Lou Gehrig’s Disease - Amyotrophic Lateral Sclerosis
Facts about Lou Gehrig’s Disease
Fifteen new cases of Lou Gehrig’s disease, which is Amyotrophic Lateral Sclerosis (ALS), is diagnosed daily in the US. ALS is a progressive neurodegenerative disease, and it affects the nerve cells of the brain and spinal cord. This disease causes patients to slowly lose the ability to move, speak, eat or even breathe. The average life expectancy after this diagnosis is from 2 to 4 years, although 10% may live 20 or more years.
The motor neurons begin in the brain, then reach the spinal cord before reaching the muscles. The motor neurons progressively degenerate with ALS. When the motor neurons die, the brain is no longer able to initiate or control muscle, so there is no voluntary movement in the arms and legs. There is no cure for ALS.
MRI with the Diagnosis of ALS
Types of ALS
There is no known cause of this disease for 90-95% of the people. Scientists believe that genetic and environmental factors are involved. The remaining 5-10% of cases are familial, which means inherited. The children that are born from parents that have this disease have a 50% chance of inherited the gene mutation and may develop ALS.
ALS can occur at any age but typically does not occur until ages 40 to 70. The familial disease may occur at age 50. More white people are affected than blacks. In Europe, this disease affects 2-3 people per 100,000 annually. Numbers are unclear for much of the world. Military vets also develop ALS at a higher rate regardless of the branch of the military where they served, and it does not matter whether they served during a war or during or peacetime.
Lou Gehrig - New York Yankees
History of Lou Gehrig’s Disease
Charles Bell described this disease in 1824. Jean-Martin Charcot, a French neurologist, discovered the disease in 1869. He was the first one to call this disease amyotrophic lateral sclerosis as he noted the connection between the symptoms and neurological problems.
This disease became known in the United States when Lou Gehrig developed this disease in 1939. Since Lou Gehrig was a well-known first baseman for the NY Yankees with an excellent hitting record, and he was known as ‘The Iron Horse’ so people knew him and cared about his life. He was diagnosed at 36 and died at age 38. Then, Stephen Hawking developed this disease in 1963.
Lou Gehrig Disease
I might have had a tough break, but I have an awful lot to live for.
-- Lou Gehrig
Symptoms and Treatment of ALS
Initially, symptoms of ALS are barely detectable. The initial characteristics are stiff muscles, muscle twitching, then, a gradually worsening muscle weakness. This is a difficult disease to diagnose and may take several months or even years.
While there is no cure, there are treatments to improve the symptoms, which is the goal at this time even though advances have been achieved. Riluzole is a medication used to extend the life of the patient by a few months.
Early symptoms of ALS:
- Coordination and strength of the arms and legs may be the first symptoms.
- Fatigue and weakness in the feet and ankles is another early symptom.
- The muscles of the arms, tongue, and the shoulders may twitch or cramp.
- Speaking, swallowing and breathing may become strained.
The patient may end up on non-invasive ventilation that will extend life for a period of time and have a feeding tube for nutrition. While these things may help the disease will continue to progress. Ultimately, most patients die of respiratory failure.
The Mayo Clinic lists the following possible symptoms:
- Muscle cramps and spasms
- Excessive salivation
- Excessive phlegm
- Sleep problems
- Uncontrolled outbursts of laughing or crying
Diagnosis and Treatment of ALS - Mayo Clinic
Studies Used to Reach a Diagnosis
Various medical tests may be conducted to differentiate this disease with other neurological diseases:
- Electromyogram (EMG) - a test to check for electrical activity in the muscles
- Nerve conduction test, which checks for the nerve's ability to send messages to the muscles
- MRI to get detailed images of the brain and spinal cord
- Urine and blood tests to look for abnormalities
- Spinal tap to get a laboratory analysis
- Muscle biopsy to rule out other diseases
Breathing care is a concern, and some people end up with a tracheostomy, Physical, occupational and speech therapy are treatments that may help the patient. Psychological and social support can help with financial problems, insurance issues, getting the appropriate equipment and emotional support may all be provided.
Slowing the advancement of this disease with medications is the only treatment at this time, but there is a vast amount of research ongoing, which hopefully will find a cure.
Treatment and Research News
Whie riluzole slows the progress of the disease, it is expensive. Spasticity may be controlled with baclofen and diazepam, but the available treatments for this disease does not reverse the damage.
Scientists seek to understand the exact mechanism that causes the motor neurons to degenerate. Research at this time have found a variety of cellular defects, which depend on the exact gene mutation. Another recent discovery shows the cells of the nervous system (glial support cells and inflammation cells) also play an important role in ALS. The ALS Association is support stem cell research using blood and skin cells.
Jingsong Zhou, a professor of kinesiology at the University of Texas, is investigating a new approach to ALS. Recently, she was awarded a $2.88 million award from the NIH to support her work that preserves the mitochondria, the way it works or dysfunctions in a diseased cell. She believes this disease affects the physiology of the whole body. Zhou is searching for the reason cells are damaged, then she hopes to test potential therapies or compounds.
Lou Gehrig's Disease (ALS): Progress and Promise in Stem Cell Disease
How to Cope with ALS
There are a few things you can do as a patient to help accept this diagnosis and live the life you have to the fullest extent.
Time to Grieve - The news that you have ALS, which is a fatal disease, if very difficult for you and your family. A period of mourning is appropriate.
Try to think beyond the physical changes as you can still lead a rewarding life despite the limitations.
Remain hopeful - Having a healthcare team will help you focus on healthy living throughout the course of this disease. Patients sometimes live 10 or more years, so maintain an optimistic outlook to improve the quality of your life.
Join an ALS support group as talking with others that have ALS can relieve some of the anxiety. There are also support groups for family members.
ALS is a difficult disease, and it is difficult to hear you have a disease that is so debilitating and has no cure. The research is ongoing throughout the world, and many researchers are looking for a cure. They have made progress.
This content is accurate and true to the best of the author’s knowledge and does not substitute for diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed health professional. Drugs, supplements, and natural remedies may have dangerous side effects. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.
© 2019 Pamela Oglesby