Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis PDF

Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Anne Kloos, PT, PhD, NCS Not for reproduction or redistribution Objectives At the end of the presentation the participant will be able to 1. Differentiate between classical A...

Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Anne Kloos, PT, PhD, NCS Not for reproduction or redistribution Objectives At the end of the presentation the participant will be able to 1. Differentiate between classical ALS and its variants 2. Justify the use of outcome measures based on current evidence for use in clients with ALS 3. Justify the use of different physical therapy treatment interventions for clients with ALS based on current evidence 4. Select appropriate physical therapy assessments and interventions for a client with ALS presented in a case study based on the client’s individual condition and stage of the disease Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS  Chapter One Differentiating Classical ALS and its Variants Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Amyotrophic Lateral Sclerosis Most common and devastatingly fatal of all motor neuron diseases Characterized by a progressive degeneration and loss of motor neurons in the spinal cord, brain stem, and motor cortex About 5,000 people in the U.S. are diagnosed with ALS each year – Worldwide, this disorder occurs in two to five per 100,000 individuals Onset: late fifties or early sixties Men are more affected than women (ratio 1.56:1) Etiology: most cases unknown but probably involves a combination of genetic and environmental factors Disease progression varies widely among affected individuals but most people die within two to ten years after diagnosis Statland et al., 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Classical Amyotrophic Lateral Sclerosis (ALS) 2/3 of ALS population; more common in men Involvement of upper and lower motor neurons Areas spared until later stages of disease: cranial nerves III, IV, VI and S1-S3 Early signs: progressive limb weakness, reduced dexterity, wasting of hands, muscle fasciculations – With additional bulbar involvement, there is wasting of the tongue, and muscles for speech and swallowing Person may complain of cramps and fatigue UMN signs and symptoms LMN signs and symptoms Yes- brainstem and spinal regions Yes- brainstem and spinal regions Statland et al., 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Primary Lateral Sclerosis (PLS) Rare condition (about 5% of those with ALS) Pathophysiology: dysfunction of corticospinal tracts leading to upper motor neuron signs and symptoms Key examination findings can include spasticity, upper motor neuron pattern weakness, and pseudobulbar findings Normal electromyography of limbs and bulbar muscles Can progress to ALS, but if no LMN signs are seen after four years, prognosis is good with life expectancy comparable to normal UMN signs and symptoms LMN signs and symptoms Yes- brainstem and spinal regions No Statland et al., 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Progressive Spinal Muscular Atrophy (PMA) 5 to 10% of those who present with LMN changes only (flaccid weakness, atrophy, fasciculation and hyporeflexia or areflexia) Presents in limbs, but bulbar palsy can develop later Mainly affects men (male:female ratio: 2:1) May be limited to one region: flail arm syndrome (cervical) or flail leg syndrome (lumbosacral) Progression varies from slow to very rapid; patients with slowly progressive spinal muscular atrophy have mean survival five to ten years UMN signs and symptoms LMN signs and symptoms No Yes – spinal regions Liewluck & Saperstein, 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Bulbar Onset ALS 1/3 of patients with ALS present with progressive bulbar palsy Affects cranial nerves IX to XII causing dysarthria and dysphagia If corticobulbar tract is affected, tongue muscle spasticity leads to dysarthria. Pseudobulbar palsy may also occur. Some patients have bulbar symptoms for extended period of time (called isolated bulbar ALS) Bulbar onset ALS more common in older females with male:female ratio 2:3 Type UMN signs and LMN signs and symptoms symptoms Progressive bulbar palsy No Yes – brainstem region Pseudobulbar palsy Yes – brainstem region No Jawdat et al., 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS ALS with Frontotemporal Dementia (FTD) Tar DNA binding Protein 43 (TDP-43) positive ubiquinated cytoplasmic inclusions identified in almost all cases of ALS and over 50% of patients with frontotemporal dementia Cognitive symptoms and dementia once thought to be uncommon ALS symptom. Recent studies suggest that – Roughly 30% of patients with ALS demonstrate mild to moderate cognitive and/or behavioral impairments without dementia – 20% of individuals with ALS will develop FTD Common deficits involve executive function, either language or personality. Causes problems with judgment, impulsivity, decreased ability to handle routine tasks. Can adversely affect compliance. Clinical course is usually more rapid in patients with ALS-FTD. Woolley and Strong, 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Familial ALS Affects 5 to 10% of patients with ALS Mutations in the following genes cause familial ALS and contribute to the development of sporadic ALS – C9orf72 gene (30 to 40% of familial ALS in the US and Europe) – SOD1 gene (15-20% of familial ALS worldwide); >90 mutations in the SOD (superoxide dismutase) enzyme alter the enzyme's ability to protect against free radical damage to motor neurons – TARDBP and FUS genes (each account for about 5% of familial cases). Other genes are ANG, ALS2, SETX, and VAPB. Boylan, 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Familial ALS (cont.) Genetic risk for ALS probably represents combined effects of multiple genes that establish a person’s overall genetic susceptibility, acting with environmental and random effects leading to disease onset Pattern of inheritance varies depending on the gene involved; most cases are inherited in an autosomal dominant pattern. Less frequently, ALS is inherited in an autosomal recessive or an X-linked dominant pattern Onset: late forties or early fifties No differences between familial and sporadic ALS on neurological examination Boylan, 2015 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Medications Disease-modifying – Riluzole: decreases glutamate release. Prolongs survival by at least a few months and slows the progress of ALS, allowing the patient more time in the higher-functioning states. Symptomatic treatment – Cramps (quinine, Dilantin, Valium, Baclofen) – Spasticity (Baclofen, Valium antrolene) – Sialorrhea (Elavil, Robinul, Atropine, Scopolamine, Botox) – Sleep disturbance (Elavil, Valium) – Depression (Elavil, Prozac, Zoloft) – Excessive laughing and crying (Elavil, Paxil, Prozac) Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS  Chapter Two Evidence-Based Examination and Treatment for the Client with ALS Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Physical Therapy Across Disease Stages Early Stage Middle Stage Late Stage Common Mild-moderate Severe muscle Severe weakness Impairments weakness in weakness some of axial and and Activity specific muscle groups; mild-mod. extremity muscles Limitations groups weakness in others Complete Difficulty with Progressive decline dependence with mobility and in mobility and ADLs mobility and ADLs ADLs towards Increasing fatigue, Dysarthria, end of stage pain dysphagia, respiratory compromise, pain PT Goals Prevent inactivity Prevent falls Maintain vital Prevent fear of Reduce mobility functions moving and limitations (i.e., Prevent secondary falling transfers, posture, complications Maintain or reaching/grasping, (pressure sores, improve physical balance, gait) contractures, pain) capacity Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Physical Therapy Assessment in ALS Due to the variety and various combinations of regions affected in ALS, the therapist’s examination must be tailored for each patient to determine his/her impairments (primary, secondary, and composite) and how they relate to activity limitations/ participation restrictions PT assessment should start early and be completed at regular intervals throughout the individual’s disease course using standard outcome measures to determine the extent and rate of progression of the disease and plan interventions in a timely manner Early and ongoing assessment allows the healthcare team to – Promote optimal health and wellness – Teach exercise to delay or prevent disease complications – Identify changes in the client’s condition early to better teach ways to modify activity and participation and maintain involvement in these areas for as long as possible Kloos et al., 2016 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Body Function and Structure Assessment Cranial nerve screen Cognitive screen (verbal fluency, Frontal Behavioral Inventory) Psychological screen (Beck Depression Inventory, Patient Health Questionnaire-2) Pain (VAS) ROM Muscle strength (MMT, Maximal Voluntary Isometric Contraction (MVIC) using strain gauge tensiometer or dynamometer) Reflexes (DTRs, Babinski, Hoffmann) Tone (Ashworth Scale) Respiratory assessment (VC, FVC, aerobic capacity and endurance testing) Fatigue (Fatigue Severity Scale) Kloos et al., 2016 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Verbal Fluency Testing For this task, subjects are asked to produce as many words as possible beginning with a specified letter in 60 seconds. This task is an assessment of the spontaneous production of words given a certain restriction. Normative values for adults are published1 1. See Tombaugh et al.(1999) article in references Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Activity Measures ALS Functional Rating Scale, ALSFRS-Revised Other functional measures (ALS Severity Scale, Norris Scale) General health1 Balance (Tinetti Mobility Test, Timed Up and Go, Berg Balance Scale) Gait (timed walk test) 30 second chair stand test Functional Status (Functional Independence Measure) Hand/UE function (Purdue pegboard, nine hole peg test) Kloos et al., 2016 1. Schwab & England Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS ALS Functional Rating Scale-R Measures ADL and global function ALSFRS-R Items Used to assess response to 1. Speech treatment or disease progression 2. Salivation Total possible score 48 with higher 3. Swallowing scores reflecting worse 4. Handwriting performance 5. Cutting food 6. Dressing and hygiene 44 consecutive ALS patients were 7. Turning in bed assessed with ALSFRS at 8. Walking baseline and then longitudinally at 9. Climbing stairs approximately every three months 10. Dyspnea totaling four visits1 11. Orthopnea – MDC = 6.74 12. Respiratory insufficiency – Excellent interrater/intrarater Find at: http://www.outcomes- reliability (ICC = 0.87 to 0.96) umassmed.org/als/alsscale.aspx Cedarbaum et al., 1999 1. Miano et al., 2004 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Schwab and England Scale of Capacity for Daily Living Schwab and England scale estimates the abilities of individuals relative to a completely independent situation One hundred percent indicates a completely independent individual and 0% indicates an individual in who is no longer functioning McRae et al., 2002 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Schwab and England Scale of Capacity for Daily Living (cont.) % Category A4 100 The patient is completely independent 90 The patient is completely independent and is able to do all chores, but with some degree of slowness, difficulty or impairment 80 The patient is completely independent in most chores, but it takes twice as long 70 The patient is not completely independent and experiences more difficulty with some chores 60 The patient is somewhat dependent 50 The patient is more dependent 40 The patient is very dependent 30 Now and then, with some effort the patient can do a few chores alone; however, much help is needed 20 The patient can do nothing alone. He or she can be a slight help with some chores 10 The patient is totally dependent and helpless; a complete invalid McRae et al., 2002 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Balance, Gait, and Fall Risk Assessment Balance tests that have been examined in the ALS population include – Tinetti Mobility Test (TMT): Lower Tinetti Balance Test scores were moderately to strongly related to lower extremity muscle weakness and activity limitations in individuals with ALS and were found to be a reliable measure for individuals in the early or early-middle stages of ALS – Timed Up and Go (TUG) study found that TUG times increased linearly over 6 months, were negatively correlated with MMT scores and functional measures, and predicted falls using a cutoff of 14 seconds in 31 patients with ALS Kloos et al., 1998; Kloos et al., 2004; Montes et al., 2007 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Balance, Gait, and Fall Risk Assessment (cont.) Postural sway during static stance in ambulatory individuals with ALS was not different than control subjects despite significant weakness and spasticity, possibly due to intact sensation Gait stability, efficiency, safety, and endurance with or without the use of orthotic and assistive devices should be assessed regularly. Timed 15-feet walk tests have been used to assess gait in clinical trials. Kloos et al., 1998; Kloos et al., 2004; Montes et al., 2007 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Participation Measures ALS Assessment Questionnaire-40 (ALSAQ-5) – Sample copies found at http://innovation.ox.ac.uk/outcome-measures/amyotrophic- lateral-sclerosis-assessment-questionnaire-alsaq/ ALS Specific QOL instrument Health-related QOL (SF-36, Sickness Impact Profile) Kloos et al.. 2016 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Support for Exercise in ALS Systematic reviews – Dal Bello-Haas & Florence, 2013 – Lui & Byl, 2009 Five moderately strong human studies support benefits (strength, function, and respiratory function) of moderate intensity exercise (aerobic and strengthening) in people with ALS; no adverse effects reported; however, there was a large dropout rate over time Animal studies suggest that moderate exercise (treadmill, running wheel) may exert a neuroprotective effect (slower progression and increased lifespan; in contrast, high intensity exercises may be harmful Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS General Exercise Guidelines Encourage individuals with ALS to continue with sports or daily activities that they enjoyed participating in prior to diagnosis, for as long as they are safely able to do it Start exercise interventions when individuals are in the early stages of the disease so that they have sufficient strength, respiratory function, and endurance to exercise without excessive fatigue Strengthening exercise programs should emphasize concentric rather than eccentric muscle contractions, at moderate resistance and intensity (e.g., 1-2 sets of 8-12 reps or 3 sets of 5 reps), in muscles that have antigravity strength (i.e., over 3 grade strength) exclusively. Avoid high-resistance exercises that increase the risk of muscle damage without an added benefit above moderate exercise. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS General Exercise Guidelines (cont.) Endurance exercise programs should emphasize moderate intensity activities (50-80% peak HR, 11-13 RPE, 3 times per week) as tolerated without inducing excessive fatigue. Rest periods are recommended, especially if continuous activity goes beyond 15 minutes. Encourage an active lifestyle when sport and other exercise activities are no longer feasible Advise patients with ALS to build up their exercise program slowly and to monitor the effects of exercise on their fatigue, pain, and ability to perform daily activities. Teach them to recognize signs of muscle overuse such as delayed onset muscle soreness, peaking between days one and five after activity; a reduction in maximum force production that gradually recovers and/or severe muscle cramping after exercise; a feeling of heaviness in the extremities; increased muscle fasciculations; or prolonged shortness of breath. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS  Chapter Three Providing PT Across the Course of the Disease: Case Study Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Mr. S Mr. S is a 60-year-old, married father of two children (ages 23 and 26), who worked as a policeman. He was in good health until six months ago when he began to experience difficulty using his right hand for writing, holding his handgun, cutting food, and buttoning his shirts. He also noticed that his walking was slower and his legs sometimes felt “heavy and tired” after going up a flight of stairs. Mr. S went to a neurologist, who ordered a variety of tests, including electrophysiology tests. Based on his clinical examination and test results, his neurologist diagnosed him with ALS. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Mr. S Initial Diagnosis Mr. S was referred by his neurologist to outpatient physical therapy with a diagnosis of probable ALS, laboratory supported. He complained that the weakness in his hands, which was worse on the right than the left side, and the stiffness in his legs were interfering with his ability to walk, go up stairs, and play basketball. He noted that it was harder to dribble and make baskets. Other than playing basketball with friends on the weekends, he stated that he did not engage in any regular exercise program. His medications included Riluzole (50 mg b.i.d.) and baclofen (10 mg b.i.d). He had health care benefits through his job with 15 outpatient physical therapy visits allowed per year. He is considered to be in the early stage. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Measures and Early Stage Disease Initial diagnosis: Mr. S complains of weakness in his arms, which is worse on the right than the left side, and of stiffness in his legs that interferes with his ability to walk, go up stairs, and play basketball – ALSFRS-R will provide a baseline measure and assess his performance on ADLs – He may have a ceiling effect on Berg. TMT would be useful for assessing his reactive balance and gait impairments – 10m walk, TUG, 30-second chair stand test, nine-hole peg will allow comparison to adults of same age without HD to better determine areas he should focus on in his PT program and will provide a baseline measure – Patient Health Questionnaire-2 and ALSAQ-5 might be useful for assessing how his diagnosis is affecting his mood and QOL Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Measures and Early Stage Disease (cont.) Body structure and function Participation – Strength/ROM – Reflexes/Tone – ALSAQ-5 – Cranial nerve screen Specific domains – Fatigue Severity Scale Activity measures – ALSFRS-R – Cognitive screen – Ten-meter walk (10MWT) – Tinetti Mobility Test – Psychologic screen – Timed up and go – Respiratory function – Berg Balance Scale – 30-second chair stand test tests – Nine-hole peg test Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS PT Examination at Initial Diagnosis At Mr. S’s first patient visit, the therapist questions him in depth about his job requirements and daily activities. He states that he has to walk a lot in the neighborhood that he patrols and has been feeling more tired at the end of the day. Right arm strength (3 to 4/5 muscle grade), left arm strength (4 to 5/5 grades), bilateral lower extremities (4+-5 proximally) Spasticity: Modified Ashworth Scale scores 1 in bilateral biceps and 1+ in bilateral hamstring and gastrocnemius muscles He performs on the low end of normal range for the 10 MWT (1.25 m/s comfortable), 30-second chair stand test (14 reps), and high end of normal on the TUG (9 seconds) and 9 HPT (right hand = 25 seconds; left hand= 24 seconds) ALSFRS-R scores: mild ADL and mobility deficits: 41/48 and Schwab and England score 90% Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS PT Treatment at Initial Diagnosis The therapist’s diagnostic impression was that the patient’s decreased hand dexterity and weakness was affecting his ability to perform ADLs, and spasticity in his legs was affecting his gait The therapist identified these areas as ones that Mr. S needed to work on – Fitness/aerobic endurance to improve overall health and fitness – Stretching and ROM exercises to maintain flexibility – Education about the disease and support groups – Energy conservation (sitting down or taking a rest when he starts to feel fatigued, taking elevator instead of stairs, etc.) – Referral to OT for hand dexterity training and assistive aids to maximize QOL Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Exercise Prescription Mr. S was advised to continue to play basketball and engage in recreational activities that he enjoyed for as long as possible – Modifications were to stand closer to the basket when shooting or to shoot underhand to utilize strength of biceps In addition, the therapist recommended a progressive walking program or possible use of hydrotherapy pool to maintain or improve strength and ROM Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Mr. S Middle Stage Disease He returns to clinic 18 months later, at which time he complains of – Pain throughout his shoulders bilaterally, right side worse than the left side, especially with overhead activities; he described the pain as “a sharp ache deep in the shoulders” – Bilateral arm weakness that interferes with doing ADLs – Neck pain, if he was sitting for long periods or riding in the car – SOB while walking, bathing, and dressing; some difficulty sleeping at night due to SOB, but not using more than two pillows; he was able to clear secretions with coughing Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Mr. S Middle Stage Disease (cont.) He was able to do most ADLs by himself but very slowly and with much effort. He used a manual wheelchair for mobility in the community, due to fatigue when walking long distances; at home, he needed minimal assistance of one person to go sit to stand and ambulated with a cane or assistance of 1 person. He noticed that his feet would drag during walking. He was no longer able to perform his home stretching program independently because of increased muscle tightness and decreased balance and muscle control. He had fallen backwards on several occasions during the past six months when ambulating without assistance; one occurred in his driveway and resulted in injury to his back. He reports that he is worried about finances and what his future holds. His wife is worried about how she will care for him when he becomes more dependent. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Measures and Middle Stage Disease Middle stage (18 months after diagnosis): Mr. S is not able to work and took an early retirement. He is able to do most ADLs by himself but very slowly and with much effort. He uses a manual wheelchair for mobility in the community due to fatigue; at home, he ambulates with assistance and has had multiple falls. – He is likely to demonstrate deficits on all activity measures due to limb weakness and spasticity – 10m walk, TUG, 30-second chair stand test, nine-hole peg will allow comparison to baseline measures to track the progression of his functional deficits and better determine areas he should focus on his PT program and will provide a baseline measure – Due to his respiratory symptoms, FVC testing would be warranted Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS PT Examination at Middle Stage At his PT visit, the therapist questions him about his falls and what precipitates them Decreased neck and limb strength, distal over proximal; neck extensors 4-, neck flexors G; right arm strength (2 to 3/5 muscle grade), left arm strength (3 to 4/5 grades), bilateral lower extremities (3 to 4/5) Decreased bilateral shoulder AROM and glenohumeral mobility Spasticity: 1+ bilateral biceps; 2 in bilateral quads, gastrocnemius The therapist notes that he is below the normal range for the 10 MWT (.90 m/s), 30-second chair stand test (9), and above normal range on the TUG (14) and 9 HPT (right hand = 30 second; left hand= 27 second) Seated FVC: 3.77 L (77% of predicted). ALSFRS-R scores: 28/48 and Schwab and England 60% (can do most chores but very slowly and with much effort; makes errors) Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS PT Treatment at Middle Stage The therapist’s diagnostic impression was that Mr. S was approaching transition from ambulation with an assistive device for short distances to dependence on a wheelchair for mobility. Pain and decreased shoulder ROM likely due to weakness and/or improper positioning. The therapist recommends – Continuation of daily walking as tolerated – Functional exercises to improve strength and endurance – AROM and positioning advice for shoulder and neck pain – Use of rollator walker, bilateral AFO’s, cervical collar – Referral to OT, Speech, pulmonary, social worker – Education about support groups for family Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Shoulder and Neck Pain The therapist implemented the following interventions to reduce the patient’s shoulder and neck pain – AROM exercises and passive stretching to neck and bilateral shoulder muscles – Gentle joint mobilizations to maintain glenohumeral mobility – Education about proper shoulder support (e.g., using arm rests on chairs or tabletop) and protection (not pulling the arms when caregiver assists him to standing from sitting) – Wearing of a soft cervical collar in the car or when neck pain increases If the patient developed adhesive capsulitis, intra-articular analgesic and anti-inflammatory cocktail injections, followed by a course of aggressive ROM exercises has successfully managed it. Ingels et al., 2001 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Cervical Weakness As Mr. S’s cervical weakness progresses, he will need a semi-rigid or rigid collar to provide adequate support. Collars that offer good support are: – Philadelphia collar™ (Philadelphia Cervical Collar Co., Thorofare, NJ) – Miami-J Collar™ (Jerome Medical, Moorestown, NJ) – Malibu Collar™ (Seattle Systems, Poulsbo, WA) If not well tolerated, the lightweight Headmaster collar™(Symmetric Designs LTD, Salt Spring Island, BC, Canada) has an open design that allows air circulation; however, it may be inadequate if rotation and lateral flexion weakness is also present Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Respiratory Interventions For Mr. S’s shortness of breath with exertional activities and during sleep, the therapist implemented the following interventions – Education on how to balance activity and rest so as not to become too fatigued – Breathing exercises Diaphragmatic breathing Resisted inspiratory exercises with spirometers (ten minute sessions two to three times per day for 12 to 16 weeks) may be beneficial for respiratory function, but evidence is inconclusive – Body positioning to optimize breathing Cheah et al., 2009; Pinto et al., 2012 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Assistive Devices Mr. S is prescribed a rollator walker because he will not have to lift it as he walks or turns. He will need to be able to activate the brakes; if he is unable to squeeze brakes, push-down brakes are preferable, or a 2WW with glides in back can be used. Disadvantage of 2WWs is that it is difficult to make turns with them. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS  Ankle-Foot Orthoses (AFO) Used Most Commonly in ALS Care Type of AFO Description Use Posterior leaf spring (PLS) Medial and lateral trim lines are Mild-to-moderate foot placed posterior to the malleoli, drop somewhat flexible Carbon-fiber lateral or Lightweight, unobtrusive Moderate foot drop posterior strut dorsiflexion (also helps with knee assist brace control) Majmudar et al., 2014 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS  Ankle-Foot Orthoses (AFO) Used Most Commonly in ALS Care (cont.) Type of AFO Description Use Floor reaction orthoses Built to leverage ground reaction Mild-to-moderate foot (FROs), such as the forces to offer a “push” at toe-off to drop with quadriceps ToeOFF braces assist with propulsion and weakness; they also compensate for ankle plantarflexion help compensate for weakness; they also create a knee ankle plantarflexion extension moment to help counteract weakness quadriceps weakness and tendency to knee buckling Hinged (articulated) Include an ankle joint; allow sit-to- Moderate foot drop stand transfers more easily than solid with or without AFOs; anti-spasticity features (such spasticity; sufficient as a plantarflexion stop) can be knee extensor incorporated as needed strength needed for optimal use Majmudar et al., 2014 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Ankle Foot Orthoses For Mr. S’s bilateral ankle dorsiflexor weakness with resultant foot drop, the therapist recommends the following – Anterior ground (floor) reaction carbon fiber AFOs because they are lightweight and supports the foot from the shin rather than the calf, so a larger size shoe is not required. In addition, if muscle is lost from the calf after the brace has been obtained, the fit is still maintained. The leveraging of the ground reaction forces offers a “push” at toe-off to assist with propulsion and will help to counteract his quadriceps weakness and tendency to knee buckling. Knee-ankle-foot orthoses for people with quadriceps weakness are not typically used in ALS due to the weight Majmudar et al., 2014 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Transfers and Mobility Mr. S is having difficulty with sit-to-stand transfers and climbing stairs due to LE weakness. To address these problems the therapist recommends the following interventions – Putting a firm cushion in his chair (two to three inches) or elevating his chair with prefabricated blocks – If that isn’t enough, he might benefit from self-powered lifting seat cushions, which are portable and relatively inexpensive – If possible, moving him to a bedroom on the first floor with access to a bathroom so that he doesn’t have to climb stairs – If that is not possible, use of chair glides or stairway lifts might be an alternative. If insurance will not reimburse for stairway lifts, some medical supply companies may rent them, or the local ALSA or MDA chapter may have recycled lifts to loan. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS ADLs Mr. S’s UE and LE weakness is impairing his ability to carry out many of his ADLs. The therapist could recommend the following adaptive equipment if not already prescribed by OT – Modified utensils, universal cuffs and mobile arm supports to assist him with feeding and eating – Bath tub seats or shower chairs, handheld shower heads, grab bars, raised toilet seats for personal care and bathing – Zipper pulls, button hooks, velcro clothing closures, and elastic shoelaces to make dressing easier – Increasing the size of a pen/pencil with a triangular grip or cylindrical foam to make writing easier and more legible – Book holders, automatic page turners, and an adjustable angle table to make reading easier Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Mr. S Late stage Disease At three years after diagnosis, home physical therapy is requested to assess caregiver demands and to make recommendations. Two years after Mr. S’s diagnosis, he underwent PEG placement due to problems with dysphagia and declining respiratory status. When his FVC declined below 50% predicted, he started using noninvasive positive pressure ventilation (Bi-Pap) at first only at night and now uses it for periods during the day. The patient’s wife states that she is feeling stressed and depressed from doing 24/7 care for her husband. The patient purchased a minivan that is equipped to transport his power wheelchair for going to church or other community events and an electric hospital bed with an anti- pressure mattress. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Measures and Late Stage Disease Late stage: Mr. S requires maximal assistance to stand and is not able to walk. He is dependent for ADLs. – He will have floor effects on the Berg and Tinetti. – 10m walk, TUG, 30-second chair stand test, nine-hole peg test may no longer be possible. – The specific domains measures may be difficult to perform due to speech impairments and respiratory weakness. If there is bulbar involvement, he will need to wear a mask that covers the nose and mouth for respiratory testing. Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Measures and Late Stage Disease (cont.) Body structure and function Participation – Strength/ROM – Reflexes/tone – ALSAQ-5 – Cranial nerve screen Specific domains – Fatigue severity scale measures Activity – ALSFRS-R – Cognitive screen – Ten meter walk (10MWT) – Psychologic screen – Tinetti Mobility Test – Respiratory function – Timed Up and Go tests – Berg Balance Scale – 30 second chair stand test – Nine hole peg test Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS PT at Late Stage At his PT visit, the therapist questions Mr. S and his wife about his mobility and safety at home. His speech is barely understandable. The patient can perform a modified stand pivot transfer from his bed/chair to his power wheelchair with maximal assistance from his wife. When standing with moderate assistance of two he is able to take a few steps but cannot walk. Strength is 0 to 1 strength in his bilateral arms and 1 to 2+ in his legs with marked atrophy and fasiculations visible in his tongue and extremities. Spasticity is two in bilateral biceps and three in his quadriceps and gastrocnemius muscles. FVC is 1.5 L or 29% of predicted and cough is weak ALSFRS-R scores: 10/48 and Schwab and England score is 10% Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS PT at Late Stage (cont.) The therapist recommends – Training his wife and caregivers in Use of a Hoyer lift for transfers Passive ROM to limbs and trunk: daily standing and weightbearing can be done to stretch ankles. Assistive coughing techniques Bed positioning (e.g., neutral position ankle splints) and turning schedule – Participating in as many activities (e.g., reading, computer games, going outside or to places in wheelchair) as possible – Referral to speech and language pathologist for communication device, social worker for respite care to relieve burden on wife Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Wheelchairs Since Mr. S’s insurance company would only reimburse for one wheelchair, Mr. S was advised by the therapist to rent a manual wheelchair or obtain a loaner from a local ALSA or MDA chapter initially. The therapist advised him to get a manual wheelchair with a lightweight frame, small wheelbase, high reclining back, and supports for the head, trunk, and extremities. As the disease progressed, the therapist ordered a power wheelchair customized to the patient’s needs, which included the following features – Tilt-in-space/recline features with a high, firm back and headrest that allowed Mr. S to shift weight and rest while in the chair – A smaller and less heavy wheelbase for ease of maneuverability – Lumbar support and a gel-filled cushion for comfort – Adjustable leg rests and removable armrests for ease of transfer – Potential mounting area for portable respirator equipment if needed Trail et al., 2001 Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Summary Initial visit through early stage, encourage Mr. S to include: – Aerobic exercise (walking, cycling, possibly treadmill) at moderate intensity (65 to 85% age-predicted maximum HR) – Functional strengthening exercises with a focus on core and postural muscle groups – Flexibility exercises with stretching of neck and trunk flexors, shoulder extensors, and internal rotators, hamstrings, gastroc/soleus muscles – Recreational and fun activities such as group walking or aquatic programs, dancing, or playing video games Educate about disease, support groups, energy conservation Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Summary (cont.) Mid-stage, encourage Mr. S to ADD to his program: Education including Mr. S and family on fall prevention strategies – Functional strengthening and balance exercises to ameliorate deficits due to muscle disuse – Use of assistive devices and orthotics to improve gait efficiency and safety Pain management if needed Breathing exercises to maintain respiratory function Adaptive devices to assist with ADLs Referrals to appropriate health care professionals Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Summary (cont.) Late-stage: Family/cargiver education for – guarding and/or assistance during transfers and gait and fall prevention strategies – Bed positioning and turning – ROM exercises – Assistive coughing and pulmonary hygiene Wheelchair prescription Lots of psychologic and emotional support to the patient, family members, and caregivers Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Physical Therapy Physical therapy should be actively involved in the care of the client with ALS from initial diagnosis and throughout the disease Do not discharge, but rather send the client home with a home program and monitor periodically as a part of skilled maintenance therapy If on reassessment the client has had a significant decline or loss of function, then institute a physical therapy plan of care for rehabilitation Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Care Plan Management Using Rehabilitation and Skilled Maintenance Therapy Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS  Thank You Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Not for reproduction or redistribution Anne Kloos, PT, PhD, NCS Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Bibliography MedBridge Advanced Physical Therapy Management of Individuals with Amyotrophic Lateral Sclerosis Anne Kloos, PT, PhD, NCS 1. 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