ELCOME! Along with my progression, this site has transformed into a three-fold purpose; first, as a source to keep friends and family updated with my condition Re: ALS . Second, as an outlet or journal, when I just want to get something off my chest, a chronological journal of sorts. Once I became more familiar with the level of ALS resources available online, I decided there wasn't a good, all-inclusive links page for anything and everything "ALS ", thus the Links page, which has become both a central focus and a source of frustration, as it can never really be complete.
For those unfamiliar, ALS literally translates as "no muscle nourishment". ALS is also known as Lou Gehrig's disease here in the USA, and MND in many other countries. Motor neuron is another name for the nerve cells that carry signals from the brain to the muscles. With the onset of ALS , the motor neurons die, and the muscles normally fed by these neurons begin to waste away.
ALS onset is normally classified as either "limb-onset", (lower motor neurons), presenting with signs of weakness in an arm/hand or leg/foot, or "bulbar", (upper motor neurons), affecting the voice, swallowing or breathing. Most PALS begin noticing a weakness in one hand, as I did, by finding it difficult to turn keys in stubborn door locks. Others may begin tripping or falling inexplicably, or experience "foot-drop". Those who are first affected by trouble speaking, swallowing or breathing, classified as bulbar, typically progress faster. The average survival rate for PALS is about 3 years, often less. PALS succumb to being unable to breathe without a respirator as the diaphragm becomes too weak for proper respiration.
ALS is hereditary in about 10% of cases. It isn't contagious, and there's currently only one FDA-approved drug to treat it, called ‘Riluzole’, and that only extends life expectancy by about three months. It costs around $18 per pill. ALS is considered an orphan disease, meaning pharmaceutical companies won't invest in research for new treatments because they won't make a profit as only 30,000 are affected in the USA.
Pretty grim prognosis, huh? Not so fast! We can't turn on the news these days without hearing of another pointless tragedy resulting in the loss of life or lives, yet with ALS, no matter the rate of progression, we usually have ample time to get our affairs in order and tell loved ones what they mean to us one last time. Even though I could very likely end up "locked-in", unable to move anything but my eyes, I know what to expect and have time to prepare.