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Amyotrophic Lateral Sclerosis-ALS PowerPoint Presentation

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Description : Check out this medical PowerPoint presentation titled "Amyotrophic Lateral Sclerosis (ALS)”.

This medical PowerPoint presentation is about amyotrophic lateral sclerosis (ALS), also known as Lou Gehrigs disease. Amyotrophic lateral sclerosis is a neurodegenerative disease that affects the nerve cells responsible for controlling voluntary muscle movement. It is a progressive disease, meaning it worsens over time, and it ultimately leads to muscle weakness, paralysis, and eventually respiratory failure.

The exact cause of ALS is not fully understood, but it is believed to be a combination of genetic and environmental factors. Some genetic mutations have been identified as risk factors for the disease, but most cases of ALS are not directly inherited.

The symptoms of ALS can vary from person to person, but they generally include muscle weakness, muscle cramps, twitching, and difficulty with speech, swallowing, and breathing. There is currently no cure for ALS, but there are treatments available that can help manage symptoms and improve quality of life for people with the disease.

Management of ALS often involves a team of healthcare professionals, including neurologists, respiratory therapists, speech-language pathologists, physical therapists, and occupational therapists. The goal of treatment is to maintain independence, improve function, and provide comfort and support for the person with ALS and their family.

Tags : Amyotrophic Lateral Sclerosis, Lou Gehrigs disease, neurodegenerative disease, Neurology, Neurology PowerPoint presentation

Published on : Jun 20, 2023 | Views : 2147 | Downloads : 1

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Slide 1-

National Hospice and Palliative Care Organization’sPalliative Care Resource SeriesPalliative Care in ALSWritten by: Mara Lugassy, MDHospice Medical DirectorMJHS Hospice and Palliative CareAssistant ProfessorDepartment of Family and Social MedicineAlbert Einstein College of Medicine
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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
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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
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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
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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.
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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.
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Multi-disciplinary ALS Care Physician (neurologist, palliative care, gastroenterologist) Nurse Social worker Occupational/Physical therapist Speech and Language Pathologist Respiratory Therapist Dietitian Psychologist
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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.
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Symptom Management Sialorrhea Thickened secretions Pain Muscle Cramps Spasticity Pseudobular affect
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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.
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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.
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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
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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
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Symptoms: Spasticity Can result in pain, contractures, falls Interfere with care Stretching, positioning, massage Muscle relaxants Baclofen, tizanidine May worsen weakness
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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
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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
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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
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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.
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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
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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.
Slide 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
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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
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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.
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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
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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.
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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
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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
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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
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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
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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
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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
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ALS Resources ALS association Multidisciplinary clinics Patient/caregiver resources Equipment lending Local organizations Home based care In person or online support communities patientslikeme.com
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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.