als

1. National Hospice and Palliative Care Organization’s
Palliative Care Resource Series
Palliative Care in ALS
Written by: Mara Lugassy, MD
Hospice Medical Director
MJHS Hospice and Palliative Care
Assistant Professor
Department of Family and Social Medicine
Albert Einstein College of Medicine

2. Amyotrophic Lateral Sclerosis (ALS):
Definition, Disease Progression and Prognosis
• Progressive fatal neurodegenerative disorder
• Destruction of upper and lower motor neurons in brain and
spinal cord
• Upper motor neuron: spasticity, clonus, hyperreflexia
• Lower motor neurons: weakness, atrophy, fasciculation
• No known cure

3. ALS:
Definition, Disease Progression and Prognosis
• 80% limb onset; 20% bulbar onset
• Progressive involvement all limbs and axial muscles
• Progressive impairment oral, pharyngeal and respiratory
muscles
• Loss of ability to speak, swallow and breathe
• Complete dependency for ADLs and near total paralysis
• Relative sparing of extraocular movements and bladder/bowel
function
• Death from respiratory failure
Tiryaki E, Horak H. ALS and Other Motor Neuron Disease. Continuum. Vol 20(5). Oct.
2014. 1185-1207

4. ALS:
Definition, Disease Progression and Prognosis
• Median survival 3-5 years
• Highly variable survival times
• Prognosis can be modified to some degree by treatment
options
• Approximately 10% of patients with ALS survive >10 years
• Predictors of poorer prognosis:
• Older age
• Bulbar onset
• More rapid progression in early stages of disease
Chio A, Logroscino G, Hardiman O, et al. Prognostic factors in ALS: A critical
review. Amyotroph Lateral Scler. 2009 ; 10(5-6): 310–323

5. Palliative Care in ALS
• Multifaceted role:
– Symptom control
– goals of care discussions
– patient and family support
– assistance with transitions of care
• Recent RTC demonstrating improvements in quality of life and
symptom burden in ALS and other neurodegenerative
diseases with early referral to specialist level palliative care
Veronese S, Gallo G, Valle A, et al. Specialist palliative care improves the quality of life in
advanced neurodegenerative disorders: NE-PAL, a pilot randomised controlled study.
BMJ Support Palliat Care. 2015 Jul 16.

6. Palliative Care in ALS
• A collaborative effort
– Important role for multiple disciplines throughout disease course
• Multidisciplinary ALS clinics:
– Early access to specialist therapies
– improved coordination of care
• Increased median survival (19 months vs. 11 months in
retrospective review of 417 patients)
• Improvement in quality of life
Aridegbe T, Kandler R, Walters SJ, et al. The natural history of motor neuron disease:
assessing the impact of specialist care. Amyotroph Lateral Scler Frontotemporal Degener
2013; 14:13-19.
Van den Berg JP, Kalmijn S, Lindeman E, et al. Multidisciplinary ALS care improves
quality of life in patients with ALS. Neurology. 2005 Oct 25;65(8)1264-7.

7. Multi-disciplinary ALS Care
• Physician (neurologist, palliative care, gastroenterologist)
• Nurse
• Social worker
• Occupational/Physical therapist
• Speech and Language Pathologist
• Respiratory Therapist
• Dietitian
• Psychologist

8. Disease Modifying Pharmacotherapy - Riluzole
• Glutamate inhibitor
• Only medication approved for treatment of ALS
• Prolongs survival (or time to tracheostomy) by 2-3 months
• Modest slowing of decline in limb and bulbar function
• Does not reverse or improve existing symptoms
• No evidence regarding impact on quality of life
• Side effects: GI symptoms, fatigue
• Monitor LFTs
Miller RG, Mitchell JD, Moore DH. Riluzole for amyotrophic lateral sclerosis
(ALS)/motor neuron disease (MND). Cochrane Database Syst Rev 2012;
3:CD001447.

9. Symptom Management
• Sialorrhea
• Thickened secretions
• Pain
• Muscle Cramps
• Spasticity
• Pseudobular affect

10. Symptom Management: Sialorrhea
• Excessive drooling
• Can be managed by:
– minimization of sweet and sour foods which stimulate the
salivary glands
– use of suction devices
– pharmacotherapy, including tricyclic antidepressants and
anticholinergic medications.
– Botulinum toxin and radiation therapy may provide benefit
in cases of severe drooling.

11. Symptom Management: Thickened Secretions
• Difficulty with expectoration and mucous plugging
• May be exacerbated by anticholinergic medications used to
treat sialorrhea.
• Thickened secretions can be managed by:
– Increasing fluid intake
– Air humidification
– Use of mucolytics, including guaifenesin and N-Acetyl
cysteine.
• Expectoration of secretions can also be facilitated by use of a
cough assist device.

12. Symptoms: Pain
• Significant impact on quality of life
– Musculoskeletal
– Immobility
– Atrophy
– Increased pressure on bones and joints
• Interventions
– Attention to positioning and repositioning
– Gentle stretching and range of motion activities
– Analgesics: acetaminophen, NSAIDS, opioids

13. Symptoms: Muscle Cramps
• Occur in up to 95%
• Stretching, positioning, massage
• Limited evidence:
– Antiepileptics (levetiracetam, phenytoin, carbamazepine)
– Benzodiazepines
– Baclofen
• Quinine – no longer recommended due to risk of toxicities
• Mexiletine
– Recent phase II randomized controlled study: reduction in cramp
frequency and intensity
Weiss MD, Macklin EA, Simmons Z, et al. Mexiletine ALS Study Group. A randomized
trial of mexiletine in ALS: Safety and effects on muscle cramps and progression.
Neurology 2016 Apr 19;86(16):1474-81.
Caress JB, Ciarlone SL, Sullivan EA et al. Natural history of muscle cramps in
amyotrophic lateral sclerosis. Muscle Nerve. 2016 Apr;53(4):513-7

14. Symptoms: Spasticity
• Can result in pain, contractures, falls
• Interfere with care
• Stretching, positioning, massage
• Muscle relaxants
– Baclofen, tizanidine
– May worsen weakness

15. Symptoms: Pseudobulbar Affect
• Sudden uncontrollable outbursts of laughter or crying
• Degeneration of corticobulbar tracts
• Occurs in multiple neurologic conditions
• Leads to embarrassment, social isolation, associated with
reduced quality of life
• Can be mistaken for depression

16. Symptoms: Pseudobulbar Affect
• Interventions:
– Education of patients and families
– Dextromethorphan-quinidine (20 mg/10 mg)
– Tricyclic antidepressants, SSRIs
Brooks BR, Thisted RA, Appel SH, et al. Treatment of pseudobulbar affect in ALS with
dextromethorphan/quinidine: a randomized trial. Neurology. 2004;63(8):1364.
Rix Brooks B, Crumpacker D, Fellus J, et al. PRISM: A novel research tool to assess the
prevalence of pseudobulbar affect symptoms across neurologic conditions. PLoS One.
2013; 8(8): e72232

17. Cognitive Impairment
• Occurs in approximately 50% of patients with ALS
• Ranges from subtle findings to dementia
• Deficits in executive function
• Memory relatively preserved
• Frontotemporal dementia in 15%
• Need to consider capacity in medical decision making/goals of
care discussions

18. Communication Deficits
• Dysarthria progressing to total loss of speech
• Significant impact on quality of life
• Source of emotional distress
• Compensatory Strategies
– Minimize distractions
– Slowing of speech
– Over articulation (pronounce each sound)
– Listener repeats understood words
Körner S, Sieniawski M, Kollewe K, et al. Speech therapy and communication device:
impact on quality of life and mood in patients with amyotrophic lateral sclerosis.
Amyotroph Lateral Scler Frontotemporal Degener. 2013 Jan;14(1):20-5.

19. Augmentative/Alternative Communication
• Augmentative communication: supports or supplements impaired
speech
– Ex. Voice amplifier in hypophonia
• Alternative communication: used in the absence of speech
– Ex. Letter board
• Needs change as disease progresses
• Early use can improve quality of life and mood in patients and
caregivers
Londral A, Pinto A, Pinto S, Azevedo L, De Carvalho M. Quality of life in amyotrophic
lateral sclerosis patients and caregivers: Impact of assistive communication from early
stages. Muscle Nerve. 2015;52(6):933

20. ALS and Nutrition
• Significant source of concern for patients and caregivers
• Multiple barriers
• Physical impairment to feeding (arm weakness)
• Progressive dysphagia
• Fear of choking
• Extensive time and energy devoted to obtaining adequate
nutrition
• BMI <18.5 associated with shorter survival
Karam C, Paganoni S, Joyce N et al. Palliative care issues in amyotrophic lateral
sclerosis: an evidence-based review. American Journal of Hospice and Palliative
Medicine. 2016 vol 33(1)84-92.

21. ALS and Nutrition:
Dysphagia and Nutrition Management
• Involvement of nutritionist and speech/swallowing therapist
• Modification of food consistency
• Add thickener to liquids
• Puree solid foods
• Multiple swallows with each bite
• Avoid distractions while eating
• Chin tuck and other techniques
• Addition of calorie rich foods
• Oral supplements

22. ALS and Nutrition: Feeding Tubes
• Consider with weight loss, insufficient oral intake, dehydration
or choking episodes
• Potential benefits
– Stabilize weight loss
– Improve overall survival time
– Possible improvement of quality of life
– Access for medications
– Can continue to eat as tolerated for pleasure without pressure of
maintaining nutrition
• Risks: complications from procedures, blockage, infection,
does not eliminate risk of aspiration.
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 American Academy of Neurology. Neurology.
2009;73(15):1218

23. ALS and Nutrition: Feeding Tubes
• Discuss early in disease course
• Very personal decision
• Consider timing of placement in context of respiratory status
• Should be placed before vital capacity falls below 50% of
predicted
• Increased procedure risks with declining respiratory status
• Review conditions under which patient might desire feeds to
be withdrawn
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 American
Academy of Neurology. Neurology. 2009;73(15):1218.

24. Respiratory Impairment
• Progressive weakness of muscles of respiration
• Hypoventilation
• Best monitored by serial vital capacities
• <50% predicted frequently associated with respiratory
symptoms
• <30% predicted risk of respiratory failure or death
• Early signs
– Daytime fatigue
– Headache
– Orthopnea
– Nighttime dyspnea
Rabinstein AA, Wijdicks EF. Warning signs of imminent respiratory failure in neurological
patients. Semin Neurol. 2003;23(1):97

25. Respiratory Impairment:
Noninvasive Positive Pressure Ventilation
• Improved survival time
– RTC 41 patients: Survival advantage of 205 days in ALS
patients without severe bulbar dysfunction using
noninvasive ventilation compared to standard care.
• Improvement in quality of life
• Improvement in symptom burden
• Variety of masks available to increase tolerance
• Less well tolerated in:
– Dementia
– Bulbar ALS
Radunovic A, Annane D, Rafiqu MK, Mustfa N. Mechanical ventilation for amyotrophic
lateral sclerosis/motor neuron disease. Cochrane Database Sys Review.2013 Mar
28;(3):CD004427.

26. Respiratory Impairment:
Management of Dyspnea
• Assessment and treatment of additional underlying causes
– pulmonary edema, pneumonia, bronchospasm
• Opioids
• Benzodiazepines
• Concurrent management of secretions
• Supplemental oxygen not recommended in absence of overt
hypoxia

27. Respiratory Impairment:
Mechanical Ventilation
• Should be discussed early, on ongoing basis
• Avoid decision making in emergent situations
• Highly personal decision, views may change over time
• Implemented in < 10% of patients
• Should also discuss possible circumstances for withdrawal
– Locked in status
– Dementia

28. End-of-Life Care
• Address patient and family concerns
– Fear of choking or suffocation
– Typically progressively decreased responsiveness due to
hypercapnia
• Reassurance regarding availability of symptom control
measures and non-abandonment
– Dyspnea
– Secretions
– Multiple routes available for administration of comfort
medications
• Referral to hospice and other supportive measures

29. ALS and Hospice
• Critically impaired breathing capacity
– Vital capacity <40% of normal
– Dyspnea at rest, orthopnea, weakened cough or other
signs of respiratory decline
– Mechanical ventilation declined
• Rapid progression of ALS and critical nutritional impairment
evidenced by
– Bedbound status
– Barely or unintelligible speech
– Dysphagia requiring purees
– Progressive weight loss
– Dependency in ADLs

30. ALS and the Military
• Increased incidence of ALS with military service, independent
of location, time of service, or branch of military
• Considered a service connected diagnosis
• Entitled to range of benefits if ALS developed during or
following military service (at least 90 days of active duty)
– regardless of branch, type of service, or location
ALS in the Military: Unintended Consequences of Military Service. Feb 4, 2013. ASL
Association.alsa.org/als-care/veterans/military-white-paper.html

31. Benefits for Veterans
• Coverage of medications, medical care, and medical
equipment
• Disability compensation
• Special Monthly Compensation
• Specially adaptive housing grant
• Automobile grant
• Adaptive Equipment
• Aid and Attendant allowance
• Dependency and Indemnity compensation

32. ALS Resources
• ALS association
– Multidisciplinary clinics
– Patient/caregiver resources
– Equipment lending
• Local organizations
• Home based care
• In person or online support communities
– patientslikeme.com

33. Summary
• ALS benefits from a multidisciplinary approach to care.
• Goals of care should be addressed early and on an ongoing
basis.
• Despite the lack of cure, interventions exist that may prolong
survival and improve quality of life.