Amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that mainly involve the nerve cells  responsible for controlling voluntary muscle movement. It attacks nerve cells called neurons in the brain and spinal cord. These neurons transmit messages from your brain and spinal cord to your voluntary muscles - the ones you can control, like in your arms and legs. At first, this causes mild muscle problems. Some people notice

• Trouble walking or running

• Trouble writing

• Speech problems

Eventually, you lose your strength and cannot move. When muscles in your chest fail, you cannot breathe. A breathing machine can help, but most people with ALS die from respiratory failure. 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. There is no cure. Medicines can relieve symptoms and, sometimes, prolong survival.

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Treatment

Although ALS has no cure, it can be managed to some extent with medication and therapy. In 1995, the U.S. Food and Drug Administration approved a medication called riluzole (Rilutek) as the first drug treatment for ALS. The drug prolongs life by several months, and it can extend the time before someone needs ventilation support. Riluzole doesn’t reverse the damage already done, and patients must be closely watched for possible side effects.

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Edaravone can slow the clinical decline in daily functioning  of people with ALS. The FDA has also approved the NeuRx Diaphragm Pacing System, which uses implanted electrodes and a battery pack to cause the diaphragm (breathing muscle) to contract, to help certain individuals who have ALS before the onset of severe respiratory failure. Other treatments are designed to relieve symptoms and improve the quality of life for people with ALS.  Drugs are available to help individuals with spasticity, pain, panic attacks, and depression.  Physical therapy, occupational therapy, and rehabilitation may help to prevent joint immobility and slow muscle weakness and atrophy.  Individuals with ALS may eventually consider forms of mechanical ventilation (respirators).  

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Other treatments for ALS aim to ease symptoms and improve quality of life. For example, an FDA-approved drug combination of dextromethorphan and quinidine (Nuedexta) helps manage intense emotional changes, such as uncontrollable laughing or crying, that are often seen with ALS.

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Because the mind remains relatively intact, people with ALS may be keenly aware of their continued loss of function. They may become anxious and depressed. Health care professionals can tailor plans for therapy and equipment to keep people as mobile and comfortable as possible.

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Suggestions for the Patients

Sleep is an important part of your daily routine—you spend about one-third of your time doing it.  Quality sleep – and getting enough of it at the right times -- is as essential to survival as food and water.  Without sleep you can’t form or maintain the pathways in your brain that let you learn and create new memories, and it’s harder to concentrate and respond quickly. Sleep is important to a number of brain functions, including how nerve cells (neurons) communicate with each other.  In fact, your brain and body stay remarkably active while you sleep.  Recent findings suggest that sleep plays a housekeeping role that removes toxins in your brain that build up while you are awake. 

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Physical therapy
Physical therapy and special equipment can enhance an individual’s independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help people fight fatigue and depression. 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 individuals conserve energy and remain mobile.

Speech therapy
People with ALS who have difficulty speaking may benefit from working with a speech therapist, who can teach adaptive strategies to speak louder and more clearly. As ALS progresses, speech therapists can help people maintain the ability to communicate. They can recommend aids such as computer-based speech synthesizers that use eye-tracking technology and can help people develop ways for responding to yes-or-no questions with their eyes or by other nonverbal means.

Some people with ALS may choose to use voice banking while they are still able to speak as a process of storing their 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
Nutritional support is an important part of the care of people with ALS. It has been shown that 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 and how to avoid foods that are difficult to swallow. People may begin using suction devices to remove excess fluids or saliva and prevent choking. When individuals 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.

Breathing support
As the muscles responsible for breathing start to weaken, people may experience shortness of breath during physical activity and difficulty breathing at night or when lying down. Doctors may test an individual’s breathing to determine when to recommend a treatment called noninvasive ventilation (NIV). NIV refers to breathing support that is usually delivered through a mask over the nose and/or mouth. Initially, NIV may only be necessary at night. When muscles are no longer able to maintain normal oxygen and carbon dioxide levels, NIV may be used 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 people increase forceful coughing, including mechanical cough assist devices and breath stacking. In breath stacking, a person takes a series of small breaths without exhaling until the lungs are full, briefly holds the breath, and then expels the air with a cough.

As the disease progresses and muscles weaken further, individuals may consider forms of mechanical ventilation (respirators) in which a machine inflates and deflates the lungs. Doctors may place a breathing tube through the mouth or 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. People may choose to be fully informed about these considerations and the long-term effects of life without movement before they make decisions about ventilation support.

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