How Is ALS Treated?
No cure has yet been found for ALS. However, there are treatments available that can help:
- control symptoms,
- prevent unnecessary complications, and
- make living with the disease easier.
You will get the best care from a multidisciplinary team of health care professionals. These teams can design an individualized treatment plan and provide special equipment. The team's goal is to keep people as mobile, comfortable, and independent as possible.
Find a Neurologist
Physicians can also prescribe medications to help manage symptoms of ALS. These symptoms include:
- muscle cramps,
- excess saliva and phlegm, and
- the pseudobulbar effect (involuntary or uncontrollable episodes of crying and/or laughing, or other emotional displays).
Drugs also are available to help you with pain, depression, sleep disturbances, and constipation.
Pharmacists can give advice on how to use your medications properly. They can also help monitor your prescriptions to avoid risks of drug interactions.
Riluzole & Edaravone
The U.S. Food and Drug Administration (FDA) has approved the drugs riluzole (Rilutek) and edaravone (Radicava) to treat ALS.
Riluzole is believed to reduce damage to motor neurons by decreasing levels of glutamate. (Glutamate transports messages between nerve cells and motor neurons.) Clinical trials in people with ALS showed that riluzole prolongs survival by a few months, particularly in the bulbar form of the disease, but does not reverse the damage already done to motor neurons.
Edaravone has been shown to slow the decline in clinical assessment of daily functioning in persons with ALS.
Physical Therapy & Speech Therapy
Physical therapy and special equipment can enhance an your independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise can strengthen the muscles that are not affected by ALS. They can improve your cardiovascular health and help you fight fatigue and depression.
These exercises can include:
- swimming, and
- stationary bicycling.
Range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles.
Physical therapists can recommend exercises that provide these benefits without overworking muscles. Occupational therapists can suggest devices—such as ramps, braces, walkers, and wheelchairs—that help you conserve energy and remain mobile.
People with ALS who have difficulty speaking may benefit from working with a speech therapist. Speech therapists can teach you adaptive strategies to speak louder and more clearly.
As ALS progresses, speech therapists can help you maintain your ability to communicate. They can recommend aids, such as computer-based speech synthesizers. These synthesizers use eye-tracking technology and can help you develop ways for responding to yes-or-no questions with your eyes or by other nonverbal means.
Some people with ALS may choose to use voice banking while they are still able to speak. Voice banking is a process of storing your own voice for future use in computer-based speech synthesizers.
These methods and devices help people communicate when they can no longer speak or produce vocal sounds.
Nutritional support is an important part of the care of people with ALS. Individuals with ALS will get weaker if they lose weight. Nutritionists can teach individuals and caregivers how to plan and prepare small meals throughout the day that provide enough calories, fiber, and fluid. They can also teach you how to avoid foods that are difficult to swallow.
You may begin using suction devices to remove excess fluids or saliva and prevent choking. When you can no longer get enough nourishment from eating, doctors may advise inserting a feeding tube into the stomach.
The use of a feeding tube also reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs.
As the muscles that help you breath start to weaken, you may experience shortness of breath during physical activity, have difficulty breathing at night, or difficulty breathing when lying down. Doctors may test an your breathing to determine when to recommend a treatment called noninvasive ventilation (NIV).
Noninvasive Ventilation (NIV)
NIV refers to breathing support through a mask over the nose and/or mouth. Initially, NIV may only be necessary at night. When your muscles are no longer able to maintain normal oxygen and carbon dioxide levels, however, you may use a NIV full-time.
NIV improves the quality of life and prolongs survival for many people with ALS.
Because the muscles that control breathing become weak, individuals with ALS may also have trouble generating a strong cough. There are several techniques to help you increase forceful coughing. This includes a mechanical cough assist devices and breath stacking.
In breath stacking, you takes a series of small breaths without exhaling until your lungs are full, briefly holds your breath, and then expels the air with a cough.
As the disease progresses and your muscles weaken further, you may consider a form of mechanical ventilation (respirators) in which a machine inflates and deflates your lungs.
Doctors may place a breathing tube through the mouth. They may surgically create a hole at the front of the neck and insert a tube leading to the windpipe (tracheostomy). The tube is connected to a respirator.
Individuals with ALS and their families often consider several factors when deciding whether and when to use ventilation support. These devices differ in their effect on a person’s quality of life and in cost.
Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS.
Choose to be fully informed about these considerations and the long-term effects of life without movement before you make decisions about ventilation support.
The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease. The NINDS is a component of the National Institutes of Health (NIH), the leading supporter of biomedical research in the world.
The goals of NINDS research on ALS are to:
- understand the cellular mechanisms involved in the development and progression of the disease,
- investigate the influence of genetics and other potential risk factors,
- identify biomarkers, and
- develop new and more effective treatments.
Scientists are seeking to understand the mechanisms that selectively trigger motor neurons to degenerate in ALS, and to find effective approaches to halt the processes leading to cell death. Using both animal models and cell culture systems, scientists are trying to determine how and why ALS-causing gene mutations lead to the destruction of neurons. These animal models include fruit flies, zebrafish, and rodents.
Initially, genetically modified animal models focused on mutations in the SOD1 gene but more recently, models have been developed for defects in the C9ORF72, TARDP, FUS, PFN1, TUBA4A, and UBQLN2 genes. Research in these models suggests that, depending on the gene mutation, motor neuron death is caused by a variety of cellular defects, including in the processing of RNA molecules and recycling of proteins, and structural impairments of motor neurons. Increasing evidence also suggests that various types of glial support cells and inflammation cells of the nervous system may play an important role in the disease.
In addition to animal models, scientists are also using innovative stem cells models to study ALS. Scientists have developed ways to take skin or blood cells from individuals with ALS and turn them into stem cells, which are capable of becoming any cell type in the body, including motor neurons and other cell types that may be involved in the disease. NINDS is supporting research on the development of stem cell lines for a number of neurodegenerative diseases, including ALS.
Familial Versus Sporadic ALS
Overall, the work in familial ALS is already leading to a greater understanding of the more common sporadic form of the disease. Because familial ALS and sporadic ALS show many of the same signs and symptoms, some researchers believe that some familial ALS genes may also be involved in sporadic ALS.
Clinical research studies supported by NINDS are looking into how ALS symptoms change over time in people with C9ORF72 mutations. Other NINDS-supported research studies are working to identify additional genes that may cause or put a person at risk for either familial or sporadic ALS.
Additionally, researchers are looking at the potential role of epigenetics in the development of ALS. Epigenetic changes can switch genes on and off, and thus can profoundly affect the human condition in both health and disease. These changes can occur in response to multiple factors, including external or environmental conditions and events. Although this research is still at a very exploratory stage, scientists hope that understanding epigenetics can offer new information about how ALS develops.
Biomarkers are biological measures that help to identify the presence or rate of progression of a disease or the effectiveness of a therapeutic intervention. Since ALS is difficult to diagnose, biomarkers could potentially help clinicians diagnose ALS earlier and faster.
Additionally, biomarkers are needed to help predict and accurately measure disease progression and enhance clinical studies aimed at developing more effective treatments. Biomarkers can be molecules derived from a bodily fluid (such as those in the blood and cerebrospinal fluid), an image of the brain or spinal cord, or a measure of the ability of a nerve or muscle to process electrical signals. The NINDS is supporting research on the development biomarkers for ALS.
New Treatment Options
Potential therapies for ALS are being investigated in a range of disease models. This work involves tests of drug-like compounds, gene therapy approaches, antibodies, and cell-based therapies. For example, NINDS-supported scientists are currently investigating whether lowering levels of the SOD1 enzyme in the brain and spinal cord of individuals with SOD1 gene mutations would slow the rate of disease progression.
Other NINDS scientists are studying the use of glial-restricted progenitor cells (which have the ability to develop into other support cells) to slow disease progression and improve respiratory function. Additionally, a number of exploratory treatments are being tested in people with ALS. Investigators are optimistic that these and other basic, translational, and clinical research studies will eventually lead to new and more effective treatments for ALS.
More information about ALS research supported by NINDS and other NIH Institutes and Centers can be found using NIH RePORTER ( projectreporter.nih.gov), a searchable database of current and past research projects supported by NIH and other federal agencies. RePORTER also includes links to publications and resources from these projects.*
*Courtesy: This content is provided and produced by National Institute of Neurological Disorders and Stroke, National Institutes of Health.
Why Choose University of Utah Health?
Our multidisciplinary team of specialists understand the challenges that come with an ALS diagnosis. We are here to help you take control of your health and support you every step of the way. The Motor Neuron Disease/ALS Clinic at University of Utah Health is recognized as a Certified Center of Excellence by the ALS Association.
Salt Lake City MDA/ALS Group Meeting
Date & Time: First Tuesday monthly, 6pm-8pm
Location: Sanderson Community Center
5709 South 1500 West Taylorsville, UT 84123
Please contact the MDA office in Boise for more information: 208-327-0107.