WHAT IS ALS?

ALS, or amyotrophic lateral sclerosis, is a progressive disease that affects nerve cells in the brain and the spinal cord. Nerve cells that control movement (called motor neurons) reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. In ALS, these nerve cells lose function and slowly degenerate.

The progressive degeneration of the motor neurons in ALS eventually leads to their ultimate destruction. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. As more motor neurons die, voluntary muscle action is progressively affected and people with ALS may lose the ability to speak, eat, move and even breathe.

ALS usually strikes people between the ages of 40 and 70, and approximately 20,000 Americans can have the disease at any given time. The average age at the time of diagnosis is 55. For unknown reasons, military veterans are approximately twice as likely to be diagnosed with the disease than the general public.

Based on U.S. population studies, a little over 6,000 people in the U.S. are diagnosed with ALS each year. That’s 15 new cases a day. According to the ALS CARE Database, 60 percent of the people with ALS in the database are men and 93 percent of patients in the database are Caucasian.

While symptoms are quite variable from person to person, it is common to see gradual onset, painless, progressive muscle weakness as the initial symptom in ALS.

One person may have trouble grasping a pen or lifting a coffee cup, while another person may experience a change in vocal pitch when speaking. ALS is typically a disease that involves a gradual onset.

We do not know what causes ALS. There are several research studies – past and present – investigating possible risk factors that may be associated with ALS. More work is needed to conclusively determine what genetics and/or environmental factors contribute to developing ALS.

It is known, however, that military veterans, particularly those deployed during the Gulf War, are approximately twice as likely to develop ALS as compared to the general population.

There is not yet a cure or treatment that that halts or reverses ALS, but scientists have made significant progress in learning more about the disease. There is one drug on the market that has helped slow progression in some patients.

Half of all people affected with ALS live at least three or more years after diagnosis. While the average survival time is three years, about 20 percent of people with ALS live five years, 10 percent will survive 10 years and 5 percent will live 20 years or more.

A.L.S., a degenerative disease that destroys motor neurons and robs its sufferers of voluntary muscle movement, sometimes over years, often virtually paralyzing them before they die.

We've heard a lot about ALS lately, but do you really know what it is? The following is directly from one of our ALS family members-

We have heard a lot about the bucket challenges and donating to the foundation but do we actually know about the disease itself and the devastation that comes with it? It really bothers me that a person of notoriety has to be diagnosed with the disease for it to bring about national attention. This illness is all around us but rarely do you find a person who knows anything about it until a family member or friend is diagnosed with it. The Batesville, Arkansas area where we live has had more than a few people diagnosed with ALS in the past 7 years including a friend of ours. This disease remains on the rare disease list but once my husband was diagnosed June 2010, it was no longer rare to us. We met the ugly monster face to face. It was always someone else's disease and we werent going to have to worry about it. So we thought ,and i am sure you are thinking the same ,it is "someone else's disease". No one is exempt from this horrible deadly disease. Once this disease takes hold it wont let go. Some have slower progression than others but the outcome is always the same. The first symptoms are usually very subtle which may include muscle weakness, slow progressive weight loss , dropping things, tripping and falling , inability to button clothes, small muscle spasms called facsiculations all over your body. Some deal with uncontrollable bouts of crying or laughter and a small percentage may have frontal lobe dementia. My husbands started with hoarseness then difficulty swallowing after he had bypass surgery in 2007. By the time most people are diagnosed they are well into the disease.

There is nothing pretty about this disease, it robs you of every muscle movement in your body with the acception of your eye movement and internal organs, especially the heart. Then there is all the secondary complications that come with it. The diaphragm is frequently affected early into the illness causing breathing difficulties, which will eventually lead to life support /ventilator. The pneumonias, urinary tract infections, kidney failure, blood pressure extremes, anemias with unexplainable blood loss only to find out your blood vessels are also affected.

My husband spent the last 2/12 years of his life in bed recieving total care. You cant breath on your own, you cant swallow, cant wipe your own tears or your nose or control other normal body functions. You food is supplied through a gastic tube, most all cant talk. The easiest way to describe what a person goes through who decides to fight until the end is laying in your own casket. My husband was tough and a fighter. He had a beautiful smile and could talk with his eyes. He lived 7 years after symptoms began and fought until the bitter sweet end- July 10th 2014 the demon monster ALS had to let go of his frail body when Jesus called him home. This is part of our story.

JG

EVERY 90 MINUTES

Every 90 minutes someone is diagnosed with amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disease that causes muscle weakness, paralysis, and ultimately, respiratory failure.

ALS, also known as Lou Gehrig’s disease, Charcot's disease, and motor neuron disease (MND), attacks certain cells in the brain and spinal cord needed to keep our muscles moving. Early signs and symptoms of ALS include:

• muscle cramps and muscle twitching

• weakness in hands, legs, feet or ankles

• difficulty speaking or swallowing

The senses, including hearing, sight, smell, taste, and touch, are not affected by ALS.

There is no single diagnostic test for ALS. However, experts in the disease, usually neurologists specializing in neuromuscular diseases, are very capable of diagnosing ALS. In some cases, they might order additional tests if the diagnosis is not clear. These include:

• electromyography and nerve conduction

• magnetic resonance imaging (MRI)

• genetic tests

• muscle biopsy

• spinal tap

• blood and urine tests

Most people with ALS live 2-5 years after their first signs of disease. About 10% of people with ALS survive at least 10 years. This variable rate of disease progression makes prognosis difficult to predict and therapies challenging to develop.

Currently, there is only a single medicine for specifically treating ALS - riluzole. The drug, marketed by Sanofi-Aventis under the name Rilutek, extends survival only about 2 to 3 months

amyotrophic lateral sclerosis 

(say: ah-my-uh-TRO-fik LA-tuh-rul skluh-RO-sis), or ALS. The official name comes from these Greek words:

• "a" for without

• "myo" for muscle

• "trophic" for nourishment

• "lateral" for side (of the spinal cord)

• "sclerosis" for hardening or scarring

So, amyotrophic means that the muscles have lost their nourishment. When this happens, they become smaller and weaker. Lateral means that the disease affects the sides of the spinal cord, where the nerves that nourish the muscles are located; andsclerosis means that the diseased part of the spinal cord develops hardened or scarred tissue in place of healthy nerves.

ALS is often called Lou Gehrig's disease after Lou Gehrig, a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s. People in England and Australia call ALS motor neurone disease (MND).

Lou Gehrig's disease damages motor neurons in the brain and spinal cord. Motor neurons are nerve cells that control muscle movement. Upper motor neurons send messages from the brain to the spinal cord, and lower motor neurons send messages from the spinal cord to the muscles. Motor neurons are an important part of the body's neuromuscular system.

The neuromuscular system enables our bodies to move and is made up of the brain, many nerves, and muscles. Things that we do every day — like breathing, walking, running, lifting stuff, and even reaching for a glass of water — are all controlled by the neuromuscular system.

Here's how the neuromuscular system works: If you want to make a fist, your brain first sends signals through upper motor neurons to the area in your spinal cord that controls your hand muscles. Then lower motor neurons in your spinal cord signal the muscles in your hand to move and make a fist.

Over time, Lou Gehrig's disease causes these motor neurons in the brain and spinal cord to shrink and disappear, so that the muscles no longer receive signals to move. As a result, the muscles become smaller and weaker. Gradually the body becomes paralyzed, which means that the muscles no longer work.

However, someone with ALS, even at an advanced stage, can still see, hear, smell, and feel touch. The nerves that carry feelings of hot, cold, pain, pressure, or even being tickled, are not affected by Lou Gehrig's disease. In some people with ALS, the parts of the brain that allow us to think, remember, and learn also are affected by the disease.

How Is the Disease Diagnosed?

Lou Gehrig's disease doesn't always begin or become worse in the same way. The disease is different for every person who has it. In general, muscle weakness, especially in the arms and legs, for Lara's father it started in his right hand and this is an early symptom for more than half of people with ALS. Other early signs are tripping or falling a lot, dropping things, having difficulty speaking, and cramping or twitching of the muscles. As the disease gets worse over time, eating, swallowing, and even breathing may become difficult.

It may take several months to know for sure that someone has Lou Gehrig's disease. The illness can cause symptoms similar to other diseases that affect nerves and muscles, including Parkinson's disease and stroke. A doctor will examine the patient and do special tests to see if it might be one of those other disorders. (It's like using the process of elimination to figure out the answer to a multiple-choice question on a test.)

One of the tests, an electromyogram, or EMG, can show that muscles are not working because of damaged nerves. Other tests include X-rays, magnetic resonance imaging (MRI), a spinal tap, and blood and urine evaluations.

Sometimes a muscle or nerve biopsy is needed. A biopsy is when a doctor takes a tiny sample of tissue from the body to study under a microscope. Examining this tissue can help the doctor figure out what's making someone sick.

How Is the Disease Treated?

Currently, there's no way to prevent or cure Lou Gehrig's disease, but a number of treatments are available to people with the disease. Medicines can control symptoms, such as muscle cramping and difficulty swallowing, and other drugs can slow the development of the disease.

Physical therapy can help people with ALS cope with muscle loss and breathing problems. Special equipment is also provided when it becomes necessary. For instance, a power wheelchair can enable a paralyzed person with ALS to get around. A machine called a ventilator can help a someone breathe.

In addition, a nurse or other health assistant may come to the person's home to provide care that the family cannot handle alone. It's normal for family members to feel upset, overwhelmed, and sad if a loved one has ALS. Counseling, as well as support from other family members and friends, can make it easier to deal with the challenges they face.

Living With Lou Gehrig's Disease

According to the ALS Association, about half of all people with ALS live at least 3 years after they find out they have the disease, and 20% (or 1 in 5) live 5 years or more. As many as 10% will survive more than 10 years."

Living with Lou Gehrig's disease is physically difficult, but it is reassuring to know that the mind is not affected. People with the disease can think as clearly as ever, are able to maintain relationships with friends and family, and should be treated respectfully and normally.

Communication can be difficult because the disease affects the person's breathing and the muscles needed for speech and arm movement. With patience, the families of patients with ALS can learn to communicate effectively with their loved one.

Researchers continue to study ALS as they try to understand why it happens, and how the disease damages the motor neurons in the brain and spinal cord. As they learn more about the disease, researchers can continue to develop new and better treatments.