Palliative Care in Amyotrophic Lateral Sclerosis (ALS)

Stephen B. Singh, MD, CCFP, Cert Pall MedAdjunct Professor, Dept. of Family MedicineQueen’s University, Kingston, ONSeptember 11, 2012

Objectives

1. Recognize signs and symptoms of ALS at various stages

2. Know about interventions that can prolong life and/or increase quality of life

3. Understand the role of an interdisciplinary team

4. Discuss the resources available in your communities

Outline

What is ALS?SymptomsInterdisciplinary TeamResources

DisclosureNo conflict of interest to declare

Case

“Fred”: 65 y.o. man, previously healthy, no family history, no medications

6 months ago: right foot drop

3 months ago: left hand fasciculations

1 month ago: right arm atrophy

2 weeks ago: progressive dysphagia to solids

Case Continued

Decreased reflexes in upper limbs, increased reflexes in lower limbs, fasciculations seen, right leg atrophic, spastic gait, no sensory loss

CT head: no obvious mass or bleed; lab investigations unremarkable

Urgent referral to neurologist, clinical diagnosis of ALS made

What is ALS?

“Lou Gehrig’s Disease” in Canada/US, “Motor Neurone Disease” in UK/elsewhere

Etiology unknown

ALS Society of Canada

What is ALS?

Progressive, terminal neurodegeneration, loss of upper and lower motor neurons

Eventual paralysis of voluntary muscles and inability to swallow, speak and breathe

Spares eyes, heart, bowel, bladder and sexual organs

Can have cognitive and behavioural changes, mimicking fronto-temporal dementia

ALS Society of Canada, Bedlack 2010

The Numbers

Age of onset 40-70 (average age 55)

Affects men and women

Incidence 2 per 100,000 per year

2500-3000 Canadians currently live with ALS

ALS Society of Canada

The Numbers

Kingston ALS clinic (Dr. J. Wee) follows 40 patients per year, with 12 new consults per year

Life expectancy typically 2-5 years20% live over 5 years, 10% live over 10 years

Mortality 2 per 100,000 per year

ALS Society of Canada, Wee 2012

Classification

Classical (sporadic) ALS: 90% of cases

Familial (genetic) ALS: 5-10%20% of these related to mutation in copper zinc superoxide dismutase (SOD1) on chromosome 21

Region of onset: limb vs. bulbar (mouth/face/throat)

Not contagious

ALS Society of Canada

Diagnosis

No specific lab or investigation

Diagnosis made by neurologist (early referral!)

Revised El Escorial criteria:Lower motor neuron (LMN) degeneration seen clinically, by EMG or biopsy; ANDUpper motor neuron (UMN) degeneration seen clinically; ANDProgressive spread of signs or symptoms within a region or to other regions

Elman 2011

Signs

Usually onset is focal and asymmetric

Can begin with limb wasting and weakness, or changes in speech and swallowing

ALS Society of Canada

LMN Signs

Muscle weakness and atrophy (including diaphragm)

Fasciculations

Muscle cramps

Hyporeflexia

Flaccidity

UMN SignsDysarthria (impairment of muscles of speaking)

Dysphagia (difficulty swallowing)

Dyspnea

Pseudobulbar affect (inappropriate uncontrollable laughter or crying)

Sialorrhea (excessive salivation)

Hyperreflexia

Outline

What is ALS?Symptoms:

DysarthriaDysphagiaDyspnea

Interdisciplinary TeamResources

SialorrheaPseudobulbar AffectPain

Back to Case

Fred was referred to interdisciplinary team

Frustrated, anxious, fearful

Team took time to explain how they could help

1. Dysarthria

Speech affected by weakness or paralysis of muscles of lips, tongue, jaw, soft palate and larynx

Slurring, hoarseness, breathy voice

Isolating and frustrating; loss of control if misunderstood or ignored

1. Dysarthria Management

Early SLP and OT referral

Augmentative and Alternative Communication (AAC):

Alphabet boardLightwriterTTYETRAN board

1. Board with Words

Permission of patient obtained by Dr. I. Stewart

1. Communication Tips

Position face to face

Establish reliable “yes” and “no”

Do not interrupt or try to finish sentences unless asked by a patient to do so

ALS Society of Canada

2. Dysphagia

Weakness of lips, tongue, masseter, soft palate or esophagus

Coughing, difficult chewing and swallowing, reduced airway protection

Drooling, malnutrition, dehydration or aspiration (increased risk of pneumonia)

2. Dysphagia Management

SLP: Swallowing assessment and adviceDietician: Texture modification, supplements

and eating strategiesOT: Adapted feeding tools, arm

supports, etc.

Hypermetabolic state:Loss of muscle mass, decreased intake, increased energy cost of activities

Assess bowel function and monitor weight

2. Tube FeedingWhen to consider?

Eating becomes exhaustingNutritional goals not being metOral intake time-consuming

G-tube, J-tube, PRG tube

Tube feeding does not eliminate risk of aspiration

Can still have oral feeds if tolerated

ALS Society of Canada, Phukan 2009

2. Tube Feeding Continued

Timing of PEG depends on breathing function, coordinated by neurologist and respirologist

Early discussion of wishes and goals of care

Tube feeding improves both quality and quantity of life

ALS Society of Canada

3. DyspneaShortness of breath, fatigue,anxiety, claustrophobia and insomnia

Speaking or eating can be difficult

Hypoventilation worse during sleep

Increasing CO2 levels headaches, somnolence, nausea

Respiratory failure is most likely cause of death

ALS Society of Canada

3. DyspneaMonitor pulmonary function (e.g. vital capacity, cough ability)

Measuring ABG helpful to guide prognosis and see if O2 warranted

In CO2 retention, target SpO2 to 88-92%

Clarify treatment goals with O2

In hypoventilation, BiPAP improves quality of sleep (including REM sleep)

ALS Society of Canada, Bede 2011, Fitzpatrick 2012

3. Dyspnea Management

Options:Medical managementNon-invasive ventilation (BiPAP)Tracheostomy and long-term invasive mechanical ventilation

3. Respiratory Secretions

Secretions compromise airway, add to discomfort and panic

Insufflator/exsufflator (Cough Assist) device

Suctioning

ALS Society of Canada

3. Dyspnea Management

BiPAP improves quality (and maybe quantity) of life

Invasive mechanical ventilation appropriate if goal is long-term survival, with supports

Advance directive discussion is key

Emphasize that “choking to death” is almost unheard of in ALS

Miller 2009

4. Sialorrhea

Normal saliva production but difficulty clearing due to impaired muscle function

Anterior pooling of secretions and poor lip seal

4. Sialorrhea ManagementAnticholinergics:

Atropine, amitriptyline, scopolamine patches (S/E xerostomia and constipation)

Good oral hygiene

Portable suction device

Botox injections into parotid or irradiation of salivary glands (Level B evidence) if medically refractory

Tracheostomy if choking is life-threateningALS Society of Canada, Miller 2009

5. Pseudobulbar Affect

Emotional lability: Uncontrolled / inappropriate laughter or crying

Due to lost inhibition of limbic motor neurons

Seen in 50% of ALS patients

Can be associated with frustration, social anxiety and social withdrawal

ALS Society of Canada

5. Pseudobulbar Affect Management

Screen for depression

Discuss social management

Consider TCAs, SSRIs, valproate or lithium

Neurodex:Combo of dextromethorphan and quinidineLevel B evidence of benefitUnder investigation for safety in the U.S.

ALS Society of Canada, Miller 2009

6. PainMuscle weakness, stiffness, immobility

Inability to maintain spinal posture

Neuropathic pain from entrapment or positioning

Muscle cramps

Jaw spasms and laryngospasm

ConstipationALS Society of Canada

6. Pain Management

Positioning and PT referral:Stretching, repositioning, passive movements to prevent stiffness

Medications:NSAIDs, opioids, anticonvulsants, TCAsConsider baclofenQuinine for spasms no longer recommendedInsufficient data re specific treatment of cramps or spasms in ALS (Level U – unknown)

ALS Society of Canada, Miller 2009

Back to Case

Fred thanks you for the info

“Is there any treatment?”

Riluzole

Riluzole prolongs trach-free survival but not shown to help quality of life

American Academy of Neurology:4 RCTs show 2-3 month benefit in survival5 cohort studies showing up to 21 month benefit

Cost $900/month, not in ODB database

Prescribed 50 mg PO BID

ALS Society of Canada, Bedlack 2010, Miller 2009

Riluzole ContinuedMechanism unclear

? inhibits glutamate release

Generally well tolerated, some nausea and fatigue

Other drugs have no evidence in changing disease course:

LithiumVitamin EAcetylcysteine

L-methionineSeleniumCreatine

Bedlack 2010

Outline

What is ALS?SymptomsInterdisciplinary TeamResources

Interdisciplinary TeamGoals are to provide information, promote function and independence, provide hope, conduct ongoing assessments, act as advocate

Patient

Family MD

Spiritual Care

Neurologist

SLP

GI

Dietician

Physiatrist PT SWResp

OT

ALS Nurse

HospicePalliative

Care

Pharmacy

Outline

What is ALS?SymptomsInterdisciplinary TeamResources

ResourcesALS Society of Canada: www.als.ca

Resources for patients/families and healthcare professionalsLocal Kingston chapter no longer in operation

Canadian ALS Research Network: www.alsnetwork.ca

ALS Association (U.S.): www.alsa.org

Canadian Hospice and Palliative Care Assoc.: www.chpca.net

ALS Clinic (Kingston)The Adult Neuromuscular Clinic (Dr. J. Wee)St. Mary's of the Lake Hospital Site340 Union Street, Postal Bldg. 3600Kingston, ON K7L 5A2Tel: 613-544-1894Fax: 613-544-8640

Operates once a monthFollows 40 patients/year, incl. 12 new consultsPatients referred upon diagnosisPatients unable to cope at home may be CCCcandidates

Wee 2012

How to Improve?

ALS Society branch no longer in operation in Kingston

Need for Augmentive Adaptive Communication (AAC) services

Funding removed by MOH from Kingston ~10 years ago

Need for hospice care for end-of-life!

Respite beds, ventilator beds

Back to Case

Fred started on Riluzole and continues to follow-up with the ALS team

Declines slowly over the next 2 years

Opts for G-tube and BiPAP

Back to Case

Eventually no longer able to tolerate BiPAP

Had decided previously not to pursue invasive mechanical ventilation

Opts to stay at home with family supports, CCAC and visiting physician

Dies at home 3 years after diagnosis, comfortably, from respiratory failure

SummaryALS is a progressive, terminal disease

Early referral to a neurologist for diagnosis, and to an interdisciplinary ALS team is key

Many symptoms have effective palliative treatments

Discuss goals of care early, especially around feeding and breathing

Always help patients maintain hope

Objectives Revisited

1. Recognize signs and symptoms of ALS at various stages

2. Know about interventions that can prolong life and/or increase quality of life

3. Understand the role of an interdisciplinary team

4. Discuss the resources available in your communities

AcknowledgementsThanks to:

Dr. J. Wee Dr. I. StewartDr. R. ViolaDr. J. Tang

References

1. ALS Society of Canada [Internet]. A guide to ALS patient care for primary care physicians. Available from: http://www.als.ca/sites/default/files/files/Physicians%20CD/A%20Guide%20to%20ALS%20Patient%20Care%20For%20Primary%20Care%20Physicians%20English.pdf

2. Bedlack RS. Amyotrophic lateral sclerosis: current practice and future treatments. Curr Opin Neurol. 2010 Oct;23(5):524-9.

3. Bede P, Oliver D, Stodart J, van den Berg L, Simmons Z, O Brannagáin D, Borasio GD and Hardiman O. Palliative care in amyotrophic lateral sclerosis: a review of current international guidelines and initiatives. J Neurol Neurosurg Psychiatry. 2011 Apr;82(4):413-8. Epub 2011 Feb 5.

4. Borasio GD, Voltz R, Miller RG. Palliative care in amyotrophic lateral sclerosis. Neurol Clin. 2001 Nov;19(4):829-47.

References

5. Elman LB and McCluskey L. Diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease. UpToDate Online. Updated Feb 2011. Accessed January 2012.

6. Fitzpatrick M. Personal communication by email. Jan 2012.

7. Maessen M, Veldink JH, van den Berg LH, Schouten HJ, van der Wal G, Onwuteaka-Philipsen BD. Requests for euthanasia: origin of suffering in ALS, heart failure, and cancer patients. J Neurol. 2010 Jul;257(7):1192-8. Epub 2010 Feb 11.

8. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ and Woolley SC. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009 Oct 13;73(15):1227-33.

References

9. Mitumoto H and Rabkin JG. Palliative care for patients with amyotrophic lateral sclerosis: "prepare for the worst and hope for the best". JAMA. 2007 Jul 11;298(2):207-16.

10. Phukan J, Hardiman O. The management of amyotrophic lateral sclerosis. J Neurol. 2009 Feb;256(2):176-86. Epub 2009 Feb 17.

11. Radunović A, Mitsumoto H, Leigh PN. Clinical care of patients with amyotrophic lateral sclerosis. Lancet Neurol. 2007 Oct;6(10):913-25.

12. Wee J. Personal communication by email. Jan 2012.