and
the road to PaIYs. J Pain Symptom Manage. 2009; 38: 27-31
11. Rodriguez KL, Young AJ. Patients’ and healthcare providers’ understanding of
life-sustaining treatment: Are perceptions of goals shared or divergent? Soc
Sci Med. 2006; 62: 125-133
12. Ziebland S, Herxheimer A. How patients’ experiences contribute to decision
making: illustrations from DIPEx (personal experiences of health and illness). J
Nurs Manage. 2008; 16: 433-439
13. Mays N, Pope C, Popay J. Systematically reviewing qualitative and
quantitative evidence to inform management and policy-making in the health
field. J Health Serv Res Policy. 2005; 10(Suppl.1): 6-20
14. Beisecker AE, Cobb AK, Ziegler DK. Patients’ perspectives of the role of care
providers in amyotrophic lateral sclerosis. Arch Neurol. 1988; 45: 553-556
15. Kristjanson LJ, Aoun SM, Oldham L. Palliative care and support for people
with neurodegenerative conditions and their carers. Int J Palliat Nurs. 2005;
12: 368-377
16. Kristjanson LJ, Aoun SM, Yates P. Are supportive services meeting the needs
of Australians with neurodegenerative conditions and their families? J Palliat
Care. 2006; 22; 151-157
17. McCabe MP, Roberts C, Firth L. Satisfaction with services among people with
progressive neurological illnesses and their carers in Australia. Nurs Health
Sci. 2008; 10: 209-215
18. Wicks P, Frost J. ALS patients request more information about cognitive
symptoms. Eur J Neurol. 2008; 15: 497-500
19. van Teijlingen ER, Friend E, Kamal AD. Service use and needs of people with
motor neurone disease and their carers in Scotland. Health Soc Care
Community. 2001; 9: 397-403
15
20. Hardiman O, Corr B, Frost E, Gibbons P, Mahon L, Traynor BJ. Access to health
services in Ireland for people with multiple sclerosis and motor neurone
disease. IMJ. 2003; 96: 200-203
21. Krivickas LS, Shockley L, Mitsumoto H. Home care of patients with
amyotrophic lateral sclerosis. J Neurol Sci. 1997; 152(Suppl. l): 82-89
22. Bolmsjo I. Existential issues in palliative care: interviews of patients with
amyotrophic lateral sclerosis. J Palliat Med. 2001; 4: 499-505
23. Brown JB. User, carer, and professional experiences of care in motor neurone
disease. Prim Health Care Research Devel. 2003; 4: 207-217
24. Hugel H, Grundy N, Rigby S, Young CA (2006). How does current care practice
influence the experience of a new diagnosis of motor neurone disease? A
qualitative study of current guidelines-based practice. Amyotroph Lat Scler.
2006; 7: 161-166
25. Hocking C, Brott T, Paddy A. Caring for people with motor neurone disease.
Inter J Therapy Rehab. 2006; 13: 351-355
26. Foley G, O’Mahony P, Hardiman O. Perceptions of quality of life in people
with ALS: effects of coping and heathcare. Amytroph Lat Scler. 2007; 8: 164169
27. Brown JB, Lattimer V, Tudball T. An investigation of patients’ and providers’
views of services for motor neurone disease. Br J Neurosci Nurs. 2005; 1: 249252
28. Hughes RA, Sinha A, Higginson I, Down K, Leigh PN. Living with motor
neurone disease: lives, experiences of services and suggestions for change.
Health Soc Care Community. 2005; 13: 64-74
29. O’Brien MR, Whitehead B, Murphy PN, Mitchell JD, Jack BA. Social services
homecare for people with motor neurone disease/amyotrophic lateral
sclerosis: why are such services used or refused? Palliat Med. 2011(a) DOI:
10.1177/0269216311398697
30. Bennett W, Mitchell D, Callagher P. Developing hospice-based, nurse-led
clinics to improve care for patients with MND. Br J Neurosci Nurs. 2009; 5:
231-234
16
31. Murphy. “I prefer contact this close”: perceptions of AAC by people with
motor neurone disease and their communication partners. ACC. 2004; 20:
259-271
32. Trail M, Nelson N, Van JN, Appel SH, Lai EC. Wheelchair use by patients with
amyotrophic lateral sclerosis: a survey of user characteristics and selection
preferences. Arch Phys Med Rehabil. 2001; 82: 98-102
33. Ward AL, Sanjak M, Duffy K, Bravver E, Williams N, Nichols M et al. Powered
wheelchair prescription, utilisation, satisfaction, and cost for patients with
amyotrophic lateral sclerosis: preliminary data for evidence-based guidelines.
Arch Phys Med Rehabil. 2010; 91: 268-272
34. Vitacca M, Comini L, Tentorio M, Assoni G, Trainini D, Fiorenza D et al. A pilot
trial of telemedicine-assisted, integrated care for patients with advanced
amyotrophic lateral sclerosis and their caregivers. J Telemed Telecare.
2010(a); 16: 83-88
35. Vitacca M, Paneroni M, Trainini D, Bianchi L, Assoni G, Saleri M et al. At home
and on demand mechanical cough assistance program for patients with
amyotrophic lateral sclerosis. Am J Phys Med Rehabil. 2010(b); 89: 401-406
36. Lopes de Almeida JP, Pinto AC, Pereira J, Pinto S, de Carvalho.
Implementation of a wireless device for real-time telemedical assistance of
home-ventilated amyotrophic lateral sclerosis patients: a feasibility study.
Telemed J E Health.2010; 16: 883-888
37. Nijeweme-d’Hollosy WO, Janssen E, Huis in t Veld R, Spoelstra J,
Vollenbroek-Hutten M, Hermens HJ. Tele-treatment of patients with
amyotrophic lateral sclerosis. J Telemed Telecare. 2006; 12(Suppl. 1): S31-34
38. Chio A, Montuschi A, Cammarosano S, De Mercanti S, Cavallo E, Ilardi A et al.
ALS patients and caregivers communication preferences and information
seeking behaviour. Eur J Neurol. 2008; 15: 55-60
39. Callagher P, Mitchell D, Bennett W, Addison-Jones R. Evaluating a fast-track
service for diagnosing MND/ALS against traditional pathways. Br J Neurosci
Nurs. 2009; 5: 322-325
40. O’Brien MR, Whitehead B, Jack BA, Mitchell JD. From symptom onset to a
diagnosis of amyotrophic lateral sclerosis / motor neurone disease;
17
experiences of people with ALS/MND and family caregivers: a qualitative
study. Amyotroph Lateral Scler. 2011(b); 12: 97-104
41. McCluskey L, Casarett D, Siderowf A. Breaking the news: A survey of ALS
patients and their caregivers. Amyotroph Lateral Scler Other Motor Neuron
Disord. 2004; 5: 131-135
42. Belsh JM, Schiffman PL. The amyotrophic lateral sclerosis (ALS) patient
perspective on misdiagnosis and its repercussions. J Neurol Sci. 1996:
139(Suppl.): S110-116
43. Silverstein MD, Stocking CB, Antel JP, Beckwith J, Roos RP, Siegler M.
Amyotrophic lateral sclerosis and life-sustaining therapy: patients’ desires for
information, participation in decision making, and life-sustaining therapy.
Mayo Clin Proc. 1991; 66; 906-913
44. Moss AH, Casey P, Stocking CB, Roos RP, Brooks BR, Siegler M. Home
ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and
patient, family, and physician attitudes. Neurology. 1993; 43: 438-443
45. Moss AH, Oppenheimer EA, Casey P, Cazzolli PA, Roos RP, Stocking CB et al.
Patients with amyotrophic lateral sclerosis receiving long-term mechanical
ventilation. Chest. 1996; 110: 249-255
46. Albert SM, Murphy PL, Del Bene ML, Rowland LP. A prospective study of
preferences and actual treatment choices in ALS. Neurology. 1999; 53: 278283
47. Rabkin JG, Albert SM, Tider T, Del Bene ML, O’Sullivan I, Rowland LP et al.
Predictors and course of elective long-term mechanical ventilation: a
prospective study of ALS patients. Amyotroph Lateral Scler. 2006; 7: 86-95
48. Munroe CA, Sirdofsky MD, Kuru T, Anderson ED. End-of-life decision making
in 42 patients with amyotrophic lateral sclerosis. Respir Care. 2007; 52: 996999
49. Albert SM, Whitaker A, Rabkin JG, Del Bene M, Tider T, O’Sullivan I et al.
Medical and supportive care among people with ALS in the months before
death or tracheostomy. J Pain Symptom Manage. 2009; 38: 546-252
18
50. Young JM, Marshall CL, Anderson EJ. Amyotrophic lateral sclerosis patients’
perspectives on use of mechanical ventilation. Health Soc Work. 1994; 19:
253-260
51. Hirano YM, Yamazaki Y, Shimizu J, Togari T, Bryce TJ. Ventilator dependence
and expressions of need: A study of patients with amyotrophic lateral
sclerosis in Japan. Soc Sci Med. 2006; 62: 1403-1413
52. Hirano Y, Yamazaki Y. Ethical issues in invasive mechanical ventilation for
amyotrophic lateral sclerosis. Nurs Ethics. 2010; 17: 51-63
53. Cazzolli PA, Oppenheimer EA. Home mechanic ventilation for amyotrophic
lateral sclerosis: nasal compared to tracheostomy-intermittent positive
pressure ventilation. J Neurol Sci. 1996; 139(Suppl.): S123-128
54. Narayanaswami P, Bertorini TE, Pourmand R, Horner LH. Long-term
tracheostomy ventilation in neuromuscular diseases: patient acceptance and
quality of life. Neurorehabil Neural Repair. 2000; 14: 135-139
55. Sundling IM, Ekman SL, Weinberg J, Klefbeck B. Patients’ with ALS and
caregivers’ experiences of non-invasive home ventilation. Adv Physio. 2009;
11: 114-120
56. Lemoignan J, Ells C. Amyotrophic lateral sclerosis and assisted ventilation:
How patients decide. Palliat Support Care. 2010; 8: 207-213
57. Ganzini L, Johnston WS, McFarland BH, Tolle SW, Lee MA (1998). Attitudes of
patients with amyotrophic lateral sclerosis and their carers towards assisted
suicide. N Engl J Med. 1998; 339: 967-973
58. Achille MA, Ogloff JRP. Attitudes toward and desire for assisted suicide
among persons with amyotrophic lateral sclerosis. Omega J Death Dying.
2003; 48: 1-21
59. Sulmasy DP, Hughes MT, Thompson RE, Astrow AB, Terry PB, Kub J et al. How
would terminally ill patients have others make decisions for them in the
event of decisional incapacity? J Am Geriatr Soc. 2007; 55: 1981-1988
60. Nolan MT, Kub J, Hughes MT, Terry PB, Astrow AB, Carbo CA et al. Family
health care decision making and self-efficacy with patients with ALS at the
end of life. Palliat Support Care. 2008; 6: 273-280
19
61. Innes A, Blackstock K, Mason A, Smith A, Cox S. Dementia care provision in
rural Scotland: service users’ and carers’ experiences. Health Soc Care
Community. 2005; 13: 354-365
62. Ytterberg C, Johansson S, Gottberg K, Holmqvist LW, von Koch L. Perceived
needs and satisfaction with care in people with multiple sclerosis: a two year
prospective study. BMC Neurology. 2008; 8: 36 doi: 10.1186/1471-2377-8-36
63. Cotterell. Striving for independence: experiences and needs of service users
with life limiting conditions. J Adv Nurs. 2008; 62: 665-673
64. Weeks JC, Cook EF, O’Day SJ, Peterson LM, Wenger N, Reding D et al.
Relationship between cancer patients’ predictions of prognosis and their
treatment preferences. JAMA. 1998; 279: 1709-1714
65. Giles S, Miyasaki J. Palliative stage Parkinson’s disease: patient and family
experiences of health-care services. Palliat Med. 2009; 23: 120-125
66. Fried TR, Bradley EH, Towle VR, Allore H. Understanding the treatment
preferences of seriously ill patients. N Engl J Med. 2002; 346: 1061-1066
67. Payne S, Burton C, Addington-Hall J, Jones A. End-of-life issues in acute stroke
care: a qualitative study of the experiences and preferences of patients and
families. Palliat Med. 2010; 24: 146-153
68. Fegg MJ, Kogler M, Brandstatter M, Jox R, Anneser J, Haarmann-Doetkotte S
et al. Meaning in life in patients with amyotrophic lateral sclerosis.
Amyotroph Lateral Scler. 2010; 11: 469-474
69. Fegg MJ, Wasner M, Neudert C, Borasio GD. Personal values and individual
quality of life in palliative care patients. J Pain Symptom Manage. 2005; 30:
154-159
20
Table No. 1. Summary of studies
Reference
n1
(N)
Location
Method
Aims of study
Main findings
Achille & Ogloff
2003
n=44
multiple locations; United
States, Canada & United
Kingdom
Quantitative crosssectional; questionnaire
and multiple instrument
measures
To investigate attitudes toward assisted
suicide among ALS patients.
To describe the characteristics of ALS
patients who considers assisted suicide.
The majority of participants felt that assisted suicide was morally
acceptable but only 7% of participants indicated they would use it if
available.
Participants’ contemplation of assisted suicide was associated with feelings
of hopelessness and depression.
Albert et al.
1999
n=121
ALS tertiary centre
NY, USA
Quantitative longitudinal
(prospective); clinical
evaluation, multiple
questionnaire and
instrument measures
To determine if ALS patients’ preferences
for care relate to later treatment choices.
Patients made choices for care and followed up on choices consistent with
their preferences but preferences also changed over time.
Patients who had a greater attachment to life despite physical decline found
life sustaining treatments acceptable.
Albert et al.
2009 / [Rabkin et
al. 2006 (n=72)]
n=71
ALS tertiary centre
NY, USA
Quantitative longitudinal
(prospective); clinical
evaluation, multiple
questionnaire and
instrument measures
To analyse the use of medical and
supportive care by ALS patients prior to
death or tracheostomy.
Patients had a desire to live and use services up to the point of death or
trachesotomy.
Choosing long-term ventilation was determined by patients’ own
perceptions of their health status, feelings of optimism and hope, positive
appraisal of life and satisfaction with life.
Only 50% patients remained hopeful and optimistic after an average of 33
months on ventilation.
Beisecker et al.
1988
n= 41
ALS / Muscular Dystrophy
tertiary centre
Kansas, USA
Qualitative; semistructured interviews
To identify ALS patients’ perspectives on
care providers.
Patients reported expectations of multidisciplinary care in the form of
information, emotional support and assistive devices.
Patients expected healthcare providers to communicate honestly and with
respect.
Patients expected providers to convey hope.
Belsh &
Schiffman 1996
n=64
USA (81%) and other
countries / ALS Digest
Survey (via email);
questionnaire
To investigate the frequency of
misdiagnosis in ALS based on patient
response.
To identify demographic and epidemiologic
factors that distinguish ALS patients who
are initially misdiagnosed.
27% of patients reported at least one misdiagnosis.
The mean length of time to receive a diagnosis was longer for patients who
received an initial misdiagnosis compared to patients who were not
misdiagnosed.
Misdiagnosis may have resulted in refusal to participate in clinical drug
trials.
1
‘n’ denotes ALS participants. ‘N’ denotes total sample (non ALS patients in addition to ALS patients).
21
Bennett et al.
2009
n=27
ALS tertiary centre Preston,
UK
Audit; questionnaire
To analyse the benefits associated with
specialist nurse lead hospice-based ALS
clinics.
Patients expressed satisfaction with hospice-based clinics and valued the
intimate environment of the hospice setting.
Bolmsjo 2001
n=8
Neurology clinic
Lund, Sweden
Qualitative; semistructured interviews
To explore existential issues for ALS
patients in palliative care.
Existential issues are of great importance for ALS patients in palliative
care.
Central to care is the need to be respected.
Brown 2003
n=6
(N=21)
3 counties southern
England (Motor Neurone
Disease Association UK
register)
UK
Qualitative; semistructured interviews
To explore lay and professional values in
ALS care.
The professional view is detached from the experience of living with ALS.
Patients’ needs are broad and person focused.
Brown et al.
2005
n=11
(N=37)
3 counties southern
England (Motor Neurone
Disease Association
register)
UK
Qualitative; structured
interviews2
Questionnaire based
survey3
To investigate whether services meet the
needs of ALS patients and their carers.
To explore expectations of service delivery
for ALS patients,carers and service
providers and compare these with services
provided.
Patients expressed dissatisfaction with timeliness of services.
Patients identified positive qualities in care professionals.
Patients reported a lack of knowledge among service providers and
deficiencies in multidisciplinary care including; specialised equipment;
availability of care co-ordinators; and respite care.
Callagher et al.
2009
n=23
ALS tertiary centre
Preston, UK
Audit: questionnaire
To evaluate the impact of a fast-tracking
system for diagnosing ALS against
traditional pathways.
Patient expressed satisfaction with privacy, time, and sensitivity when
provided with a diagnosis.
Cazzolli &
Oppenheimer
1996
n=75
Pennsylvannia, Ohio (local
ALSA chapter registers)
ALS support group, West
Virginia
USA
Qualitative; structured
interviews
To compare the use of tracheostomy
ventilation and nasal positive pressure
ventilation among ALS patients.
All patients who chose nasal-IPPV expressed satisfaction with choosing
nasal-IPPV.
The majority of patients using long term mechanical ventilation received
tracheostomy without advance directives in place.
The majority of patients would not have chosen long term mechanical
ventilation due to burden of care.
Chio et al. 2008
n=60
ALS tertiary centre
Survey; questionnaire
To evaluate preferences for communication
Patients reported satisfaction with communication of the diagnosis.
2
3
Interviews with ALS participants only
Not used with ALS participants
22
(N=120)
Turin, Italy
and information seeking behaviour in ALS
patients and their carers.
Patients had a desire to obtain information and search for information both
within and outside health care services.
Foley et al. 2007
n=5
ALS tertiary centre Dublin,
Ireland
Qualitative; semistructured interviews
To explore the concept of QoL for ALS
patients and effect of healthcare services on
perceived QoL.
Patients reported health care services to have a positive effect on QoL.
Ganzini et al.
1988
n=100
ALS tertiary centre
Oregon, USA
Quantitative crosssectional; multiple
questionnaire and
instrument measures
To determine attitudes about assisted
suicide among ALS patients.
Feelings of hopelessness predicted patient preference for assisted suicide.
Patients who preferred assisted suicide had greater distress, were less
religious and had a lower quality of life than those who did not wish to
hasten death.
Hardiman et al.
2003
n=94
Ireland
(Irish Motor Neurone
Disease Association
register)
National survey (via
telephone); questionnaire
To identify access to health care services
for ALS patients in Ireland.
Patients reported low to moderate levels of access to community based
services.
Private health insurance was of no advantage to obtaining services or
specialised assistive devices and patients accessed services from the
voluntary sector to provide support.
Patients were unaware of the specific roles of healthcare professionals.
Hirano et al.
2006
n= 184
Japan
(Japenese ALS Association
register)
Mixed method;
Semi-structured interviews
(n=27), multiple
questionnaire and
instrument measures
(n=157)
To examine needs and experiences of ALS
patients on long-term ventilation.
Patients reported fear about using mechanical ventilation and about the
potential for ventilation to increase burden of care on their families.
Psychosocial support with ventilation fostered hope.
Hirano &
Yamazaki 2010
n=50
Japan
(Japanese ALS Association
register)
Mixed method;
Semi-structured
interviews, multiple
questionnaire and
instrument measures
To examine decision-making for ALS
patients in mechanical ventilation.
Patients’ decision to undergo mechanical ventilation was associated with
family support.
Patients who did not receive sufficient information about mechanical
ventilation reported conflicting feelings about ventilation and/or regret
about having undertaken ventilation.
Patients who received education had similar levels of hope pre- and post
ventilation. Those who did not receive education had low levels of hope.
Hocking et al.
2006
n=7
Auckland, New Zealand
Qualitative; semistructured interviews
To explore the ALS patients’ experience of
living with ALS and of their care.
Patients reported feelings of loosing control when confronted by numerous
healthcare professionals.
Developing and maintaining relationships with service providers required
time, effort and trust.
23
Hugel et al. 2006
n=13
Neuroscience tertiary
centre
Liverpool, UK
Qualitative; semistructured interviews
To explore the experiences of and issues
surrounding the diagnosis of ALS for ALS
patients
Despite satisfaction with the delivery of the diagnosis, patients reported
delays in receiving a diagnosis of ALS.
Patients reported poor co-ordination between services following the
diagnosis.
Hughes et al.
2005
n=9
(N=29)
ALS tertiary centre
London, UK
Qualitative; semistructured interviews
To explore the llived experience of ALS
patients and their carers including their
experiences of health care services.
To identify how delivery of services could
change to meet user need
Patients reported uncertainty about services and poorly timed
multidisciplinary care.
Patients reported concerns about professionals’ detached approach and
their lack of knowledge about ALS.
Patients reported delays in receiving a diagnosis which resulted in
problems accessing entitlements to care.
Kristjanson et al.
2005, 2006
n=119
(N=876)
Australia (Victoria,
Queensland & Western
Australia) – voluntary
association registers
Survey; questionnaire with
multiple instrument
measures
To identify supportive and palliative care
needs among progressive neurological
patients (including ALS) and their carers.
To determine extent to which services meet
the needs of patients and their carers.
Patients who received tailored services were the most satisfied.
ALS patients were more receptive to care than other neurological groups.
ALS patients were the least satisfied with services compared to other
neurological groups.
ALS patients emphasised more needs across a range of services
(information, specialised equipment and financial assistance) than other
neurological groups.
Krivickas et al.
1997
n=98
ALS tertiary centre,
Cleveland, USA and
Eastern Ohio ALSA
chapter
Survey; questionnaire,
structured interview (via
telephone)
To assess the utilisation and availability of
home care services for ALS patients
Over 50% of patients surveyed did not receive home care services.
Patients who received home care reported services to be inadequate and
poorly timed.
Lemoignan &
Ells 2010
n=9
ALS tertiary centre
Montreal, Canada
Qualitative; semistructured interviews
To explore the decision-making process in
assisted ventilation for ALS patients.
Patients’ decision-making was determined by perceptions about outcomes
(symptom relief versus life sustaining), severity of illness, and social and
financial supports and by personal values (relationships, autonomy and
quality of life) and fears about adapting to ventilation.
Lopes de
Almeida et al.
2010
n=4
Neurological rehabilitative
centre Lisbon, Portugal
(servicing catchment ALS
clinics)
Preliminary trial with
clinical evaluation.
To evaluate the feasibility of a telemedicine
assistive device for home ventilated ALS
patients
Patients reported satisfaction with a telemedicine assistive device.
4
Not reported
24
McCabe et al.
2008
n= 15
(N=138)
Australia (Victoria,
Queensland & Western
Australia) – voluntary
association registers
Qualitative; semistructured interviews
To investigate the types of support
networks and services used by patients with
progressive neurological disorders
(including ALS) and their carers.
ALS patients reported a strong desire for both basic care services and a
broad range of professional expertise.
McCluskey et al.
2004
n=144
(N=257)
Pennsylvania (local ALSA
chapter register)
USA
Survey; questionnaire
To evaluate how ALS patients and their
carers rate their experience of receiving the
diagnosis of ALS
The majority of participants rated physician performance as average to
poor.
Patients had greater satisfaction when more time was given to disclosing
the diagnosis.
Moss et al. 1993
n=19
(N=71)
Specialist ALS centres and
non specialist services
Illinois, USA
Quantitative longitudinal
(prospective); structured
interviews
To identify outcomes, costs and attitudes
towards assisted ventilation among ALS
patients, carers and providers.
The majority of patients were glad they had chosen assisted
ventilation
Patient s valued extended life provided by ventilation and were
positive about ventilation despite its physical limitations.
Assisted ventilation placed burden on families.
Moss et al. 1996
n=50
Specialist ALS centres and
non specialist services
(Illinois, Ohio,
Pennsylvania, West
Virginia)
Ventilator care programme
(California),
USA
Quantitative crosssectional; structured
interviews
To examine advance care planning and
outcomes of ALS patients.
Patients valued additional life afforded by ventilation but recognised
limitations of ventilation on sustaining quality of life.
The majority of patients completed advance directives and nearly all
patients wanted them.
Most patients wanted to stop ventilation under certain circumstances.
Munroe et al.
2007
n=42
Neuromuscular clinic
Boston, USA
Quantitative longitudinal
(retrospective); chart
review
To determine when end-of-life issues are
discussed with ALS patients.
Decisions about end-of-life care were delayed for patients.
Patients’ decision-making was independent of disease severity.
Murphy 2004
n=15
(N=28)
Scotland
(Scottish Motor Neurone
Disease Association
Register)
Qualitative; semistructured interviews5,
video recordings
To explore ALS patients’ and their
partners’ perceptions of ACC.
Patients reported dissatisfaction with poorly timed provision of ACC and
inadequate training to use ACC.
Patients found ACC restrictive as it affected intimacy with communication
partners.
5
Interviews with ALS participants only
25
Narayanaswami
et al. 2000
n=8
(N=19)
Tennessee, USA
(Long-term ventilation
domiciliary care)
Survey; questionnaire
To assess neuromuscular disease patients’
acceptance and quality of life on long-term
ventilation.
Patients with ALS were more ambiguous toward assisted ventilation than
patients with Duchenne Muscular Dystrophy.
Patients reported concern about the costs associated with assisted
ventilation.
Nijewemed’Hollosy et al.
2006
n=4
ALS rehabilitation clinic
Enschede, The Netherlands
Questionnaire; response
categories
To investigate ALS patients’ use of and
attitudes towards tele-medicine.
Patients expressed satisfaction with the use of telemedicine and perceived
telemedicine as time efficient.
Patients felt that traditional face-to-face contact with physicians was still
required to discuss psychosocial and emotional issues.
Nolan et al. 2008
n=16
(N=32)
Specialised teaching
hospital
Baltimore, USA
Qualitative (descriptive);
structured interviews with
‘control preference’
measure
To compare ALS patients’ preferences for
involving family in the decision making
process at end-of-life care and the actual
involvement by family at time of death.
The majority of patients issued an advance directive.
Patients who opted to make decisions independently were more likely to
have family report that decisions were made in this style.
Patients who preferred shared decision making with family were more
likely to have family report that decisions were made more independent of
a shared decision making style.
Survey decision making
questionnaire and
qualitative interviews6
O’Brien et al.
2011(a), O’Brien
et al. 2011(b)
n=24
(N=42)
ALS tertiary centre
Preston, UK
Qualitative; semistructured interviews
To identify factors related to uptake of
social care services for ALS patients.
To explore ALS patients’ and carers’
experiences of care between symptom onset
and diagnosis.
Patients’ desire to maintain control and normality and uncertainties around
service provision delayed use of social services.
Patients reported problems about the continuity of home services and felt
that social services were unfamiliar with ALS.
Patients reported a lack of sensitivity among physicians when
communicating the diagnosis.
Patients reported delays in diagnosis due to slow detection of early
symptom presentation by general practitioners.
Silverstein et al.
1991
n=38
ALS tertiary centre
Chicago, USA
Quantitative longitudinal
(prospective); structured
questionnaires
To identify if ALS patients seek
information about end-of-life treatments.
To identify if decisions made by ALS
patients about their care remains stable
overtime.
Patients’ desire for information and participation in decision-making
remained stable.
Preferences for end-of-life treatment changed overtime.
Preferences for care were independent of functional status and
demographic variables.
Sulmasy et al.
2007
n=32
(N=147)
Two specialised teaching
hospitals
Baltimore, USA
Quantitative longitudunal
(prospective); control
preference measure, QoL
To determine how terminally ill patients
(including ALS) opt to have carers and
physicians participate in their decision
Patients mostly opted for shared decision making but leaned more
independently from carers.
Decision control did not change significantly in the proceeding 6 months.
6
Not conducted with ALS participants
26
New York, USA
questionnaire & general
health questionniare
making and how this changes overtime.
Patients opted for physicians to make best-interest decisions at time of
diagnosis but overtime, some patients opted for physicians to make
decisions based on their judgements.
Sundling et al.
2009
n=7
(N=15)
University Hospital
Huddinge, Sweden
Qualitative; semistructured interviews
To explore ALS patients’ and carers’
experiences of non-invasive home
ventilation.
Patients viewed home ventilation positively but also had contradictory
emotions about being dependent on a ventilator.
Patients’ experiences of the benefits of ventilation were strongly
influenced by home support.
Trail et al. 2001
n=42
ALS tertiary centre
Houston, USA
Survey; questionniare
To determine ALS patients’ preferences for
wheelchair mobility.
Patients reported positive outcomes including a positive impact on their
well-being and increased independence in everyday life.
van Teijlingen et
al. 2001
n=153
Scotland
(Scottish Motor Neurone
Disease Association
Register)
National survey; structured
interview questionnaire
To examine service use and needs of ALS
patients and their carers in Scotland.
Patients reported long waiting periods for access to multidisciplinary care.
The majority of patients reported unmet needs in services and accessed
voluntary sector support to alleviate burden of care.
Vitacca et al.
2010a, 2010b
n=40
(N=80)
ALS tertiary centre Brescia,
Italy
Multiple outcome study
(satisfaction assessed by
structured telephone
interview)
To examine the benefit of tele-medicine
care for a cough assist programme for ALS
patients and their carers.
75% of patients reported high levels of satisfaction with programme.
Use of the programme reduced the frequency of hospitalisation and cost of
care for patients.
Ward et al. 2010
n=32
ALS / Muscular Dystrophy
tertiary centre
North Carolina, USA
Survey; questionnaire
To determine characteristics of and
satisfaction with powered wheelchair
mobility for ALS patients.
Patients demonstrated high levels of powered wheelchair utilisation and
expressed high levels of satisfaction with powered mobility.
Patients appreciated the capacity of powered mobility to accommodate to
their changing needs.
Wicks & Frost
2008
n=247
(N=334)
PatientsLikeMe.com
internet website
Survey (via email);
questionnaire
To indentify what ALS patients and their
carers know about ALS.
To identify if ALS patients and their carers
thought they received sufficient information
about ALS.
Patients reported receiving information about physical symptoms as
opposed to cognitive symptoms.
Patients indicated a preference for more information about all symptoms of
ALS.
Young et al.
1994
n=13
ALS tertiary centrre
Vancouver, Canada
Mixed method:
Qualitative; semistructured interviews
Quantitative;
questionnaires and
To explore ALS patients’ perspectives on
mechanical ventilation.
Patients’ decisions to avail of mechanical ventilation were determined by
their views on; the potential of ventilation to improve quality of life; ability
to control discontinuation of ventilation; and likelihood of ventilation to
sustain life.
Decisions against ventilation were influenced by patients’ perceptions
27
instrument measure
about potential adverse effects on quality of life and on burden of care.
Few patients sought comprehensive medical opinion on ventilation.
Objective measure of disability was not associated with decision-making.
28