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ALS

what exactly is

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.

ALS (amyotrophic lateral sclerosis) is also known as Charcot's Disease, MND (Motor Neuron Disease) and commonly known as “Lou Gehrig’s Disease” in the US after the famous New York Yankee Baseball player who was diagnosed on June 19th 1939, his 36th birthday. 

 

When neurologist Jean-Martin Charcot, MD, first peered into the tissues of his patients lost to ALS in 1865, he noticed clear signs of progressive neuronal damage that stretched from the brain to the brain stem (upper motor neurons) to the spinal cord (lower motor neurons) and atrophy of neighboring muscles.

 

Scientists now understand that this neurodegeneration is extremely complicated and occurs through several mechanisms.

A-myo-trophic comes from the Greek language. "A" means no. "Myo" refers to muscle, and "Trophic" means nourishment – "No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.

 

Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control. Examples of voluntary movements are making the effort to reach for a smart phone or step off a curb. These actions are controlled by the muscles in the arms and legs.

 

There are two different types of ALS, Sporadic and Familial.

  • Sporadic which is the most common form of the disease in the U.S., is 90 – 95 percent of all cases. It may affect anyone, anywhere.

  • Familial ALS (FALS) accounts for 5 to 10 percent of all cases in the U.S. Familial ALS means the disease is inherited. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease.

 

Living With ALS

The initial symptoms of ALS can be quite varied in different people. One person may have trouble grasping a pen or lifting a coffee cup, while another person may experience a change in vocal pitch when speaking. ALS is typically a disease that involves a gradual onset.  The rate at which ALS progresses can be quite variable from one person to another. Although the mean survival time with ALS is three to five years, many people live five, 10 or more years. Symptoms can begin in the muscles that control speech and swallowing or in the hands, arms, legs or feet. Not all people with ALS experience the same symptoms or the same sequences or patterns of progression. However, progressive muscle weakness and paralysis are universally experienced.

Gradual onset, painless, progressive muscle weakness is the most common initial symptom in ALS. Other early symptoms vary but can include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches, and/or uncontrollable periods of laughing or crying.  When the breathing muscles become affected, ultimately, people with the disease will need permanent ventilatory support to assist with breathing.

Since ALS attacks only motor neurons, the sense of sight, touch, hearing, taste and smell are not affected. For many people, muscles of the eyes and bladder are generally not affected.

Early signs of ALS
Most people with ALS first feel muscle cramps, spasms or twitching (fasciculations) in one of their arms or legs. Other signs include weakness in the hands and feet or loss of balance. This form of the disease is called limb-onset ALS.

About 25% of people with ALS first have trouble talking clearly - slurring words. This form of the disease is called bulbar-onset ALS.

Middle stages of ALS
As the disease spreads, many muscles weaken and start to stiffen. Range of motion exercises will likely be recommended to help keep muscles loose and prevent the formation of contractures and muscle pain.

People with ALS might tire more easily. Breathing may be affected. A BiPAP machine or a phrenic pacer might be suggested, particularly to help improve sleeping. A feeding tube might be suggested to help meet nutritional needs. Medications might be also recommended to control emotions (pseudobulbar affect) or reduce muscle spasms.

People with bulbar-onset often work with a speech therapist to keep talking longer. People with limb-onset ALS may rely on a cane, walker, or wheelchair due to difficulties walking and maintaining balance.

Late stages of ALS
As the disease progresses, muscles become paralyzed. Most people with ALS require a wheelchair to get around and may communicate through assistive devices using an eye-tracking device or a letter board.

People with late-stage ALS are often cared for in hospices or at home. Some people with ALS choose invasive ventilation to help keep them breathing. Most people lose the battle with ALS due to respiratory failure.

ALS is a complex multi-system disease. A growing number of ALS clinics are deploying multidisciplinary teams to care for people with ALS to meet their physical, emotional, and nutritional needs. These teams include physical, respiratory, speech and occupational therapists to help people with ALS breathe easier, keep moving, and stay connected. Palliative care specialists support people with ALS and their caregivers.

Breathing devices may also improve quality of life and extend survival. Non-invasive ventilatorssuch as a BiPAP machine help people with ALS breathe better, sleep better and boost survival by about a year according to some estimates. Phrenic pacers (NeuRX DPS) might also help people sleep better and extend survival according to clinical observations about 16 months after NIV use is initiated. Clinical trials are ongoing to further evaluate the NeuRX DPS and identify which people with ALS might benefit from them.

Certain forms of exercise are also becoming routine. Range of motion (stretching) is general practice for people with ALS to prevent muscle pain and the formation of contractures. Emerging aerobic workouts might improve quality of life and help reduce functional decline. A clinical trial evaluating the benefits of certain forms of exercises including stationary cycling and weight training remains ongoing.