ALS—amyotrophic lateral sclerosis—also called Lou Gehrig’s disease, is a neurological disease characterized by the degeneration of motor neurons in the brain and spinal cord, causing muscle weakness and eventual paralysis of voluntary muscles. Appropriate treatment can slow the progression of the disease, allowing patients to maintain independence for longer, but there is no way to stop or cure it. Here’s a look at how the stages of ALS develop.
Early Stages
The early stages of ALS can often pass before a diagnosis is even made. ALS is an extremely variable disease so it appears varyingly in different patients. For the most part, ALS appears in one of two ways. Limb-onset ALS, or "bottom up," is the more common form. Initial symptoms become more obvious in the muscles of the arms and legs. This might be a sudden weakness, trembling, twitching, or cramping of the muscles (also called "fasciculations"), making it difficult to accomplish everyday tasks such as picking up your toothbrush. Bulbar-onset ALS, or "top down," generally begins in the head or neck and occurs in about a quarter of ALS patients. Swallowing may become difficult or patients may experience a slurring of the speech.
Most often, symptoms either affect one area of the body more strongly, or they occur more globally and less severely. Other common early warning signs include a loss of balance, tripping, falling, and increased clumsiness. Fatigue, cramps, or twitching in the shoulder or tongue muscles and fighting to maintain solid posture are also common.
Middle Stages
As ALS progresses, more and more muscles begin to lose strength and mass. This is called "atrophy." As muscles atrophy, more and more activities become difficult. Driving becomes impossible. Eating, chewing, and swallowing grow more and more difficult. As muscle weakness spreads and intensifies, some muscles become completely paralyzed. Breathing, despite being an involuntary action, becomes compromised as the muscles of the diaphragm, chest, and abdomen grow weaker. This respiratory insufficiency may be especially pronounced in a prone position, requiring artificial ventilation during sleep. Additionally, the joints surrounding unused muscles may "contracture," or become stiff, sore, and misshapen.
Despite the many physical changes and challenges, most involuntary functions remain intact. For example, most ALS patients retain bladder and bowel control. Additionally, the mind remains conscious and active, despite difficulty communicating. Some patients, however, do develop pseudobulbar affect, a disorder characterized by uncontrollable bouts of laughter or tears inappropriate to the situation or unrelated to the patient’s feelings.
End Stages
As a progressive disease, ALS continues to become worse over time. More and more muscles become paralyzed. A feeding tube may be necessary to maintain hydration and prevent malnutrition. Continuous artificial ventilation may be necessary as the chest muscles atrophy. Respiratory insufficiency may cause fatigue, dizziness, and difficulty focusing or remembering things as the body craves oxygen. Speech may become virtually impossible as talking becomes completely incomprehensible with the paralyzation of those muscles.
The average life expectancy for ALS patients is about 3-5 years with the disease. However, because of the varying ways the disorder develops in different patients, this number can double or triple. For example, while Lou Gehrig lived only 2 years after being diagnosed, Stephen Hawking is still alive more than 50 years after being diagnosed with juvenile onset ALS. New medications can extend both length and quality of life. However, ALS-related fatalities occur most often from respiratory failure. It is possible to suffer from severe malnutrition or dehydration without feeding tubes, as well.
