Amyotrophic lateral sclerosis is a neurodegenerative disease that causes the deterioration of nerves in the body. It often starts in a localized area such as the hands but then spreads to the rest of the body causing numerous challenges for the diagnosed individual.
The disease affects around 5,000 people each year in the USA and is eventually fatal in all cases as there is no cure. As the nerves break down, they are unable to send signals to the muscles. This means, for example, that individuals will find their mobility severely impaired as the disease progresses.
It also affects areas such as speech and swallowing and even the ability to breathe. There is no functional part of the human body that is not served by nerves. Without them, they are unable to function. This is a progressive disease which ends in death, more often than not from respiratory failure.
ALS, often referred to as Lou Gehrig’s Disease (particularly in the USA) is not curable, although we now know more about the condition than ever before thanks to continuous research efforts.
Certain drugs have been approved that can help to slow down the progression of ALS, but they are unable to reverse any damage already caused by the disease.
The average life expectancy for someone who has been diagnosed with ALS is between 3 and 5 years. More than half of people who develop the disease die within three years and only about 10% live more than ten years.
5% of people will live for 20 years or longer. One famous example of someone living a long time with ALS is the late professor Stephen Hawking.
The eminent scientist lived for more than 55 years with ALS. He was diagnosed in his early 20s and this may be a reason why he survived for so long. It’s believed that ALS can progress slower in cases whereby the patient was diagnosed at a relatively young age.
ALS is progressive in its nature. But the rate in which it progresses and how it affects the body will vary from individual to individual.
Some people may first notice issues with slurred speech. Others may initially experience muscle weakness. In other cases, issues with balance may be the first noticeable symptom.
How fast the disease develops after initial diagnosis varies considerably from person to person. There may be months where there is no further loss of function but the disease can often suddenly progress quickly.
Some people may see small improvements, but these rarely last beyond a period of 12 months.
Respiratory failure the most common cause of death amongst ALS patients. Essentially the nerves to the lungs deteriorate and the individual is unable to get enough oxygen into their system. Exhalation is also difficult in the later stages of the disease which means patients are also unable to effectively expel carbon dioxide out of their lungs.
Pneumonia is another big issue that can affect those diagnosed with ALS. Lung infections can be difficult to get rid of, especially if the patient is unable to cough up phlegm and remove fluid from their lungs. Pneumonia itself can weaken the muscles leading to respiratory failure.
Those with later stage ALS have difficulty swallowing and food and drink can end up passing into the windpipe by mistake causing an infection. Individuals may also suffer from malnutrition and dehydration which may contribute to eventual death.
Others with ALS can find that the nerves serving areas like the heart deteriorate first and this leads to heart failure.
There is generally a wide range of factors that actually lead to death following an ALS diagnosis. A lot depends on the progression of the disease and the level of care is being received.