How Is Amyotrophic Lateral Sclerosis Treated?

Amyotrophic lateral sclerosis (ALS) is a disease of the nervous system that gets worse over time. It is also known as Lou Gehrig's disease, after a baseball player who was famous in the 1920s and '30s and had to retire due to ALS.^1,2

ALS is a disorder of the motor neurons. Motor neurons are nerves. They run from your brain to your spinal cord and then to muscles throughout your body. ALS destroys motor neurons in the brain and spinal cord. This leads to:^1,3

• Loss of muscle control
• Muscle weakness
• Wasting of the muscles

There is no known cause in most people with ALS, but some cases are inherited.^1,3

ALS often begins with muscle twitching, stiffness, weakness in the arms or legs, or slurred speech. Eventually, all muscles are affected, leaving the person unable to move, speak, eat, or breathe. Most people die 3 to 5 years after symptoms first appear. But up to 1 in 10 lives more than 10 years.^1,3

Types of treatments

ALS is a complicated disease that requires a large team of specialists. A typical care team will include a neurologist, dietitian, social worker, pharmacist, nursing care managers, and respiratory, physical, occupational, and speech therapists. Outcomes can be better if the care team specializes in ALS.⁴

There is no cure for ALS. There is also no way to reverse the damage done to the nerves. However, there are treatments to slow progression of the disease, prevent complications, and improve quality of life.¹

FDA-approved drugs

The US Food and Drug Administration (FDA) has approved 2 drugs to treat ALS:^3,5,6

• Riluzole (Rilutek®, Tiglutik®, Exservan™) reduces damage to nerves. It also increased lifespan by a few months in clinical trials. It is the only treatment known to improve survival.
• Edaravone (Radicava®, Radicava ORS®) has been shown to slow the decline in daily functioning. It seems to help the most when treatment begins in the early stages of ALS.

People with ALS also need other treatments, including medicines, to manage:^3,4

• Muscle cramps and stiffness
• Extra saliva and phlegm
• Uncontrolled episodes of crying, laughing, or other emotional eruptions
• Pain
• Depression
• Fatigue
• Sleep problems
• Constipation

Respiratory therapy

People with ALS eventually begin to have shortness of breath and problems breathing at night or when lying down. This happens as the breathing muscles become weak. When this occurs, doctors prescribe a BiPAP (bilevel positive airway pressure) device.^3,7

BiPAP devices provide noninvasive ventilation. They deliver air through a mask placed over the nose, or nose and mouth. The device may be used only at night at first and gradually full time.^3,7

As the muscles that control swallowing become weak, suction devices are needed to remove excess fluids and saliva.^3,4

A machine that inflates and deflates the lungs may be recommended. This may be called a ventilator, a respirator, or mechanical ventilation. Being on a ventilator may prolong life, but people with ALS often decline this option.^3,7

Physical, occupational, and speech therapy

Physical therapy, exercise, and stretching can help a person with ALS build strength in unaffected muscles and improve stiffness and range of motion.³

Occupational therapy can help the person find devices that help them conserve energy and remain mobile. Some of the devices most commonly used include ramps, braces, walkers, and wheelchairs.³

Problems with speaking and swallowing are common as ALS begins to affect muscles in the face, mouth, and neck. Speech therapy may help the person speak louder and more clearly. Writing, alphabet boards, and devices that work through eye or hand controls may replace verbal communication.^3,4

Some people are able to use a system called a brain-computer interface. These machines use the person's brain activity to communicate or control equipment like a wheelchair.³

Nutrition

Weight loss and nutrition are important concerns in people with ALS. Problems swallowing, poor appetite, and an inability to feed themselves as arms weaken are common. A nutritionist can help caregivers create smaller, more frequent meals that are easy to swallow and high in nutrition.^3,4

When eating is no longer possible, a feeding tube (g-tube) may be recommended. This helps prevent choking and reduces the chances of developing pneumonia due to fluid getting into the lungs.^3,4

Other supportive treatment

As muscle weakness gets worse, people with ALS may use a variety of supportive treatments, such as assistive devices. These devices help make daily activities and movement possible and include:^3,4,7

• Canes, ankle/foot orthotics, crutches, walking frames, and wheelchairs
• Higher toilet seats
• Bathtub and bed lifts
• Pressure-relieving mattress
• Hospital bed
• Headrests
• Special eating utensils, grips, and holder

Palliative care and hospice care

Palliative care and hospice care are often confused. Palliative care is medical care that keeps the person as comfortable and functional as possible during treatment. Hospice care focuses on keeping the person comfortable when all treatments have stopped.⁵

Palliative care for ALS adds an extra layer of support as ALS progresses and symptoms get worse. This type of care includes emotional, spiritual, and practical support to the patient and their caregivers and family. This may lead to improved quality of life and fewer and less serious hospitalizations.⁵

Hospice care may begin at the end of life, usually when death is expected in 6 months or less. End-of-life planning should begin earlier than hospice and be revisited at least twice a year.

There are many new ALS treatments being studied. Talk to your doctor about clinical trials that may be available.