Supporting Clients With ALS: Presentation And Management

Editor's note: This text-based course is a transcript of the webinar, Supporting Clients With ALS: Presentation And Management, presented by Amber L. Ward, MS, OTR/L, BCPR, ATP/SMS, FAOTA.

*Please also use the handout with this text course to supplement the material.

Learning Outcomes

• After this course, participants will be able to evaluate between the medication and research options and real-world implications for the management of clients with ALS.
• After this course, participants will be able to evaluate 3 options for client management with desired occupations.
• After this course, participants will be able to evaluate common occupational therapy adaptations for appropriate use with clients with ALS.

Introduction

It's good to see you all. To provide a bit more information, I am a full-time employee at an ALS neuromuscular clinic, and I'm excited to be able to share this information. I have a bit of an advantage since I am full-time and able to work with folks with ALS. I'm excited that we're going to be working through some of what you hear online, what you hear in the news, and what is actually happening. We'll also evaluate three options for client management, considering their desired outcomes and common adaptations. Because this class is advanced, we won't be creating all the buttonhooks and other traditional items, but rather some unusual, interesting, homemade, and other unique adaptations. 

ALS Overview

In the United States, ALS is often referred to as Lou Gehrig’s disease, named after the baseball player who was one of the most well-known people to have it. Elsewhere in the world, it is known as motor neuron disease; both terms refer to the same condition. It affects the brain and spinal cord, specifically the motor neurons—their function, health, and efficiency—in both regions.

This diagnosis is one of exclusion. Based on the presenting symptoms, clinicians must systematically rule out every other plausible cause. An EMG is typically performed, which involves inserting a needle into the muscle to evaluate how the muscle functions and responds. Extensive blood testing is conducted to assess for heavy metals, toxins, and other abnormalities that could explain the symptoms. Some conditions can mimic ALS and vice versa, which can lead to delayed recognition.

Delays often begin with the care pathway itself. If I develop ankle weakness, I am unlikely to see an ALS specialist first. I might start with my primary care clinician, get referred to orthopedics or physical therapy, and wait to see if symptoms improve. When they do not, I may return to orthopedics and be offered surgery or referred elsewhere for further evaluation. By the time I reach a neurologist, months—sometimes a year—may have passed. During this period, we try different interventions while time moves on, and symptoms may progress.

I have seen people undergo procedures for spinal stenosis, other spinal issues, or carpal tunnel syndrome. These problems may indeed be present, but they are not always the underlying cause of the weakness. A key clinical indicator is that ALS generally does not cause sensory changes. Back problems and many other conditions often do, but ALS typically does not. I worked with a client who had bilateral carpal tunnel surgery despite having no sensory symptoms—only weakness. That absence of sensory complaints is unusual for carpal tunnel, which raised concerns and, in retrospect, was a signal to look more closely for ALS. It is also important to acknowledge that no one wants to deliver this diagnosis, so clinicians will appropriately and thoroughly explore every other possibility first.

ALS Symptoms

You may experience symptoms in your arms and legs, which is called limb onset. Symptoms can also start in the trunk or neck, or present as changes in speech, swallowing, or breathing. A key feature of an ALS diagnosis is the presence of both upper motor neuron and lower motor neuron signs. To refresh what that means: upper motor neuron signs include increased reflexes, spasticity, and increased tone—imagine a more spastic presentation, somewhat similar in feel to what is seen in cerebral palsy. Lower motor neuron signs include weakness, muscle atrophy, reduced or absent reflexes, and lower tone.

You do not need to have a lot of each; it is not all or nothing. You have changes to your voice, weakness in your tongue or lips, and at the same time an ankle jerk that is brisk enough to “kick” during a reflex exam. You may experience stiffness and tightness, resembling mild spasticity, in one bicep, and foot drop on the other side. The key is that you show signs in multiple areas, with some features pointing to increased reflexes and tone, and others pointing to decreased reflexes, weakness, and atrophy. For an ALS diagnosis, you must have both upper and lower motor neuron signs.

Another key aspect of the symptoms is that they typically involve the voluntary muscles. Breathing, swallowing, speaking, and moving your limbs are voluntary functions and are commonly affected. By contrast, you do not have voluntary control over digestion or autonomic responses like heat and other sensory reactions, which helps explain why sensory changes are generally not part of ALS.

You may also experience cognitive or behavioral changes, as well as pseudobulbar affect. Pseudobulbar affect refers to emotional expression that is out of proportion to the situation, such as uncontrollable laughing or crying, and it is common in ALS.

Progression can move in any direction, which is why ALS can look very different from person to person. In a large clinic, it can seem like 40 different conditions in 40 people. One person might start with foot drop and see weakness travel up that same side; another might develop neck extensor weakness along with speech and swallowing difficulties. Symptoms may spread from one foot to the opposite arm, affect both arms, or move from proximal to distal or distal to proximal. Because the pattern and speed vary so widely, it is challenging to give firm timelines about what will happen next or how long I have to live.

While ALS is typically not a sensory condition and usually does not cause pain as a primary symptom, a small percentage of people do report pain. More often, any discomfort is secondary—related to being more sedentary, muscle tightness and cramping, prolonged sitting, and reduced mobility—rather than pain directly caused by the disease itself.

ALS- An Umbrella

ALS is, interestingly, an umbrella term. Several variants do not neatly fit the requirement of having both upper and lower motor neuron signs. If classic ALS requires evidence of both, it raises the question of what to call it when someone lacks one set of findings or does not meet all the criteria at a given time. Some people show predominantly upper motor neuron signs—they are notably spastic with increased reflexes—while others show mostly lower motor neuron signs, where weakness and atrophy predominate and reflexes are reduced. These patterns can still fall within the ALS spectrum and typically progress at a pace similar to classic ALS, just with a dominant phenotype.

There is a form that involves only upper motor neuron signs throughout the disease course. It progresses much more slowly, often over 30 to 50 years, and is marked by significant spasticity and a spastic gait. Management may include interventions like a baclofen pump to control spasticity. If you have only lower motor neuron signs with ongoing progression, the diagnosis would be progressive muscular atrophy (PMA). PMA usually follows a longer trajectory—often 20 to 30 years—characterized by weakness, markedly decreased reflexes, and pronounced atrophy.

There can also be a purely bulbar presentation, limited to speech, swallowing, and breathing. This condition, pseudobulbar palsy, may progress more slowly and can sometimes plateau without spreading beyond those functions. Another variant is bibrachial (shoulder) diplegia, where you may experience isolated upper body weakness for many years before other regions are affected, eventually leading to more generalized weakness and bulbar symptoms. The opposite pattern—sometimes called lower body diplegia—presents as long-standing lower extremity weakness that can resemble paraplegia before progression extends to other areas.

What many people, including those outside of neuromuscular subspecialties, may not realize is the breadth of this umbrella. When someone has a diagnosis like PMA but refers to it as ALS, confusion can arise about prognosis and management—especially when the individual is functioning relatively well 15 years in. Recognizing these variants helps set appropriate expectations and guides care planning within the broader ALS spectrum.

ALS Facts

Approximately 10% of ALS cases are familial, resulting from identifiable genes that are inherited within families. About 90% are sporadic, with no known cause and no family history.

The average life expectancy reported in the literature is two to five years. However, I have cared for people with classic ALS who live much longer and others who progress more quickly. At any given time, about 30,000 Americans are living with ALS. There are regions with higher apparent incidence, and when we talk about potential causes, I will come back to that. While onset is most common between ages 40 and 70, in my 22 years in clinic, it has seemed that people are being diagnosed at younger ages, and as our world changes, many diseases—including ALS—appear to be seeing upticks.

Because ALS primarily affects voluntary muscles, the last muscles often involved are in the face, and very often the eye muscles. People can become locked in. A tracheostomy and ventilator do not stop the disease from progressing. Suppose you lose the ability to communicate because you no longer have reliable eye movements or small, consistent muscle twitches to signal yes or no. In that case, you can enter a locked-in state. The five senses—hearing, vision, smell, taste, and touch—are generally spared, and sensory loss is typically not part of ALS.

If you are around 70, you are more likely to have other conditions like neuropathy, spinal stenosis, or injuries from past accidents—so sensory changes can occur. Still, those are usually not due to ALS itself. Most people with ALS maintain bowel and bladder control. A small number experience bladder or bowel dysfunction, more often flaccid, occasionally spastic, but that is much less common.

ALS Causes

If you are the kind of person who wants clear answers, I know you are asking two things. What is the cause, and what is the cure? When examining causes, several avenues are currently under active study. At the cellular level, researchers focus on free radicals and glutamate, which operate at microscopic scales within the cells. In some individuals, an autoimmune component may be present. For others, exposure to toxins, certain viral infections, or significant life stress may play a role. We also know that about ten percent of cases are genetic.

When I refer to genetic risk factors or triggers, I mean that this disease, like many others, may involve a genetic vulnerability that requires additional events to be expressed. Think about someone who has never smoked, lives a healthy life, and still develops lung cancer. One theory suggests that a gene does not guarantee the disease, but certain life events can trigger it, and further events can then accelerate the process. In this framework, the triggers could include the stress of a spouse dying, living near a lake with a specific algae bloom, working around toxic chemicals, being a high-level athlete, and other exposures. My genetic risk might require twenty-five triggers, yours might require fifteen, and someone else might need ten. We may never reach that number, or we may accumulate many. This multi-hit model is currently the leading theory, although it is not the only one.

There are other observed associations. Participating in high-intensity exercise, such as professional-level sports, for many years may be associated with an increased risk. Military service also appears to carry a higher risk, even for people who have never seen combat. There are many of these patterns, but no single unifying cause has been identified. Given that, the hit idea makes practical sense to me as a way to consider risk accumulation across genetics, environment, infections, toxins, stress, and lifestyle over time.

Cognitive/Behavioral Symptoms

For a long time, the prevailing belief was that people with ALS had no cognitive changes and were trapped in their bodies. That is not accurate. Around half of people with ALS experience some degree of cognitive or behavioral symptoms, and about ten percent develop full frontotemporal dementia.

These changes differ from the memory loss pattern typically seen in Alzheimer’s disease. The issues are more frontal in nature. People may exhibit decreased empathy and experience language difficulties, such as trouble finding words or spelling errors. On simple category fluency tasks, such as naming as many animals as possible in one minute, they may produce only a few words and then stall. Behaviorally, they may become more apathetic, impulsive, inconsiderate, irritable, rude, or paranoid, and they may develop a strong need for control. Some individuals are only mildly affected and appear simply more apathetic. Others meet criteria for true frontotemporal dementia with multiple symptoms present.

Memory can be relatively preserved, but impairments in judgment, executive function, and language can make learning and understanding new material harder. This becomes very clear when we move beyond basic yes-or-no communication to high-level assistive technology. In the past, someone could nod for yes, shake their head for no, or use one or two finger signals, which could mask cognitive difficulties because the questions were simple. When I ask someone to use a new eye-gaze system to communicate, browse the internet, answer calls, and manage daily digital tasks, limitations can arise. If they cannot spell reliably, cannot retrieve words, or make frequent grammatical errors, operating that technology becomes much more difficult. We are recognizing more often that significant cognitive and behavioral changes affect many people with ALS.

If you work with someone who has ALS, watch for these patterns and educate caregivers and family members. It is easy to attribute changes to depression or the stress of the diagnosis, and those can certainly be present. However, when irritability, paranoia, apathy, and other behavioral features persist despite treatment for mood and anxiety, it suggests there is more going on than a reaction to illness. Many people do not realize these cognitive and behavioral issues are part of the ALS spectrum, which can lead to misunderstandings and the belief that the person is simply being difficult. Helping families understand what they are seeing can make care more effective and compassionate.

Pseudobulbar Affect

Pseudobulbar affect refers to emotional expressions that are far out of proportion to the situation. It occurs in stroke and brain injury as well as other conditions, and in ALS, it often presents as episodes of uncontrollable laughing or crying.

I have watched someone move from a touch of sadness to intense sobbing within moments, completely overwhelmed. I have also heard a person say they were not that sad while they were openly weeping. Laughter can escalate the same way. I can come across as the funniest therapist in the room because the laughing becomes extreme. At first, it may seem lighthearted, but it quickly becomes overwhelming. People struggle to catch their breath, and tears stream down their faces, and social interactions become very difficult.

There is a medication called Nudexta that helps many people with pseudobulbar affect. A large percentage responds well, making it a worthwhile treatment option to discuss.

Dysarthria

Dysarthria is very common. Speech becomes garbled and progressively harder to understand. It can sound like there are marbles in the mouth, and it often worsens with fatigue over the course of the day and over time. One challenge I frequently encounter is the disconnect between how clearly a person sounds in their own mind and how others perceive them. Even with dysarthria, people know exactly what they intended to say, so it is frustrating when they are not understood. Familiar caregivers may still understand them, while the rest of us struggle to comprehend. Helping someone transition to communication tools so that others can understand them can be a hard sell, because many prefer to have people keep guessing, lip-reading, or listening harder.

Hearing technology can help in some situations, but it does not fix the core problem when lip, cheek, and tongue weakness reduce enunciation and volume. Some people with a spastic presentation, such as primary lateral sclerosis, develop a tight, strained voice because the same increased tone that affects the limbs also affects the speech muscles. Eventually, many people become anarthric and can no longer speak. They may still vocalize with sounds or moans. Some choose to write. I have seen people fill notebook after notebook. Low-tech options like a dry-erase board or a boogie board, which is like an adult Etch A Sketch that you write on and clear repeatedly, can be very effective.

Higher-level options are often available. Phones and tablets are ubiquitous, and simple text can be the fastest solution. Many clients type a message and turn the screen around for me to read. There are free, general-purpose apps that support communication, and voice dictation can help when articulation is still adequate. Dedicated augmentative and alternative communication devices, known as AAC, typically start with touch screens and can be accessed in many ways. People may use head pointing, eye gaze, or switch scanning. Word prediction helps increase speed. When language is affected by frontotemporal dementia, picture-based communication can be more effective, sometimes using photos of the person’s own familiar items to support understanding and choice making.

Cognitive changes matter for communication planning. Someone may begin with touch access and spelling, but as word finding and sentence construction become more challenging, operating a robust communication system can become difficult. Many people eventually rely on simple yes-or-no systems for reliability and speed.

Voice banking is an option that allows individuals to record multiple sentences in various contexts, enabling their synthesized voice on a device to sound like their own. The result still has a synthetic quality, but it preserves elements of the person’s natural sound. Standard device voices are serviceable but impersonal. Newer tools that use artificial intelligence can sometimes create a usable voice even if formal voice banking was not completed early enough. With permission and appropriate sources, systems can learn from prior recordings such as talks, interviews, or voicemails and produce a voice that is surprisingly close to the original. Even short recordings can yield a meaningful approximation, which has been a welcome development for people who start the process later.

Dysphagia

Swallowing problems are common. The difficulties can involve lip closure, chewing, moving food to the back of the mouth, and forming an effective bolus. Most of the time, people are not silent aspirators until they are very weak or using a tracheostomy and a ventilator. They typically cough forcefully when something goes down the wrong way, which is a cue to change the consistency of food or liquid or to stop eating by mouth and rely more on the feeding tube. Because of this, I do not routinely send every person for a modified barium swallow. Many decide to take small bites and sips for pleasure and to enjoy taste and smell, while adjusting safety based on their cough response.

Feeding tubes are very common in ALS, and timing matters. If a feeding tube is placed under general anesthesia and the person’s respiratory status is too low, they may not be able to come off the breathing tube afterward. If they also do not want a tracheostomy, they may not be candidates for a late feeding tube. That is why I encourage earlier placement while breathing tests are still adequate. A PEG is a percutaneous endoscopic gastrostomy, and a RIG is a radiologically inserted gastrostomy. Anatomic variations sometimes make radiologic assistance preferable. Anesthesia plans can vary, and some surgeons will modify the approach or allow use of the person’s own noninvasive ventilation mask during the procedure. These decisions depend on the surgeon and the individual’s respiratory status.

Diet texture changes usually start with challenges to dry and crumbly foods, such as rice and crackers, and then extend to chewy foods like steak. Thin liquids are not always the main problem. Items that require more oral manipulation can be harder to control. Softer, moister foods tend to be easier as symptoms progress. When a feeding tube is placed early, many people still eat by mouth for enjoyment while using the tube for extra water, protein, and medications. Over time, more calories can shift to the tube as needed.

Nutrition is therapy in ALS. The condition is hypermetabolic, so people burn through calories quickly and require higher protein, fat, and total calorie intake to maintain their weight. Nearly everyone I see at diagnosis has lost weight, sometimes by forty to one hundred pounds, and often unintentionally. That can look like a positive change to someone who was already trying to slim down, but in ALS, unplanned weight loss is a red flag. When intake does not meet demand, the body catabolizes its own muscle tissue, which accelerates muscle weakness. Adequate calories can support strength and are associated with better survival. This is one of the few conditions in which I encourage people to load smoothies with calories and protein, to choose full-fat options, and to prioritize weight maintenance or gain.

Body habitus plays a role in risk and resilience. Many people I meet were previously very fit with few chronic conditions, so they do not have much reserve to lose. Higher body weight appears to be somewhat protective, although it is not absolute. People closer to normal or low body weight have less margin before the metabolic demands of ALS begin to erode muscle and function.

Respiratory

Respiratory symptoms are common, and they shape many day-to-day decisions. Dyspnea is shortness of breath. Orthopnea is shortness of breath when lying flat. These issues are why I often recommend a hospital bed or an adjustable bed. Positioning helps with breathing, swallowing, and managing secretions. Suppose someone uses a power wheelchair with tilt to relieve pressure. In that case, they may avoid tilting because they feel they cannot breathe comfortably, especially if they only use their respiratory device at night. That behavior increases the risk of pressure injuries, not solely from direct pressure, but because respiratory limitations interfere with the necessary postural changes.

Noninvasive ventilation typically starts at night. A CPAP provides continuous positive airway pressure and is primarily used for treating sleep apnea. A BiPAP provides different pressures for inhalation and exhalation. A volume ventilator goes further by synchronizing with the person’s breathing intent, making support more comfortable and comprehensive. Many volume ventilators can be used throughout the entire course of care, including after tracheostomy, eliminating the need to change machines. Mask choice matters. Some use a full face mask, others a nose and mouth mask, and others a nasal mask. Fit is as critical as it is for sleep apnea, and ultimately, many people need continuous support.

Use typically progresses from nights only, to nights plus daytime naps, to periods of quiet rest such as watching television, and then to more frequent daytime use. Eating by mouth may become hard to coordinate with the mask. If a person does not want to use a mask or pursue tracheostomy and ventilator support, they will face essential decisions earlier if respiratory symptoms are prominent.

Airway clearance tools are essential. A high-frequency chest wall oscillation vest helps mobilize secretions, especially when deep breaths and movement are limited. A cough assist device helps augment a weak cough when the diaphragm and thoracic muscles cannot generate sufficient force. Suction can help when secretions reach the mouth but cannot be cleared. Nebulized medications may be helpful in selected cases. Secretions can be excessive or insufficient, and individuals may experience alternating episodes of drooling and dry mouth, necessitating ongoing adjustments.

ALS is a disease of muscles, including the diaphragm and thoracic muscles. It is not a primary lung disease. Oxygen saturation on a fingertip may be low, but the problem is often inadequate ventilation rather than insufficient oxygen content. The solution is to inflate the lungs and move air effectively, not to add supplemental oxygen. Supplemental oxygen administered in isolation can further depress respiratory drive in individuals whose primary issue is neuromuscular weakness, potentially worsening the situation. In the hospital, I advocate for placing the person on their own mask and ventilator or on an appropriately sized ventilator rather than reflexively applying oxygen alone. For coexisting lung diseases such as COPD, chronic bronchitis, or asthma, oxygen therapy may be appropriate, sometimes in conjunction with ventilatory support. For straightforward ALS without intrinsic lung disease, the focus should be on ventilation.

Preferences vary, and some people feel more comfortable with oxygen regardless. My role is to educate and prepare. I provide a clear plan for urgent care and emergency visits that explains the need for noninvasive ventilation, encourages bringing personal equipment to the hospital, and outlines steps to avoid unnecessary oxygen without adequate ventilatory support. This planning helps prevent complications and keeps care aligned with the person’s goals.

Breathing Testing

Breathing tests are often part of routine care, and many clinics perform them at each visit, typically once a quarter. You may encounter abbreviations in a chart, so I would like to clarify a few. FVC stands for forced vital capacity. For this test, I ask someone to take a deep breath in, seal the device in their mouth, and exhale for as long and as forcefully as possible, much like blowing up a balloon until they are entirely out of air. We repeat it three times. FVC reflects how well the diaphragm and lungs can move air and is one of the most commonly used measures. There are other tests as well, and they can be helpful when someone’s FVC looks acceptable, but they still feel air hungry or short of breath. Those additional measures can support insurance qualification for respiratory devices before symptoms become severe.

Peak expiratory flow is essentially a measure of cough strength, and I use it to help justify devices like cough assist. I also educate people about mouthpiece ventilation, which I wish were better known. This setup connects to a volume ventilator and runs continuously. When someone needs support, they place their mouth on the mouthpiece and receive assisted breaths. If they are tilting back in the wheelchair, feeling air hungry while talking, or just need a boost, they can take a few assisted breaths and then continue their activity. It is a valuable way to avoid wearing a mask that can interfere with communication and eating. Some ventilators require a separate setting for mouthpiece use compared to a mask, while others can manage both. Many people keep one machine at the bedside and another mounted on the wheelchair so they have support available wherever they are.

ALS Stages

I am going to walk you through a discussion of the early, middle, and late stages in simple terms.

Early Stage

Early symptoms are often subtle. I may start tripping or notice a slight weakness in my ankle. I might feel unsteady when hiking or have trouble going up on my toes to reach a high shelf. I may notice muscle twitching, cramps, or that my grip is weaker. I used to be the one everyone handed jars to, and now I struggle to open them as easily. Some people begin with thumb weakness that affects fine motor tasks. Others notice shoulder weakness or hip weakness that makes it harder to rise from low chairs. In the early stages, a mix of twitching, cramping, atrophy in one area, and increased tone in another can occur, which can resemble many other conditions.

It is common to explain early changes away. I may think I've overexerted myself, pushed too hard at the gym, or strained something. Athletes tend to seek evaluation sooner because they are familiar with how their bodies usually perform and can sense small changes, whereas many of us wait. After all, minor ankle fatigue or hand weakness seems easy to rationalize. This contributes to delays in diagnosis, especially when other issues, such as mild carpal tunnel syndrome or arthritis, are also present and seem to explain the symptoms.

The medical term for muscle twitching is fasciculations. It is essential to remember that occasional twitches, such as an eye twitch or a thumb twitch, caused by fatigue, are common and not a sign of ALS on their own.

Middle Stage

In the middle stage, fasciculations become unmistakable. Everyone gets occasional twitches, but this is different. You can often see muscles rippling and rolling with frequent visible twitching. Changes are more obvious to others. You may start using a cane or a walker. Speech and swallowing can worsen, so dysarthria and dysphagia become more pronounced. Fine motor tasks, such as fastening buttons, working zippers, tying shoes, and opening packages, become more challenging. Shortness of breath may occur, and more adaptive equipment may be necessary to manage daily activities.

Discomfort can increase, often from spending more time sitting, though pain can sometimes be part of ALS itself. Fatigue can be significant, similar to what people report in multiple sclerosis. Safety becomes a priority. If leg weakness is present, everyday tasks such as standing in the shower, getting out of bed, maintaining balance, or navigating stairs can feel risky. This is also when bigger questions surface. You may need to consider whether your home will be suitable for long-term use, especially if it has multiple levels. You may also need to think about continued employment and caregiving responsibilities. This stage often makes the future feel more concrete, and proactive planning becomes essential.

Late Stage

In the late stage, most people use a power wheelchair. For those with frontotemporal dementia, minimal space at home, or a preference not to move independently, a manual wheelchair with tilt can provide comfort.

Muscles that were previously spastic in classic ALS can become too weak to show spasticity. As overall weakness advances, muscles become more flaccid, and the upper motor neuron signs that were once noticeable tend to diminish because the muscles no longer respond strongly enough to display them. Communication becomes difficult. A feeding tube is common. If respiratory support is needed for more than about twenty hours a day, a tracheostomy and ventilator may be considered.

If respiratory or swallowing function cannot meet basic needs and interventions such as volume ventilation or a feeding tube are not desired, hospice and supportive care become essential to maintain comfort. Decision-making in this phase focuses on goals, values, and what day-to-day life should look like.

Technology can preserve independence. There are several methods to controlling a power wheelchair, including head arrays, sip and puff, switch scanning, and eye control. The same access methods can also extend to environmental control, allowing you to manage devices such as phones, lights, doors, and communication systems. When reliable access to mobility and communication is possible, some people find more reason to consider tracheostomy and ventilator support, as the quality of life can still be meaningful. These choices are highly individual, and there is no single correct answer.

ALS Medical Management

Medical management focuses on extending function, managing symptoms, and coordinating care. There is no cure, but several treatments can help.

Riluzole is the most common long-term medication. It is associated with modestly more prolonged survival and is generally well tolerated. The main concerns are liver function abnormalities and occasional nausea, so periodic labs are essential. Many clinicians start riluzole early, even when ALS is only suspected, because earlier use is linked with better preservation of function and overall survival.

Edaravone is a newer option that can slow motor decline for some people. Tolerability varies in real-world use, and not everyone perceives a benefit. Decisions about continuing it are individualized based on side effects, logistics, cost, and observed effects.

Tofersen is a targeted therapy for a specific familial ALS gene. Only a subset of familial cases carries that mutation; however, among gene-positive individuals, studies show a meaningful benefit. If genetic testing confirms the relevant mutation, this therapy may be appropriate.

Relivrio was removed from the market in 2024 after confirmatory data failed to support the benefit, and side effects were a concern.

Across studies, multidisciplinary care consistently improves outcomes. When neurology, respiratory therapy, physical therapy, occupational therapy, speech language pathology, nutrition, and social work are integrated, survival and quality of life improve. Ventilatory support when indicated, timely feeding tube placement to maintain weight and hydration, and proactive therapy and equipment planning are essential to achieving this benefit.

Symptom management is also essential. Medications can reduce sialorrhea to help with drooling, address spasticity, treat depression and anxiety, and manage cramps, sleep disruption, constipation, and pain. These agents do not alter the disease course, but they can substantially improve daily life and enhance the effectiveness of other therapies.

ALSFRS-R

A widely used tool in clinics is the ALS Functional Rating Scale, Revised. Multidisciplinary clinics typically administer it at every visit, about every three months. Large databases collect these scores, which allow researchers and clinicians to track how function changes over time.

The scale helps quantify progression as a slope over time. A steeper slope indicates faster progression. A shallower slope indicates slower progression. For most people, that slope tends to remain relatively consistent across the disease course. If you are a fast progressor early on, you tend to remain fast, and the same holds for slow or intermediate progression. In clinical trials, if a treatment changes the slope, that suggests the treatment had an effect.

The scale is not perfect. It is not very sensitive in some areas, and scoring used to vary across clinics. That has improved with clearer scoring criteria. The scale includes items such as speech, saliva management, swallowing, handwriting, cutting food, dressing, and hygiene. Each item is scored from four, meaning normal, to zero, meaning complete loss of function for that item. With twelve items, your total score reflects your overall function, and repeated measurements show how it changes over time.

Adaptive strategies and equipment can influence certain item scores. For example, changes in writing tools, grips, or paper can improve performance on handwriting. As a result, more explicit rules now define how to assign scores, ensuring more consistent results across clinics. People sometimes closely track their total score. If someone begins care with a low score compared to another person who starts with a higher score, it can reflect how far along they are at diagnosis, especially since diagnosis can take months to years. When reading ALS research, you will frequently see this scale referenced because it provides a common way to measure function and progression.

Research

I would now like to review some of the research.

Exercise

Exercise in ALS is a nuanced topic, and studies often have small sample sizes or preclinical designs rather than large, randomized controlled trials. A few practical principles are helpful.

High-intensity exercise can hasten deficits. After strenuous workouts, healthy muscles tear and then repair. In ALS, repair is less efficient, and motor neurons are already stressed, so trying to outwork the disease can backfire. Observational data also suggest that long-term, high-level, repetitive training in elite athletes may be associated with a higher incidence and earlier onset of certain conditions in some individuals. Hypermetabolism adds another layer. If weight is already dropping, pushing hard increases energy expenditure and can worsen weight loss, which is linked to faster weakness.

A reasonable clinic guideline is to lift only when the limb is at least a three out of five or better in strength. For example, lifting weights overhead when arm elevation against gravity is not possible does not make sense. Be cautious with eccentric loading, which is the muscle work used to control lowering or deceleration, such as descending stairs, lowering in push-ups, and specific machines. Eccentric work is demanding and can lead to excessive fatigue.

Favor submaximal, moderate activity. Avoid max rep and feel the burn goals. Emphasize endurance, lower resistance with higher but comfortable repetitions, compound movements that recruit multiple muscle groups without exhausting any single one, and pacing that prevents a post-exercise crash. Aquatics, gentle aerobics, and recumbent cycling can be effective options. You should recover within about an hour. If recovery takes longer, the session was too intense.

Expect day-to-day variability. Set Good Day, Middle Day, and Tough Day plans that define safe limits for time, repetitions, resistance, and tasks. Prioritize function first. If energy is limited and a busy day includes housework or time with grandkids, count that activity and scale back formal exercise. The goal is to enhance quality of life and preserve capabilities, not to deplete reserves.

A recent meta-analysis suggests that resistance training offers the most significant benefit for functional outcomes. In contrast, aerobic training provides the most crucial benefit for breathing measures, such as diaphragm performance. Translate that into a personalized program that blends gentle resistance for daily tasks with light to moderate aerobic work for ventilatory health.

Build a personalized plan with the care team. Occupational therapy can frame exercise around meaningful daily activities, focusing on functional exercises, while physical therapy can incorporate resistance and aerobic elements safely. Education is key. Address fall risk, water safety if using pools, and energy conservation. Reassure that it is acceptable to spend limited energy on valued activities rather than formal workouts. Many people with ALS have been very active and social in fitness communities, so acknowledging the emotional and social impact of scaling back and offering support to stay engaged in adapted ways is essential.

Pain

A review by Kwok reports wide variability in pain prevalence, roughly fifty to eighty percent, and pain can occur at any point in the disease course. Greater pain is associated with worse function and lower quality of life. Sources differ. For some, cramps and spasticity are the primary symptoms. For others, fasciculations are uncomfortable. Some experience neuropathic pain described as burning, aching, or a deep ache that is more directly related to ALS.

Secondary contributors are common. Muscle atrophy and immobility alter joint mechanics. The shoulder is especially vulnerable. Instability can trigger pain, which in turn leads to guarding and may progress to a frozen shoulder. Prolonged sitting and restricted positions compound the problem. People who cannot lie flat due to orthopnea often remain on the same pressure points, may be unable to stand, and need to keep the head of the bed elevated. They may also rely on a recliner and avoid tilting their wheelchair. The risk of pain and pressure injuries becomes high. Frequent assisted weight shifts, pressure redistribution surfaces, and careful positioning help reduce these complications.

Effectiveness of OT

Evidence points to multidisciplinary care improving outcomes, which aligns with greater access to occupational therapy across the disease course. A 2014 systematic review in AJOT highlighted that integrated teams correlate with better results, in part because OT can consistently address emerging problems with stretching, home exercise, task analysis, and equipment optimization.

The use of a power wheelchair illustrates the OT's focus on real-world function. Comfort, daily task performance, and device ease of use are central to sustained independence. Positioning, pressure management, and feature programming such as tilt, recline, and alternative drive controls directly affect participation in self-care, communication, and community activities.

Hospice involvement benefits from OT as well. The review recommended including OT on the hospice team or at least allowing targeted visits focused on positioning, comfort, transfer training, caregiver education, and safety. Even limited OT input at this stage can reduce pain, prevent pressure injuries, and support meaningful activity.

Research volume remains limited, but the clinical throughline is consistent. OT maintains function by adapting tasks, environments, and tools, as well as by educating caregivers. Interventions span energy conservation, joint protection, splinting, seating and mobility, communication access, environmental control systems, and routines that preserve autonomy and dignity.

Rehabilitation

Rehabilitation for regaining strength has limited evidence beyond expert opinion and a few reviews, mainly because many people with ALS do not get stronger over time. There is a difference between disease-related weakness and deconditioning from inactivity. Falls, balance problems, and fear of movement often lead to prolonged sitting, which adds a separate layer of weakness.

When weakness stems from hospitalization or illness, such as pneumonia requiring a two-week hospital stay, gentle and cautious rehabilitation can be an appropriate approach. The key is dosing. Pushing too hard is counterproductive, and a standard three hours of inpatient therapy per day is usually excessive. Expectations should be calibrated to the cause. If the decline is primarily due to bed rest rather than disease progression, meaningful gains are still possible. Many people can recover close to their prior level of function after targeted, moderate therapy, even though the underlying disease will continue to progress.

Wheelchairs

You may see my name on several wheelchair studies, as that is my area of focus. I work in a wheelchair seating clinic, so I have a steady stream of participants. About every five to ten years, I publish something on power wheelchair use and satisfaction. My most recent project tested a smartphone-based alert to prompt regular pressure relief. It did not go well. People with ALS and their caregivers are already managing an enormous load. By the time a power chair is needed, many feel they cannot handle another task. Hourly alerts were frustrating. Some participants did not own smartphones, others were unfamiliar with downloading apps, and some lacked reliable internet. More than half of the participants died before the study concluded. Conducting longitudinal research in this population is very challenging, and I published on those practical barriers.

Another ongoing challenge is securing insurance coverage not only for current needs but for foreseeable needs as the disease progresses. Payers often resist features or devices that will be essential in the near future, which creates delays and disrupts continuity of care. As an occupational therapist, my primary focus is on function and participation. The exact device is less critical than enabling community access and daily life. If a scooter, a manual chair, or a power chair helps someone get out of the house and become engaged, that is the right move while we work toward the optimal long-term solution. Matching the person to the right mobility system and access method requires professional evaluation, iterative fitting, and advocacy to protect safety, independence, and quality of life.

Other Research

Stem cell clinics are proliferating rapidly, and many are charging substantial fees for injections that lack solid scientific evidence. The body clears injected stem cells efficiently, much like it clears a virus, so paying twenty thousand dollars for unproven injections is not a good use of resources. Legitimate trials do exist, and I direct people toward regulated studies rather than pay-to-participate offerings.

Active research is robust. Teams are testing individual drugs and drug combinations, studying proteins and gene targets, and working towards the development of reliable biomarkers in blood or cerebrospinal fluid. Genetics remains a significant focus, both for understanding risk and for matching people to targeted therapies.

A fascinating frontier is brain-computer interfaces. An implantable device designed to read signals from motor areas and trigger actions is nearing market entry, with the initial goal of restoring communication for people at very low functional levels. The pace of innovation is fast, and I encourage staying connected to academic centers, registered clinical trials, and multidisciplinary ALS clinics to separate credible advances from hype.

ALS Information

For reliable, current information on ALS, I begin with organizations that keep their materials up to date and grounded in clinical practice. The Muscular Dystrophy Association includes ALS within its mission, and its site offers clear overviews of the disease, care standards, and research updates. The ALS Association provides national-level resources, education, and connections to certified clinics, support groups, and equipment loan programs. In some regions, clinics are aligned with ALS United rather than the ALS Association. Several states and local chapters transitioned to ALS United, so the exact website you use can vary by location. For example, in North Carolina, the chapter is ALS United North Carolina. Regardless of affiliation, people can access resources wherever they find them most helpful, but clinic funding and support often run through one organization or the other.

For practical, day-to-day guidance, I often recommend Your ALS Guide. The materials are primarily written by occupational therapists, physical therapists, and speech-language pathologists, and they translate clinical recommendations into checklists, how-to articles, and step-by-step instructions. Whether it is preparing for a first clinic visit, setting up a home for safety and access, choosing communication tools, or planning for mobility and respiratory support, these guides help people and caregivers put plans into action.

Assessments

Some patients enjoy tracking their grip strength with a hand dynamometer and comparing it between their right and left hands over time. I have seen that kind of data spark helpful conversations about asymmetry and function. I still recall alerting our neurologist about a patient whose dominant hand and thumb were significantly weaker than their nondominant side. There was no sensory loss, no pain, and fasciculations were present. Someone had suspected multiple sclerosis, but the pattern pointed toward ALS. The patient later received the diagnosis at our center. As tricky as that news was, I was grateful that a wheelchair visit led to the proper referral.

I use standardized measures when they add value. The Barthel focuses on basic activities of daily living. The DASH captures arm, shoulder, and hand function. Range of motion and manual muscle testing help me follow trends. I also prioritize a functional lens. I look closely at what someone can do today, what matters most to them, and what I see coming next. That perspective guides energy budgeting, equipment choices, and timing. Because I typically see people every three months in a multidisciplinary clinic, serial observation and targeted problem-solving often take precedence over completing every possible assessment at each visit.

My role blends evaluation and treatment with a strong emphasis on practical solutions. I am partially grant-funded through ALS United, the MDA, and other sources, which lets me spend time on education, handouts, training, and real-world trials without always needing to bill. I maintain a lending pool of devices, allowing people to borrow and test them before making a commitment. When someone says, I do not need that yet, I explain that I would not recommend it if I did not expect it to help soon. Readiness varies, so I try to put the tool in their bag. If it makes it home, it is far more likely to be used than if it stays in my clinic. Many people lack the time or extra money to chase down items later, and they would rather not think about equipment until it is necessary. Specialized occupational therapy in a multidisciplinary clinic means anticipating needs, smoothing the path, and keeping people engaged in the activities that matter most.

ALS Multidisciplinary Clinic OT

If you are working with someone who has ALS in home health or outpatient care, connect with a multidisciplinary clinic in your region. Those teams welcome collaboration and can offer guidance, resources, and coordinated planning. Many clinics do not have full-time staff across every discipline so that responses may align with clinic days rather than daily availability. Typical follow-up occurs about every three months, which complements community-based OT. Use that cadence to coordinate equipment trials, education, and timing for referrals such as seating and mobility, communication access, nutrition, and respiratory support. Proactive communication keeps everyone aligned and helps the person receive the right services at the right time.

OT Intervention

Short, targeted courses of occupational therapy are well-suited to meet the needs of ALS patients. When progression brings a new problem, such as a fall, shoulder pain, loss of balance, or difficulty with a specific task, a brief episode of OT or PT at home or in outpatient care can address it. After the issue is managed and training or equipment is in place, therapy pauses. Months later, as new challenges emerge, therapy resumes for another focused burst. This serial approach respects billing requirements and keeps goals concrete and measurable while matching the evolving nature of a progressive disorder. A forthcoming talk on goal setting for progressive conditions will explore how to structure these focused episodes to solve immediate problems and set people up for the next stage.

Outpatient/Rehabilitation

Outpatient referrals often arrive for weakness without a precise diagnosis. Standard exercise programs may be prescribed to restore strength and endurance. If the underlying issue is ALS, aggressive strengthening can worsen fatigue and function. Unexplained asymmetric weakness without sensory loss that is attributed to spine problems should raise a small flag. A gentle suggestion to consult neurology can help ensure the right workup. Encounters can be task-specific. A visit for wheelchair evaluation, a shoulder pain episode, or post-hospitalization deconditioning after a pneumonia or feeding tube placement can each warrant focused rehabilitation. Home health is well-suited to safety evaluations, caregiver training, fall prevention, energy conservation, and the setup of adaptive equipment. Collaboration with the ALS clinic OT is valuable. Loaner equipment may be available, and coordinated trials let the community therapist train with the exact devices the person will use.

Home Health

In home health, there is room for more visits and more meaningful targets. Focus areas include pain management, safety, energy conservation, fall prevention, positioning, caregiver training, and equipment setup. Plans of care should reflect the realities of ALS. Goals such as increasing strength to five out of five are not appropriate and will prompt a revision. Goals should emphasize functional outcomes through equipment and technique rather than raw strength.

Examples include improving independence with dressing and bathing using adaptive tools and seated setups, increasing safe transfers with a sliding board or mechanical lift, reducing pain with positioning and a pressure redistribution cushion, increasing communication efficiency with a text to speech app or AAC device, improving nutrition access by establishing safe mealtime strategies and integrating feeding tube routines, increasing respiratory safety by training on cough assist and suction and coordinating mask use during daily tasks, improving home navigation by configuring a power wheelchair with tilt, recline, leg elevating functions, and programmed drive profiles, increasing caregiver capacity through training in body mechanics, transfer methods, and equipment use, and reducing fall risk by implementing a home safety plan that includes lighting, pathway clearance, grab bars, and footwear. Frame progress as improved use of equipment, adoption of alternate techniques, and reduced caregiver burden. These goals align with the disease course, support quality of life, and give clear, measurable outcomes for each episode of care.

Best Practices

Best practices center on occupational engagement that preserves roles, routines, and meaningful activities, with technology introduced early enough to be effective. A robotic arm, such as the JACO, can restore simple yet essential actions like scratching an itch, which is precisely the kind of functional gain that improves daily life. The guiding question is always what is worth the time, effort, and money, whether that is eating independently, completing grooming, operating a phone, writing a message, or moving safely around the home. The emphasis shifts away from exercise as the primary goal and toward functions that maintain autonomy.

Early introduction of equipment prevents a crisis. Ramps, bathroom modifications, entry solutions, and bedroom reconfigurations are far more complex and costlier when rushed. Trialing tools before they are urgently needed reduces waste and lets people learn while they still have the bandwidth. Clients and caregivers consistently value problem-solving, creativity, responsiveness, availability for questions, and practical know-how. Many are overwhelmed and often say they never would have thought of a given solution on their own. A lending pool of low-tech and mid-tech devices reduces friction and increases follow-through.

A mobile arm support illustrates how targeted devices extend independence by offloading the weight of the arm, allowing feeding, grooming, typing, and switch or touch access to remain possible. Pairing this with thoughtful positioning, appropriate seat-to-table height, and stabilized dishware further improves success. Similar thinking applies across daily tasks. Eating and drinking can be supported with built-up or angled utensils, universal cuffs, stabilized dishware, long straws, and early discussion of feeding tube use for hydration, medication, and calories while continuing to enjoy eating as safely. Grooming and hygiene can benefit from electric toothbrushes with enlarged handles, elbow supports, spray shower heads with toggles, long-handled sponges, seated shower setups, and no-step entries.

Meanwhile, toileting can be eased with bidet seats featuring armrests, drop-arm commodes, and transfer aids. Dressing is simplified with elastic laces, zipper pulls, magnetic closures, seated techniques, and slide sheets. Writing and computer access improve with keyguards, large key keyboards, trackballs, head mice, eye tracking, switch scanning, word prediction, and speech to text. At the same time, articulation allows for all movements that the person can reliably reach. Phone and environment control expand through voice assistants, smart plugs, smart locks, automated doors, adjustable beds, and integrated wheelchair power and mounts to keep controls in reach. Transfers and mobility are supported with gait belts, rails, transfer poles, slide boards, stand-assist devices, portable lifts, and a staged plan for power mobility, including alternative drive controls and programmed tilt and recline for pressure relief and breathing. Positioning and pressure management rely on contoured seating, adjustable backrests, pressure redistribution cushions and mattresses, scheduled weight shifts with practical prompts, and caregiver education for microshifts when full tilt is not tolerated. Energy conservation strategies, such as task simplification, seated workstations, batching tasks, utilizing delivery services, and timing high-value activities during optimal energy windows, help preserve participation.

Goal setting in progressive disease prioritizes adoption and mastery of equipment and techniques, reduction of caregiver burden, fewer falls, less pain, faster and more reliable communication, safer nutrition, and sustained engagement in valued roles. The outcome that matters is being able to do what is essential with the least strain for as long as possible.

Adaptations

Some adaptations are less common but very useful. A bidet in this country typically replaces only the toilet seat and lid, connects to the existing water supply, and extends a nozzle under the seat. Because it replaces the seat, a traditional clamp-on riser will not work. If you clamp a riser on top of a bidet seat, you will end up spraying the ceiling. Plan for height by starting with a taller, comfort height toilet or by raising the toilet from underneath. A Toiletvator is a three-and-a-half-inch base that installs under the entire toilet, using longer bolts, to provide the necessary height without interfering with the bidet. When ordering a bidet, choose a model with a remote rather than controls fixed to the seat. People often need a remote that can be placed forward or to the side, or one that a caregiver can operate, rather than reaching back to press small buttons next to the seat.

For urinary management, PureWick utilizes a small external wick and a suction device to draw urine away and into a collection canister. It can be very effective, but it is not typically covered because the device cost exceeds the allowable code, so suppliers will not bill it. It remains a cash item and is expensive.

For bathing, consider tub slider systems such as TubBuddy or ShowerBuddy. These systems leave a frame in the tub or shower, a bridge from the commode or bathroom floor, and allow transfer on a rolling shower chair. The upfront cost can be several thousand dollars, but they can avoid a complete bathroom renovation and can be installed quickly. Team Gleason, a charity founded by Steve Gleason, has helped some clients obtain bathing equipment like this when insurance does not.

For clothing management, anticipate the progression and adapt garments rather than relying on tools that will soon be too hard to use. Instead of button hooks, convert closures to magnets. Velcro tends to collect debris and can be too strong to separate. Magnetic closures can be sewn in behind existing buttons or purchased in adaptive clothing. Add pull loops at the waistband of underwear and pants to allow hooking the hand or thumb and pulling garments up without a pinch grasp. Loops can also be used on bra straps, allowing both thumbs to hook and bring the straps together with less fine motor demand.

For eating, think across the timeline. Start with enlarged handles made of foam, then bend the utensils to reduce wrist motion. Next, secure the utensils with a universal cuff. Later, introduce powered assistance, such as a mobile arm support or a robotic feeder like Obi. If powered devices are not feasible, raise the plate and support the elbows so the hand is closer to mouth level, which reduces the effect of gravity and extends independence. The same positioning concept helps with grooming. When proximal shoulder and elbow weakness limit sustained reaching, set up grooming tasks seated at a table with elbow support so the hands are near the face. Wash your hair with a handheld sprayer while seated, and use supports to minimize the time spent with your arms unsupported. Shirts can be laid face down on the table so arms slide into sleeves with elbows supported.

For meal preparation and kitchen tasks, pre-open jars, bottles, and containers. Decant heavy items such as milk or juice into smaller bottles that provide a single serving and are easier to lift. Use a folding rolling cart to move groceries from the car to the kitchen, to take dishes to the table, or to transport laundry, which is safer than carrying items while using a walker. Many carts fold and store easily, allowing them to be pushed with both hands, which improves safety.

Provide anchor points for transfers and bed mobility to ensure stability. Sometimes, the most valuable feature of a hospital bed is simply something solid to hold onto. Add loops or straps to the bed frame, headboard, or side rails to give leverage. Standard bed canes work on the outside of the bed, but people also need something to pull against when rolling the other direction. Lower overly tall beds by removing box springs or using low-profile foundations, so that feet contact the floor for safer sit-to-stand transitions. Add straps to everyday tools to secure the hand when the grip is unreliable. Two interlocked Velcro loops can secure an electric razor to the hand for pennies; the same idea can attach a rake, a dressing stick, or a kitchen tool, extending participation in valued tasks.

Adapt leisure by identifying what truly matters about the activity. If golf is about spending time with friends, enjoying fresh air, and being part of a group, then driving the cart, keeping score, and hosting watch parties may deliver the same joy without the physical demands of swinging clubs. If gardening is about tending to plants and being outdoors, consider moving a few tomatoes into large planters on a porch at wheelchair height. By clarifying the essence of the activity, it becomes easier to design practical ways to incorporate it into a person’s life.

Range of Motion/Pain Management

This is core occupational therapy work. Range of motion and pain management matter, but the goal is to preserve the motion that is truly necessary for function. If raising an arm overhead causes pain every time, stop forcing that motion. Use a reacher to relocate items to lower shelves and reconfigure the task. Painful overhead stretching can inflame tissue, destabilize an already weak shoulder, and exacerbate the condition. The shoulder is often weak and unstable, sometimes to the point of subluxation, so external support may be needed to approximate the joint and reduce strain. When the arms are globally weak and hang heavily, support that unloads the limb protects the joint and reduces pain.

Neck extension weakness is common. Most people do not want a rigid collar that locks the head as if for whiplash. What helps is a place to rest the chin without feeling trapped. A padded wire frame cervical collar offers that balance. It supports the chin while allowing some movement and is designed for neuromuscular weakness rather than trauma immobilization. Thoughtful selection of supports, along with task and environment changes, reduces pain, preserves essential motion, and keeps daily activities feasible without provoking injury.

Home Modifications

Plan ahead for access, because what seems manageable today often becomes the barrier that forces crisis decisions. A half bath on the main level may not fit a chair, so consider where a full bath and a bed can be on the same floor. Count every step to enter, measure clearances, and map real routes in and out for all weather conditions. Look for a second egress and consider the impact of rain, ice, and snow so caregivers and equipment can move safely. Poorly placed grab bars and ad hoc fixes can be worse than nothing; height, angle, stud anchoring, and approach path matter more than just having a bar on the wall.

Portable shower systems can bridge the gap when bathroom renovations are impossible. A freestanding pan with a curtain enclosure connects to a sink for warm water, and a small pump returns grey water to the drain. Setups in a kitchen, garage, or utility area can provide real showers without demolition. They are not cheap, but they are far less than a remodel and often less than tub slider systems.

Be cautious with stairlifts. They can make sense briefly, but ALS progression often brings head control loss, trunk instability, and the need for a power wheelchair on each level. A stairlift solves the stairs while stranding mobility devices on the wrong floor and becomes a sunk cost. Tight stair geometry, door placements, and short landings can make installation impossible in any case. When structure, rental rules, or Section 8 constraints preclude major modifications, aim for safe and workable alternatives. A bed in the front room, a bedside commode, and carefully arranged transfers may be the realistic plan. None of this is ideal, but planning early allows families to choose the least disruptive option before an emergency dictates their course of action.

High Tech

High-tech access is advancing quickly. An eye-gaze tablet can now drive a power wheelchair, and if someone already uses eye-gaze for communication on a Windows-based device, such as a Tobii system, the same platform can often handle both communication and driving. Apple-based systems are not yet compatible with wheelchair eye gaze control, so a Windows option or a dedicated driving tablet is needed. The forward camera helps by showing what the tablet blocks from view, and the on-screen controls gray out directions you cannot safely choose. Success depends on cognition, reliable eye control, and ocular range of motion. Some people lack sufficient eye movement or endurance for precise gaze, and sometimes the caregiver cannot manage the technical setup, which limits feasibility despite the potential.

Modern complex power wheelchairs typically include Bluetooth and often feature infrared capabilities. That means the chair’s drive interface can also act as a universal access hub. The same joystick, head array, or switch system that steers the chair can pair to a phone or tablet, control a voice assistant, act as a computer mouse, and operate TV or environmental controls through IR. With expanded electronics enabled, a person can navigate the home, answer messages, adjust lights and doors, and use a computer without changing input methods. Insurers sometimes resist funding these expanded electronics, and some suppliers shy away from exposing the full capabilities because they do not want to be responsible for configuring every device in a person’s digital life. When that happens, loop in the state assistive technology program or a specialized AAC and access team to handle training, pairing, and long-term support. The wheelchair becomes the gateway to mobility, communication, and environmental control when the ecosystem is configured deliberately.

Case Examples

Case Example 1

Spasticity dominated his days. Legs and trunk braced themselves against nothing in particular, tone ratcheting up with every small demand, and underneath it all, the slow erosion of strength made everything heavier. Speech came out thick and effortful. He refused the device because he wanted to be understood as he had always been, and the frustration of not being understood spilled over into meanness that was really a result of disinhibition and fear. Meals became choke points, the throat clamping down when he needed it to open, so the feeding tube went in early and quietly saved him from the constant struggle with calories and pills.

Standing changed the weather. He paid out of pocket to add powered standing to his chair, and the first time he rose up it was like someone opened a window. Tone softened a notch. Pressure eased. Bowels behaved. He cycled up and down through the day because he could, because control matters when so much does not yield, and even his attention seemed more straightforward in those minutes upright. The chair became more than mobility. It was a lever he could pull to feel better without needing to ask anyone for help.

At home, the air was tense. His wife moved through rooms in a practiced arc, doing a hundred things that kept them afloat, and he snapped when words failed him. Burnout crept in while wearing ordinary clothes. It arrived in the pause before she answered, in the way she rubbed her eyes on the way to the kitchen, in the quiet admission that she had not signed up for decades. People often imagine ALS as a rapid decline. Primary lateral sclerosis and frontal changes stretch time out and bend it in ways that are hard to live with. Control becomes a battlefield. Small choices become skirmishes. A plan is a kind of mercy.

When the days went better, tone was managed on purpose. Medications were scheduled, not just chased. Positioning at night reduced the number of insults that woke the muscles into guarding. The standing feature anchored a routine that gave his nervous system predictable inputs. Communication got a backup, whether he liked it or not, with significant phrases ready on a screen and a simple way to say yes or no when speech fell apart. The house responded to him through switches and voice, so fewer arguments started with I can do it myself. Social work found short stretches of respite, and aides learned the lift so his wife did not strain her back with every transfer. They wrote down the red lines that would mean it was time to change the plan, and that made the present a little easier to carry.

On bad days, nothing seemed to fit. He pushed for one more stand when it was not safe. She tried to keep the peace and watched the clock. The difference between those days and the better ones was not character. It was a matter of tone, fatigue, and whether the scaffolding held. The chair still rose, and when it did, he looked out at the room from a different height, and for a moment, both of them breathed.

Case Example 2

She was young, and the disease moved quickly. Bulbar symptoms came first, with speech thinning to a whisper and then to fragments that no one could catch. Swallowing was unsafe, and breathing was shortened by effort. Pseudobulbar affect colored the days, laughter breaking through at the wrong times, tears coming harder and more often. Her neck could no longer hold her head, her shoulders giving way so that her face pointed toward the floor. A collar helped for minutes, and then pressed her jaw into a weight-bearing job it was never built to do, aching more than it supported.

She could still walk, and that mattered to her. Walking meant she was not done, so the idea of a wheelchair landed like a verdict. The problem was that walking did nothing to alleviate her headache or improve her voice. Without a place to rest her chin and mount a communicator, she was trapped inside her body while she paced the house. Texting got slower as her hands weakened, and the family strained to read meaning in every gesture. Children waited for answers that took too long to form. Her spouse tried to steady the day and found there was nothing solid to hold.

The argument was not really about wheels. It was about what counted as giving up. The loaner chair sat there like a dare, and the conversation kept coming back to tools. A chair can hold a head, carry a device, keep oxygen close, and still be something you step out of to stand when it is safe. It does not pass a sentence. It solves a problem. Occupational therapy turns that truth over gently until it can be believed. Use the tool that enables you to do the thing. Mount the talker. Rest the neck. Save your strength for the parts of the day that matter.

Bulbar onset compresses time. Decisions that other phenotypes can postpone arrive in a tight window. Feeding tubes, noninvasive ventilation, voice and message banking, and emergency plans for aspiration all rise to the surface within months. The slope and the direction decide the shape of a life more than any average ever could. Finger weakness can stretch for years before breathing is threatened. Bulbar disease asks its most challenging questions first. She stood in that hard place, wanting to walk because walking was proof, and learning that proof does not keep you fed or heard. The day she rode out of the clinic in the loaner chair with her head supported and her device speaking in her voice, the house sounded different when she came home. It was not a surrender. It was air getting in and words getting out.

Conclusion

Staying ahead is the craft. Watch the slope, anticipate the next barrier, and meet it with whatever works, from a strip of Velcro to a scrap of splinting plastic to a borrowed device that buys six more months of independence. Money and time are scarce, so ingenuity prevails. Loner closets stretch budgets and turn maybes into yeses. The work is the purest kind of occupational therapy—problems named out loud and solutions built with what is at hand, then rebuilt when the ground shifts again. ALS is a terrible disease, and still, the impact of one well-timed adaptation can be astonishing. A head supported so words can be spoken. A transfer made safe so a partner’s back is spared. A meal finished without fear. If the chance comes to stand with someone through this, bring all of it—creativity, patience, pragmatism, and that unwavering focus on doing what matters.

Questions and Answers

Do you run into insurance denials because ALS has a poor prognosis?

Denials do happen, but they are often less about prognosis and more about a general deny-first strategy. Many insurers assume that a first denial will deter most people from pursuing the request, so persistence is crucial. Appealing, providing clear functional justifications, and involving the clinic team can help turn denials around.

Are loan closets usually run by ALS clinics?

Often yes. Many ALS clinics maintain loan closets with grant support, which means more items to trial and borrow. Muscular Dystrophy Association clinics or community organizations may also run loan closets.

Is insurance coverage the most challenging part of treatment?

It is one of the most challenging aspects. A practical approach is to “play the game” with scheduled re-evaluations every three to six months, creative but measurable goals, and short bursts of therapy tied to new problems. Even if continuous treatment is not covered, repeated focused episodes are typically approved and effective.

Does frequent re-evaluation really help with approvals?

Yes. Regular re-evaluations document change, justify new goals or equipment, and align with payer expectations. This cadence supports coverage for serial, problem-focused therapy.

Did Stephen Hawking live so long because he had a spastic form of ALS?

His presentation is generally described as a slow-progressing motor neuron disease with predominant weakness rather than classic rapidly progressive ALS. Some people, for reasons not fully understood, progress very slowly or plateau, especially when there is limited respiratory involvement. A minimal subset even shows partial reversal. Research is ongoing to understand why some phenotypes progress far more slowly.

Have a great day, everyone. Hope you join us again.

References

See additional handout.