majmudar2014.pdf

INVITED REVIEW REHABILITATION IN AMYOTROPHIC LATERAL SCLEROSIS: WHY IT MATTERS SALONY MAJMUDAR, MD,1 JASON WU, MD,1 and SABRINA PAGANONI, MD, PD1,2 1 Department of Physical Medicine and Rehabilitation, Spaulding Rehabilitation Hospital, Harvard Medical School, Boston, Massachu- setts, USA 2 N...

INVITED REVIEW REHABILITATION IN AMYOTROPHIC LATERAL SCLEROSIS: WHY IT MATTERS SALONY MAJMUDAR, MD,1 JASON WU, MD,1 and SABRINA PAGANONI, MD, PD1,2 1 Department of Physical Medicine and Rehabilitation, Spaulding Rehabilitation Hospital, Harvard Medical School, Boston, Massachu- setts, USA 2 Neurological Clinical Research Institute (NCRI), Massachusetts General Hospital, Boston, Massachusetts, USA Accepted 6 February 2014 ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a fatal neu- with ventilatory muscle failure as the most com- rodegenerative disease that results in a constellation of prob- lematic symptoms and a high patient and caregiver burden. mon cause of death.3 The only U.S. Food and Multidisciplinary care includes rehabilitation interventions that Drug Administration (FDA)–approved drug for have the goal of assisting people to teach their fullest potential ALS, riluzole, only confers a modest survival bene- despite the presence of a disabling disease. Given the progres- sive nature of ALS, the clinician must be aware of the expected fit.4,5 Thus, most of the care of people with ALS disease trajectory and apply appropriate interventions at each (PALS) is supportive and centered around symp- stage. This review will present rehabilitation strategies that can tom management, making ALS an incurable, yet be utilized to maximize patient independence, function, safety, and quality of life, and to minimize disease-related symptoms. treatable disease. The role of bracing, exercise, assistive devices, and adaptive equipment will be discussed. At each disease stage, an experi- REHABILITATION AS MULTIDISCIPLINARY CARE enced rehabilitation team is well positioned to make a signifi- cant impact on the life of ALS patients. Rehabilitation is the process of assisting a per- Muscle Nerve 50: 4–13, 2014 son to maximize function and quality of life. Although there are no curative treatments for ALS, rehabilitation can assist people to continue to “Can you cure ALS?” function independently and safely, manage their “I am afraid I can’t.” symptoms, and, most importantly, live a fulfilling “What else can you offer then?” life despite having a disease that is known to “ Rehabilitation for ALS? I thought there is nothing you shorten lifespan. Therefore, rehabilitation matters can do for this disease.” to PALS, because it enables them to reach their fullest potential despite the presence of a disabling It is this type of conversation with patients and disease. Further, it is likely that rehabilitation will their families that prompted this review on the become even more essential when caring for PALS importance of rehabilitation in amyotrophic lateral in the near future, as more treatments will hope- sclerosis (ALS). ALS is a fatal neurodegenerative fully be developed to delay disease progression disease that produces a constellation of symptoms, and prolong lifespan. including muscle weakness, wasting, fatigue, spas- The ALS practice parameter of the American ticity, cramps, muscle twitches, dysphagia, dysarth- Academy of Neurology (AAN) currently recom- ria, respiratory failure, and, in some patients, mends early referral of PALS to a multidisciplinary cognitive and mood changes. The disease typically clinic.6 The multidisciplinary clinic is the ideal set- leads to death within 3–5 years after diagnosis,1,2 ting where rehabilitation needs can be assessed and coordinated.7 In specialized ALS clinics, the Abbreviations: AAC, augmentative and alternative communication; AAN, team often includes a physician, physical therapist American Academy of Neurology; ADL, activities of daily living; ALS, (PT), occupational therapist (OT), speech lan- amyotrophic lateral sclerosis; ALS-FRS, Amyotrophic Lateral Sclerosis Functional Rating Scale; AFO, ankle–foot orthosis; FRO, floor reaction guage pathologist (SLP), respiratory therapist, orthosis; FDA, Food and Drug Administration; IADL, independent activities nurse coordinator, and social worker.8 Additional of daily living; KAFO, knee–ankle–foot orthosis; MBS, modified barium swallow; MCP, metacarpophalangeal; NPPV, non-invasive positive pres- specialists may be available, such as nutritionists, sure ventilation; NSAID, non-steroidal anti-inflammatory drug; OT, occupa- orthotists, pulmonologists, gastroenterologists, tional therapist; PALS, people with ALS; PEG, percutaneous endoscopic assistive technology experts, home modification/ gastrostomy; PLS, posterior leaf spring; PT, physical therapist; RIG, radio- logically inserted gastrostomy; SLP, speech language pathologist; SOD1, designer experts, psychologists, and palliative-care superoxide dismutase 1; TENS, transcutaneous electrical nerve stimulation providers. This type of comprehensive approach Key words: braces; exercise; multidisciplinary care; physical therapy; rehabilitation ideally optimizes health-care delivery by consolidat- This study was supported by a Career Development Award (2K12HD00 ing diverse skill sets, providing continuity and con- 1097-16 to S.P.) from the National Institutes of Health. sistency of care, and interfacing with primary-care Correspondence to: S. Paganoni; e-mail: [email protected] physicians and community-based providers.6 ALS C 2014 Wiley Periodicals, Inc. V Published online 9 February 2014 in Wiley Online Library (wileyonlinelibrary. rehabilitation specialists therefore include physi- com). DOI 10.1002/mus.24202 cians and allied health-care professionals who work 4 ALS Rehabilitation MUSCLE & NERVE July 2014 as a team to address the multifaceted needs of early on, whereas they may not have problems PALS. Given the complexity of the disease and the with limb strength until later in the course of variability in presentation and disease course, it is their disease. Here we use case studies to high- best to involve professionals who have experience light how rehabilitation interventions are critical with ALS, can address current problems, and can toward maximizing patient function, safety, inde- provide anticipatory guidance regarding future pendence, comfort, and quality of life. One needs. Various studies have suggested that multi- should keep in mind that the specific type and disciplinary care is associated with increased sur- timing of these interventions must be individual- vival time; higher quality of life; and increased ized to address the patient’s symptoms and rate of utilization of riluzole, non-invasive positive pres- progression. sure ventilation (NPPV), feeding tubes, and adapt- ive equipment.9–11 CASE STUDY 1: EARLY-STAGE ALS Multidisciplinary care should ideally start early Mary is a newly diagnosed ALS patient. She is 45 in the disease course and should be approached in years old, otherwise healthy, and has a busy life with a a problem-oriented fashion. The goal is to focus full-time administrative job and 2 young children. She on what the patient needs most at any particular likes to exercise regularly, drives to work every day, and time in the course of the disease to maintain maxi- often walks with her kids to soccer practice. For the last 6 mum function and quality of life. Thus, it is critical months, she has been experiencing right leg and arm to frequently reassess rehabilitation strategies and weakness, as well as fatigue. She tripped and fell twice modify them according to changes in disease sta- on uneven terrain while playing with her kids. She is tus. As an example, in the early stages of ALS, exhausted by the end of the day and has a hard time pre- therapists can educate patients on energy- paring dinner for her family. Cutting food and opening conservation techniques and intermittent use of jars is increasingly difficult. She must ask her husband braces when performing demanding activities. for help with buttons and zippers. She also wonders Later, therapists may need to modify braces or rec- whether she can continue to exercise, an activity she ommend additional adaptive equipment and enjoys, because it keeps her connected with 2 friends with mobility aids to adapt to evolving weakness. Rather whom she has been exercising for a long time. than writing generic therapy prescriptions, it is In the early phases of ALS, patients may develop best to address specific problems as they arise mild, but progressive, muscle weakness, fatigue, (e.g., gait training, transfer training, wheelchair poor endurance, and performance limitation.12 In evaluation, etc.). Of note, insurance carriers limit limb-onset ALS, weakness typically begins asymmet- the number of therapy sessions for which people rically in specific upper or lower body muscles. are eligible in a given period of time. Ideally, Fall Assessment and Lower Limb Orthoses. Lower PALS patients should develop a therapeutic alli- body weakness, often unilateral or bilateral foot ance with a therapist or group of therapists early drop, is associated with increased work of ambula- in the disease so that they can have a point of con- tion as the patient compensates with a circum- tact for future needs. However, in-person visits ducted, steppage, or hip hiking gait to avoid need to be spaced over time. This model is very tripping and falling. Proximal leg weakness may different from the rehabilitation of other diseases, also be present, making it difficult to independ- such as acute orthopedic injuries, where the focus ently perform a sit-to-stand transfer, such as when is on concentrating therapy sessions in a relatively getting out of a car or a low chair. Further, muscle short period of time to obtain maximal recovery. weakness may be compounded by spasticity. Due to the progressive nature of ALS, a completely Changes in gait pattern, difficulty with transfers, different rehabilitation paradigm needs to be and spasticity all increase energy expenditure and implemented and requires involvement of thera- contribute to fatigue. Altogether, these factors pro- pists who are familiar with the complexities of the duce a substantially increased risk of falls. disease. Rehabilitation assessment of early lower body weakness begins with a thorough initial evaluation THE GOAL OF THIS REVIEW of gait, balance, manual motor strength, range of Other investigators have reviewed the individ- motion, and tone to assess fall risk and optimize ual roles of each rehabilitation specialist in the remaining function. Daily functional activities are care of PALS. Our goal here is to address the util- reviewed to identify areas for intervention. Evalua- ity of multiple rehabilitation tools and strategies tion and modification of the home and workplace in the different phases of ALS. The presenting environments (such as removing carpets and instal- symptoms and disease course can vary substan- ling night lights) can enhance safety and promote tially. As an example, PALS with bulbar onset may independence. It is important to note that, if falls have significant difficulties with communication occur, a resultant injury and a subsequent period ALS Rehabilitation MUSCLE & NERVE July 2014 5  Table 1. Ankle–foot orthoses (AFOs) used most commonly in ALS care. Type of AFO Description Use Posterior leaf spring (PLS) Medial and lateral trim lines are placed posterior to Mild-to-moderate foot drop. the malleoli, somewhat flexible. Carbon-fiber lateral or posterior Lightweight, unobtrusive. Moderate foot drop (also helps with knee strut dorsiflexion assist brace control). Floor reaction orthoses (FROs), Built to leverage ground reaction forces to offer a Mild-to-moderate foot drop with quadriceps such as the ToeOFF braces “push” at toe-off to assist with propulsion and weakness; they also help compensate for compensate for ankle plantarflexion weakness; ankle plantarflexion weakness. they also create a knee extension moment to help counteract quadriceps weakness and tend- ency to knee buckling. Hinged (articulated) Include an ankle joint; allow sit-to-stand transfers Moderate foot drop with or without spasticity; more easily than solid AFOs; anti-spasticity fea- sufficient knee extensor strength needed tures (such as a plantarflexion stop) can be for optimal use. incorporated as needed. of immobilization can be associated with signifi- demanding activities such as walking long distances cant functional decline, making fall prevention a or traversing uneven terrain. A variety of orthoses high priority in ALS care. Fatigue can be addressed for the lower limbs are available. Ankle–foot ortho- by recommending energy conservation techniques ses (AFOs) assist people with ankle dorsiflexion such as pacing, taking rest breaks, and using brac- weakness and are the most commonly prescribed ing and adaptive equipment. braces for PALS (Table 1). Some AFOs can also Lightweight braces (orthoses) and adaptive enhance knee control. Knee–ankle–foot orthoses equipment can be used on an intermittent basis (KAFOs) may also provide knee stability in people early in the disease course, when weakness is still with quadriceps weakness. However, they are usu- mild, to help conserve energy and assist at times of ally too heavy and cumbersome to be effective in ALS. The involvement of physical therapists and orthotists with experience in ALS care is crucial to Table 2. Adaptive equipment for ADLs/IADLs. identify the brace that best suits the individual Activity Examples of adaptive equipment patient’s needs. Importantly, the brace may need adjustments over time as the patient’s functional Meal preparation Large-handled utensils, rocker knives, and self-feeding cutting boards, bendable utensils, status is likely to evolve. A few sessions of gait train- universal cuffs for holding utensils, ing with a skilled therapist is highly recommended lightweight drinking cups, straw hold- to optimize braced gait. When using braces, ers, long straws, reachers and grab- patients should be instructed to perform skin bers, scoop dishes, plate guards, checks every time the brace is used to identify any non-skid pads, mobile arm supports (devices to support the arm and possible skin irritation early on and to allow appro- assist with self-feeding, computer priate brace modifications. Skin evaluation should keyboard use, and other activities). be part of routine follow-up care. Because patient Dressing Button hooks, zipper pulls, Velcro fas- tolerability for braces is variable, their feedback on teners, sock aids, elastic shoelaces, the comfort and fit of the orthosis is critical to long-handled shoe horns, dressing sticks. enhance compliance. Grooming and Strap-fitted hairbrush, long-handled personal hygiene comb, cylindrical foam applied to the Adaptive Equipment for Activities of Daily Living and handle of multiple bath tools to facili- Hand Orthoses. Upper body weakness ranges from tate grip, lightweight electric shavers, proximal weakness of shoulder muscles to distal and toothbrushes, floss holders, long-handled sponges. weakness involving wrist and intrinsic hand Reading and writing Book easels, tilt-top overbed tables, muscles. Hand weakness interferes with activities manual or automatic page-turning that require fine motor skills such as grasping, devices, rubber thumbs, pen holders, gripping, and pinching.12 Patients may have diffi- pencil grips, foam cylinders placed culties with doorknobs, buttons, and zippers, and around writing instruments to facilitate grip, writing splints, dry-erase writing with writing, cutting food, and opening cans and boards. jars. A variety of tools are available to assist with Leisure activities Many adaptations exist for activities activities of daily living, with the goal of prolonging such as gardening, golfing, and fish- independence for as long as possible (Table 2). ing. Many different types of adaptive Unfortunately, most insurance carriers do not sports are also available. cover the cost of these devices. Inevitable disease 6 ALS Rehabilitation MUSCLE & NERVE July 2014  Table 3. Hand orthoses (splints) used most commonly in ALS care. Type of splint Description Use Resting hand splint Lightweight; may be used during the day and/or at Prevention of wrist and finger flexion contractures. night to maintain proper muscle length in patients with wrist and intrinsic hand muscle weakness. Anti-claw Limits metacarpophalangeal (MCP) extension and Reduction of “claw hand” deformity; improvement improve grasp by keeping the joints flexed. of grasp. Volar cock-up Supports the wrist in 20–30 of extension. Improvement of grasp in people with wrist extensor weakness. Short opponens Keeps the thumb in an abducted and opposed Improvement of grasp in people with thumb abduc- position. tion and extension weakness. progression needs to be considered when making ance/strengthening, and balance exercises recommendations for all equipment. Guidance (Table 4). from an OT with experience in ALS is crucial to Stretching and range-of-motion exercises are ensure that the most appropriate device is selected part of the standard of care for PALS. Neuromuscu- and to avoid unnecessary expenditures on equip- lar specialists should encourage simple daily stretch- ment that is not likely to benefit the patient. Hand ing exercises that target the major joints starting orthoses (splints) may also be considered to assist early in the course of the disease. Experience shows with activities impacted by intrinsic hand muscle that a regular, proactive stretching program helps weakness (Table 3). prevent development of painful and function- limiting contractures, especially at the shoulder and ankle joints. When ambulation is no longer possi- Exercise. PALS often inquire about exercise, ble, daily standing and weight-bearing with assis- because physical activity is often an integral part tance provides effective stretching of the ankles. of the pre-morbid lifestyle for many. PALS often The use of night-time splints to keep the hands and ask whether exercise is safe, whether it can help ankles in a resting, neutral position can supplement slow their disease, and what type of exercise is daily stretching and range-of-motion exercises. recommended for their particular condition. The With respect to aerobic and strengthening exer- types of relevant exercise include stretching and cise, disease-specific guidelines are not available, as range-of-motion exercises, aerobic training, resist- the evidence regarding benefits or harm of Table 4. Types of exercise relevant to patients with ALS. Exercise Description Benefits Practical considerations Flexibility Stretching; range Part of the standard of care for Encourage regular stretching and range-of-motion of motion. prevention and management of exercises early in the course of disease. Care- contractures; might also help giver participation is needed when muscle weak- reduce pain and spasticity. ness prevents the patient from performing program independently. Strengthening Repeated mus- Potential role in maintaining mus- Avoid high-resistance exercise. A practical cle actions cle strength and delaying onset approach is to find a weight that the patient can against of functional impairment. lift comfortably 20 times. Then ask the patient to resistance. perform 2 or 3 sets of 10 repetitions each with that weight. Progression to heavier loads depends on the stage of disease. Do not exer- cise muscles that do not have antigravity strength. Avoid eccentric exercise. Aerobic Dynamic activity Potential role in reducing decondi- Select a mode of exercise with minimal risk of injury using large tioning and improving functional from falling (e.g., recumbent stationary bike as muscle independence, mood, sleep, opposed to treadmill). Aerobic exercise should groups. spasticity, and quality of life. be performed at a moderate, submaximal level. A practical approach is to begin with bouts of 10 minutes of exercise 2 or 3 times a week and pro- gress as tolerated. If the patient cannot talk com- fortably during exercise, the program is too vigorous. Balance Balance training Potential role in fall risk reduction. Perform under supervision of a physical therapist. using different modalities. ALS Rehabilitation MUSCLE & NERVE July 2014 7 exercise in ALS is limited.13 However, a few gen- period. It is therefore important to carefully plan eral exercise recommendations can be made based services while keeping in mind the likelihood of on preclinical data, small human studies, and disease progression and future needs. research on exercise in other neuromuscular dis- CASE STUDY 2: MIDDLE-STAGE ALS eases. Preclinical evidence gathered in the trans- genic mutant superoxide dismutase 1 (SOD1) John is a 65-year-old man who was diagnosed with mouse model of ALS has suggested that moderate ALS 3 years ago. He has managed his gait difficulties by endurance exercise can delay disease onset and using bilateral AFOs, a cane, and, more recently, a increase survival.14–16 High-intensity endurance walker. He has trouble with transfers and is now looking training, on the other hand, was counterproductive into wheelchair options. Since his last visit, he has devel- in these mice.16,17 In a study in humans, Drory oped worsening leg stiffness and back pain. He is having and colleagues suggested that a daily program of a hard time swallowing and has dropped a few pounds 30 minutes of moderate-intensity aerobic exercise of weight. His speech has become slower and more improves function.18 This study included 25 PALS effortful. who were randomized to perform a moderate daily In the case of John, the disease has progressed program of activities, such as walking, stationary considerably. He presents with a multitude of reha- bicycling, or swimming, as opposed to avoiding any bilitation needs. The combined expertise of physi- physical activity beyond their usual daily require- cians, nurses, and therapists from different ments. At 3 months after study initiation, PALS disciplines is now essential to allow him to main- who performed regular exercise showed less deteri- tain mobility; address secondary complications, oration on the ALS Functional Rating Scale (ALS- such as pain and discomfort; and manage progres- FRS) and the Ashworth Spasticity Scale.18 At 6 sive bulbar symptoms. months, there was no significant difference Transfers. Patients with progressive leg weakness between groups, although a trend toward less dete- have difficulty with transfers (e.g., from sitting to rioration was observed in the exercising group.18 standing or from bed to chair). The inability to Another 6-month, randomized, controlled trial transfer safely and efficiently needs to be tested moderate resistance exercise in 27 PALS. addressed promptly, as it can prevent patients The exercise group had significantly better func- from getting out of bed or leaving their homes, tion, as measured by total ALS-FRS scores and thus hampering their ability to participate in social quality of life, without adverse effects, as compared events and leading to decreased quality of life. with subjects who received usual care.19 Commonly available transfer aids are described in These studies suggest that moderate exercise Table 5. Patient and caregiver education and train- may be safe for PALS. General practical exercise ing in proper transfer techniques is essential to considerations are summarized in Table 4. When maximize safety and prevent potential soft tissue PALS engage in an exercise program, it is impor- injury and pain for the patient. Caregiver burden tant to monitor for signs of overexertion. Excessive during transfers is also an important consideration. or prolonged post-exercise fatigue, muscle pain, or Poorly performed transfers can predispose the soreness are indicators that the patient is overwork- caregiver to musculoskeletal strain and injury, ing. Post-exercise fatigue should not interfere with which in turn has a negative effect on patient care. daily activities. If a patient has fatigue or pain that lasts longer than 30 minutes after exercise, the Assistive Devices for Ambulation and exercise program needs to be modified. Wheelchairs. As leg weakness progresses, PALS In summary, in the case of Mary, a woman with eventually require the use of assistive devices for early-stage ALS described in case study 1, early ambulation, ultimately transitioning to a wheel- referral to therapists with experience with ALS is chair. Assistive devices for ambulation include essential to maximize function and improve quality canes, crutches, and walkers (Table 6). Canes pro- of life. Therapy may include education on energy vide the least stability and are used when there is conservation techniques, safety, and exercise. only mild lower extremity weakness or imbalance. Lower limb braces can be considered to improve Walkers provide the most support (Table 6). The gait efficiency and safety. Adaptive tools for hand decision as to which walking aid to prescribe, how- weakness can promote independence with house- ever, depends not only on the degree of weakness hold chores and work-related tasks. Education on in the lower limbs but also on upper body and home and workplace modifications, community grip strength. Canes are available in a variety of resources, and expected progression is important styles and sizes of hand grips. Offset canes have a to allow the patient and family to plan accordingly. flat handle that can be built up to allow for better Unfortunately, reimbursement for therapy is often grip. Crutches have limited use in ALS, as their limited to a set number of sessions in a given time use requires a high degree of upper body strength, 8 ALS Rehabilitation MUSCLE & NERVE July 2014  Transitioning to a wheelchair is ultimately Table 5. Transfer aids. needed in ALS and may be challenging from both Equipment for transfers Use a psychological and logistical point of view (Table 7). Manual wheelchairs may be used in the early Firm cushions (2–3 Allow the hips to be higher than stages to help conserve energy when traveling long inches thick) knees when sitting, thereby facili- tating sit-to-stand transfers. distances. However, one should keep in mind that, Swivel cushions Lightweight seats that swivel in both as the disease progresses, the patient will probably directions (allow car transfers). develop difficulty propelling the wheelchair in a Self-powered or elec- Cushions that assist in rising to relatively short period of time. Of note, at present, tric lifting cushions standing; their use requires good Medicare and most private insurers limit reim- trunk control and balance. Powered recliner lift Assist in rising to standing by using bursement to only 1 wheelchair every few years. chairs an electric control, can also be Therefore, one may wish to rent or borrow a man- installed in cars and vans. ual wheelchair instead of purchasing one. Power Sliding boards Assist in low-level transfers; can be scooters are sometimes considered as an alterna- used alone if the patient has good tive to a manual wheelchair, especially for outdoor upper extremity strength and sit- ting balance or with assistance use and energy conservation when one needs to from a caregiver. traverse longer distances. However, scooters should Transfer belts (safety Allow caregivers to provide safe be recommended with caution, because reimburse- belts) transfer and ambulation assis- ment for a scooter will preclude reimbursement tance; belts are positioned around for a power wheelchair. This is an important finan- the patient’s waist and hips and prevent traction on the shoulders, cial consideration, as most PALS will ultimately which may be painful; they also need a power wheelchair. Power scooters may be ease caregiver burden and poten- an option for those who can borrow or afford to tial musculoskeletal strain when purchase one (most scooters are available for assisting the patient. Belts with $700–$2000 depending on the model). Some handles are often preferred. Stairway lifts and chair For multilevel homes when patients patient organizations maintain loaner closets that glides cannot negotiate stars safely; they allow patients to borrow pieces of equipment. are costly and require an extra However, other important limitations regarding wheelchair at each level of the the use of scooters should be discussed with ALS staircase; patient may require patients. Good upper limb and trunk strength is assistance to transfer. Mechanical lifts Allow for safe lifting of people who needed to drive them. In addition, they cannot be do not have the preserved modified for disease progression or to accommo- strength to transfer independently; date other equipment such as mounted trays and they may be electric or hydraulic; electronic equipment. Finally, because of their they are attached to a sling system long wheelbase and wide turning radius, there may with head support. Tools for bed mobility Facilitate repositioning while in bed be difficulty using them indoors. and getting in and out of bed As mobility continues to decline, most patients (e.g., step stools, bed canes, hos- will need a power wheelchair. The design and pre- pital beds, and overhead tra- scription of a power wheelchair should be coordi- pezes). Electric hospital beds allow nated with a physical therapist who is experienced adjustment of total bed height in addition to head and knee in ALS to ensure that the appropriate components adjustments. are selected to accommodate current needs and anticipate future modifications (Table 7). Proper seating and positioning are essential to ensure a coordination, and energy. Wheeled walkers, as comfortable and functional sitting posture, allow opposed to standard walkers, do not need to be for maximal breathing capacity, and prevent sec- lifted and are preferred in ALS. However, one ondary injuries, such as skin breakdown and back must ensure that the patient can maneuver them pain. Transitioning to power mobility may require safely. If a 4-wheeled walker is used, the patient a number of corresponding environmental modifi- needs to be able to activate the brake system. Most cations, because power wheelchairs do not fit into 4-wheeled walkers are equipped with squeeze narrow doorways and standard cars. Ramps are brakes, which may be hard to use if the patient has needed to enter the home if there are steps, and a a weak grip. Alternatively, one can choose a 4- modified van is needed for transportation. Many wheeled walker with push-down brakes (secured individuals do not have the financial means to pur- when the patient loads his or her weight on the chase a modified van and thus utilize a back-up walker) or a 2-wheeled walker with glides in the manual wheelchair for travel or use accessible pub- back. lic transportation, if available in their community. ALS Rehabilitation MUSCLE & NERVE July 2014 9  Table 6. Assistive devices for ambulation (mobility aids). Device Type Practical considerations Canes  Wooden (standard, affordable). Patients must have adequate upper body and arm strength to  Aluminum (light, adjustable length). use a cane safely. Canes are typically used on the side oppo-  Offset (flat handle, easier to grip). site to the affected leg. They can be used on stairs. Patients  Quad (provide greater stability, but heavy to lift may need to negotiate stairs on an angle and 1 step at a time. and therefore rarely used in ALS). One should lead with the stronger limb on flat ground and when ascending stairs, and with the more affected limb when descending stairs (“up with the good and down with the bad”). Crutches  Axillary (rarely used in ALS). Limited use in ALS because they are heavy, and their use  Forearm (Canadian, Lofstrand; limited use in requires preserved arm and trunk strength along with proper those with mainly leg weakness). balance. Walkers  Standard. Standard walkers are rarely used in ALS because they lack  Wheeled (2 or 4 wheels). wheels and must be picked up and lowered to advance for- ward, causing fatigue. Wheeled walkers do not need to be lifted and are preferred in ALS as long as the patient can maneuver them safely. Four-wheeled walkers should be equipped with brakes for safety. They may have an additional attached seat, which the patient can use when fatigued. If the patient cannot use the grip brakes due to hand weakness, 2- wheeled walkers with glides in the back can be used. Having a back-up manual wheelchair is also impor- disturbance is a typical symptom of ALS, over time tant in case the power chair needs repairs or patients can develop secondary musculoskeletal upgrades, which is a common occurrence. pain and discomfort due to poor mobility, subopti- mal transfer techniques, spasticity, loss of range of Musculoskeletal Symptoms. Although progressive motion, difficulty with positioning in bed or in the muscle weakness in the absence of pain or sensory wheelchair, and contractures. A careful history combined with detailed neurologic and musculo- skeletal exams can help identify the pain genera- Table 7. Wheelchairs. tor(s) and direct treatment. It should be noted, Type Practical considerations however, that recommendations for treatment of Manual Manual wheelchairs should be light- pain and spasticity in ALS are based mostly on weight or ultra-lightweight. With dis- expert opinion, as very few studies have been con- ease progression, patients have a ducted to analyze the efficacy of specific medica- hard time propelling the wheelchair. tions or other interventions.20–22 Consider renting or borrowing manual Pain is reported by some PALS even in the wheelchairs instead of purchasing one. Removable wheels make it eas- early stages of their disease.23 Common sites of ier to transport wheelchair in a small pain include the low back, the neck, and the vehicle. shoulder area.24 Loss of range of motion, subluxa- Transport Transport wheelchairs are light and tion, and contractures in the shoulders are particu- (companion) inexpensive, but must be pushed by larly frequent complications of ALS, and yet they a caregiver. They fold and fit into a car’s trunk. Many families borrow or are probably preventable with the institution of a buy one as a back-up chair for travel. regular stretching and range-of-motion program Power Power wheelchairs do not fit into stand- early in the course of the disease. The use of ard cars (modified van is needed for shoulder approximation sleeves can help minimize transportation). Ramps are required shoulder subluxation and pain. Simple modifica- to enter homes. Wheelchairs can be equipped with ventilators and aug- tions, such as adjusting the seating system or arm mentative and alternative communi- rests of the wheelchair, provide additional comfort cation devices. Tilt-in-space and pain relief. In the upper limbs, patients may mechanism is used for pressure and complain of hand pain due to finger contractures pain relief. Multiple drive controls are or finger flexor tenosynovitis (the latter being available to allow users with different degrees of weakness to control the responsive to local steroid injections). Resting power wheelchair (e.g., joystick, head splints may be used to allow the hands to rest in a array, eye gaze). neutral position and can be used throughout the day or at night. For all wheelchairs, positioning and modifications with disease progres- sion are essential to ensure proper seating and avoid secondary compli- In the legs, discomfort can be associated with cations such as skin breakdown and back pain. spasticity, dependent edema, loss of range of 10 ALS Rehabilitation MUSCLE & NERVE July 2014 motion, and contractures. If dependent edema is a offered to document penetration and/or aspira- problem, leg elevation, massage, and compression tion and to help educate patients on swallowing stockings may provide some relief. Gentle sus- strategies. tained muscle stretches, weight-bearing with assis- From a rehabilitation perspective, there is no tance, and night-time neutral position splinting evidence that exercise improves swallowing func- may be used for spasticity at the ankles. In addi- tion. Compensatory techniques, however, can be tion, one can use dynamic splints, which provide a used to reduce aspiration risks, including pro- low-load, prolonged-duration stretch to prevent longed chewing time, head tilt, and chin tuck. ankle contracture development. In wheelchair Behavioral strategies include taking small bites and users, back pain can often be relieved by providing sips, alternating bites of solid food with sips of liq- adequate lumbar support and good cushioning. uid, sitting upright, avoiding talking while eating, Some features of power wheelchairs can help with and paying increased attention to each individual pain management. Power-activated leg rests can swallow. These recommendations, however, need help maintain hamstring length and ease back to be strictly individualized, as there is no pre- pain. Tilting the wheelchair relieves pain from glu- scribed regimen that is appropriate for all patients teal pressure. For those with advanced weakness, with dysphagia. Education about types of food and the bed should be fitted with pressure relief over liquids that are easier to chew and swallow is essen- bony prominences to avoid pain and pressure tial. As an example, soft, moist foods are easier to ulcers. Foam wedges can be used to facilitate proper swallow than dry, crumbly, or chewy foods. Thicker positioning. liquids (fruit nectar, smoothies) are easier to han- Pharmacologic treatment of pain and spasticity dle than water. A modified diet might be recom- is indicated in some patients, although evidence mended, ranging from soft (tender foods) to supporting one regimen versus another is lack- pureed (blenderized foods). ing.21 Depending on the pain generator, one can Dieticians can help monitor a patient’s caloric use medications for neuropathic pain (such as intake and recommend additional supplements to gabapentin, pregabalin, tricyclic antidepressants, maintain body weight. Strategies to help avoid duloxetine, and topical lidocaine or capsaicin) or weight loss include eating smaller meals with high- non-steroidal anti-inflammatory medications calorie snacks at scheduled times, choosing calorie- (NSAIDs) if there is evidence of an inflammatory dense foods, and adding high-calorie supplements. process such as arthritis or bursitis. As-needed or When oral intake becomes inadequate, too effort- standing doses of acetaminophen may augment or, ful or fatiguing, and/or compromises safety, alter- if there are contraindications, replace the use of native routes for nutrition may be considered, NSAIDs. Oral medications for spasticity include including percutaneous endoscopic gastrostomy baclofen, tizanidine, benzodiazepines, and canna- (PEG) or radiologically inserted gastrostomy (RIG) binoids. As an adjunct to medications, strategies to tubes. The risk of gastrostomy tube placement alleviate pain and spasticity include stretching, increases when functional vital capacity falls to bracing, massage, topical heat and ice (given alone