Lou Gerhig's Disease

Our nervous system is made up of billions of cells called neutrons. They can be damaged or destroyed by accidents or disorders. As we grow older, many of them wear out. Neutrons, unlike other tissue cells, cannot be replaced, and neuralgia (pain along the path of the nerve). Some problems of the nervous system are caused by functional disorders. Lou Gehrig's along with many others are the result of neuron degeneration.

Lou Gehrig's disease, or amyotrophic lateral sclerosis, is a rare condition caused by death of some of the nerves responsible for moving the muscles. The main symptom is paralysis. The muscles gradually waste away, and movement becomes weak and uncontrolled. The disease may affect muscles controlling speech, swallowing, and breathing as well as those controlling the limbs. In this case the first noticeable symptom is slurred speech.

As yet we do not know what causes this motor neuron disease and there is no cure. It occurs most often in men between 55 and 60. It often begins with the hands and spreads to other parts of the body. Patients may live for five years or more, but become progressively weaker physically, although they may remain mentally normal. Physical therapy is of some help (Marshall 1995).

Amyotrophic Lateral Sclerosis (ALS), also called Lou Gehrig's disease, is a progressive neuromuscular disease that weakens and eventually destroys motor neurons (components of the nervous system that connect the brain with the skeletal muscles). Skeletal muscles are involved with voluntary movements, such as walking and talking. The motor neurons transmit the command to move from the brain to the skeletal muscles, which respond by contracting.

A person with ALS usually presents with problems in dexterity or gait resulting from muscle weakness, or with difficulty speaking or swallowing. Sphincter control, sensory function, intellectual ability, and skin integrity are preserved. Patients become paralyzed and often require ventilation and surgery to provide a new opening in the stomach (gastrostomy). Loss of respiratory function is ultimately the cause of death.

Approximately 30,000 patients in the United States currently have ALS. The disease has no racial, socioeconomic, or ethnic boundaries. The life expectancy of ALS patients is usually 3 to 5 years after diagnosis. ALS is most commonly diagnosed in middle age and affects men more often than women.

Risk factors include an inherited genetic defect, which accounts for 5-10% of cases of familial ALS (FALS) in the United States. FALS is linked to a genetic defect on chromosome 21. This gene codes for an enzyme called superoxide dismutase (SOD), an antioxidant that protects motor neurons from free radical damage (i.e., molecules introduced to the body, or produced by body processes that interact and cause cellular damage). More than 60 different mutations that cause SOD to lose its antioxidant properties have been found. However, only 40% of familial ALS cases are linked to SOD mutations, so there may be other unknown genetic defects involved.

In the United States, 90-95% of ALS cases are sporadic. Sporadic ALS appears to be increasing worldwide. The causes are not clear, yet some evidence suggests that the immune system may be involved. Excessive levels of glutamate can overstimulate motor neurons and cause them to die. Glutamate is one of the most important neurotransmitters for healthy brain function. Neurotransmitters are chemicals that transmit signals from one nerve to another.

In Guamanian ALS, a dietary neurotoxin is the risk factor. The suspected neurotoxin is an amino acid (BMAA) found in the seed of the cycad Cyas cirinalis, a tropical plant found in Guam, which was used to make flour and was a major dietary component during the 1950s and the early 1960s, when this type of ALS had an exceptionally high incidence.

The cause of ALS is not completely understood. Researchers and physicians suspect viruses, neurotoxins (especially in Guamanian ALS), heavy metals, DNA defects (especially in familial ALS), immune system abnormalities, and enzyme abnormalities (Gardner 2005).

You've got serious health problems and decide you need an exit strategy. You've got some hard-to-pronounce fatal illness like amyotrophic lateral sclerosis Lou Gehrig's disease and you want to go out with dignity, not with tubes in your body so you can eat and breathe. Or end up in a wheelchair using some machine so you can speak. That's not who you want to be. You've got to get things set up so you can make the call when you've had enough and it's time to go. It's not something you're comfortable talking about or even thinking about, but the alternatives are painful, scary. You're worried you'll end up with Florida Gov. Jeb Bush praying at your bedside, telling your family to back off and let him handle this. You're having nightmares in which congressman Tom DeLay and Sen. Bill Frist decide it's best for the government to deal with your problems. They're holding news conferences to let the country know they really care about you because they're kind, compassionate individuals. They get the president to personally appoint "Brownie" to handle your case. These guys will keep you going until they think you've had enough. But this isn't what you want. Why bother to live if you can't play softball or eat sushi? Or yell at your kids and give your wife a hard time? So you decide that when it gets to the point where you need all these toys and tubes to keep you going you've got to make the hard decision. You're going to be tough to the end. That was last year. Now you're sitting there with a feeding tube pouring some sludge-like paste into your stomach. You're playing with this little machine where you punch in words and it can speak for you, you can even do curse words in Italian. You've got a cane to help you get around, and you know a wheelchair is not far off. At times you're depressed, angry that you need these things. But mostly you're feeling this weird sense of being challenged, and you want to fight back. All these little tools are a pain, but you can still deal with that stuff. The feeding tube gives you the strength to keep going. So you're thinking about all you would have missed if it weren't there. You wouldn't have gone out Saturday night to a church dance. That means you wouldn't have seen the pastor jumping around the dance floor wearing a bright red shirt and matching socks, rocking out to Surfin' USA. And, more important, you wouldn't have seen Iowa's lieutenant governor grabbing the lead in the church's hula hoop contest. Then there's the news, something you care about. You would have missed this one: Sen.