Symptom management is a major component of the care of ALS. Identifying and addressing problematic symptoms can minimize their effects on a person’s function, health, and quality of life.
There are many resources and technologies that can allow people with ALS to live their lives more fully. Below we have written some guidelines for caring for the symptoms associated with ALS. Please use these resources as a starting point for conversations with your clinician.
Early Respiratory Issues
ALS causes weakness in many muscles in the body, including the muscles involved in breathing and swallowing. Weakness in these muscles can cause respiratory problems, making breathing more difficult. Respiratory problems are among the most serious symptoms of ALS and should not be ignored. The ALS Multidisciplinary Clinic offers pulmonary care services to help treat respiratory issues. If you begin to have difficulty breathing or swallowing, tell your physician right away. You should also inform your doctor right away if you develop a fever or if you having a change in the color of your phlegm, as these can be early signs of respiratory infection.
Early Signs of Respiratory Problems
Shortness of breath may be the first respiratory symptom that you experience. You may notice shortness of breath when you are engaged in vigorous physical activities like running, exercising, walking long distances, or climbing stairs. You may also have difficulty breathing at night which can interfere with your sleep and cause fatigue or morning headaches. You may find yourself waking frequently throughout the night. Difficulty breathing at night occurs when your chest muscles relax too much to assist with breathing during the dreaming phase of sleep.
Problems with swallowing can also interfere with breathing. When the swallowing muscles are weakened, food and saliva are more easily inhaled (aspirated) into the windpipe which leads to choking. Aspiration of food or saliva can also introduce bacteria into the lungs, increasing the risk of respiratory infection and pneumonia. Fever can be a sign of respiratory infection and should be reported immediately so your doctor can prescribe a course of antibiotics.
Treating Early Respiratory Symptoms
If you are experiencing any difficulties with breathing or swallowing, inform your physician. A breathing test, called a vital capacity (VC), will be used to measure the amount of air you can blow out. Most ALS clinics routinely administer this test to monitor any changes that may occur.
Another common breathing test performed in the clinic is the maximum inspiratory pressure (MIP), which measures the strength of your breathing muscles. Additional tests that may be performed include oxygen saturation levels of your red blood cells (called pulse oximetry) using a small probe placed on your finger throughout the night at home, an overnight sleep study in a sleep lab, or a blood sample to measure your oxygen and carbon dioxide levels.
The results of these breathing tests are used to decide when to recommend a treatment called non-invasive positive pressure ventilation (NIV). This therapy uses a ventilator which is called either BiPAP or VPAP, depending on the manufacturer. Research has shown that using NIV when the VC is at or below 50% of normal improves the quality of life and prolongs survival for many people with ALS (Bourke S, et al. 2006). Newer studies (Carrat`u P, et al.,2009) would support introducing it earlier. The question when to the best start using NIV, continues to be studied.
NIV is often applied by putting a mask over your nose or over your nose and mouth. There are, however, a variety of appliances that can be used and our goal is to find the one that works best for you. NIV assists weakened breathing muscles by using forced air pressure to help fill your lungs with air. NIV may be uncomfortable at first and it may take some time to get used to wearing the mask. Your respiratory therapist may initially recommend using it while relaxing during the day to help with this adjustment. A heated humidifier with the NIV prevents dryness of the mouth, nose, and throat which are commonly reported side effects. The goal of using NIV is to use it a minimum of four hours a night. As you get used to the device, most go on to use it throughout the entire night.
As the respiratory muscles weaken, coughing becomes more difficult making it difficult to expel phlegm from your lungs. If you have difficulty coughing effectively, a cough-assist machine may be helpful. This device, sometimes called a mechanical insufflator-exsufflator (MIE), works by producing a deep breath of air followed by negative pressure (suction) which helps you cough up phlegm. Using a cough-assist machine can help prevent lung infections by keeping the lungs clear of mucus
If you develop a fever, notice a change in the character of your sputum, or have an acute change in your breathing, contact your physician immediately. These can be symptoms of respiratory infection. If you have a respiratory infection, the best success to clear it quickly is to begin a course of antibiotics immediately.
Late Respiratory Issues
As ALS progresses, respiratory problems often worsen. Shortness of breath may occur more often, including while at rest during the day. When this happens, it can be helpful to start using NIV during the day as well as at night. Your respiratory therapist can help you with alternative appliances for daytime use, such as a mouthpiece.
When it becomes necessary to use NIV for most of the day, it is time to consider a tracheostomy. This involves a surgical procedure to create an opening into your windpipe (trachea) through which a plastic tube is inserted. This tube allows air to pass directly into your trachea. Unlike the mask used for NIV which can be taken off, this tube stays in place at all times. A ventilator can be attached to the tube for breathing assistance as needed. This tube can also be used to clear phlegm from your lungs and to prevent food or saliva from going into your lungs by accident.
A ventilator with a tracheostomy may add years to your life as it can fully support your breathing. It will not, however, reverse the progression of your disease or prevent further weakening of the muscles throughout your body. Some people with a tracheostomy are able to speak, but this varies among individuals. Similarly, some people are able to eat and swallow with a tracheostomy while others cannot. For people who cannot eat or take in all of their nutrition by mouth, a feeding tube is inserted into the stomach for nutrition.
Whether or not to have a tracheostomy and to use a ventilator is an individual decision. You can discuss any questions or concerns you may have with your physician.
Choosing to use a ventilator, whether it is with NIV or with a tracheostomy, does not mean that you must continue to use breathing assistance indefinitely. As your ALS progresses, you can decide to discontinue ventilation and allow nature to take its course. Ventilation can be discontinued at home with hospice care to ensure that you are comfortable and do not experience any breathing distress. Sedation is provided before the ventilator is removed and is continued until breathing stops naturally. Usually, family and friends are present so that they can say goodbye and witness their loved one’s passing. This can be a very peaceful and loving experience.
It is always a good idea for people with ALS, especially those on ventilation, to appoint a healthcare proxy who can make medical decisions on their behalf if they become unable to make or express decisions themselves. You should make your wishes about important aspects of treatment and end-of-life decisions known to your healthcare proxy. It is important to keep an open, continuing dialogue with your healthcare proxy as your thoughts and feelings may change over time.
Speech and Swallowing
Problems communicating with others and difficulties with eating and drinking are frustrating symptoms that people with ALS experience. As the disease progresses, it weakens the muscles of the mouth and throat by causing atrophy (a decrease in the size of the muscle) or spasticity (decrease in control caused by “tightening” of the muscles). These changes affect various aspects of the speech (dysarthria) (dĭs-är'thrē-ah) and swallowing (dysphagia) (dĭs-fā'jah).
What is Dysarthria?
Dysarthria refers to changes in speech that result from the muscle weakening. 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 person’s 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 in ALS can vary greatly from person to person. Eventually, however, everyone with ALS experiences some degree of dysarthria.
A speech-language pathologist (SLP) is someone who is specifically trained to assess, treat, and manage the speech and swallowing difficulties that people with ALS experience. SLPs are an integral part of the ALS Multidisciplinary clinic at MGH and are available to answer your questions. As part of the clinic team, the SLP develops approaches to deal with problems in speech and swallowing that are related to ALS or cognitive changes (reasoning and processing information) that can result from ALS. The SLP can also recommend devices to assist with communication and maintenance of proper nutrition and provide ongoing education to you and your family about current and anticipated needs. A primary goal of the SLP in ALS care is to help you maintain your ability to communicate successfully, thereby improving or maintaining your quality of life.
When should I see a Speech-Language Pathologist?
If you begin to have problems with speaking or swallowing, an early meeting with an SLP may help. For your benefit, schedule an SLP consultation when you first begin to notice symptoms or changes or ask to meet with our SLP at your next clinic visit.
How Does an SLP Make a Speech Assessment?
The SLP will ask you to perform some simple, non-invasive tests that enable them to 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.
How Do I Manage Changes in Speech?
The first step in managing changes in your speech is to have an assessment by an SLP. One of the SLP’s primary roles is to help people with ALS maintain the ability to communicate successfully. This may include teaching ways to compensate for difficulties with speaking, helping with the decision to use an augmentative communication device and providing training for augmentative communication devices.
Techniques to Improve Speech
People with ALS who only have minor problems with speaking can learn techniques that will make his or her speech easier to understand. For example, there are techniques for speaking in a group setting. Over-articulation or exaggerated pronunciation can help speech be better understood. As dysarthria progresses, people with ALS need to learn new techniques to compensate for further changes in speech.
An augmentative communication device is a means of supplementing or replacing speech. These devices enable people who are either unable to speak or unable to be easily understood, to communicate 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.
If your speech is normal, or only slightly impaired, you may choose to “bank” your voice so that it can be used by an electronic augmentative device in the future. 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 type of augmentative communication system involves the typing of letters, words, or phrases into a computer which then uses your own voice (as opposed to a computer-generated voice) to speak the words and phrases out loud.
Problems with Swallowing
Since the same muscles used for speaking are used for eating and drinking, almost everyone with ALS will eventually also experience some problems with swallowing. Swallowing involves a complex network of muscles and nerves in the mouth and throat that work together to move food from the mouth down into the stomach while also preventing us from inhaling our food and choking.
People with ALS experience changes in swallowing as a result of weakness in the lips, tongue, soft palate, pharynx (the muscles in the throat that squeeze foods through to the esophagus or food pipe), and the larynx (the upper part of the airway that needs to close quickly to prevent choking). Even when all of these muscles are working well, a person with ALS can still have trouble swallowing if their respiratory muscles are affected and they have trouble coordinating breathing and swallowing.
The types of changes in swallowing that occur are fairly predictable, however, the rate at which they occur is not. For example, an individual with ALS may experience an initial decline in his or her ability to swallow effectively and then may experience a period where the symptoms remain stable.
Managing Problems with Swallowing
The first step in managing changes in swallowing is to have an SLP perform an assessment. Together with other members of the ALS multidisciplinary team, the SLP can help you manage your overall nutrition and swallowing. Even if there are no significant changes in swallowing, it is important to be aware of, and maintain, proper nutrition early in the disease.
An SLP can help you maintain your nutrition by teaching you techniques which will improve your ability to swallow. An SLP can also advise your family members and other caregivers on ways to help you eat and drink.
How is Swallowing Evaluated?
The SLP will observe and evaluate your ability to eat and drink. By watching you drink a glass of water and eat either a cookie or dish of pudding your ability to hold food and liquid in your mouth, to chew, and to swallow can be evaluated. The SLP will watch for obvious difficulties, such as coughing and choking, but also less obvious indications of difficulty. These may include the inability to efficiently manage the food in your mouth, leakage from your mouth, and other muscle weakness. Based on what is observed, they may recommend a change in food or beverage consistency as well as other recommendations such as changing head position during swallowing or changing liquids and solids. This type of screening is usually done in the clinic.
If your health team is concerned about weight loss or breathing problems, or if there are changes in speech that are indicative of early swallowing problems, a full swallowing assessment may be performed. This assessment is called a modified barium swallow (MBS). The MBS can offer a more detailed understanding of the problem than the SLP can provide through simple observation. The MBS test also helps the person with ALS to better understand and visualize the extent of the swallowing problem.
The MBS test is performed by an SLP and a radiologist. This test is painless and requires no advance preparation (i.e. you can eat and drink before the test.) You will be asked to swallow small sips of liquid and have small bites of solid food of different consistencies. Each of these liquids and solids contains a small quantity of barium to make it visible on the x-ray screen. A video is made up of all the x-rays from the MBS so the SLP and radiologist can observe what happens. Immediately after the test, the SLP will review the test with you and provide recommendations to help with your swallowing.
Techniques to Help with Swallowing
The SLP will make recommendations that will help you eat and drink safely. These may include preferable food consistencies, changes in the position of your head while eating and drinking, and maneuvers that may help keep your airway closed during swallowing.
Why Does a Person with ALS Need a Feeding Tube?
A feeding tube is an essential element of care when ALS has progressed to the point where it interferes with a person’s ability to swallow food and fluids. A feeding tube can reduce the stress and exhaustion of trying to swallow when swallowing becomes too difficult. Initially, it may supplement eating, or if swallowing is causing choking, may be used to replace eating. It can reduce the risks of choking and pneumonia by allowing another method of getting sufficient calories. A feeding tube can stabilize weight or help to recover lost weight. When a person has a feeding tube, it permits them to receive most of their nutrition through the tube, allowing them to eat foods that are enjoyable and safe to swallow, in amounts that feel comfortable for them.
How Important is it to Keep My Weight Stable?
New research is exploring this question as our understanding of the relationship between good nutrition and improved survival increases. It is clear that a loss of body mass is associated with faster progression of the disease (Jawaid A, et al. Amyotrophic Lateral Sclerosis, 2010: Early Online, 1-7).
How Does a Feeding Tube Work?
A feeding tube is a small tube that goes from outside the skin of the abdomen directly into the stomach. Most people who have a feeding tube take a balanced liquid nutritional supplement that comes pre-mixed in cans. Each 240 ml can is identified by a number indicating the concentration that when multiplied by 240 determines the number of calories in the can. For example, a 1.5 concentration had 360 calories. When a large syringe without a plunger is connected to the feeding tube, each can is poured directly into the syringe and by gravity it goes directly into the stomach. This is commonly referred to as a bolus feeding. Some individuals have a slow feeding delivered over a longer period of time with a simple pump connected to the feeding tube that controls the flow. This is frequently set up to occur when the person is asleep at night. The supplement is slowly dripped into the stomach overnight.
Where is a Feeding Tube Placed?
The feeding tube is surgically inserted into the stomach through the abdomen beneath the left side of the ribcage and held in place internally in the stomach. The external portion of the tube, which is usually 6 inches long, has a cap or a three-way valve (stopcock) on the end to prevent leakage.
What Else, Besides Supplement, Can Go in the Tube?
Nutritional supplements do not provide all the fluid that the body needs. Water can also be given through the tube. Water is usually added after the nutritional supplement to provide additional hydration and to flush any residual supplement from the tube.
Medications can also be given through the feeding tube. Pills can be crushed and mixed with warm water and poured into the tube. Some medications can be ordered in liquid form. However, some medications, particularly long-acting medications, must be taken whole and cannot be crushed. 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?
Insurance generally pays for the nutritional supplement when more than half of the calories a person receives for the day is given through the feeding tube.
Will Others Notice?
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.
When Is the Time to Begin Using a Feeding Tube?
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 percent of normal. Someone who already has begun having swallowing, choking and other eating concerns should consider having one placed earlier, before starting to lose weight.
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 procedure for inserting them is different.
The term PEG is often used interchangeably with the terms “feeding tube” and “gastrostomy tube,” but not all feeding tubes are actually PEGs.
Inserting a PEG tube
To insert a PEG tube, another tube with a light at its end (endoscopy tube) is inserted down the esophagus (the “food pipe”) into the stomach. The light at the end of this tube shines through the stomach and skin, directing the surgeon to the spot where the PEG tube should be inserted. The surgeon makes a small incision through the skin and the wall of the stomach and inserts the PEG tube through it. The tube with the light is removed from the stomach once the PEG tube has been inserted.
Inserting a RIG tube
A RIG tube is inserted with the aid of fluoroscopy, a type of x-ray that allows the interventional radiologist to view real-time moving images of the patient’s internal organs. Fluoroscopy enables the radiologist to find the right spot to insert the RIG tube through the skin into the stomach. To help identify the intestines (so they can be avoided), barium is given the night before. During the procedure a small tube is inserted into the nose and directed into the stomach to inflate the stomach; this tube is removed once the procedure is completed. During the procedure, the stomach is positioned near the surface of the skin. This “gastropexy” is held in place with sutures called T – tacs. About 14 days after the RIG tube has been inserted, the T-tacs are removed.
Inserting a feeding tube: What are the risks?
Inserting a feeding tube is a surgical procedure that requires anesthesia. For most people with ALS, the primary risk during the procedure is respiratory distress. This is due to any respiratory limitation that a person may have from their ALS. Anesthesia affects a person’s regulation of breathing. Lying flat can also make breathing more difficult for ALS patients.
During the feeding tube insertion, the anesthesiologist monitors the person’s breathing. Some individuals with few respiratory limitations may require only light sedation and no assistance with breathing. Others whose breathing is more impaired might need the assist of non-invasive ventilation (NIV) during the procedure. Individuals with more distressed breathing will usually receive only a local anesthesia and not be sedated. Each person is individually assessed by the anesthesiologist and a decision is made with the patient about the type of anesthesia that will be given.
Should a person experience respiratory distress, a tube may be temporarily inserted into the airway through the mouth or nose to help them breath during the procedure. Placing a temporary tube in the airway is called intubation. Intubation allows the airway to stay open and permits a ventilator to assist with breathing while the person is sedated. When the procedure is completed and the anesthesia has worn off, the intubation tube is removed. To prevent the possibility of needing intubation, you will be encouraged to consider having a feeding tube placed before developing significant respiratory concerns.
Other risks that are possible, but more rarely seen include bleeding, perforation of the intestine and infection.
Is One Tube Safer to Place?
At MGH, our ALS physicians recommend a RIG tube for most of our patients. A RIG tube is significantly smaller than a PEG tube. A smaller tube requires less anesthesia to inserted. This helps to minimize the risk of respiratory complications that can result from anesthesia. Additionally, the endoscopic tube that is used in a PEG procedure is relatively large and may be more difficult for patients during the procedure, especially those persons who are already having swallowing and breathing issues.
Care and Maintenance of a Feeding Tube
Feeding tubes are generally easy to care for. For the first two or three days after the feeding tube has been inserted, the tube may be uncomfortable. Until the incision heals, this discomfort is usually well controlled with medication.
The site where the tube enters the body is cleaned with soap and water. All feeding tubes experience some seepage; small amounts of liquid from the stomach travel along the outside of the tube to the surface of the skin, which can produce a crusty area on the skin around the site where the tube enters the body. This can be removed with soap and water.
Sometimes gastric juices from the stomach can cause irritation or even break down the skin around the feeding tube. Applying an antacid paste to the irritated skin can help prevent further damage and allow the skin to heal. Antacid paste is made by letting a small amount of liquid antacid sit for awhile so that it settles, then pouring off the liquid that rises to the top.
The tube itself is kept clean and clear of clogs by flushing water through it. Water is used so that any residual supplement will be rinsed from the tube. If a clog does develop in the tube, often cola inserted into the tube and left to sit will dissolve it.
Although the feeding tube is secured internally, if pulled hard enough, it can be pulled out. Reasonable care needs to be taken to secure the tube when it is not in use to avoid accidentally pulling it out. Curling it up and taping it to the body will help keep it from being accidentally pulled. If the tube is accidentally pulled out, it should be replaced as soon as possible. Waiting too long will result in the tract from the skin to the stomach sealing off. This can occur in a matter of hours.
Occasionally, as the body tries to heal the tract from the skin to the stomach, it produces extra tissue that extends outside the skin. This tissue may be tender and uncomfortable and can bleed easily. However, it can be removed during an office visit through a simple procedure, cauterizing it with silver nitrate. There is some mild and temporary discomfort associated with this.
Other Types of Feeding Tubes
What is a Mic-Key or Button?
Mic-Key is the brand name of a type of feeding tube that is often called a “button” because its opening lies flush with the skin. A cap is used to access the tube.
Many of our ALS patients ask about the Mic-Key because it is less obvious than the RIG or PEG tubes, which extend outside the body. Because a RIG tube requires less anesthesia and poses less of a respiratory risk than inserting a PEG tube, our MGH ALS physicians consider this advantage of the RIG tube to outweigh the improved cosmetics of the Mic-Key.
What is a Gastrojejunal Tube (GJ tube)?
A gastrojejunal tube, or GJ tube, is a feeding tube that is inserted into the stomach and continues through the small intestine. The tube is inserted like either the RIG or PEG, but it is directed further down the gastrointestinal tract beyond the stomach and into the jejunum (beginning of the small intestine). This tube is used when there is concern that the person may vomit when nutritional supplement goes directly into the stomach. Vomiting poses the additional risk that the person may inhale vomit into the lungs This can result in choking and/or pneumonia. Because the GJ tube bypasses the stomach, this risk is reduced. Nutrients are supplied through a GJ tube more slowly than through a RIG or PEG and a feeding pump may be required.
What is a Nasogastric Tube (NG tube)?
A nasogastric tube (NG tube) is a tube that is inserted through the nose into the stomach. NG tubes are typically intended to remain in place only for a short period (day to weeks). However, NG tubes that are specifically intended as feeding tubes are smaller and made of more supple material. Nasogastric feeding tubes can be used for several weeks at a time.
For a person with ALS, a NG tube may be temporarily used for feeding until a RIG or PEG tube can be arranged to prevent weight loss or dehydration if the individual is becoming malnourished.
Anxiety and Depression
As with any serious disease, ALS has an immense impact on a person’s life as well as their family members or caregivers.
Depression and anxiety are common psychological reactions to the disease, although there are other emotion-related challenges that can be directly related to the disease itself.
How Common Is Depression in People With ALS?
Clinical depression is not as prevalent or severe as might be expected (ALS Journal vol 8: 4, 2007. 243-54). Researchers have found, for example, that depression is found less frequently in individuals with ALS than in another neurological disease, multiple sclerosis (MS) (Shifner and Babigian, 13). However, depression, when present, has the ability to significantly affect the life of any one individual and is more commonly seen in situations involving loss or threat of loss.
In general terms, individuals experience depressive symptoms mainly as a reaction following the communication of an ALS diagnosis. There is a higher tendency for depression to appear at the beginning of disease than towards the end. A high number of people with ALS tend have a more positive attitude toward life, even as their disease advances.
Researchers have also found that the presence of 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 (Neurology 2005; 65:62-67).
Another finding is that depression in individuals with ALS is not associated with disease severity, although a worsening of their physical functioning is clearly related to levels of their psychological distress.
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, family members may also be affected by depression. This may or may not include the patient. In a study of depression in ALS subjects and their caregivers, depression increased much more for caregivers than for patients. Around 10 percent of caregivers had depression at the beginning of the study, whereas 9 months later, it almost doubled.
It is not unusual that the person who is depressed is the last to realize it. Symptoms may come on slowly and almost appear to be normal after awhile.
People who are depressed usually exhibit a very low mood that is part of all aspects of their life. There is the inability to enjoy activities that formerly were enjoyed. Depressed persons may be preoccupied with, or constantly have thoughts and feelings of worthlessness, inappropriate guilt or regret, helplessness, hopelessness and self-hatred. Other symptoms include poor concentration and memory, withdrawal from social situations and activities, reduced sex drive and thoughts of death or suicide.
The inability to sleep normally is common and often a person wakes very early and is unable to get back to sleep. Oversleeping may occur, but is less common. Appetite often decreases, with resulting weight loss, although increased appetite and weight gain occasionally occur. The person may report multiple physical symptoms such as fatigue, headaches or digestive problems. However, very 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 the ongoing difficult events in their lives.
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 frequently respond to treatment.
The management of depression must be tailored for each individual patient. Treatment is built on a combination of addressing precipitating problems, antidepressant medication and therapeutic counseling.
Antidepressants are often part of the therapy. No one antidepressant has been shown to be superior than another, and any one medication is often chosen on a personal or family history of use of antidepressants or on the presence or absence of possible side effects. An example of this is the use of tricyclic antidepressants to help reduce saliva production, to help a person sleep better at night or to help with controlling nerve pain. A serotonin uptake receptor inhibitor (SSRI) can sometimes be avoided because of the 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 each day and to take it at the same time to keep blood levels of the medications stable. Antidepressants are generally started at lower doses and then steadily increased. Many antidepressants can safely be increased every week or two. It is common for an antidepressant to take three to four weeks to begin working and longer to become fully effective (six to eight weeks); however, some individuals respond much earlier. It is important to give any antidepressant an adequate trial.
Although individuals may not respond to any 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. Most antidepressants, when they are discontinued, should be gradually tapered over a two to four week period. Patients should call their doctor if they feel a need to discontinue taking the medication, 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 and Cognitive Changes in ALS
ALS may be accompanied by changes in the person’s behavior and overall cognitive functioning or their ability to process information. These features may occur to different degrees in any one individual, from barely noticeable to seriously affecting their ability to stay safe and manage their affairs.
The disease causes these symptoms because of its effect on the frontal-temporal part of the brain. Like other aspects of the disease, symptoms may not be present or noticeable earlier in the disease process, though they may become more apparent in time. It is not well understood how often these symptoms occur in ALS, but as the disease is better understood, it is being more recognized than previously reported.
There do not appear to be any particular risk factors for why it might develop in some patients, but not others. Psychological testing is frequently used to determine the presence of cognitive changes. These tests try to determine if there are changes in personality towards a more rigid thinking process, slow psychological processes (like in decision making and answering questions), difficulties understanding new information, problem solving, dividing one’s attention (the inability to do two things at the same time) and emotional changes. It is important to determine if one or some of the above changes are due to the disease process or with a person’s attempt to cope in a stressful situation.
Helping the individual manage when there are personality changes can be very difficult for family members. The degree of the changes will, to a greater or lesser degree, affect their degree of flexibility and ability to understand information that is important in making medical decisions and decisions centered around safety.
Calling upon the medical, social services and nursing staff involved in the care of the person, as well as relying upon the psychological supports of a psychiatrist or therapist, may prove helpful. Most importantly, involving other family members in the care and support of the patient should be considered. The emotional support of the immediate family members involved in providing care is essential.
Inappropriate Crying and Laughing
Involuntary emotional expression disorder (IEED) is a syndrome in which a person’s affect and expressions of emotions occurs independently from the real emotions the person is feeling. This syndrome in ALS is often referred to as a Pseudobulbar Affect (PBA).
A typical presentation of IEED is the occurrence of uncontrollable and unpredictable crying and/or laughing, and the lack of an apparent cause to trigger such responses. These episodes may alternate between tears and laughing quite rapidly, and they can start and stop quite abruptly. The syndrome may sometimes be under-recognized or 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.
Treatment is available for 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.
Movement & Exercise
A physical therapist (PT) is a healthcare professional who is trained to evaluate and treat problems related to movement and function. In our clinic, the physical therapists have experience working with persons with ALS and other neuromuscular diseases, and are familiar with many of the specific issues they may have. These issues may include questions about activity and exercise, difficulty walking or with transfers, balance problems, difficulty with daily functional activities (eating, dressing, bathing, etc), weakness or stiffness (spasticity) in muscles and tightness or pain in joints.
Our physical therapists will meet with you during your multidisciplinary clinic visit to discuss any questions you have surrounding exercise and movement, including stretching and general exercise guidelines, fall prevention, balance, and functional activities. They can also assess the need for different types of equipment that may help you with mobility and/or equipment for your home. The ALS clinic physical therapist can also help identify the need for local PT and/or Occupational Therapy (OT) and help you find a local PT or OT.
The physical therapist visit in the ALS Clinic is a consultation and is not meant to replace any ongoing physical therapy treatment, either as an outpatient or in your home. Rather, the physical therapist in the ALS clinic is available as a resource to you, and to your home or outpatient PT, to help you manage changes in your function on a long-term basis. If you are seeing a PT or OT (outpatient or home visits) locally, our physical therapists are available to talk with your local therapist and act as a resource for them regarding your plan of care.
When should I see a Physical Therapist?
If you have questions regarding exercise guidelines, stretching, or other activity recommendations, it may be helpful for you to meet with a physical therapist during your clinic visit to discuss these questions. If you are experiencing difficulty with balance, walking, endurance, transfers, or functional activities, it is likely a good time to seek the input of a physical therapist, either locally or at your next clinic visit. If you have fallen or stumble sometimes, seeing a physical therapist for consultation may help to identify the cause of these incidents, and suggest a plan going forward to minimize your risk of falls. This plan may include ongoing PT treatment (locally, near your home, or in your home), a device (such as an ankle brace or other assist) to help you clear your feet when walking, or other techniques or treatment options. If you fall in-between clinic visits, please DO NOT WAIT until the next clinic visit to tell your neurologist and/or the ALS clinic physical therapist about your fall. We recommend that you call us to let us know right away, so we can help you problem-solve and identify local resources, such as PT and OT, to help maximize your function and decrease your risk of further falls.
Why is exercise important for the patient with Neuromuscular Disease?
A common perception regarding patients with neuromuscular disease is that since the muscles won't get stronger, it will not help to exercise, and 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.” Based on what we currently know, it seems sensible to exercise with discretion and stop before reaching the point of exhaustion. Researchers are also investigating what type of exercise is safest and most tolerated in patients with ALS. 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) because of disease.
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 (movement in which another person assists)
Dos and Don’ts in Exercise for people with Neuromuscular Disease:
- Do take short rest periods throughout the day and 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
Note: 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 1 hour after exercise, you should feel like you could do the exercise again (back to baseline level of energy).
At some point in the disease process, most people with ALS find that various types of assistive devices, “gadgets” or mobility equipment may be of use to them. “Equipment,” in this broad sense, encompasses everything from a wide-handled knife to a key turner to a walker to a completely motorized wheelchair. What 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 the things you are having difficulty with, from balance and walking to tasks related to your daily activities.
Assistive Devices, or “mobility equipment,” includes devices to help with walking and getting around, stairs, transfers, and other movement. Many people with ALS develop weakness in their ankles, and an Ankle-Foot Orthosis (AFO or ankle brace) may be helpful to improve balance, gait and safety. For those with balance difficulties, often a cane or walker helps to give them a little 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 danger of falling or extreme fatigue from long-distance walking. A folding “transport” wheelchair may be a good option for you 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 and you need another way to get around. It’s important, when obtaining a custom wheelchair, 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.
There are a variety of devices that may help with daily activities as well. Depending on what you are having difficulty doing, our physical therapists can help you find a device that can help make activities easier, including tying shoes (elastic shoelaces or different types of shoes), turning keys (key turners), getting dressed (shoe horns, sock aides, button and zipper aides), and writing (pens, keyboards, etc).
Changes in function or balance
Changes in physical function or balance are most often a part of the ALS disease process. Although we can not change the disease itself, our physical therapists can work with you to maximize your balance and safety, minimize your risk of falls, and help with different aspects of daily function. We can also work with your home or outpatient Physical or Occupational Therapist regarding treatment plans.