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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.