Handling Lou Gehrig’s Disease
Your arms feel weak. Your speech is slurred. Your muscles are twitching and sometimes cramping. You could have Lou Gehrig’s disease.
Lou Gehrig disease a.k.a. amyotrophic lateral sclerosis is a progressive neuromuscular condition that affects voluntary muscle movement such as picking up a book, walking or swallowing, and eventually leads to paralysis. The nerves that control motor activity degenerate in the brain and spinal cord, resulting in progressive wasting of the muscles. One loses the ability to walk, talk, and swallow. Most patients die within two to five years of the first symptoms and death comes from suffocation.
Many retain their cognitive abilities during the course of the disease. There is no cure for ALS. And if a patient is willing to go on a ventilator, some patients lived 15 years or more with the condition such as scientist Stephen Hawking.
Some researchers have found that many people, especially those people who start off with the disease in their throat, the bulbar onset, the people who start with difficulty swallowing and chewing, those people can develop frontotemporal dementia.
While the cause of ALS is unknown, one theory is excess glutamate in the body. Glutamate is a chemical in the body that accumulates in the fluid between brain cells, but when nerve cells that control motor activity become overexcited, they eventually die. The brain uses glutamate to send signals from one neuron to another. Most of the time dietary sources of glutamate are harmless.
Another belief regarding the cause of ALS, is that the disease is caused by the degeneration of cells in the spinal cord and brain caused motor neurons, which control all the voluntary muscles in the body. As the cells deteriorate, the person loses the ability to move, speak and breathe, although patients typically do not lose sensation in their limbs.
Although no one knows for sure, it is estimated that 12,000 to 15,000 people in the United States have ALS, according to the Centers for Disease Control and Prevention. Every year, doctors tell about 5,000 people that they have it.
About 5-10 percent of ALS cases occur within families. This is known as familial ALS and it means two or more people in a family have ALS. People with familial ALS don’t fare as well as those with unrelated ALS, and live only one to two years after diagnosis.
Some scientists believe that those without a familial connection to the disease may have a genetic predisposition to the disease that is activated later in life.
In ALS, the brain and spinal cord nerve cells that energize the muscles are unexplainably killed, making patients prisoners of their own bodies.
Other symptoms of the disease include tripping and falling, hand weakness or clumsiness, weakness in your legs, feet, or ankles, slurred speech or trouble swallowing, inappropriate crying, laughing or yawning, and muscle cramping and twitching in your arms, shoulder and tongue.
Some tests that can rule out other diseases are:
1. An electromyogram—where your doctor inserts a needle electrode through the skin into various muscles. The test evaluates the electric activity of your muscles when they contract and when they are at rest.
2. Nerve conduction study—measures your nerves ability to send impulses to muscles in different areas of your body. This test can determine if you have nerve damage or certain nerve or muscle diseases.
3. MRI—an MRI produces detail images of your brain and spinal cord using radio waves and a powerful magnetic field.
4. Blood and urine tests—analyzing certain blood and urine samples in the laboratory may help your doctor eliminate other possible causes of your signs and symptoms.
5. Spinal tap—your doctor will remove a sample of your spinal fluid for laboratory testing.
6. Muscle biopsy—evaluates if you have muscle disease rather than ALS. While under local anesthesia, a small portion of your muscle is removed and sent to the lab for analysis.
As far as treatment goes, the drug Rilutek seems to be effective in slowing the progression of the disease and it has been approved by the Federal Drug Administration.
Don’t be afraid to seek out help in terms of a support group or counseling to deal with this disease. It can be devastating when you hear your diagnosis and it is easy to feel overwhelmed with the possibility of managing ALS symptoms. Surround yourself with friends and family who want to help. Don’t go through this alone.
