Experience of Services as a Key Outcome in Amyotrophic Lateral
Sclerosis (ALS) Care: The Case for a Better Understanding of Patient
Experiences.
Foley, Geraldine, Timonen, Virpi and Hardiman, Orla – all of Trinity College Dublin
Published in American Journal of Hospice and Palliative Medicine 2012
Corresponding author:
Geraldine Foley
HRB Research Fellow
School of Social Work and Social Policy
Arts Building
Trinity College Dublin
Ireland
foleyg3@tcd.ie
geraldinefoley63@gmail.com
Acknowledgements:
This work is funded by the Health Research Board (HRB) of Ireland
1
Experience of Services as a Key Outcome in Amyotrophic Lateral Sclerosis (ALS)
Care: The Case for a Better Understanding of Patient Experiences.
Abstract
People with amyotrophic lateral sclerosis (ALS) frequently express dissatisfaction
with services. Service user satisfaction with services in ALS care is not always
measured and service user perspectives are not usually included when evaluating
outcomes of care. There is a lack of consensus on what constitutes satisfaction for
service users in ALS care. To date, healthcare professionals’ conceptualisation of
outcomes in ALS care has excluded measures of service user satisfaction with
services. Exploring the context of the ALS service user experience of care will identify
a conceptual framework that will include the domains of satisfaction with care for
service users with ALS. An instrument that draws on the ALS service user perspective
of services, developed on the basis of qualitative investigation, should be used to
measure satisfaction with services.
Key words
Amyotrophic lateral sclerosis, motor neurone disease, outcomes, satisfaction,
experiences, services
2
Introduction
Amyotrophic lateral sclerosis (ALS), otherwise known as motor neurone disease
(MND), is a rapidly progressive neurological disorder that results in diffuse motor
neuron degeneration within the central nervous system. Up to 60% of people with
ALS also experience cognitive impairment, and 15% of the population develop a
fronto-temporal dementia. The life expectancy in ALS is 3-5 years. Physical disability
affects limb and bulbar function and end-of-life is generally due to respiratory failure
1.
The care approach in ALS is palliative from the point of diagnosis 2. A combination of
symptomatic management, nutritional and respiratory support, assistive devices,
and psychological support including advance directives are considered components
of ALS care 3.
Outcomes in healthcare are defined as measures of the value of service intervention
4.
Outcome measures are usually independent of the service user perspective and
include measures pertaining to survival, disease severity and cost, all of which are
objective and unambiguous outcomes in healthcare. Healthcare services aim to
prolong survival for service users and limit the impact of disease severity. Costbenefit analysis is undertaken to measure the effect of care in relation to the cost of
care.
The role of healthcare professionals in ALS is care is to provide services that are not
only responsive to disease progression but also to service users’ perceptions about
3
services 2. Service users who live with a terminal illness may have different
perspectives on care than service providers 5. Furthermore, experiences of care have
a strong influence on outcomes for service users with terminal illness 6. Service users
with advancing illness shift towards values of self direction and benevolence 7. They
may choose interventions based on their perspective and decide about care in the
context of the needs of those who support them.
In recent years, there has been a shift towards incorporating service users’
perceptions of services (including satisfaction) when judging benefits of care 8.
Service user satisfaction is defined as an evaluation of distinct healthcare dimensions
9.
Some healthcare services research now compares satisfaction with care against
outcomes 10 or measures service user satisfaction with care as an outcome measure
11.
Despite positive outcomes in ALS multidisciplinary care, literature that reports on the
ALS service user perspective suggests, for the most part, dissatisfaction with services.
In highlighting this disparity between outcomes and experiences, we draw on a
systematic review of the literature pertaining to service user experience of care and
care preferences in ALS
12
and on defined web-based data from the service user
perspective. We define multidisciplinary care in ALS and report on the effects of ALS
multidisciplinary care. We show that studies of outcomes in ALS multidisciplinary
care for the most part neglect the service user perspective. We offer suggestions as
to why research in ALS to date has not investigated the relationship between
outcomes of care and service user satisfaction with services or indeed measured
4
service user satisfaction with services as an outcome of care. We hypothesise that
the inclusion of measures that tap into the ALS service user perspective would yield a
different, and fuller, picture of outcomes. We conclude by recommending the
construction of an outcome measure that draws on the ALS service user perspective
on services. A qualitative investigation of the parameters of the ALS service user
experience of services is the first step in developing this new outcome measure.
Satisfaction and dissatisfaction with ALS services: the service user perspective
The authors undertook a systematic review of recent literature pertaining to service
users’ perceptions of and preferences for care in ALS
12.
This review identified 43
studies which reported from the service user perspective, of which 20 dealt primarily
with service user satisfaction with services. Here, we focus on these 20 studies
pertaining to service user satisfaction with services and studies published on ALS
service user satisfaction with services since we completed our review.
Many studies have identified dissatisfaction with services. Most service users request
services, but then confront various problems. These include: absence of specialised
(tertiary) care and care co-ordinators; limited access to different healthcare
disciplines and assistive devices; and a lack of knowledge about ALS among health
care professionals
13-19.
Inadequate home care and/or respite care are also
problematic for service users
20-21.
Delays in diagnosis are common
22-23.
Some
service users also report dissatisfaction with the way in which the diagnosis is
provided and feel they receive inadequate information about the disease and care
options following diagnosis
24-25.
However, some studies report satisfaction with
5
disclosure
26-28.
Studies that focus on the use of assistive devices report satisfaction
with the use of these devices 29-35.
Service user satisfaction with services has also been captured by defined web-based
datasets. An online questionnaire to 247 people with ALS 36 found that service users
wished for more information about ALS than what was provided to them. Service
users who share health information online, experience greater satisfaction with
services and have better outcomes from their perspective 37.
Outcomes in multidisciplinary ALS care
Multidisciplinary care in ALS refers to specialised care which comprises a number of
different healthcare professionals including consultant physicians (in neurology,
respiratory,
gastroenterology,
and
palliative
care),
specialist
nurses,
physiotherapists, occupational therapists, speech and language therapists, clinical
nutritionists, and social workers. Specialised multidisciplinary ALS clinics are typically
secondary or tertiary care facilities and provide both diagnostic and treatment
services. They co-ordinate care and interface with primary healthcare professionals
and the ALS voluntary sector 38.
Multidisciplinary care in ALS achieves positive outcomes for service users and service
providers
39-46.
Positive outcomes include prolonged survival
hospital admissions and in length of hospital stay
43.
39-43
and reduction in
ALS multidisciplinary care also
improves quality of life for service users in psychological and social domains
without increased cost to services
45.
44
Intensive multidisciplinary intervention may
6
also be beneficial in limiting the effects of impairment on patient activity levels
46.
The majority of these studies have achieved better outcomes when compared to
care which is not multidisciplinary
39,40,43-45.
One population based study found no
difference in survival between multidisciplinary care and general care
47.
Multidisciplinary care is also associated with well managed care at end-of-life 48 and
better management of ALS symptoms 49 in line with ALS practice parameters 50-52.
What explains the paradox between positive outcomes in ALS multidisciplinary
care settings and dissatisfaction with services?
It is difficult to gain an understanding of how service user satisfaction with services
relates to other outcomes in ALS multidisciplinary care because no studies have
tested the relationship between them. However, there is a disparity in the literature
between predominantly positive outcomes on the one hand and generally low
service user satisfaction on the other hand. We have identified a number of possible
explanations for this.
The majority of studies that report positive outcomes in multidisciplinary ALS care
have done so based on survival and cost
39-43,45.
One out-come study reports from
44,
but no outcome studies report on
the service user perspective on quality of life
service user satisfaction with services. Whilst the North American ALS Patient Care
Database 49,53 has measured service user satisfaction with services, only one study 18
has used a generic patient satisfaction with care measure and only few studies (using
Likert scales) have measured satisfaction with specific aspects of care 27,29,30,33-35. It is
also noteworthy that many studies which report on service user satisfaction with
7
services have included participants who may not have accessed ALS multidisciplinary
care 14,15,18-21,23,24.
Service user satisfaction
The outcomes used in ALS multidisciplinary outcome studies until now (survival, cost
and physical activity), are conceptually different from satisfaction. Satisfaction with
services is subjective; cost and survival are objective. Survival in ALS can be clearly
defined and death rate is the least variable and most easily identifiable measure of
survival in ALS 54. Cost of services (both direct and indirect) in ALS can also be clearly
defined, usually by actual costs 45 or by estimated costs 55.
Satisfaction is a more complex construct than survival and cost. Satisfaction with
health care services is defined as the extent to which service users’ expectations of
services are met across specific domains of care. It constitutes an emotional and/or
cognitive evaluation of care. Satisfaction is influenced by numerous factors (including
education, expectations of services and acceptance of illness) 56 which have not been
controlled for in existing studies on satisfaction with services in ALS. Satisfaction is
also influenced by the effects of illness and moderated by the effects of available
treatments. It reflects the complex interplay of expectations and experiences of
health care services. Although a number of quality of life (QoL) measures
57-60
and a
meaning of life measure 61 are used in ALS to capture service users’ views on what is
important to them 62-64, they are rarely used to evaluate their perceptions of services
or indeed the impact of care from their perspective. This is hardly surprising as QoL
8
measures are self appraisals of wellbeing
65
and can exist independently of specific
experiences of health care services.
One possible factor for influencing outcomes and satisfaction with services could be
care provided by specialised ALS centres versus general neurology care, and it is
important to note that the studies that have yielded positive outcomes in
multidisciplinary care sampled service users who attended specialised ALS clinics
45.
39-
It is also of relevance that the majority of multidisciplinary clinic settings that have
sought to measure service user satisfaction report high level of service user
satisfaction with healthcare services
49,53
and/or satisfaction with respect to distinct
components of care 26-35.
Measuring satisfaction with services for terminally ill service users
Expert consensus for clinical and service user metrics in palliative care services
includes patient satisfaction 8. However, the lack of a conceptual basis for service
user satisfaction has resulted in the development of many different measures
66.
Service providers and researchers who measure satisfaction among people with lifelimiting conditions conduct patient satisfaction surveys (questionnaires) that assess
either overall satisfaction with services (thus aggregating all aspects of care into a
global measure of satisfaction) or satisfaction with one or very small number of
specific aspects of care, such as assistive devices, information or care providers
67.
Satisfaction questionnaires may comprise only domains of care deemed important
by service providers and neglect domains of care which service users consider
meaningful 68. The use of generic palliative care outcome tools themselves may not
9
necessarily be sensitive to the needs of specific groups including people with ALS 69.
ALS is always terminal and in comparison to other conditions (including cancer),
there is more certainty regarding the period of time to death. Hence, it is
conceivable that the domains of satisfaction with services for ALS service users may
differ from the domains of satisfaction with services for other groups who live with
terminal illness.
Conceptualisation of ‘outcomes’ and ‘patient satisfaction’ in ALS care
The previous section demonstrated that researchers and service providers in ALS
care have to date largely neglected measuring satisfaction with services because of a
lack in standard approaches to measuring satisfaction. Neither have researchers and
service providers yet tested the relationship between outcomes in ALS care with
satisfaction with services because their conceptualisation of outcomes pays little
attention to the service user experience. Whilst service providers may view
satisfaction in ALS as an important component within care, they generally do not
view satisfaction with care as an outcome that is beneficial to facilitating the process
of care.
The need to identify the domains of satisfaction with care in ALS
The dominant view in ALS is that service users’ perspective on care should remain
central to the care process 2. Despite this, the experience of services for ALS service
users is poorly understood and few studies have investigated the experiences of
services from the service user viewpoint. What constitutes domains of satisfaction
with services for people with ALS and what influences these domains is unknown.
10
How context e.g. relationship with service providers, cognitive impairment,
psychological well-being, coping strategies, severity of illness, symptomatic
treatment, carer support, expectations and carer views on services may affect the
service user perspective on services, remains unclear. Whilst context does not
necessarily determine experiences and/or set the course of interaction between
service users and service providers, it does incorporate a set of conditions which
influence how people respond to healthcare services.
Satisfaction with life and QoL measures are used in ALS care, but no studies have
indentified the key domains of ALS service user satisfaction with health and social
care services, let alone provided a substantive explanation of how people with ALS
experience care services. The parameters of the ALS service user experience of
healthcare services have not yet been mapped out. Apart from a limited number of
qualitative studies on perceptions about services
meaning of care in ALS
73,74
15-17,19,20,22,25,28,70-72
and the
little is known about how people with ALS interpret,
approach and experience their healthcare services. Further research is required to
pinpoint the key parameters of the ALS service user experience of health and social
care services. Grounded theory is a research method which builds concepts and
theory from qualitative data 75. The use of grounded theory method to identify the
key parameters of the ALS service user experience of services will facilitate the
development of a theoretical framework that explains how people with ALS
understand and use their healthcare services. This framework will include the
domains of service user satisfaction with health and social care services in ALS. Such
a framework will not only incorporate relevant domains of care but also account for
11
variation, that is, dimensions of the service user experience within these domains.
Importantly, grounded theory method is concerned with process, that is, variation in
response according to context
75.
Response shifts (which are recaliberations of
patients’ internal views usually associated with changing circumstance 76) have been
identified in ALS
62.
This process of adaptation is poorly understood. Hence, a
qualitative investigation of the parameters of service users’ experiences of services
through grounded theory method will shed further light on this phenomenon.
Conclusion
This article has shown that studies on outcomes in ALS care have to date paid little
attention to service users’ perceptions of services. We argue that the inclusion of
measures that include the ALS service user perspective on services would yield a
different, and fuller, picture of outcomes. We recommend the development of a new
instrument for measuring ALS service users’ satisfaction with services based on an
improved understanding of their experience of healthcare services. Identifying the
parameters of ALS service users’ use and understanding of services through
qualitative research will unearth the key domains of satisfaction with healthcare
services in ALS. A qualitative investigation will be the first step in grounding such a
measure in the lived experiences and perceptions of ALS service users. Open-ended
questions will be posed to respondents who will be invited to recount their
experience and views at length; the ensuing qualitative interview data will be
analysed with the help of open, axial and selective coding, a largely inductive process
which will yield the main categories of the ALS service user experience and a theory
to explain relationships between these categories. The main parameters of the
12
service user experience will be translated into a set of questions and responses
(scales) that measure those parameters 77 and tested for reliability and validity with
a variety of ALS populations as per standard survey design principles. This in turn will
provide a measure to be used in quantitative studies of service user satisfaction with
ALS healthcare services. The use of such a measure with large and randomly sampled
populations where key variables are controlled for will for the first time, give an
accurate and representative picture of ALS service users’ views on the services that
they receive.
The measurement of the key domains of care through the use of such a scale will
enable researchers and service providers to measure key components of the service
user experience of ALS services and to test the relationship between service users’
satisfaction with services and other outcomes of care. This will help to improve the
quality of care and promote services that are driven by service user need. It will also
improve the utility of ALS services and be a useful tool in the cost-benefit analysis of
ALS healthcare services.
References
1. Chio A, Logroscino G, Hardiman O et al. Prognostic factors in ALS: a critical
review. Amyotroph Lateral Scler. 2009; 10: 310-323
2. Oliver D, Borasio GD, Walsh D. Palliative care in amyotrophic lateral sclerosis.
From diagnosis to bereavement. 2nd ed. Oxford: University Press; 2006
13
3. 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
4. Kane RL. Understanding Health Care Outcomes Research. 2nd ed. Sudbury,
MA: Jones Bartlett; 2006
5. 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
6. Normand C. Measuring outcomes in palliative care: limitations of QALYs and
the road to PaIYs. J Pain Symptom Manage. 2009; 38: 27-31
7. 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
8. Weissman DE, Morrison RE, Meier DE. Center to advance palliative care
palliative care clinic clinical care and customer satisfaction metrics consensus
recommendations. J Palliat Med. 2010; 13: 179-184
9. Pascoe GC. Patient satisfaction in primary healthcare: a literature review and
analysis. Eval Program Plan. 1983; 6:185-210
10. Alazri MH, Neal RD. The association between satisfaction with services
provided in primary care and outcomes in Type 2 diabetes mellitus. Diabet
Med. 2003; 20: 486-490
11. Howard M, Goertzen J, Hutchison B, Kaczorowski J, Morris K. Patient
satisfaction with care for urgent health problems: A survey of family practice
patients. Ann Fam Med. 2007; 5: 419-424
14
12. Foley G, Timonen V, Hardiman O. Patients’ perceptions of services and
preferences for care in amyotrophic lateral sclerosis. A review. Amyotroph
Lateral Scler. 2012; 13: 11-24
13. Ng L, Talman P, Khan F. Motor neurone disease: disability profile and service
needs in an Australian cohort. Int J Rehabil Res. 2011; 34:151-159
14. 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. Brown JB, Lattimer V, Tudball T. An investigation of patients’ and providers’
views of services for motor neurone disease. B J Neuro Nurs. 2005; 1: 249-252
16. 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. 2012; 26: 123131
17. 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
18. 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
19. Murphy J. “I prefer contact this close”: perceptions of AAC by people with
motor neurone disease and their communication partners. ACC. 2004; 20:
259-271
15
20. 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
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. Hugel H, Grundy N, Rigby S, Young CA. 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 Lateral
Scler. 2006; 7: 161-166
23. Belsh JM, Schiffman PL. The amyotrophic lateral sclerosis (ALS) patient
perspective on misdiagnosis and its repercussions. J Neurol Sci. 1996; 139:
110-116
24. 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
25. O’Brien MR, Whitehead B, Jack BA, Mitchell JD. From symptom onset to a
diagnosis of amyotrophic lateral sclerosis / motor neurone disease;
experiences of people with ALS/MND and family caregivers: a qualitative
study. Amyotroph Lateral Scler. 2011; 12: 97-104
26. Callagher P, Mitchell D, Bennett W, Addison-Jones R. Evaluating a fast-track
service for diagnosing MND/ALS against traditional pathways. B J Neuro Nurs.
2009; 5: 322-325
16
27. Chio A, Montuschi A, Cammarosano S et al. ALS patients and caregivers
communication preferences and information seeking behaviour. Eur J Neurol.
2008; 15: 55-60
28. Beisecker AE, Cobb AK, Ziegler DK. Patients’ perspectives of the role of care
providers in amyotrophic lateral sclerosis. Arch Neurol. 1988; 45: 553-556
29. 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
30. Ward AL, Sanjak M, Duffy K 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
31. Vitacca M, Paneroni M, Trainini D et al. At home and on demand mechanical
cough assistance program for patients with amyotrophic lateral sclerosis. Am
J Phys Med Rehabil. 2010; 89: 401-406
32. Lopes de Almeida JP, Pinto AC, Pereira J, Pinto S, de Carvalho M.
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
33. 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
17
34. Gruis KL, Wren PA, Huggins JE. Amyotrophic lateral sclerosis patients’ selfreported satisfaction with assistive technology. Muscle Nerve. 2011; 43: 643647
35. Hossler C, Levi BH, Simmons Z, Green MJ. Advance care planning for patients
with ALS: Feasibility of an interactive computer program. Amyotroph Lateral
Scler. 2011; 12: 172-177
36. Wicks P, Frost J. ALS patients request more information about cognitive
symptoms. Eur J Neurol. 2008; 15: 497-500
37. Wicks P, Massagli M, Frost J et al. Sharing health data for better outcomes on
PatientLikeMe. J Med Internet Res. 2010; 12: e19
38. 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
39. 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,
40. Lee JRJ, Annegers JF, Appel SH. Prognosis of amyotrophic lateral sclerosis and
the effect of referral selection. J Neurol Sci. 1995; 132: 207-215
41. Sorenson EJ, Mandrekar J, Crum B, Stevens JC. Effect of referral bias on
assessing survival in ALS. Neurology. 2007; 68: 600-602
42. Rodriguez de Rivera FJ, Oreja Guevara C, Sanz Gallego I et al. Outcomes of
patients with amyotrophic lateral sclerosis attending a multidisciplinary care
unit. Neurologia. 2011. DOI:10.1016/j.nrl.2011.01.021
18
43. 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
44. Van den Berg JP, Kalmijn S, Lindeman E et al. Multidisciplinary ALS care
improves quality of life in patients with ALS. Neurology. 2005; 65: 1264-1267
45. Van der Steen I, Van den Berg JP, Buskens E, Lindeman E, Van den Berg LH.
The costs of amyotrophic lateral sclerosis, according to type of care.
Amyotroph Lateral Scler. 2009; 10: 27-34
46. Calzada-Sierra DJ, Gomez Fernandez L. The importance of multifactorial
rehabilitation treatment in amyotrophic lateral sclerosis. Revista de
Neurologia. 2001; 32: 423-426
47. Zoccolella S, Beghi E, Palagano G et al. ALS multidisciplinary clinic and
survival; results from a population-based study in Southern Italy. J Neurol.
2007; 254: 1107-1012
48. Mandler RN, Anderson FA, Miller RG, Clawson L, Cudkowicz M, Del Bene M.
The ALS patient care database: insights into end-of-life care in ALS.
Amyotroph Lateral Scler Other Motor Neuron Disord. 2001; 2: 203-208
49. Miller RG, Anderson F, Brooks BR et al. Outcomes research in amyotrophic
lateral sclerosis: lessons learned from the amyotrophic lateral sclerosis
clinical assessment, research, and education database. Ann Neurol. 2009;
65(suppl): S24-S28
50. Miller RG, Jackson CE, Kasarskis EJ 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
19
Standards Subcommittee of the American Academy of Neurology. Neurology.
2009; 73: 1218-1226
51. Miller RG, Jackson CE, Kasarskis EJ et al. Practice parameter update: the care
of the patient with amyotrophic lateral sclerosis: multidisciplinary care,
symptom management, and cognitive/behavioural impairment (an evidencebased review): report of the Quality Standards Subcommittee of the
American Academy of Neurology. Neurology. 2009; 73: 1227-1233
52. Andersen PM, Borasio GD, Dengler R 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.
53. Miller RG, Anderson FA, Bradley WG et al. The ALS Patient Care Database:
Goals, design, and early results. Neurology. 2000; 54: 53-57
54. Gordon PH, Corcia P, Lacomblez L et al. Defining survival as an outcome
measure in amyotrophic lateral sclerosis. Arch Neurol. 2009; 66: 758-761
55. Lopez-Bastida J, Perestelo-Perez L, Monton-Alvarez F, Serrano-Aguilar P,
Alfonso-Sanchez JL. Social economic costs and health-related quality of life in
patients with amyotrophic lateral sclerosis in Spain. Amyotroph Lateral Scler.
2009; 10: 237-243
56. Fakhoury WK. Satisfaction with palliative care: what should we be aware of?
Int J Nurs Stud. 1998; 35:171-176
57. O’Boyle CA, McGee HM, Hickey A, O’Malley K. Individual quality of life in
patients undergoing hip replacement. Lancet. 1992; 339: 1088-1091
20
58. Hickey AM, Bury G, O’Boyle A, Bradley F, O’Kelly FD, Shannon W. A new short
form individual quality of life measure (SEIQol-DW): application in a cohort of
individuals with HIV/AIDS. BMJ. 1996; 313: 29-33
59. Cohen SR, Mount BM, Strobel MG, Bui F. The McGill Quality of Life
Questionnaire: a measure of quality of life appropriate for people with
advanced diseases. A preliminary study of validity and acceptability. Palliat
Med. 1995; 9: 207-219
60. Simmons Z, Felgoise SH, Bremer BA, et al. The ALSSQOL. Balancing physical
and non-physical factors in assessing quality of life in ALS. Neurology; 67:
1659-1664
61. Fegg MJ, Kramer M, L’hoste S, Borasio GD. The Schedule for Meaning in Life
Evaluation (SMiLE): Validation of a new instrument for meaning-in-life
research. J Pain Symptom Manage. 2008; 35: 356-364
62. Clarke S, Hickey A, O’Boyle C, Hardiman O. Assessing individual quality of life
in amyotrophic lateral sclerosis. Qual Life Res. 2001; 10: 149-158
63. Chio A, Gauthier A, Montuschi A et al. A cross sectional study on
determinants of quality of life in ALS. J Neurol Neurosurg Psychiatry. 2004;
75: 1597-1601
64. Fegg MJ, Kogler M, Brandstatter M et al. Meaning in life in patients with
amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2010; 11: 469-474
65. Bromberg MB. Quality of life in amyotrophic lateral sclerosis. Phys Med
Rehabil Clin N Am. 2008; 19: 591-605
66. Dy SM, Shugarman LR, Lorenz KA, Mularski RA, Lynn J. A systematic review of
satisfaction with care at the end of life. J Am Geriatr Soc. 2008; 56: 124-129
21
67. Sen S, Fawson P, Cherrington G et al. Patient satisfaction in the disease
management industry. Dis Manag. 2005; 8: 288-300
68. Aspinal F, Addington-Hall J, Hughes R, Higginson IJ. Using satisfaction to
measure the quality of palliative care: a review of the literature. J Adv Nurs.
2003; 42: 324-339
69. Hughes RA, Aspinal F, Higginson IJ et al. Assessing palliative care outcomes
for people with motor neurone disease living at home. Int J Palliat Nurs.
2004; 10: 449-453
70. 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
71. Lemoignan J, Ells C. Amyotrophic lateral sclerosis and assisted ventilation:
How patients decide. Palliat Support Care. 2010; 8: 207-213
72. Young JM, Marshall CL, Anderson EJ. Amyotrophic lateral sclerosis patients’
perspectives on use of mechanical ventilation. Health Soc Work. 1994; 19:
253-260
73. Bolmsjo I. Existential issues in palliative care: interviews of patients with
amyotrophic lateral sclerosis. J Palliat Med. 2001; 4: 499-505
74. Brown JB. User, carer, and professional experiences of care in motor neurone
disease. Prim Health Care Res Devel. 2003; 4: 207-217
75. Corbin J, Strauss A. Basics of qualitative research. 3rd ed. Thousand Oaks:
Sage; 2008
76. Wilson I. Clinical understanding and clinical implications of response shift. Soc
Sci Med. 1999; 45: 1577-1588
22
77. Coyle J, Williams B. An exploration of the epistemological intricacies of using
qualitative data to develop a quantitative measure of user views of health
care. J Adv Nurs. 2000; 31: 1235-1243
78. Logroscino G, Beghi E, Hardiman O et al. Effect of referral bias on assessing
survival in ALS. Neurology. 2007; 69: 939; author reply 939-940
23