What is ALS/Lou Gehrig’s disease?
ALS is a motor neuron (nerve cell) disease also commonly known as Lou Gehrig's disease. How long you can live with ALS (Amyotrophic lateral sclerosis) may depend on the cause of the condition.
How long you can live with ALS (Amyotrophic lateral sclerosis) may depend on the cause of the condition. Most people with ALS that occurs seemingly at random will live for about three to five years after seeing the first symptoms. If you have familial ALS, though, you may only live for one or two years after symptoms appear.
Younger people tend to live longer following diagnosis.
ALS is a motor neuron (nerve cell) disease also commonly known as Lou Gehrig’s disease. The condition got its name from Lou Gehrig, the famous baseball player who retired in 1939 due to ALS. ALS is a condition that affects your muscles’ nerve cells. It causes the nerves that supply the upper and lower body muscles to stop working and die. The affected muscles eventually weaken and become paralyzed.
ALS is classified into two main types:
- Sporadic ALS. This type of ALS results from unknown causes and without any family history of the condition. It affects about 9 in 10 people with the condition.
- Familial ALS. This type of ALS is inherited from close family members. It affects only a small number of people in the U.S.
Most people find out that they have ALS when they are between 55 and 75 years old. ALS occurs more in men than in women. However, recent studies have shown that this difference has been decreasing.
Familial ALS affects males and females equally. Research by the CDC shows that five to ten percent of all ALS cases are familial.
Main symptoms of ALS
The symptoms of ALS mostly go unnoticed during the initial stages, but as it progresses, the symptoms of weakness and atrophy become more obvious. The first symptoms are usually seen in the upper and lower limbs. You may start experiencing difficulty doing simple things like writing, unbuttoning your shirt, or even turning a key in a lock. If the symptoms affect your legs, you may start tripping, stumbling, and walking/running in an awkward manner.
Some of the early symptoms of ALS include:
- Cramping of the muscles
- Difficulty chewing and swallowing
- Slurred or nasally speech
- Twitching leg, arm, shoulder, or tongue muscles
- Weakening of the arm, neck, leg, or diaphragm muscles
When the ALS symptoms begin by showing in the arms or legs, it’s called “limb onset.” If the symptoms start showing in your speech or swallowing, it’s known as “bulbar onset.” The muscle weakness and atrophy usually spread to other parts of the body as ALS progresses.
Symptoms that may occur after the condition progresses include:
- Body movement issues
- Dysphagia (difficulty swallowing)
- Dysarthria (difficulty forming words or speaking)
- Dyspnea (difficulty breathing)
- Rapid weight loss
While different people with ALS may get these symptoms at a varying sequence and rate, the condition usually progresses into paralysis. That means that you will eventually not be able to use your limbs to walk, lift things, or even get in and out of bed. Since ALS causes difficulty chewing and swallowing, you may become malnourished and lose weight pretty fast.
ALS also causes people to become more active mentally in processes like problem-solving, remembering, reasoning, and understanding. Because of that, you become more aware of what's happening to you, leading to mental issues like anxiety and depression. Some individuals also experience issues with language, decision-making, or even dementia (after some time passes). Eventually, people with ALS become unable to breathe on their own and will require a ventilator (a machine that helps with breathing).
Main causes of ALS
The exact causes of ALS are unknown. However, some studies suggest some links between ALS and factors like environmental exposure and genetic inheritance.
Risk factors for getting ALS
While the exact cause of ALS is unknown, some factors may put you at a higher risk of getting it. Some of these risk factors are:
- Age. As seen earlier, ALS symptoms are most common in individuals between the ages of 55 and 75. The condition, though, may also occur in people of any age.
- Gender. ALS is more likely to occur in males than in females. However, the difference reduces as people age.
- Ethnicity. ALS is seen more in people of Caucasian and non-Hispanic descent.
- Veterans. Being a military veteran has been found to increase a person’s chances of getting ALS due to exposure to things like environmental toxins.
How is ALS diagnosed?
Due to the similarities of ALS symptoms to other motor neuron diseases (like stroke and Parkinson’s disease), this condition may take some months to diagnose. When you get to your doctor, they will conduct some examinations and special tests to verify if you have ALS or any of the other motor neuron conditions.
Here are some of the tests:
- EMG (electromyogram)
- Spinal tap
- Blood test
- Urine analysis
- Muscle or nerve biopsy
- MRI (magnetic resonance imaging)
How is ALS treated?
There is no known cure for ALS. However, the treatment options available can help you manage the symptoms and make life easier. ALS treatment mainly involves supportive care. Your doctor may recommend:
- Medications. You may get medications like Riluzole (oral), which reduces damage to neurons, or Edaravone (intravenous), which slows the progression of ALS and improves daily physical functioning.
- Physical and occupational therapy. This may help improve your independence and safety with special equipment (like ramps and wheelchairs) and gentle, low-impact aerobic exercise (like walking and swimming).
- Communications support. This involves getting help from a speech therapist or devices like computer-based speech synthesizers and the brain-computer interface (BCI).
- Nutritional support. Nutritional support involves working with a nutritionist who teaches your caregiver how to plan and make the appropriate meals. If you can’t chew and swallow, your doctor may recommend using a feeding tube.
- Breathing support. Depending on your condition, your doctor may recommend using noninvasive ventilation (NIV) or mechanical ventilation (respirator). Noninvasive ventilation is breathing support given using a mask over the nose or mouth, while mechanical ventilation involves a machine that inflates and deflates the lungs using a tube inserted through the windpipe.