Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, fatal neurological disease which attacks the motor neurons (nerve cells) which control voluntary muscles. The disease belongs to a group of disorders known as motor neuron diseases (MND), which are characterized by the gradual degeneration and death of motor neurons. Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that pass impulses from the brain to the voluntary muscles of the body.



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What is ALS?
What are the symptoms of ALS?
Who gets ALS?
How is ALS diagnosed?
What is the survival Rate of ALS?
How can I participate in Clinical Trials?

If there are any other questions I have not addressed, feel free to visit the Feedback page and ask. If I don't have the answer I will try to find it. The following are FAQs that people frequently ask me regarding my own condition, Theses answers were valid as of June 20th, 2012. As my condition deteriorates I will continue to update my answers.

Can You walk?
How do You type?
How do you drive?
What type of wheel chair do you use?
Do You have any difficulties eating?
How many pillows do You use?
Do you have trouble with blankets and sheets?
How could you go so long not knowing you had a serious illness?
Do You have any family members with neurological disorders?
Do You exercise?

   

What is (ALS) Amyotrophic Lateral Sclerosis?

Amyotrophic lateral sclerosis (ALS), also referred to as Lou Gehrig's disease in the U.S. and Canada and motor neuron disease throughout the rest of the world, is a form of motor neuron disease caused by the degeneration of upper and lower motor neurons, located in the ventral horn of the spinal cord and the cortical neurons. The condition is often called Lou Gehrig's disease in North America, after the New York Yankees baseball player who was diagnosed with the disease in 1939. The disorder is characterized by rapidly progressive weakness, muscle atrophy and fasciculations spasticity, dysarthria, dysphagia, and respiratory compromise. Sensory functions generally are spared, as is autonomic and oculomotor activity. ALS is a progressive, fatal, neurodegenerative disease with most affected patients dying of respiratory compromise and/or pneumonia after 2 to 3 years; although some die within a year from the onset of symptoms, and occasional individuals have a slower progression and survive for many years.

The disorder causes muscle weakness and atrophy throughout the body caused by degeneration of the upper and lower motor neurons. Unable to function, the muscles weaken and atrophy. Affected individuals may ultimately lose the ability to initiate and control all voluntary movement, although bladder, bowel and the muscles responsible for eye movement are usually, but not always, spared. Cognitive function is generally spared for most patients although about 5% of it's victims also have frontotemporal dementia (FTD). A higher proportion of patients have more subtle cognitive changes which may go unnoticed but are revealed by detailed neuropsychological testing. Sensory nerves and the autonomic nervous system, are generally unaffected meaning the majority of people with ALS will maintain sight, hearing, touch, smell, and taste. Bladder and bowel functions are also rarely affected by ALS.


   

What are the symptoms of (ALS) Amyotrophic Lateral Sclerosis?

Early symptoms usually include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches and uncontrollable periods of laughing or crying. The hands and feet may be affected first, causing difficulty in walking or using the hands for the activities of daily living such as dressing, washing and buttoning clothes. The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include twitching, cramping, or stiffness of muscles, muscle weakness affecting an arm or a leg. Muscle wasting gradually spreads to the muscles of the trunk of the body, a typical first indicator is difficulty in lifting your feet off the ground as in climbing stairs. The disease eventually affects swallowing, chewing and breathing. Complete paralysis eventually results, usually occurring within two to five years of diagnosis. When the diaphragm is attacked, the patient is unable to breathe for him/herself, and faces permanent ventilator support in order to survive. ALS attacks only 'motor' neurons. Sight, touch, hearing, taste, smell and muscles of the eyes and bladder are generally not affected. Sexual function and drive are not affected. The mind is not affected, and remains sharp despite the progressive degenerating condition of the body.


   

Who gets (ALS) Amyotrophic Lateral Sclerosis?

ALS is a disorder that affects the function of nerves and muscles. A little over 5,600 people in the U.S. are diagnosed with ALS each year. (That's a new victim every 90 minutes.) It is estimated that as many as 30,000 Americans have the disease at any given time. 80% of the people with ALS are men and 93% of patients are Caucasian. Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. However, cases of the disease do occur in persons in their twenties and thirties. Generally though, ALS occurs in greater percentages as men and women grow older. ALS is 20% more common in men than in women, however with increasing age, the incidence of ALS is more equal between men and women. Military veterans are more likely to contract ALS, amyotrophic lateral sclerosis than the rest of the population, regardless of when they served in uniform, according to a report released by The ALS Association at a hearing of the Senate Appropriations Subcommittee. Men and women with any history of military service in the last century are twice as likely to contract ALS than men and women who did not serve in the military. Some studies suggest that Air Force veterans are slightly more likely to suffer from this disease. There is a high incidence of ALS cases in Guam and the Trust Territories of the Pacific. Between 10% and 20% of ALS cases are considered Familial meaning hereditary. When Familial ALS is the case, victims will have a 50% chance of passing it on to their children, likewise there would have been a 50% chance that one of their parents also suffered from ALS.


   

Diagnosing Amyotrophic Lateral Sclerosis

ALS is difficult to diagnose because the symptoms are similar to those of other neuromuscular disorders, many of which are treatable. Typically the diagnosis of ALS otherwise known as Lou Gehrig's Disease is the elimination of all other possible causes for the symptoms. The diagnosis is usually based on a complete neurological examination and clinical tests. The neurological exam usually shows evidence of muscle weakness (localized or widespread, depending on the extent of the disease). The exam also reveals muscle atrophy. Because ALS affects the skeletal, voluntary muscles, the neurological exam usually does not reveal abnormalities in the sensory reflexes (i.e., vision, hearing, taste, smell, touch, or bowel and bladder control). Nerve conduction velocity (NCV) and electromyography (EMG) help diagnose nerve and muscle disorders. These tests, which can be used in combination, are often referred to as EMG/NCV studies. NCV is administered before EMG and measures the speed at which nerves transmit electrical signals. During NCV, electrodes are placed on the skin over a nerve that supplies a specific muscle or muscle group. A mild, brief electrical shock is delivered through the electrode and the response of the muscle is detected, amplified, and displayed. The strength of the signal is also measured. Neurological conditions can cause the NCV to slow down or to be slower on one side of the body. The strength of the response also provides clues to help with diagnosis and to determine the extent of the disease. In ALS, the speed of the nerves is usually slowed, but compound muscle action potentials (CMAPs) may be decreased. EMG measures nerve impulses within the muscles. The EMG test is often referred to as the 'Pin Test'. Tiny electrodes are placed in the muscles in the arms and legs and the electronic responses are observed using an instrument that displays movement of an electric current (oscilloscope). As muscles contract, they emit a weak electrical signal that can be detected, amplified, and tracked, providing information about how well the muscles are working. These responses are abnormal in cases of ALS. Tests may be performed to rule out other neurological disorders. Magnetic resonance imaging (MRI scan) may be used to rule out spinal cord or brainstem disease. Blood tests may be done to detect the presence of heavy metals such as lead in the blood. Laboratory tests may detect abnormal proteins or hormone levels associated with other neurological diseases. A lumbar puncture or spinal tap may be performed to analyze the cerebrospinal fluid for genetic abnormalities.


   

Survival rate of Amyotrophic Lateral Sclerosis

Although the life expectancy of an ALS patient averages about two to five years from the time of diagnosis, this disease is variable and many people live with quality for five years and more. More than half of all patients live more than three years after diagnosis. About ten percent of people with ALS will live for up to ten years, and five percent will live 20 years or more. There are people in whom ALS has stopped progressing.
Steve Wells is Canada’s longest-living survivor of the disease who was diagnosed in 1980. 32 years ago.
Marilyn Cooper is the longest known American survivor of ALS. Cooper was diagnosed in 1975. 37 years ago.
Stephen Hawkings The world renowned theoretical physicist from Great Britain was diagnosed in 1963, doctors diagnosed the then 21-year-old with amyotrophic lateral sclerosis. 49 years ago


   

Info on clinical trials

With no effective ALS treatments currently available in the marketplace, many ALS patients opt to participate in clinical trials. The ALS Therapy Development Institute doesn't officially sponsor or endorse any specific clinical trial, it does however provides unbiased and up-to-date information about many known ALS trials taking place worldwide. You can search their database by type and category and compare trials side by side. Clinical trials are not limited to new drugs. There are trials taking place for a wide range of products such as communication devices, Bio-marker imaging, assistive devices, nutrition, breathing aids and other technologies that may potentially be beneficial.

Click here for: ALS TDI

The ALS Association is another good source for information on clinical trials. An ALS clinical trial expert can be reached at (877) 458-0631 Monday through Friday between 9 AM. and 5 PM. Eastern Time and by e-mail at alstrials@partners.org

Click here for: ALSA.ORG

The Northeast ALS Consortium (NEALS) is one more source you may want to look into. Clinical trials are the only way to develop new treatments for people with ALS. People with ALS who enroll in a clinical trial are contributing to improved health care for everyone with the disease. Even when the results of a trial are negative, we learn that much more about the disease, and how to look for more promising new treatments. To access the NEALS Database visit the following link and use the pull down box to select Amyotrophic Lateral Sclerosis (ALS).

Click here for: NEALS Consortium

IF YOU HAVE ALS AND HAVE NOT REGISTERED WITH THE NATIONAL ALS REGISTRY PLEASE DO SO NOW!
THIS IS THE MOST IMPORTANT STEP YOU WILL TAKE. IF You have ALS please register.
Click here for : ALS Registry

   

Can You walk?

Only barely, I can make it 10 - 15 feet, however there is always a major risk of falling. Here is the progression as best as I can remember: In 2002 I recall having a difficult time lifting my feet, running was no longer an option. I started falling frequently, about once every month or 2. In 2009 walking a half mile was a major effort and would wipe me out, I was now falling about 2 or 3 times a month. In 2011 I began using a walker and would still fall at times, January of 2012 I began using a wheel chair. I do still fall at times going from my bed to the john. After a while you get smart and make sure you always have a suitable object to fall towards, such as a couch, counter top, wall etc. I have not sustained any injuries from falling in quite some time.



   

How do You type?

So far I can type without any extra technology, my fingers are clumsy, I use the back space button alot. I learned to type back in the early 70s I use a combination of the way I learned in school and the hunt and peck method, more hunt and peck. 2 fingers and my right thumb have been severely affected, but even a monkey can type with 7 fingers.



   

How do you drive?

I have not had any problems driving, I have however had great difficulty getting into the drivers seat. I have hand controls but I can drive without them. My ankles may be weak, but they function. If I get my right heel in the correct position my foot easily pivots from gas to brake. I always try to keep a step ahead of my disease, so I practiced with the hand controls. I am now more comfortable with hand controls, but if I am going to hit a button for windows, stereo, a/c, mirrors etc... etc... I either set the cruise control or use my foot for the gas. People are often surprised to hear me say the hand controls are easier to use. This is only because my leg muscles are so weak and the motor skills are fading.



   

What type of wheel chair do you use?

I have the most pimped out Hoveround in the state. I traded a model airplane for it because I was to broke to pay attention. I added 2 headlights underneath the seat, 3 strobe lights and red neon ground effects. People young and old hoot and holler when they see me cruising down the street at night. I added a holder for my cane, and built a portable ramp which can get me up a 3 or 4 inch curb. You will notice that the ramp stows away under the seat.


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Do You have any difficulties eating?

Yes I do! I can't get enough. I have added on about 30 pounds in 6 months. Being a diabetic I have had to cut out sugar, pasta and bread. Worst of all no Mt. Dew. Diabetes is new to me, I friggen hate it. I want cheesecake dammit! I can swallow just fine. Sign me up for an egg eating contest because I can swallow hard boiled eggs whole.



   

How many pillows do You use?

Four and a half. One is tiny. Doctors ask this question to judge breathing difficulties. People tend to prop their head up high to aid in breathing. I breath okay, so here is the real deal. One pillow under my head, the small one fills the gap created by the curvature of my neck. I kinda hug one pillow to keep my arm propped up, and another pillow goes under my hand just to keep my elbow bent. I don't know why a straight elbow bothers me. The last pillow goes under my leg. I got so sick of explaining this to doctors that I now just say "ONE".



   

Do you have trouble with blankets and sheets?

Oh dear God, yes. If you are healthy you wont understand, if you have lower motor neuron involvement you get it, I know you do. My legs can get tangled up in the covers so bad that you may as well just put shackles on my ankles. This can be so frustrating. Sometimes I just have to grin and bare it, even though I am so uncomfortable I just have to deal with it. I don't have the strength or coordination to straighten them out.



   

How could you go so long not knowing you had a serious illness?

I am an idiot. I used to jog 1.5 to 2 miles, 3 to 5 nights a week. Not bad for a pack a day smoker. In my mid to late 30s I had to give it up due to lower back pain. By my 40th birthday I couldn't run across the street. over the next 8 years walking gradually became more difficult. I just assumed I was getting older and this was natural due to the fact that I quit the only excercise I ever did. By 46 or 47 years old I kept thinking Man if I woke up feeling like this 10 years ago, I would have checked myself in to the hospital. I truly believed God just had me on the accelerated aging program. Like I said, I am an idiot.



   

Do You have any family members with neurological disorders?

Nothing hereditary. I do have a cousin who was an EMT in New York City and suffered some issues from working the World Trade Centers. My fathers uncle had Parkinson's but he was not a blood relative to me.



   

Do You exercise?

I started to. In 2009 I bought a bicycle, and would do a 90 year old woman's version of deep knee bends, not very deep. Riding the bike got harder every day. I needed a step stool or curb to get on the bike. I kept saying "That which doesn't kill me, will make me stronger. And, I don't feel any stronger". I suspected that I was doing more harm than good, so I gave it all up and sold the bike. Again my instincts were correct. My doctors have told me to not do any strenuous exercise. I was told to do stretching exercises to maintain range of motion.







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