ALS, short for amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects the nerve cells in the brain and spinal cord. It is also known as Lou Gehrig’s disease, named after the baseball player who was diagnosed with the condition in the 1930s. ALS gradually damages nerve cells that control muscle movement, causing muscle weakness, and ultimately leading to muscle atrophy and paralysis.
According to the ALS Association, approximately 5,000 people in the United States are diagnosed with ALS each year, and as many as 30,000 Americans may have the disease at any given time. Although ALS can affect anyone, it typically strikes people between the ages of 40 and 70, and men are slightly more likely to develop the disease than women.
The Symptomatology of ALS
One of the earliest signs of ALS is muscle weakness or stiffness, which may manifest in the hands, feet, arms, or legs. Other early symptoms include difficulty speaking, swallowing, or carrying out fine motor tasks, such as writing or buttoning a shirt. As the disease progresses, the muscles in other parts of the body, including the face, chest, and abdomen, begin to weaken. Eventually, people with ALS may experience difficulty breathing, and typically die from respiratory failure within three to five years from the onset of symptoms.
Muscle weakness
The muscle weakness associated with ALS is typically progressive and may affect muscles in the limbs, trunk, throat, and face. Some people may initially experience only mild weakness, while others may experience more severe weakness that affects their ability to perform everyday tasks. In some cases, muscle twitching or cramping may also be present.
Difficulty speaking and swallowing
As ALS progresses, muscle weakness in the tongue, throat, and mouth may make it difficult to speak and swallow. People with ALS may begin to slurr their words, have difficulty projecting their voice, and may cough or choke while eating or drinking.
The Faces of ALS: Who is Affected?
ALS affects people of all races and ethnicities, and its cause is not yet fully understood. There is currently no cure for the disease, and treatments are focused on alleviating symptoms and improving quality of life. Here are a few examples of individuals who have been affected by ALS:
- Stephen Hawking: The famed physicist was diagnosed with ALS at age 21, and was given just a few years to live. He went on to live with the disease for over 50 years, becoming one of the world’s most renowned scientists in the process.
- Pete Frates: The former college baseball player became an advocate for ALS research after being diagnosed with the disease in 2012 at age 27. He helped popularize the ALS Ice Bucket Challenge, which raised millions of dollars for research into the disease.
- Dwight Clark: The former San Francisco 49ers wide receiver was diagnosed with ALS in 2017, and passed away from the disease in 2018 at age 61.
- Pat Quinn: The co-founder of the ALS Ice Bucket Challenge was diagnosed with the disease in 2013 at age 30. He passed away from the disease in 2020 at age 37.
Diagnosis and Treatment Options for ALS
Currently, there is no single test that can definitively diagnose ALS, and the process of diagnosis is typically a multi-step process. Doctors may perform a physical exam, electrical tests to measure muscle function, and imaging tests, such as MRIs or CT scans, to rule out other possible causes of symptoms. In some cases, a biopsy of muscle or nerve tissue may also be performed to aid in diagnosis.
There is no cure for ALS, but a range of treatments are available that can help manage symptoms and improve quality of life. Some of the most commonly used treatments include:
Medications
Several medications may be prescribed to help manage symptoms of ALS, such as muscle cramping, spasticity, or drooling. These may include muscle relaxants, anti-spasticity drugs, or medications to help with speech or swallowing.
Physical therapy
Physical therapists can help people with ALS maintain strength and flexibility, and provide exercises and stretches that can improve mobility and ease muscle tension or stiffness.
Assistive devices
A range of assistive devices are available to help people with ALS maintain independence and carry out everyday tasks, such as eating, dressing, or communicating. These may include specialized beds, wheelchairs, communication devices, and adaptive utensils.
Research and Awareness Efforts
Although there is no cure for ALS, ongoing research efforts are focused on developing new treatments and ultimately finding a cure for the disease. The ALS Association and other organizations fund research into the genetic and environmental factors that contribute to ALS, with the goal of identifying new targets for treatment.
As awareness of ALS has increased, so too have efforts to raise funds for research and support for people with the disease. The ALS Ice Bucket Challenge, which went viral in the summer of 2014, involved people dumping buckets of ice water on their heads and challenging others to do the same or donate to ALS research. The campaign raised over $200 million worldwide and contributed to the development of new treatments and therapies for ALS.
FAQs About ALS
- What are the early warning signs of ALS? Early signs of the disease may include muscle weakness, stiffness or cramping, difficulty speaking or swallowing, or a change in handwriting or gait.
- What causes ALS? The precise cause of the disease is not yet fully understood, but it is thought to involve a combination of genetic and environmental factors.
- What is the life expectancy for someone with ALS? Most people with ALS live 2-5 years after the onset of symptoms, although some may live longer.
- Is there a cure for ALS? Currently, there is no cure for ALS, but a range of treatments are available to help manage symptoms and improve quality of life for people with the disease.
- Can ALS be prevented? There is currently no way to prevent ALS, but ongoing research may help identify new approaches for prevention and treatment.
References:
- ALS Association. ALS Facts and Figures. Retrieved from https://www.als.org/understanding-als/facts-and-figures.
- National Institute of Neurological Disorders and Stroke. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. Retrieved from https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet.
- Wijesekera, L. C., & Leigh, P. N. (2009). Amyotrophic lateral sclerosis. Orphanet journal of rare diseases, 4, 3. https://doi.org/10.1186/1750-1172-4-3