Amyotrophic lateral sclerosis (ALS)
Posted on: June 30, 2019
At first, ALS causes mild muscle problems. Some people may notice:
Trouble walking or runningTrouble writingSpeech problems
As the disease progresses, the person loses strength and eventually cannot move. Intellectual abilities remain intact although emotions may be effected. In the later stages, amyotrophic lateral sclerosis (ALS) affects the nerves/muscles that control breathing. At end-stage, ALS is considered a hospice-appropriate disease. Most patients eventually pass away from respiratory depression.
The Centers for Disease Control and Prevention (CDC) estimate that between 14,500 and 15,000 people in the United States (U.S.) had ALS in 2016, with around 5,000 people receiving a diagnosis annually. Worldwide, it is thought to affect between 2 and 5 people in every 100,000.
Most people with ALS will live for 3 to 5 years after first experiencing symptoms, but around 10 percent of patients will live for another 10 years or longer.
The disease usually strikes between age 40 and 60. More men than women get it. No one knows what causes ALS. It can run in families, but usually it strikes at random. At present, there is no cure. Medications may help relieve symptoms.
MORE INFORMATION FROM THE ALS ASSOCIATION:
ALS is a neurodegenerative disease that usually attacks both upper and lower motor neurons and causes degeneration throughout the brain and spinal cord. A common first symptom is a painless weakness in a hand, foot, arm or leg, which occurs in more than half of all cases. Other early symptoms include difficulty with speech, swallowing or walking.
As the disease progresses, it robs patients of the use of their hands, arms and legs, as well as their ability to speak, swallow and, ultimately, breathe on their own. Familial ALS (genetically handed down between generations) represents between five to ten percent of all cases. The rest happen spontaneously and mysteriously, making seemingly random attacks on previously healthy adults. The simple fact is, ALS can strike anyone, anytime.
Even though options for treating ALS have emerged in recent years, physicians still have very limited choices. Studies suggest that patients’ length of survival and quality of life are enhanced by night-time breathing assistance early in the course of the disease and by aggressive application of alternate feeding options to assure good nutrition once swallowing becomes difficult.
Recent years have brought a wealth of new scientific understanding regarding the physiology of this disease. There are currently four drugs approved by the U.S. FDA to treat ALS (Riluzole, Nuedexta, Radicava, and Tiglutik). Studies all over the world, many funded by The ALS Association, are ongoing to develop more treatments and a cure for ALS.
Recently Diagnosed With ALS?
I Have ALS. Now What?
A short set of essays written by someone with ALS for others who have just been diagnosed with the disease. It tries to anticipate the most common issues faced by persons living with ALS in the first weeks and months following the diagnosis, and point you to helpful sources of support.
Recommended Steps after Diagnosis
Recognize that ALS is bigger than any one person or family. You will need help. There are agencies and organizations out there whose specific purpose is to help. Find them and take advantage of what they can offer.ALS is difficult to diagnose. If you were not diagnosed by a neurologist specializing in neuromuscular diseases, request a referral for a confirmation of the diagnosis. Request an appointment with your local ALSA Certified Center or ALS Clinic. Connect with your local ALS Association (NC 877-568-4347) Chapter and/or The National ALS Association (800-782-4747) and request information about services and publications available.Contact your medical insurance provider and request that you be assigned a case manager to be your primary point of contact. Contact an attorney who specializes in elder care law and make an appointment to discuss legal and financial planning recommendations. In the United States: If you served in the military, it is crucial that you contact the Department of Veteran Affairs (800-827-1000) regarding eligibility for health, vocational rehabilitation and disability programs and services. Substantial benefits are now available for those with an ALS diagnosis.
If you are employed, contact your state Vocational Rehabilitation Division and request information about available services.
HOW DOES ALS PROGRESS?
Many people want to know how quickly ALS progresses. This is another difficult aspect of this disease as no two individuals present exactly the same. Some people have severe involvement in one area before it spreads, while others experience rapid progression throughout their body. In some people, the disease progresses very slowly. In general, people with ALS live about three to five years after they experience the first sign of weakness. This is a generalization, which is based on averages. People with ALS can live anywhere from a few months to decades depending upon disease changes and the types of medical care and assistive devices they choose. ALS is different in each person and will run an individual course.
A person with ALS may develop severe weakness (medical term: paralysis) of all muscles in the arms and legs and the muscles of breathing, swallowing, and speaking. However, every person is different. Some people have severe weakness of one area, but little in others (e.g., unable to swallow but still able to walk and drive), but other individuals may demonstrate a similar severity of involvement of different areas. Some patients have the disease that progresses very slowly, while others have changes that happen more quickly. Clinicians have difficulty predicting how a person’s ALS will progress at the time the diagnosis is made; therefore, they continually see the individual and assess symptoms with each visit. Your ALS physician cannot fully predict the time course of the disease and cannot entirely predict in what order the body will be affected. Although no ALS healthcare provider can know for sure, it is generally true that individuals who experience a more rapid onset will have a more rapid course of the disease, while individuals with a slow onset will likely experience slow progression.
In general, people with face and tongue (medical term: bulbar) involvement have a shorter life span due to the problems with loss of function in this area. It is also generally true that the spread occurs from one body part to the next. So for instance, if the disease starts in the legs it would be expected that the arms would be affected next. Symptoms do not begin all at once or suddenly. Many people fear that they will wake up paralyzed, but symptoms do not change overnight. Some family members may notice an abrupt change, but this is more often due to the person attempting to compensate (counteract) the gradually worsening weakness until the muscle can no longer support an important function in daily life (for instance climbing stairs or getting up from a chair). It is important to take notice of any changes. Discuss them early with your ALS specialists because they may be able to help treat the symptoms and prepare you for upcoming changes. Eventually, you will likely need help with most movements, including getting out of bed, moving onto a chair, getting dressed, showering, eating, and toileting. This is due to the loss of the motor neurons causing paralysis. People lose muscle mass and as a result, lose body weight. Weight loss is also due in part to increased need for calories, decreased ability to eat adequately because of swallowing difficulties, as well as arm and hand weakness, which impacts the ability to feed oneself.
Some of the symptoms you may experience include difficulty with:
■ Going up and down stairs
■ Getting up from a chair
■ Playing sports
■ Moving onto and off the commode (toilet)
■ Walking (especially on uneven surfaces) and occasional falls
■ Lifting items or holding a pen
■ Holding a glass
■ Typing on a keyboard or using eating utensils
■ Swallowing liquids or foods
■ Sleep (poor sleep can result from stress, inability to get into a comfortable position, or from difficulty breathing)
■ Breathing (shortness of breath) with activity or when lying down
■ Speech (being poorly understood because of slurring words or speaking slowly)
■ Decision-making or planning
■ Crying or laughing too much (medical term: pseudobulbar affect) The symptoms listed above are discussed in more detail in the other resource guides published by The ALS Association. (See Living with ALS Resource Guides below.)
Pain in ALS
By Ileana Howard, MD
Does ALS cause pain?
The answer is yes, although in most cases it does so indirectly. From what we know at this time, the disease process in ALS only affects the nerve cells controlling strength (motor neurons) in the brain, spinal cord, and peripheral nerves. Fortunately, these nerves don’t send pain signals back up to the brain. Unfortunately, there are several reasons that the weakness associated with ALS can cause pain:
Weak muscles can cause extra strain on muscles and joints, which often causes pain. This is most common in the neck, shoulders, and back.Immobility or the inability to change positions or roll in bed can cause abnormal skin pressure and result in pain or wounds.Medical treatments, like feeding tube or tracheostomy tube placement, can cause discomfort. In most cases, this is temporary following a tube placement.Constipation is common in ALS and can be really uncomfortable.Muscle cramps, tightness, or spasms.
The good news is…
That most of this pain is treatable, and there are a wealth of options at hand to minimize pain and maximize quality of life and function for persons with ALS.
What are some of the strategies to treat or manage pain symptoms in ALS?
Stretching or range of motion. Loose muscles are less likely to cramp or spasm. Stretching is important to maintain range of motion and prevent permanent tightening (contractures) of the muscles or joints (such as frozen shoulder).Non-medication treatments. Vibration, electrical stimulation, and heat can be used to help relax tight, spastic, or painful muscles. Taping or bracing can help stabilize weak muscles causing pain. These tools are often available over-the-counter or through a therapist or by prescription. The effect usually lasts several minutes, but can be more effective if used with stretching.Medications. Over-the-counter pain medications are usually safe to take in ALS. When these don’t work, specific medications are available for cramps, spasms, and pain. Please work with your ALS doctor to discuss options.Injections. When pain is focused in a specific joint or part of the body (e.g. shoulder or spine) sometimes injections can be helpful. Rehabilitation medicine doctors (physiatrists) often perform these types of injections, although other providers may offer them as well.Wheelchair positioning. When muscles aren’t working to support the joints and body, seating and positioning in a wheelchair can often be customized to allow pressure relief and support to vulnerable areas.A routine bowel regimen. An ounce of prevention is really worth a pound of cure when it comes to the gut. Daily bowel programs, consisting of fiber or bulking medication along with bowel-stimulating medications if needed, can help ensure regularity despite immobility in ALS. Occasionally, tube feeding formulas need to be changed if they cause stomach upset, constipation or diarrhea. Keep in mind that some pain medications, especially narcotics, can cause worsening or even severe constipation unless they are accompanied by routine bowel medications.Acupuncture. This treatment has been around for thousands of years, and has been found to be helpful for muscle spasms in other neurologic diseases- therefore, it might be helpful to control some pain symptoms in ALS.Palliative care/hospice. Palliative care is a growing specialty of medicine focused on improving quality of life for persons with severe illness. Hospice providers can also help manage pain and other troublesome symptoms at home for persons with advanced stages of ALS who don’t wish to have life-prolonging measures like mechanical ventilation.
Remember that your ALS care team is your partner in this journey, and is available and willing to help. Communicate often and honestly with your team, and inform them when treatments are not working!
The ALS Association thanks and acknowledges Ileana Howard, MD, for sharing her time and expertise on this factsheet.
Mobility and ALS
Freedom of movement – walking, going up and down stairs, getting off the floor, completing daily activities, exercising—these are just a few of the activities that many of us take for granted and are able to perform without limitation. For an individual with ALS, these often become difficult or even impossible to do. As weakness develops in the legs, trunk muscles or diaphragm, active movement can become limited. Working with a physical therapist to learn range of motion and stretching is essential to maintain muscle length, joint mobility, and decrease spasticity. It is recommended that a family member, friend, or caregiver be instructed on how to safely perform these exercises as part of a home exercise program. As the need arises, the physical therapist will also provide education and training in the proper fit and use of an appropriate assistive device to maximize safety and independence.
The use of a cane or walker may become necessary to provide assistance with walking due to changes in balance and/or strength. Weakness in the muscles of the neck or trunk cause postural changes resulting in forward trunk lean and potentially increasing the risk of falls. A walker can provide assistance in these situations to support the body and decrease the effort of maintaining upright posture. Orthotic devices, such as an ankle foot orthosis (AFO), can aide in foot clearance when lower leg weakness prevents safe foot clearance and foot drop is present. A certified orthotist will fabricate a custom brace to meet the specific needs of the individual.
As walking becomes more limited due to increased weakness, decline in balance, or changes in respiratory status, a transport chair or scooter can be used for mobility, especially when going longer distances or in the community. When use of an assistive device is no longer safe or appropriate, a physical therapist or occupational therapist can provide recommendations and assessment for a custom power wheelchair. An Assistive Technology Professional (ATP) works with the therapist to complete the evaluation, perform a home assessment and order the necessary components for a customized power wheelchair designed specifically for the individual. The power wheelchair becomes a vital tool in promoting greater independence and functional mobility.
When transfers become more difficult, using equipment such as a Get-U-Up lift or Hoyer lift can provide a safe means for a caregiver to assist with mobility. Both devices can be used for surface-to-surface transfers, while the Hoyer can be used to lift a person off the floor. Training for either device can be performed by a therapist, ATP, or home health care agency. A hospital bed is often recommended for positioning due to changes in respiratory status or to aide in bed mobility. Comfort and prevention of secondary complications is of utmost importance.
Ongoing assessments by a physical and/or occupational therapist are essential to maintain functional independence to the fullest extent possible. This is achieved by monitoring changes in physical function, providing recommendations for a home program, educating about the prevention of secondary complications, and assisting in obtaining the proper adaptive equipment to promote energy conservation and improved quality of life. These activities should be part of every person’s care plan, both short term and longer term.
Written by Jennifer McKune, MPT
The ALS Association thanks and acknowledges Jennifer McKune, MPT, for sharing her time and expertise on this factsheet.
Managing Excessive Saliva
Patients who experience swallowing problems often notice that they seem to salivate more. The fact is, they may not be salivating more, but the saliva is pooling in the mouth because of an inability to swallow it. Excessive saliva can be one of the most frustrating symptoms of ALS to manage. It can also be life threatening, since it frequently causes choking, especially at mealtimes when saliva secretion is increased and chance of aspiration is greatest.
Early in the course of the disease, excess salivation can be controlled by simply being aware of the problem and making a conscious effort to swallow the saliva or wipe it away with tissues. To some degree, these problems can be managed by controlling the intake of very sweet or very sour foods that cause hypersecretion. It may also be helpful to increase or decrease (as the case may be) foods of high water or fluid content.
With progression of the disease, however, patients may find that excess saliva has become a nuisance and an embarrassment, and needs to be controlled by other means. One helpful measure is to have a suction machine available in the home. Modern technology has provided portable, battery-operated suction machines for those “on the go”.
Your physician may prescribe certain medications to control saliva. The following is a list of prescription medications that have been used successfully in controlling saliva. If a single dose does not work, a combination may be tried. Most common side effects of these drugs are mild sedation, dizziness, difficulty in urination, and tachycardia. They are in the order most often used.
Glycopyrrolate (Robinul) 1-2 mg. every four hours. Robinul is also available in injectable form – 0.1 mg. every four hours or 3-4 times/day. Maximum dose – 0.2 mg 4 times/day.Propantheline (Pro-Banthine) 15 mg. one half hour before meals three times a day.Amitriptyline (Elavil) 10 mg. three times a day or 10-25 mg. at bedtime. Amitriptyline is also available in injectable form – 2 to 5 mg. IM to start. Elavil is also used as an antidepressant.Nortriptyline HCL (Pamelor) 10-25 mg. at bedtime.Scopolamine (Transderm Scop) transdermal patch 1.5 mg. programmed to deliver medication over 72 hour period. Reported by patients to reduce saliva by 75-80%. Caution – may cause glaucoma.
In addition, the use of Imipramine (Tofranil) 50-150 mg. at bedtime to reduce anxiety and promote sleep, has also been noted to reduce choking on saliva at night.
THIS INFORMATION SHOULD BE DISCUSSED WITH THE PATIENT’S PHYSICIAN.
The ALS Association does not promote, endorse or encourage the use of any of these medications. We are simply providing information.
Living with ALS Resource Guides
Reading all 11 resource guides at one time can be overwhelming to persons with ALS and their families. We highly recommend that you only access the information that pertains to your present circumstances.
Once you have reviewed one or more of The ALS Association’s Living with ALS Resource Guides, you may have questions and you may want to know where to go for even more information. Feel free to contact The ALS Association via our toll-free number at (800) 782-4747 or e-mail firstname.lastname@example.org. With the vast network of ALS Association chapters, certified centers and clinics across the nation, there is likely an ALS Association affiliate in your region to help you. Use our locator tool to find an ALS Association affiliate. (Visit www.alsa.org)
What is ALS? An Introductory Resource Guide for Living with ALS:
This resource guide provides an overview of ALS, what it is, and how it affects your body. It provides information on what kind of resources are available to help you deal with ALS more effectively.
This resource guide addresses the psychological, emotional, and social issues that you must face when your life is affected by ALS. It provides information on how to cope with the many lifestyle changes and adjustments that occur when you live with ALS.
This resource guide addresses how thinking and behavior may be affected by ALS and how these changes can impact disease course, symptom management, and decision making.
This resource guide reviews areas where careful planning and decision making will be required and will provide you with resources to help you and your family plan for the future.
This resource guide provides strategies and helpful hints to better navigate health insurance and benefits. While understanding insurance and benefits may feel overwhelming, the guidelines outlined here should help simplify the process for you.
This resource guide discusses a variety of symptoms that may affect you when you have ALS. As the disease progresses, various functions may become affected and it is helpful to understand potential changes so that you know what to expect and how to manage these new changes and symptoms.
This resource guide covers the range of mobility issues that occur with ALS. It discusses exercises to maximize your mobility, as well as how to adapt your home and activities of daily living to help you function more effectively.
This resource guide will help you understand how swallowing is affected by ALS and what you can do to maintain nutrition for energy and strength and to keep your airway open.
This resource guide covers how speech can be affected by ALS and explores a variety of techniques, technologies, and devices available for improving communication. By maintaining communication with others, you continue to make a significant difference in their lives, while retaining control of your own.
This resource guide explains how breathing is affected by ALS. Specifically, it will teach you the basics of how the lungs function, the changes that will occur, and how to prepare for the decisions that will need to be made when the lungs need maximal assistance.
This resource guide examines thoughts and feelings about dying and end of life. Approaching end of life is difficult and support is critical to help sort out feelings, expectations, and plans. By talking to friends, family, professionals, and planning and communicating your wishes, you can help prepare for the best possible end-of-life phase.
When Do I know that Amyotrophic Lateral Sclerosis (ALS) is Hospice-Appropriate?
The patient meets at least one of the following (1 or 2)
1. Severely impaired breathing capacity with all of the following findings:
Dyspnea at restVital capacity less then 30%Requirement for supplemental oxygen at restThe patient declines artificial ventilation
OR 2. Rapid disease progression with either a or b below: Rapid disease progression as evidenced by all of the following in the preceding twelve (12) months:
Progression from independent ambulation to wheelchair or bed-bound statusProgression from normal to barely intelligible or unintelligible speechProgression from normal to pureed dietProgression from independence in most or all Activities of Daily Living (ADLs) to needing major assistance by caretaker in all ADLs
a. Severe nutritional impairment demonstrated by all of the following in the preceding twelve (12) months:
Oral intake of nutrients and fluids insufficient to sustain lifeContinuing weight lossDehydration or hypovolemiaAbsence of artificial feeding methods
OR b. Life-threatening complications demonstrated by one or more of the following in the preceding twelve (12) months:
Recurrent aspiration pneumonia (with or without tube feeding)Upper urinary tract infection (Pyelonephritis)SepsisRecurrent fever and antibiotic therapyStage 3 or 4 decubitus ulcer(s)
In the absence of one or more of the above finding, rapid decline or co-morbidities may also support eligibility for hospice care.