I describe ALS as a slow motion death sentence. Here’s how it works:
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease… is a disease that causes the death of neurons controlling voluntary muscles. Some also use the term motor neuron disease for a group of conditions of which ALS is the most common. ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscles decreasing in size.
After at least 24-months since probable onset and the first breathing problems, and over a year since the original diagnosis, I have all of that. Some people with ALS live for a decade or more; some only a few years.
But it’s more complicated than that.
No cure for ALS is known. The goal of treatment is to improve symptoms.
Since there is no cure and those with ALS live only a few years, it can be described as a slow motion death sentence with relatively rapid debilitation.
But it’s more complicated than that.
About 10% of all cases of ALS begin before age 45 (“young-onset” ALS), and about 1% of all cases begin before age 25 (juvenile ALS). People who develop young-onset ALS are more likely to be male, less likely to have bulbar onset of symptoms, and more likely to have a slower progression of disease.
I’m old already.
Late onset (after age 65) is associated with a more rapid functional decline and shorter survival.
Uh oh.
Respiratory-onset ALS is a rare variant that accounts for about 3% of all cases of ALS, in which the initial symptoms are difficulty breathing (dyspnea) with exertion, at rest, or while lying down (orthopnea). [Traditional] symptoms tend to be mild or absent at the beginning.
That is where my symptoms began. Respiratory onset. Other muscles are far less affected.
It is more common in males.
It sucks to be a male sometimes.
Respiratory-onset ALS has the worst prognosis of any ALS variant; in a population-based study, those with respiratory-onset had a median survival of 1.4 years and 0% survival at 10 years.
Uh oh.
I’m at the median already.