American Journal of Hospice

& Palliative Medicine®

End-of-life Characteristics and Palliative 1-5
ª The Author(s) 2017
Reprints and permission:
Care Provision for Patients With Motor sagepub.com/journalsPermissions.nav
DOI: 10.1177/1049909117735832
Neuron Disease journals.sagepub.com/home/ajh

Hon Wai Benjamin Cheng, MBBS, MRCP, FHKCP1, Oi Man Iman Chan, MSW1,
Chun Hung Red Chan, BSc, MSc, FHKAN2, Wan Hung Chan, APN2,
Koon Sim FUNG, RN2, and Kar Yin Wong, RN2

Abstract
Motor neuron disease (MND) is a neurodegenerative disease and manifested as progressive decline in physical, respiratory,
swallowing and communication function, and ultimately death. Traditional model of care was fragmented and did not match with
multifacet needs of patients and carers. Furthermore, there could be lack of integrated care at end of life for patients with MND in
most lower- and middle-income countries or in places with inadequate palliative care (PC) coverage. In view of this, a special
workgroup for patients with MND, which includes neurologist, respiratory physician, rehabilitation specialist, and PC physician
was formed in Hong Kong since year 2011. In various disease phase, each specialty team plays a leading role in coordinated care of
patients with MND. From July 2011 to June 2017, a total of 52 patients with MND were referred for PC; 41 deceased patients
with MND were included into data analysis. Major cause of death remains pneumonia (54.8%) and respiratory failure (40.5%).
Most of the patients with MND (66.7%) died in acute ward and neurology units, with only 11.9% dying in PC units and hospices.
The PC team plays a major role in advance care planning (ACP), and most patients had their ACP documented at second or third
PC clinic visit (93.8%). Patients with MND often have limitations in mobility, swallowing difficulty, respiratory insufficiency
requiring ventilator support, and various psychosocial needs. This highlighted the importance of early PC referral.

Keywords
neurology, motor neuron disease (MND), amyotrophic lateral sclerosis, palliative, multidisciplinary team, end of life (EOL),
Hong Kong

Introduction coordination in the care of patients with MND.2 The National

Motor neuron disease (MND) is a neurodegenerative disease
characterized by loss of motor neurons in the spinal cord, brain
stem, and motor cortex. Clinically, it is manifested as progres-
sive decline in physical, respiratory, swallowing and commu-
nication function, and ultimately death.
Motor neuron disease is a life-limiting illness with challen-
ging progressive course that results in a wide range of ever-
changing spectrum of care needs. The symptoms in MND are
diverse and challenging. They include weakness, spasticity,
limitations in mobility and activities of daily living (ADLs),
communication deficits, and dysphagia; and in those with bul-
bar involvement, respiratory compromise, fatigue and sleep
disorders, pain, and psychosocial distress. The average survival
ranged from 2 to 5 years from onset, although some people may
live for up to 10 years. Because of its diverse symptoms, persons
with MND are often taken care by multiple specialties and
disciplines including neurologist, chest physician, rehabilitation
specialist, palliative medicine specialist, physiotherapist, occu-
pational therapist, and dietitian. 1 There was lack of
Service Framework was developed by the Department of
Health in United Kingdom to provide quality requirements for
the inspection authorities to use in measuring local progress for
long-term neurological conditions (LTNCs).3 It advocates the
need for integrated care in managing LTNCs including
MND. The guidelines issued by Royal College of Physicians
(London) for persons with LTNCs recommend the interface
between neurology, rehabilitation, and palliative care (PC)
to address the diagnostic, restorative, and palliative phases
of illness.3
1
Medical Palliative Medicine (MPM) Unit, Department of Medicine and
Geriatrics, Tuen Mun Hospital, New Territories, Hong Kong
2
Palliative Home Care Team, Tuen Mun Hospital, New Territories, Hong Kong
Corresponding Author:
Hon Wai Benjamin Cheng, MBBS, MRCP, FHKAM, Medical Palliative Medicine
(MPM) Unit, Department of Medicine and Geriatrics, Rehabilitation Block,
Tuen Mun Hospital, New Territories, Hong Kong.
Email: benchw@hkstar.com
2 American Journal of Hospice & Palliative Medicine® XX(X)
Figure 1. Motor neuron disease workgroup model in Hong Kong
neurologist, rehabilitation specialist, and PC team each playing a
leading role along MND disease course. MND, motor neuron disease;
PC, palliative care.
Materials and Methods
Traditional model of care for patients with MND was fragmen-
ted and did not match with patients’ multifacet needs. In view
of this, a special interest group for MND that includes neurol-
ogist, respiratory physician, rehabilitation specialist, and PC
physician was formed in 2011. In various disease phase, each
specialty team plays a leading role in coordinated care of
patients with MND (Figure 1). For instance, neurologist plays
a major role in MND diagnosis and treatment initiation/monitor-
ing. Rehabilitation specialist plays a leading role in ADLs func-
tion maintenance, home modification, liaison with respiratory
physician in ventilator use, and liaison with gastroenterologist
on artificial feeding options. Palliative care physicians play a
leading role in the later disease course, including symptoms man-
agement, psychosocial support for patients with MND and their
family members, bereavement support, and end-of-life (EOL)
preparation.
The aims of the group are to:
 promote and implement best practice interdisciplinary care,
 enable the dissemination of MND-specific information
to other health-care professionals, and
 encourage the sharing of expertise in managing patients
with MND.
The key focus areas of the group are supporting patients
to maintain quality of life and basic ADLs by fast-track refer-
ral to rehabilitation team once diagnosis is ascertained.
Patient assessment was standardized with special attention
to communication, swallowing, nutrition, mobility, and
respiratory support. Counseling on disease education and
acceptance by patient and carer was strengthened and priority
to maintain an independent living through early rehabilitation
intervention was emphasized. The evolving needs to compen-
sate for progressive loss of function is anticipated via regular
rehabilitation assessment and timely support from allied
health staffs with special interest in communication augmen-
tation and mobility or seating device equipped with appropri-
ate use of environmental control unit. Coordination among
various specialists and allied health professionals is vital. This
is achieved by key workers to maintain contact with the
patients and liaise with other team members and services.
Formal and informal communication channels are established
to ensure a well-concerted care adjusted according to patients’
changing needs.
In order to address the gaps in MND care, an MND taskforce
comprising neurologist, rehabilitation specialist, chest physi-
cians, and PC specialist was formed in Tuen Mun Hospital,
Hong Kong. During the course of the illness, neurologists
assess, diagnose, and manage the disease. Rehabilitation spe-
cialists assist with disability management and adaptive equip-
ment provision, for example, strategies and aids for mobility
and ability to perform ADLs, procedures for spasticity. Pallia-
tive care team helps in the management of distressing symp-
toms, providing emotional, psychological, and spiritual support
as needed, and support the family in bereavement. Involvement
of PC team at an earlier stage of disease is important to allow
discussion of advance care planning (ACP) before the person
loses the ability to communicate.
All patients with confirmed diagnosis of MND by neurolo-
gists at Tuen Mun Hospital, Hong Kong, would be recruited
into the interdisciplinary workgroup as described above. Tuen
Mun Hospital is an integrated regional hospital in Hong Kong
which serves a population of around 1.2 million. The institu-
tional review board of New Territories West Cluster approved
this study. A retrospective review was made of patient records
from all recruited patients with MND, between July 2011 and
June 2017. Medical records of all identified patients would be
reviewed retrospectively, by collecting data including demo-
graphic variables, treatment received, reasons and timing of PC
team referral, cause of death and place of death, feeding option
received, namely, comfort feeding, nasogastric tube, or percu-
taneous endoscopic gastrostomy (PEG)/radiologically inserted
gastrostomy (RIG), and ventilator support, namely, no ventila-
tor required, noninvasive positive pressure ventilation
(NIPPV), or invasive ventilation. Collected data were analyzed
with SPSS version 16.0 for Windows. The statistical signifi-
cance level was set at P < .05 unless otherwise specified.
Descriptive statistics were used to characterize the sociodemo-
graphic and clinical features of the whole sample. Means, stan-
dard deviations (SDs), and ranges for all scale variables
measured in the study were calculated for all patients.
Results
During the study period from July 2011 to June 2016, there
were 51 patients with MND referred for palliative care. Nine
Cheng et al 3

Table 1. Demographic and Health Variables of Study Participants.a patient (2.4%) died of sudden cardiac arrest with no identified
cause, and 1 patient (2.4%) died of peritonitis secondary to
n % Mean SD
PEG leakage. In all, 95.2% (n ¼ 40) patients with MND had
Age (years) 42 59 11.5 do-not-attempt cardiopulmonary resuscitation (DNACPR)
Sex order in place before their death, while 38.1% (n ¼ 16) of
Female 14 33.3 deceased patients had signed advance directives (ADs).
Male 28 66.7
Feeding option received
Comfort feeding 20 47.6 Discussion
Nasogastric tube 16 38.1
Gastrostomy (PEG/RIG) 6 14.3 In our study, major cause of death in patients with MND
Ventilator support remains pneumonia (54.8%) and respiratory failure (40.5%).
No ventilator support 17 40.5 Respiratory failure in patients with MND is caused by weak-
NIPPV 15 35.7 ness in respiratory and bulbar muscles and can be made worse
Invasive ventilation 9 21.4 by aspiration and bronchopneumonia. Being the major cause of
Place of death
death in these patients, early detection is important, as ventila-
Acute ward setting 28 66.7
Convalescent and rehabilitation ward 6 14.3 tion support can prolong survival.4,5
PC ward and hospice 5 11.9 Early assessment of respiratory function includes history,
Home 3 7.1 physical examination, overnight pulse oximetry, and spirometry
Immediate cause of death with respiratory pressures. Nocturnal desaturation can suggest
Pneumonia 23 54.8 hypoventilation, and blood CO2 and bicarbonate levels will give
Respiratory failure 17 40.5 confirmation. The forced vital capacity is used to detect respira-
Cardiac arrest 1 2.4
tory muscle weakness. A fall to less than 50% predicted is asso-
Peritonitis secondary to PEG leakage 1 2.4
Do-not-attempt cardiopulmonary 40 95.2% ciated with significant respiratory impairment. If checked supine,
resuscitation in place before death a >25% decrease suggests diaphragmatic weakness.6 Respiratory
Documented ACP in place 32 76.2 pressures correlate with respiratory muscle weakness.7 A max-
Signed AD in place 16 38.1 imal inspiratory pressure of <60 cm H2O is a predictor of
reduced survival. A sniff pressure can be used as surrogate.
Abbreviations: ACP, advance care planning; AD, advance directive; NIPPV,
noninvasive positive pressure ventilation; PC, palliative care; PEG, Patients with MND ultimately have recurrent aspiration as
percutaneous endoscopic gastrostomy; RIG, radiologically inserted their ability to clear secretions in the upper and lower airways
gastrostomy; SD, standard deviation.aN ¼ 42. becomes impaired. Assisted cough techniques need to be taught
to patients and caregivers for effective sputum clearance.8
Assisted cough device, suction machine, and mucolytic drugs
patients were still alive at data analysis. Forty-two patients can alleviate respiratory symptoms.9 Pneumococcal and influ-
were finally included in our study. Patient characteristics are enza vaccination can reduce pulmonary infections.7
depicted in Table 1. There were 28 males (66.7%) and Ventilation support has been shown to prolong survival10
14 females, with a mean age of 59.0 years (SD ¼ 11.5). All and will be needed in most patients eventually. More than half
recruited patients were Chinese. The median time from MND of patients with MND in our study received ventilator support
diagnosis to death was 14 months (range 1-66 months), while (35.7% received noninvasive ventilation [NIV] and 25.4%
the median time from PC referral to death was 4 months (range received invasive ventilation). This needs to be thoroughly
0.5-37 months). discussed with the patients and caregivers, including the asso-
Feeding option and ventilator support since diagnosis ciated ethical considerations, costs, and home ventilation and
were analyzed. Twenty patients maintained on comfort associated care. A multidisciplinary team care approach is sug-
feeding (47.6%), 16 patients received feeding via nasogas- gested, including psychological support.11
tric tube (38.1%), and 6 patients were put on feeding via The NIV with bilevel intermittent positive pressure ventila-
gastrostomy (PEG or RIG). Seventeen patients (40.5%) tor is most cost-effective in patients with MND.12 The NIV
did not receive ventilator support since MND diagnosis, improves respiratory function and quality of life, reversing
15 (35.7%) patients received NIPPV, while 9 (21.4%) chronic respiratory fatigue, decreasing decline in FVC, and
patients received invasive ventilation. relieving atelectasis.13 It alleviates the symptoms of hypoven-
Most of the patients with MND (n ¼ 28, 66.7%) died in tilation, reversing hypercapnia.14 The associated problems for
acute ward and neurology units, 14.3% (n ¼ 6) died in con- NIV are nasal bridge soreness, abdominal bloating, claustro-
valescent and rehabilitation settings, with only 11.9% (n ¼ 5) phobia, anxiety, and excessive salivation.15
dying in PC units and hospices; 3 patients (7.1%) attained home Invasive ventilation via tracheostomy is used in patients
death and none of them died in intensive care unit. In our study, with severe bulbar dysfunction and in those who failed NIV.
pneumonia remained the leading cause of death and accounted It allows for suction of secretion and avoids facemask problems
for more than half (n ¼ 23, 54.8%) of the diagnoses. Another but predisposes to recurrent infection, wound site infection and
17 patients with MND (40.5%) died of respiratory failure. One bleeding, and tracheoesophageal fistula formation.16
4 American Journal of Hospice & Palliative Medicine® XX(X)
Table 2. Palliative Care Model for Patients With MND and Caregivers.
Session Target of Each PC Clinic
1 To orientate patient and caregivers to the program and create a
trusting relationship between caregivers and PC team
members, enhance caregiver psychosocial support and
medical knowledge, introduce concept of ACP and ADs
2 and 3 To follow up previously identified problems, assess for ongoing
caring and emotional issue(s), formulation of ACP and signing
of AD for patients who show preparedness
4 and To follow-up on symptom progression, arrangement of regular
onwards pulmonary function test and NIV titration, review on feeding
plan and tolerance, regular review of signed AD and
documented ACP
Abbreviations: ACP, advance care planning; AD, advance directive; MND, motor neuron disease; PC, palliative care; NIV, non-invasive ventilation.
Role of PC Physician in MND Workgroup
As the disease progresses, a palliative approach is required to
ensure that early discussions around future care management
decisions and ACP are held. Symptomatic management
approach is adopted for both patients and their relatives in order
to achieve holistic care.
The PC actually begins at the time of diagnosis for patients with
poor prognosis. Discussions about prognosis and EOL issues can
be conceptualized as a process of ongoing conversation over time,
rather than a single discussion. Advance care planning topics such
as invasive respiration support or surgical procedures for tube feed-
ing were discussed. As the patients become more dependent, ser-
vices would be modeled at maintaining quality of life and relieving
carer’s stress. Nutritional support, home medication, communica-
tion devices, and respite service advice are provided. At the final
stage of the disease, the patients would be handed over to the
palliative team, with subsequent periodic review. The team would
focus on symptomatic relief in the terminal phase, which includes
pain, dyspnea relief, and dysphagia/dysarthria management.
The PC clinic adopted a proactive, comprehensive, and multi-
disciplinary approach for both patients and caregivers. It emphasized
symptom advice and prevention because patients with MND could
Role of PC Nurse and Social Worker in MND Care Model
– Palliative care nurse: assessment of patient’s symptom
burden; introduction of PC service, team members, and
types of services; knowledge of MND and related
problems; patient medical care aspect (symptom control,
feeding options, ventilator support); patient and caregiver
psychological aspect; use of pamphlets to enhance
adherence to diet and ventilator recommendations;
home care visit, physiotherapy, clinical psychologist
referral for intervention.
– Social worker: assessment of patient and caregiver social
background with a demographic data sheet; family social
support; financial assessment; counseling, community
service referral, coping skill training, respite care for
intervention
– Palliative care nurse: regular symptom burden
assessment, monitoring to adherence to dietary and
ventilator recommendations, management of the
patient’s symptoms and skills in coping with them,
assessment of psychological aspect
– Social worker: assessment of social support and caring
issue(s), orientation in stress management, improvement
in communication skills in family, orientation of caregivers
to relaxation methods, interventions as needed
– Palliative care nurse: regular symptom assessment,
arrangement of pulmonary function test and NIV
titration; review on feeding plan and tolerance; make
appropriate referral to respiratory physicians and
gastroenterologists
– Social worker: regular review of signed AD and
documented ACP, end-of-life preparation and
bereavement support to deteriorating patients
have a heavy symptom burden. A previous study showed that higher
symptom scores of patients were associated with increased caregiver
burden as well.17 Caregivers were often stressed when handling
medical emergencies of patients.18 To address the multifacet needs
of patients with MND and their carers, a standardized PC clinic
follow-up model was set up, with reference made to our previous
success in taking care of patients with end-stage renal failure.19
Each PC clinic visit consists of a 30-minute session of
patient’s joint clinic follow-up with a nurse, social worker, and
physician. The PC nurse and social worker assessed each
patient/caregiver pair before physician consultation and on the
same day of the patient clinic appointment for the sake of
caregiver convenience. Each patient with MND and their care-
givers were interviewed by both a social worker and PC nurse
(usually alternately) in a consultation room. The PC nurse
assessed the symptom burden by the Memorial Symptom
Assessment Score, provided symptom advice with the use of
pamphlets, monitored adherence to drug treatments, ACP dis-
cussions, and psychosocial–spiritual support. The role of the
social worker included giving social support and advice con-
cerning financial issues and difficulties in placing the patient in
home care and arranging respite care for caregivers. The goals
and contents of MND PC clinic are summarized in Table 2.
Cheng et al
In our study, it was found that 76.2% (n ¼ 32) of patients with
MND had documented ACP, while 38.1% (n ¼ 16) had signed
AD. In our PC clinic model as shown in Table 2, the ACP process
involves detailed explanation and development of rapport between
PC team and patients. In all, 87.5% (n ¼ 28) of documented ACP
and 93.8% (n ¼ 15) of signed AD were indeed completed at the
second or third PC clinic sections. This reminded us on the
importance of early PC referral of patients with MND, as the
ACP process often requires a few clinic sections before final
agreement and completion. From our database, we had a rela-
tively low percentage of patients with signed AD (38.1%). This
could be attributed to the relatively late PC referral (with a
median of 4 months at first PC consultation before death).
Besides, this could be influenced by Chinese cultural value
on death and dying. In traditional Chinese culture, death was
sensitive, and mentioning it was sacrilegious and to be
avoided.20 This might contribute to a higher percentage on
ACP discussion, but a lower acceptance on signing an AD.
Conclusion
Motor neuron disease is very different for every patient. It may
start in different areas of the body and progress in different pat-
terns and at different rates. There is no “recipe” for managing
MND. Therefore, reviewing and assessing the needs of patients
and their families, information provision, and grasping the oppor-
tunity for discussion about their needs are essential. Although
MND offers an optimal model of coordinated interprofessional
care, there could be lack of integrated care at EOL in most lower-
and middle-income countries. Besides, the transition of care
between care settings remains a concern. With the major cause
of death contributed to pneumonia and respiratory failure, often
patients with MND died in non-PC settings. The ACP remains a
cornerstone in MND care and therefore PC team should be
involved early in the care model of patients with MND.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, author-
ship, and/or publication of this article.
References
1. Rooney J, Byrne S, Heverin M, et al. A multidisciplinary clinic
approach improves survival in ALS: a comparative study of ALS
in Ireland and Northern Ireland. J Neurol Neurosurg Psychiatry.
2015;86(5):496-501.
2. Van den Berg JP, Kalmijn S, Lindeman E, et al. Multidisciplinary
ALS care improves quality of life in patients with ALS. Neurol-
ogy 2005;65(8):1264-1267.
3. Clinical Standards Department at the Royal College of
Physicians. Long-term Neurological Conditions: Management
at the Interface Between Neurology, Rehabilitation and Palliative
Care - National Guidelines (Concise Guidance to Good Practice).
5
Royal College of Physicians of London. 2008. https://www.
rcplondon.ac.uk/guidelines-policy/long-term-neurological-
conditions. Accessed September 28, 2017.
4. Borasio GD, Gelinas DF, Yanagisawa N. Mechanical ventilation
in amyotrophic lateral sclerosis: a cross-cultural perspective.
J Neurol. 1998;245(suppl 2):S7-S12; discussion S29.
5. Sherman MS, Paz HL. Review of respiratory care of the patient
with amyotrophic lateral sclerosis. Respiration. 1994;61(2):61-67.
6. Alle SM, Hunt B, Green M. Fall in vital capacity with weakness.
Br J Dis Chest. 1985;79(3):267-271.
7. 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 Standards Subcommittee of the Amer-
ican Academy of Neurology. Neurology 2009;73(15):1218-1226.
8. Lyall RA, Moxham J, Leigh N. The control of symptoms. Dys-
pnoea. In: Oliver D, Borasio GD, Walsh D, eds. Palliative Care in
Amyotrophic Lateral Sclerosis (motor neurone disease). Oxford,
UK: Oxford University Press; 2000:43-62.
9. Gordon PH. Amyotrophic lateral sclerosis: pathophysiology,
diagnosis and management. CNS Drugs. 2011;25(1):1-15.
10. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ,
Gibson GJ. Effects of non-invasive ventilation on survival and
quality of life in patients with amyotrophic lateral sclerosis: a
randomised controlled trial. Lancet Neurol. 2016;5(2):140-147.
11. Sivak ED, Cordasco EM, Gipson WT. Pulmonary mechanical
ventilation at home: a reasonable and less expensive alternative.
Respir Care. 1983;28(1):42-49.
12. Radunovic A, Annane D, Rafiq MK, Mustfa N. Mechanical ven-
tilation for amyotrophic lateral sclerosis/motor neuron disease.
Cochrane Database Syst Rev. 2013;(3):CD004427.
13. Radunovic A, Annane D, Jewitt K, Mustfa N. Mechanical venti-
lation for amyotrophic lateral sclerosis/motor neuron disease.
Cochrane Database Syst Rev. 2009;(4):CD004427.
14. Kleopa KA, Sherman M, Neal B, Romano GJ, Heiman-Patterson
T. BiPAP improves survival and rate of pulmonary function
decline in patients with ALS. J Neurol Sci. 1999;164(1):82-88.
15. Rafiq MK, Proctor AR, McDermott CJ, Shaw PJ. Respiratory
management of motor neurone disease: a review of current prac-
tice and new developments. Pract Neurol. 2012;12(3):166-176.
16. Goldstein LH, Atkins L, Leigh PN. Correlates of quality of life in
people with Motor Neuron Disease (MND). Amyotroph Lateral
Scler Other Motor Neuron Disord. 2002;3(3):123-129.
17. Alvarez-Ude F, Valdés C, Estébanez C. Health-related quality of life of
family caregivers of dialysis patients. J Nephrol. 2004;17(6):841-850.
18. Beanlands H, Horsburgh ME, Fox S, et al. Caregiving by family
and friends of adults receiving dialysis. Nephrol Nurs J. 2005;
32(6):621-631.
19. Chan KY, Yip T, Yap DY, et al. Enhanced psychosocial support for
caregiver burden for patients with chronic kidney failure choosing
not to be treated by dialysis or transplantation: a pilot randomized
controlled trial. Am J Kidney Dis. 2016;67(4):585-592.
20. Cheng HWB, Li CW, Chan KY, Ho R, Sham MK. Bringing
palliative care into geriatrics in a Chinese culture society–results
of a collaborative model between palliative medicine and geria-
trics unit in Hong Kong. J Am Geriatr Soc. 2014;62(4):779-781.