How to Recognize and Manage ALS Symptoms

Respiratory Symptoms in the Early Stages of ALS

ALS causes muscle weakness, impacting various muscles, including those responsible for breathing and swallowing. This weakness can lead to respiratory problems, which are among the most serious symptoms of ALS and should not be ignored.

If you notice any of the early signs of respiratory problems listed below, contact your doctor immediately:

• Shortness of breath: This most often occurs during activities like running, exercising, walking long distances, or climbing stairs.
• Fatigue and morning headaches: Difficulty breathing can disrupt sleep, leading to frequent awakenings during the night. This occurs because the chest muscles relax too much and can’t assist with breathing during the dreaming phase of sleep.
• Difficulty swallowing: This may result in food and saliva being more easily inhaled (aspirated) into the windpipe, increasing the risk of choking. Aspiration can also introduce bacteria into the lungs, raising the likelihood of respiratory infections and pneumonia.
• Fever: If you notice a fever, it could be a sign of a respiratory infection. Reporting this symptom to your doctor allows for quick intervention, often involving a course of antibiotics.
• Change in color of phlegm: Monitor for any unusual discoloration.

Respiratory Symptoms in the Late Stages of ALS

As ALS progresses, breathing problems often worsen. Shortness of breath may occur more often, including while at rest during the day.

Treating Early-Stage Respiratory Symptoms

If you are experiencing these symptoms, inform your physician right away. Diagnostic tests may include:

• Vital capacity (VC) breathing test: This measures the amount of air you can blow out, and is routinely administered in ALS clinics to monitor changes.
• Maximum inspiratory pressure (MIP) test: This test measures the strength of your breathing muscles.
• Pulse oximetry: This test examines the saturation levels of your red blood cells. This may involve a small probe on your finger throughout the night, an overnight sleep study, or a blood sample for oxygen and carbon dioxide level measurement.

The results of these breathing tests are used to decide whether a treatment called non-invasive positive pressure ventilation (NIV), a treatment using a ventilator, is recommended. Our goal is to identify the most suitable option for your condition.

Non-invasive positive pressure ventilation

NIV typically involves wearing a mask over your nose and mouth. NIV assists weakened breathing muscles by applying forced air pressure to help fill your lungs with air. While NIV may feel uncomfortable at first, your respiratory therapist will help you adjust to it. The goal is to use NIV for a minimum of four hours a night, gradually progressing to full-night usage.

Mechanical insufflator-exsufflator

As ALS weakens respiratory muscles, it becomes difficult to cough and expel phlegm from the lungs. A cough-assist machine, sometimes called a mechanical insufflator-exsufflator (MIE), may be recommended. This device facilitates coughing by delivering a deep breath of air followed by negative pressure (suction), assisting in clearing phlegm. Using a cough-assist machine can help prevent lung infections by keeping the lungs clear of mucus.

Treating Late-Stage Respiratory Symptoms

If your breathing problems worsen, it can be helpful to start using NIV during the day as well as at night. Your respiratory therapist can help explore alternative daytime devices, like a mouthpiece, to enhance your comfort.

Tracheostomy for ALS

In some cases, considering a tracheostomy may become necessary. This surgical procedure involves creating an opening into your windpipe (trachea) through which a plastic tube is inserted to allow air to pass directly into your trachea. Unlike the mask used for NIV, this tube stays in place at all times. A ventilator can be attached to assist with breathing and clear phlegm to prevent food or saliva from going into your lungs.

While a tracheostomy with a ventilator can greatly assist with breathing, it doesn’t reverse ALS progression or prevent muscle weakening. Speech abilities, as well as the ability to eat and swallow, vary among individuals with a tracheostomy. For people who cannot eat or gain nutrients by mouth, a feeding tube is inserted into the stomach.

Deciding whether to undergo a tracheostomy and use a ventilator is a personal choice. You can discuss any questions or concerns you may have with your physician to make informed decisions about your care.

Can ALS patients choose to stop ventilation?

As your ALS progresses, you can decide whether or not you would prefer to discontinue ventilation. This process, guided by our hospice care team, can be carried out at home to ensure comfort and minimize any breathing distress. The removal of the ventilator is done under sedation and is continued until breathing stops naturally. Family and friends are often present, providing a peaceful and loving atmosphere.

For those with ALS, especially those relying on ventilation, appointing a health care proxy is advisable. The proxy can make medical decisions if the patient becomes unable to express preferences. You should clearly communicate your treatment and end-of-life preferences to your health care proxy. It is important to keep an open, continuing dialogue with your health care proxy as your thoughts and feelings may change over time.

People with ALS often encounter frustrating symptoms related to communication and eating/drinking. As the disease progresses, it can lead to muscle weakening, a decrease in the size of the muscle (atrophy), and/or a decrease in control due to muscle tightening (spasticity) in the mouth and throat. These changes impact speech (dysarthria) and swallowing (dysphagia).

What Is Dysarthria?

Dysarthria refers to changes in speech that result from weakened muscles. Dysarthria can interfere with your ability to:

• Produce clear sounds (articulate)
• Draw an adequate breath to speak which results in decreased volume of speech
• Control the quality of the sound (timber) or what your voice sounds like
• Maintain the natural rhythm of speech (prosody), which helps others understand the intended message
• Control the escape of air from the nose, which affects how nasal the person sounds (resonance) making it difficult to produce certain sounds such as m, n, and ng

The rate of progression of dysarthria due to ALS can vary greatly from person to person. Eventually, everyone with ALS experiences some degree of dysarthria.

What Is Dysphagia?

Dysphagia is the inability to pass food and liquid easily from the mouth, into the throat, and down into the esophagus to the stomach during the process of swallowing.

Schedule a Consultation with a Speech-Language Pathologist

If you're experiencing challenges with speaking or swallowing due to ALS, it is recommended that you meet with a Speech-Language Pathologist (SLP). MGH SLPs play a crucial role in our team, specializing in assessing, treating, and managing speech and swallowing difficulties associated with ALS. Schedule an consultation when you first begin to notice symptoms or ask to meet with our SLP at your next clinic visit.

Some of the services they provide include:

• Developing effective strategies for managing ALS-related speech and swallowing issues
• Recommending supportive devices for communication and nutrition management
• Providing ongoing education for you and your family about your condition
• Addressing cognitive changes related to reasoning and information processing

The SLP will ask you to perform some simple, non-invasive tests so they can evaluate your speech, including vocal quality, overall intelligibility, breathing, and coordination of muscle movement. These tests may include tasks such as reading aloud, speaking naturally, producing certain sounds in isolation (such as “papapa”), and holding the sound “ah” as long as possible. The SLP will also assess the strength of your lips, tongue, and soft palate (the back part of the roof of the mouth) by having you perform simple oral movements, like pushing your lips against a tongue depressor. The SLP will repeat some or all of these tests at each visit to track changes.

Learn Techniques to Improve Speech

People with ALS who only have minor speech difficulties can learn techniques that will make his or her speech easier to understand. For example, over-articulation or exaggerated pronunciation can help speech clarity and comprehension. As dysarthria progresses, people with ALS need to learn new techniques to compensate for further changes in speech.

Use Communication Devices

An augmentative communication device can help to supplement or replace speech. These devices enable people who are either unable to speak or unable to be easily understood communicate more effectively. An augmentative communication device can be something as simple as an alphabet board or as complex as a computer system driven by a laser-guided mouse that produces the sound of a person’s own voice.

It is recommended that people with ALS undergo an assessment for augmentative communication devices when their speech is just beginning to change, as it takes time and energy to transition into using an augmentative device. Working with insurance providers, securing funding, obtaining the device, learning how to use it, and making the emotional adjustment to this new means of communication are all best accomplished when you are still able to communicative effectively and do not feel like you are pressed for time.

Usually, the evaluation for augmentative communication devices is conducted at a facility or clinic other than the ALS Multidisciplinary Clinic at MGH. The full evaluation takes two to three hours and must be conducted by a speech pathologist and/or occupational therapist with expertise in high-tech augmentative devices. These clinicians usually know whether insurance will cover the cost of the devices and they can help advocate or appeal for devices through insurance or other funding sources.

Preserve Your Voice for Future Use

If your speech is normal, or only slightly impaired, you may choose to “bank” your voice for potential future use with an electronic augmentative device. Voice banking involves reading words and phrases into a computer recording system. Later, these words and phrases can be used as the voice output of a computerized augmentative communication system. This system allows you to type letters, words, or phrases into a computer which then uses your own voice for spoken communication rather than a computer-generated voice.

Since eating and drinking requires the same muscles as speaking, almost all patients with ALS will eventually experience complications with swallowing. The process of swallowing is intricate, involving a network of muscles and nerves working together to move food from the mouth to the stomach while preventing inhalation into the lungs and potential choking.

ALS-related changes in swallowing often result from weakness in the lips, tongue, soft palate, pharynx (the throat muscles that guide food through the esophagus or food pipe), and larynx (the upper part of the airway that closes quickly to prevent choking). Even when these muscles are working well, respiratory muscle weakness can disrupt the coordination between breathing and swallowing, causing difficulties for patients with ALS.

While the types of changes in swallowing are fairly predictable, the rate at which they occur varies. For example, an individual with ALS may initially experience a decline in effective swallowing, followed by a period where symptoms stabilize.

Schedule a Consultation with a Speech-Language Pathologist

The first step in managing changes in swallowing is to have an SLP perform an assessment. Working collaboratively within the ALS multidisciplinary team, the SLP will create strategies for maintaining proper swallowing, ensuring adequate nutrition. Even if there are no significant changes in swallowing, it is important to establish a nutritional plan early in the disease.

The SLP will teach you techniques to enhance your ability to swallow and provide guidance to your family and caregivers on how they can support your eating and drinking experience.

During an assessment, the SLP observes and evaluates your eating and drinking capabilities. This involves watching you consume water, a cookie, or a dish of pudding to assess tasks like holding, chewing, and swallowing. The SLP will watch for obvious difficulties, such as coughing and choking, and subtler indications of muscle weakness, such as difficulty managing food efficiently or experiencing leakage from the mouth. Based on their evaluation, they may recommend lifestyle changes, including adjustments to food or beverage consistency and alterations in head position during swallowing. This type of screening is usually done in the clinic.

If your team is addressing weight loss, breathing, or other swallowing problems, they may recommend a comprehensive swallowing assessment known as a modified barium swallow (MBS). This test offers a more detailed understanding of the problem than simple observation.

The MBS is a painless test performed by an SLP and a radiologist. You can eat and drink before the test, as it requires no advance preparation. During the test, you will be asked to swallow small sips of liquid and bites of solid food of different consistencies, each containing a small amount of barium to ensure visibility on the x-ray screen. Immediately after the test, the SLP and radiologist will review a video that is made up of all the x-rays from the MBS and will provide recommendations to help you eat and drink safely. These recommendations may include:

• Specific food consistencies
• Changing the position of your head while eating and drinking
• Techniques to keep your airway closed during swallowing

Will I require a feeding tube because of ALS?

In the later stages of ALS, when swallowing becomes difficult, a feeding tube may be recommended. This tube can either supplement or entirely replace oral feeding.

Benefits of a feeding tube include:

• Reducing the risks of choking and pneumonia while ensuring proper nutrition intake
• Stabilizing or aiding in the recovery of lost weight
• Facilitating the intake of high amounts of nutrition while allowing for some oral food consumption

The best time to place a feeding tube for a person with ALS is when their breathing is not severely impaired and before any significant weight loss occurs. A physician will only recommend inserting a feeding tube when the procedure is unlikely to cause any complications. Generally, the safest time to insert a feeding tube is at or before the forced vital capacity (FVC)—a measure of breathing function—falls below 50% of normal.

How does a feeding tube work in ALS?

The feeding tube is surgically inserted into the stomach through the abdomen beneath the left side of the ribcage, securing its internal position. The external portion of the tube, which is usually six inches long, has a cap or a three-way valve (stopcock) on the end to prevent leakage.

Most people with a feeding tube consume a balanced liquid nutritional supplement that comes in pre-mixed cans. Each 240 ml can is labeled with a concentration number, which confirms the calories it provides when multiplied by 240. For example, a 1.5 concentration can contain 360 calories.

In a method known as bolus feeding, a large syringe without a plunger is connected to the feeding tube which allows for the liquid supplement to go directly into the stomach. Some individuals opt for a slower feeding method, which is achieved when a pump is connected to the feeding tube to regulate the flow over an extended period. This slow drip is often set up to occur during sleep, providing a gradual supplement intake overnight.

Water can also be given through the tube. It is administered through the feeding tube after the nutritional supplement to provide additional hydration and to flush any residual supplement from the tube.

Some medications can be given through the feeding tube by crushing and mixing them with warm water or ordering them in liquid form. However, some medications, particularly long-acting medications, must be taken whole. Check with your medical team first about which of your medications can and cannot be given through a feeding tube.

Does insurance cover nutritional supplements for ALS?

Insurance generally pays for the nutritional supplement for ALS patients when more than half of the calories a person receives for the day is given through the feeding tube.

Will anyone notice my feeding tube?

The feeding tube is easily concealed by clothing. The external portion of the feeding tube can be curled up and taped to the body when not in use.

What kind of feeding tube is best for me?

There are two main types of feeding tubes: the radiologically inserted gastrostomy (RIG), and the percutaneous endoscopic gastrostomy (PEG). Both RIG tubes and PEG tubes are inserted directly into the stomach through the abdomen and held in place internally. However, the procedures for inserting these tubes differ.

• Inserting a PEG tube: The term PEG is often used interchangeably with “feeding tube” and “gastrostomy tube,” but not all feeding tubes are PEGs. During the insertion of a PEG tube, an endoscopy tube is guided down the esophagus into the stomach. The light at the end of the endoscopy tube shines through the stomach and skin, directing the surgeon to the precise spot where the PEG tube should be inserted. The surgeon makes a small incision through the skin and stomach wall, through which the PEG tube is inserted. Once successfully placed, the endoscopy tube is removed.
• Inserting a RIG tube: Fluoroscopy, a type of x-ray providing real-time moving images of internal organs, is used to guide the placement of the RIG tube. This method allows the interventional radiologist to pinpoint the right spot to insert the RIG tube through the skin into the stomach. To enhance visibility of the intestines during the procedure, barium is administered the night before. A small tube inserted through the nose directs air into the stomach, facilitating its positioning near the skin's surface. This 'gastropexy' is secured with sutures known as T-tacs. Approximately 14 days post-RIG tube insertion, the T-tacs are removed, completing the procedure.

At MGH, ALS physicians frequently recommend a RIG tube for patients, as it is significantly smaller than a PEG tube and requires less anesthesia during insertion. This approach aims to minimize the potential risk of respiratory complications associated with anesthesia. Additionally, the endoscopic tube used in a PEG procedure is larger, potentially posing challenges for patients, especially those already experiencing swallowing and breathing difficulties.

What are the risks of a feeding tube?

The surgical procedure to insert a feeding tube requires anesthesia. For most people with ALS, the primary risk is respiratory distress due to the impact of anesthesia on breathing regulation. Lying flat can also make breathing more difficult for ALS patients.

During the procedure, the anesthesiologist will monitor your breathing and tailor the type of anesthesia based on your individual needs. Some with few respiratory limitations may require only light sedation, while others with more impaired breathing might need non-invasive ventilation (NIV) assistance. Those experiencing respiratory distress may undergo temporary intubation through the mouth or nose to aid breathing during the procedure. Intubation keeps the airway open, allowing a ventilator to assist while the person is sedated. Once the procedure is complete and anesthesia wears off, the intubation tube is removed.

Choosing to have a feeding tube placed before developing significant respiratory concerns may prevent the need for intubation.

Other risks that are possible but rare include bleeding, perforation of the intestine, and infection.

How do I take care of my feeding tube?

Taking care of your feeding tube is generally straightforward. After insertion, any discomfort is typically well managed with medication until the incision heals.

Here are some care tips:

• Clean the entry site: Use soap and water to clean the area where the tube enters the body. Seepage and a crusty area may occur around this spot, which is normal.
• Managing irritation: Gastric juices from the stomach can sometimes irritate the skin around the tube. Applying an antacid paste can help prevent further damage and promote healing. You can make an antacid paste by letting a small amount of liquid antacid settle, then pouring off the liquid that rises to the top.
• Flushing the tube: Keep the tube clean by flushing water through it. If a clog develops from residual supplement, cola inserted into the tube and left to sit can often dissolve it.
• Securing the tube: The tube can be accidentally pulled out if not secured. Curl it up and tape it to the body when not in use to prevent accidental removal. If the tube is pulled out, it should be replaced as soon as possible to avoid the risk of the tract from the skin to the stomach sealing off. This can happen within a few hours.
• Managing extra tissue: Occasionally, the body may produce extra tissue around the tube entry site. This tissue can be tender and may bleed easily. However, it can be easily removed during an office visit through a simple procedure, providing mild and temporary discomfort."

Are there other types of feeding tubes to consider?

In addition to the PEG and RIG tubes, there are several other types of feeding tubes to consider, each with its own features:

• Mic-Key (button) feeding tube: Mic-Key is a brand of feeding tube known as a button, because its opening lies flush against the skin. A cap is used to access the tube. Many of our ALS patients ask about the Mic-Key because it is less visible than the RIG or PEG tubes. While less visible than RIG or PEG tubes, the advantage of reduced anesthesia and respiratory risk with RIG tubes often outweighs the cosmetic appeal of Mic-Key.
• Gastrojejunal (GJ) feeding tube: Inserted into the stomach to the beginning of the small intestine (jejunum), the GJ tube is recommended when there's a risk of vomiting during direct stomach feeding. This type reduces the risk of inhaling vomit into the lungs, lowering the chances of choking and pneumonia. Nutrients are supplied more slowly, and a feeding pump may be necessary.
• Nasogastric (NG) feeding tube: Inserted through the nose into the stomach, NG tubes are typically meant for short-term use. However, specifically designed feeding NG tubes, being smaller and more flexible, can be used for several weeks. In ALS, an NG tube may serve temporarily until a RIG or PEG tube is arranged to prevent malnourishment.

Facing a serious disease like ALS can have a profound impact on both the individual diagnosed and their loved ones, significantly affecting their mental well-being.

Depression and anxiety are common psychological reactions to the disease. Additionally, there are other emotional challenges that stem from the disease that can occur.

Depression in People with ALS

Clinical depression is not as prevalent or severe as might be expected. Researchers have found, for example, that depression is found less frequently in individuals with ALS than in another neurological disease, multiple sclerosis. However, depression, when present, can significantly affect quality of life and is more commonly seen in situations involving loss or threat of loss.

Individuals most often experience depressive symptoms following their ALS diagnosis, particularly at the beginning of disease. Despite the challenges posed by ALS progression, a high number of people with ALS tend to maintain a positive attitude toward life.

Researchers also have found that depression in patients with ALS doesn’t seem to be related to a person’s spiritual beliefs, the presence of a spouse, caregiver/partners, their financial situation, the psychological health of their partners or their participation in hospice care. Another finding is that depression in ALS isn't linked to disease severity, but rather to a decline in physical functioning. The presence or absence of depression was not more common among those individuals who expressed a wish to have a hastened death, and the authors of the study concluded that this desire may be part of a broader phenomenon they call end-of-life despair, which is characterized by hopelessness, but not other depressive symptoms.

Because ALS affects everyone in the family, loved ones may also experience depression. In a study of depression in ALS patients and their caregivers, depression increased much more for caregivers than for patients. Around 10% of caregivers had depression at the beginning of the study, whereas nine months later, it almost doubled.

How do I recognize depression?

Often, the person with depression is the last to realize it. The symptoms may show up slowly and eventually start feeling like they're just a regular part of life.

People who are depressed may:

• Have a low mood in all aspects of life
• Lose interest in activities that once brought joy
• Harbor persistent thoughts and feelings of worthlessness, inappropriate guilt or regret, helplessness, hopelessness, and self-hatred
• Struggle with poor concentration and memory
• Withdraw from social situations and activities
• Notice a diminished sex drive
• Have thoughts of death or suicide
• Be unable to sleep, wake up early and be unable to fall asleep again, and/or oversleep
• Lose weight loss as a result of low appetite
• Gain weight as a result of increased appetite
• Experience fatigue, headaches, or digestive problems

Often, the patient may not admit to feeling sad, as they may consider it another burden to the family members or a weakness of character. The following two questions can help identify depression:

• Over the past two weeks, have you ever felt down, depressed or hopeless?
• Over the past two weeks, have your felt little interest or pleasure in doing things?

If the answer is yes to both questions, depression might be present. Diagnosis and treatment can help restore one’s ability to enjoy life again, even as they deal with ongoing difficult events in their lives.

Behavioral and Cognitive Changes in Patients with ALS

ALS can lead to changes in a person’s behavior and overall cognitive functioning, affecting their ability to process information. The severity of these changes can range from subtle to significantly impacting their ability to stay safe and manage their affairs.

These symptoms result from ALS affecting the frontal-temporal part of the brain. Like other aspects of the disease, these symptoms may not be noticeable in the early stages but can become more noticeable over time. The frequency of these symptoms in ALS is not well understood, but there is growing recognition of their occurrence compared to previous reports.

There is no evidence of specific risk factors explaining why some people with ALS develop cognitive and behavioral changes while others do not. Cognitive changes are often diagnosed using psychological testing. These tests try to determine shifts in personality towards more rigid thinking, slow psychological processes (such as decision making and answering questions), difficulties in understanding new information, problem solving abilities, difficulties dividing attention (inability to do two things at the same time), and emotional changes. It is important to determine if changes are due to the disease process or a person’s attempt to cope in a stressful situation.

Involuntary Emotional Expression Disorder

Involuntary emotional expression disorder (IEED) is a syndrome where a person's display of emotions happens independently of their actual feelings. When IEED occurs in patients with ALS, it is often referred to as a Pseudobulbar Affect (PBA).

IEED typically appears as uncontrollable, unpredictable crying and/or laughing with no apparent cause. These episodes may quickly alternate between tears and laughing, and they can start and stop abruptly. IEED may sometimes be misunderstood as depression or bipolar disease. IEED should be managed differently than other mood disorders.

The cause of the disorder remains unclear, although there is general agreement that it is the result of an injury to the brain pathways that control expression of emotions. This syndrome can present in a number of neurological diseases like stroke or traumatic brain injury, multiple sclerosis (MS), as well as dementias, Parkinson’s disease and ALS. Both patients and those close to them should be aware of this syndrome, since it can be very disruptive and as distressing as the physical symptoms of the disease.

Depression and ALS

Identifying depression in people with ALS offers considerable hope. In contrast to many symptoms and complications of the disease, the episodes of depression are not permanent and often improve with treatment.

The management of depression must be tailored to the individual. Treatment is built on a combination of addressing precipitating problems, antidepressant medication, and therapeutic counseling.

No single antidepressant has been shown to be superior to another, and the choice of medication is often based on personal or family history of antidepressant use or the presence or absence of possible side effects. An example of this is the use of tricyclic antidepressants to reduce saliva production, which helps improve sleep and nerve pain. A serotonin uptake receptor inhibitor (SSRI) is sometimes avoided because of its potential to affect sexual desire, although this class of medication is frequently prescribed when anxiety is also a major symptom.

Once prescribed an antidepressant, it is important to take it every day at the same time to stabilize blood levels. Antidepressants are generally started at lower doses and then steadily increased. Many antidepressants can be safely increased every week or two. It is common for an antidepressant to take three to four weeks to take effect and six to eight weeks to become fully effective. It is important to give any antidepressant an adequate trial.

Although individuals may not respond to one antidepressant, adding or switching to another antidepressant can increase the chances of success. Many individuals who are successfully treated remain on treatment for at least six months.

Antidepressants should not be discontinued abruptly, but rather gradually over a two-to-four-week period. Patients should call their doctor before stopping any antidepressant medication.

Psychotherapy is also an integral part of the care and management of depression. Counseling attempts to address the way that a person sees themselves and to help patients get in control of their disease and their lives. Psychotherapy can be very effective, but it requires time and commitment. Often, a person will also start on an antidepressant medication in addition to seeing a therapist.

Behavioral/Cognitive Changes and ALS

Helping the individual manage personality changes can be very difficult for family members. The severity of the changes will affect their degree of flexibility and ability to understand information that is important in making medical and safety decisions.

It is recommended to consult with the medical, social, and nursing team involved in the care of the person, as well as seek out a psychiatrist or therapist. It is also important to involve other family members in the care and support of the patient, as their emotional support is essential.

Involuntary Emotional Expression Disorder and ALS

Treatment is available for IEED/PBA. Results from clinical trials support the use of Nuedexta, an FDA approved drug for emotional lability (Annals of Neurology 2010; 68:693-702). Although the syndrome is not a symptom of depression, antidepressants have also been used in the clinical setting and appear helpful.

Changes in Function or Balance

Changes in physical function or balance are most often a part of the ALS disease process.

A physical therapist (PT) is a health care professional with expertise in assessing and addressing movement and functional issues. At MGH, our physical therapists are experienced in addressing concerns that arise for patients with ALS and other neuromuscular diseases. This includes assistance with questions related to activity and exercise, challenges in walking or transferring, issues with balance, difficulties in daily functional activities (such as eating, dressing, bathing, etc.), muscle weakness or stiffness (spasticity), and discomfort or pain in joints.

Our physical therapists will meet with you during your multidisciplinary clinic visit to discuss exercise and movement and provide guidelines for stretching, fall prevention, balance, and functional activities. They can also assess the need for equipment that may help with mobility at home, such as an ankle brace. If you fall in-between clinic visits, please do not wait until the next clinic visit to tell your MGH neurologist or physical therapist. We will help you identify local resources to help improve your function and decrease your risk of future falls. The ALS clinic physical therapist can also connect you with a local PT and/or occupational therapy (OT), if needed.

The physical therapist visit in the MGH ALS Clinic is a consultation and is not meant to replace any ongoing physical therapy treatment, whether as an outpatient or at home. Rather, the clinic physical therapist is available as a resource to you and your home or outpatient PT to help you manage long-term changes in your function. If you are currently receiving PT or OT, either through outpatient visits or home sessions, our physical therapists are available to communicate with your local therapist. They can act as a resource, providing support and information regarding your plan of care.

Exercising with ALS

How does exercise help patients with ALS?

A common misunderstanding regarding patients with neuromuscular disease like ALS is that exercise is not helpful because the muscles won’t get stronger. Recommendations regarding exercise for those with neuromuscular disease have long been controversial. Some experts believe it’s impossible to overdo it, while others believe exercising to exhaustion can “burn out” remaining motor neurons before their time. Some physical therapy experts have raised questions about whether it’s wise to put too much demand on a gradually decreasing number of motor neurons, which have to do the work that would normally be done by many more such cells. In the past several years, some small research studies have shown that moderate exercise, both aerobic and light strengthening, may be beneficial. More research is needed (and is currently planned) to determine how much exercise is enough and how much is “too much.” Researchers are also investigating what type of exercise is safest and most tolerated in patients with ALS.

Based on what we currently know, we recommend exercising with discretion and stopping before reaching the point of exhaustion. We feel that appropriate exercise is very beneficial for patients with neuromuscular disease, for the following reasons:

• To prevent joint stiffness and maintain joint mobility, allowing for easier performance with self-care tasks.
• To keep muscle tissue that is not affected by neuromuscular disease healthy and strong. Healthy muscle tissue can better support the weak muscles.
• To maintain cardiopulmonary fitness as much as possible.
• To possibly delay muscle atrophy (decrease in muscle size).

Beneficial types of exercise (within individual activity tolerance):

• Active movement
• Low impact activity, such as stationary bicycle, pool exercises, walking, movement
• Light resistance, if using weights
• Assisted movement

Dos and don’ts for exercising with Neuromuscular Disease:

• Do take short rest periods throughout the day
• Do space activities over several hours
• Do save energy for necessary daily activities
• Don’t perform exercise to the point of severe fatigue
• Don’t “feel the burn” or push yourself until you are sore after exercise or the next day

You should not feel pain, sore muscles or exhaustion from any exercise performed the day before. If you do, you are exercising too hard.

An exercise program should not interfere with ability to complete daily tasks. A good rule of thumb is if you rest for 30 minutes to one hour after exercise, you should feel like you could do the exercise again (back to baseline level of energy).

Assistive Equipment and Mobility Devices

At some point in the disease process, most people with ALS find that various types of assistive devices, gadgets, or mobility equipment are needed. Equipment encompasses everything from a wide-handled knife to a key turner to a walker to a completely motorized wheelchair. The equipment you will find useful, and at what point you will need it, is very individualized. Our physical therapists are here to help you figure out if there is equipment that can help you with various activities, from balance and walking to daily tasks, including tying shoes, turning keys, getting dressed, and writing.

Assistive devices, or mobility equipment, are devices that help with getting around, climbing stairs, transfers, and other movement. Many people with ALS develop weakness in their ankles, and an ankle-foot orthosis (ankle brace) may help improve balance, gait and safety. For those with balance difficulties, often a cane or walker helps provide support and can greatly improve balance, walking ability and safety.

If walking is very difficult, a wheelchair can allow you to continue to do the things you enjoy without falling or becoming extremely fatigued. A folding transport wheelchair may be a good option if you can walk short distances but have difficulty with longer treks. A custom manual or power wheelchair is appropriate if walking is becoming too difficult in general. When obtaining a custom wheelchair, it’s important to be evaluated by an experienced physical therapist who is a specialist in wheelchairs. We can help you find a wheelchair clinic or specialist PT in your area.