Rockin' For A Cure

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Founder, Steve Weekes
(1961-2005)

 

 

 

What is ALS?

And 38 other frequently asked questions about the disease.

This information was initially put together by Steve Weekes to help his
family, friends, and other patients understand more about the disease. 


What is ALS?

Amyotrophic Lateral Sclerosis (ALS) is a fatal neuromuscular condition characterized by progressive muscle weakness, muscle wasting, fasciculations and increased reflexes, resulting in total paralysis of all voluntary muscles.

 

OVERVIEW:

ALS is a rapidly progressive, fatal neuromuscular disease that attacks motor neurons in the spinal cord and lower brain, which transmit signals from the brain to the voluntary muscles throughout the body.

When motor neurons die because of ALS, the ability of the brain to control muscle movement is lost. When muscles fail to receive messages, they weaken, atrophy, and die.

 

The most basic activities and function of daily living, such as, walking, talking, breathing, writing, reaching, bending, squatting, jumping, twisting, pushing, pulling, grabbing, pinching, squeezing, holding, lifting, all become extremely difficult tasks, even monumental, and eventually cannot be done at all.

 

There is no known cure.


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What do the letters ALS stand for?


ALS stands for Amyotrophic Lateral Sclerosis.

Like many medical terms, it comes from Greek words.

"A"               = without
"Myo"           = muscle
"Trophic"          = nourishment
"Lateral"          = side (of the spinal cord)
"Sclerosis"          = hardening or scarring


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When was ALS first discovered? 


ALS was first described in scientific literature in 1869 by the French neurologist Jean-Martin Charcot.

 

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Are there are other names for ALS?


ALS is commonly known in the US as Lou Gehrig’s Disease.

In England, it is known as MND, (Motor Neurone Disease).

The French refer to ALS as Maladie de Charcot.

 

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Is there a cure for A LS?

 

  • There is no cure for ALS

 

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How long do people with ALS live?

  • About twenty percent of people with ALS live five years or more and up to ten percent will survive more than ten years and five percent will live 20 years. 
  • There are people in whom ALS has stopped progressing and a small number of people in whom the symptoms of ALS reversed.

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What are the symptoms of ALS?

 
ALS symptoms include the following:

  • Dropping things, tripping, stumbling and falling.

  • Abnormal fatigue of the arms and legs.

  • Loss of both muscle control & strength in the hands, arms, feet and legs.

  • Difficulty with speaking, swallowing and breathing.

  • Muscle twitching and cramping.

  • Chronic fatigue

  • Brisk reflexes

  • Uncontrollable periods of laughing or crying.

 

ALS is characterized by both upper and lower motor neuron damage. Symptoms of upper motor neuron damage include stiffness (spasticity), muscle twitching (fasciculations), and muscle shaking (clonus). Symptoms of lower motor neuron damage include muscle weakness and muscle shrinking (atrophy).

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 Is ALS painful?

The motor neuron damage and muscle atrophy caused by ALS is not painful.

  • Some of the effects of ALS may be painful.

  • Common pains as a result of ALS include pressure sores, muscle cramps, joint contractures, constipation, burning eyes, swelling feet, and muscle and joint aches.  

 

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Is ALS contagious?


ALS is not contagious.

 

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Is ALS hereditary?


At least 10% of ALS cases are hereditary.

This is called familial ALS. Generally, we define familial ALS as two or more cases in the same bloodline. In familial ALS, the disease is autosomal dominant, meaning that if a parent has ALS, their children have a 50% chance of inheriting the defective gene. While the risk of inheriting the defective gene is 50% for each child of an affected person, not all people with the defective gene will develop the disorder. Twenty percent of familial ALS cases have a specific gene defect in the SOD (super oxide dismutase) gene.

90% of ALS cases have no familial link, and they are called sporadic ALS.  

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What causes ALS?


The cause of ALS is unknown.

Researchers know that an excess of a neurotransmitter called glutamate clogs the synapse of the nerve cell preventing transmission of neural impulses.

The cause of this neurotransmitter problem remains a mystery.

 

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Who gets ALS?

 

ALS occurs in both sexes and all races.

Men are much more likely to get ALS than women.

Older people are more likely to get ALS than younger people. 

 

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Does ALS affect the mind?

 


  • There are many mixed opinions on this subject. Especially in current discussions.

  • It is now known that some ALS patients do experience dementia, but it is unknown whether ALS was the cause of the dementia.

  • Past ALS literature describes the mind as being "completely unaffected."

  • Many current ALS specialists are starting to take a much closer look at this statement.

 

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Does ALS affect the bladder and bowel?

Until recently, it was thought that the bowel and bladder were completely unaffected,

But as research continues on, this belief is changing rapidly.

Many ALS patients are now reporting that there is a lack of bowel control.  

 

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How is ALS diagnosed?

ALS is diagnosed through a process of elimination and takes several months.

Early ALS symptoms may be similar to a number of other neuromuscular diseases. Diagnostic tests include MRI (magnetic resonance imaging), EMG (electromyogram), muscle biopsy, and blood tests. In order for a definitive diagnosis of ALS to be made, damage must be evident in both upper and lower motor neurons. When three limbs are sufficiently affected, the diagnosis is ALS.  

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How long does it take to diagnose ALS?

The diagnosis of ALS typically takes months and in some cases years.

Many of the initial symptoms are similar to other neuromuscular diseases.

Diagnosing ALS is a process of elimination, which is time consuming.

 

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What treatments are available?

 

Riluzole, the first treatment to alter the course of ALS, was approved by the FDA in late 1995. This antiglutamate drug was shown scientifically to prolong the life of persons with ALS by at least a few months. More recent studies suggest Riluzole slows the progress of ALS, allowing the patient more time in the higher functioning states when their function is less affected by ALS.

 

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How common is ALS?

The incidence of ALS is about two per 100,000.

This means that for every 100,000 people, two will get ALS. The prevalence of ALS is about eight per 100,000. This means that if you look at a population of 100,000 people, eight of them will be living with ALS. In the United States, approximately 30,000 people are living with ALS. There are about 5,000 new cases per year, or 15 per day.

(As ALS is terminal and incurable, death rates are very close to incidence rates.)

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Are there different types of ALS?

There are three types of ALS.

The most common type is called sporadic ALS. This type of ALS is not hereditary and accounts for 90% of ALS cases.

 

Familial ALS is hereditary, is passed on by a dominant gene and accounts for nearly 10% of ALS cases.

 

The third type of ALS is called Guamian and is related to the high incidence of ALS on the island nation of Guam.

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Is ALS always a fatal disease?

 

ALS is always fatal.

There are a few rare cases where the disease progression plateaus or stops.

Concerns of accurate diagnoses have been suspect in these rare cases.

If an ALS patient opts for a ventilator, he or she can live for many years with the disease under the care of qualified nursing personnel.

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Do ALS patients suffer painful, suffocating death?

In the majority of cases, ALS patients die painlessly and peacefully, often in their sleep. If an ALS patient does not opt for a ventilator to assist breathing, death is usually a result of insufficient oxygen, which leads to a build-up of carbon dioxide in the blood. The build up of carbon dioxide has a narcotic effect on the patient making him or her sleepy. Many ALS patients also opt for medications to ease breathing discomfort and anxiety. ALS patients often choose to die in a hospice setting or at home where their loved ones can comfort them.

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 What is the cause of death for ALS patients?


A common cause of death among ALS patients is respiratory failure or cardiac arrhythmias due to insufficient oxygen.

Another common cause of death is respiratory infection such as pneumonia. The risk of respiratory infections increases as weakened diaphragm and chest muscles make it more difficult to clear the lungs.

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Is ALS a disease of the muscles or nerves?


ALS is a disease of the motor nerves, specific nerve cells in the brain and spinal cord that control voluntary movement.

Motor nerves attach to muscles and when the motor nerves gradually degenerate and die, the muscles no longer receive nerve impulses. Because of the nerve death, the muscles atrophy and waste away.

When an ALS patient first notices neurological symptoms, more than half of the motor neurons may already be dead. The body has a remarkable ability to compensate for nerve loss and new nerves grow at the same time other nerves are dying. Eventually, nerve death becomes so pervasive that muscle paralysis is the result.   

 

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Which muscles are affected first?


Most ALS patients first notice muscle weakness in either the arms or the legs (32 percent in the arms and 36 percent in the legs.)  This is called limb-onset ALS.

Approximately 25% of ALS patients have difficulty speaking as their first symptom.  This is called bulbar ALS because it involves the corticobulbar area of the brainstem. ALS is a very variable disease, and there are also cases affecting breathing first, without any other symptoms.

Approximately 7 percent have difficulty breathing (dyspnea) as their first symptom.

 

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Are there muscles that are not affected?


ALS does not affect all muscles.

Bowl and bladder control remains intact, as does sexual function. The heart muscle is unaffected. The muscles of the eyes are the last ones affected and, in some cases, are not afflicted.

 

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 How does ALS typically progress?


ALS progression can vary considerably with different patients.

A common progression is as follows:

Difficulty walking resulting in the use of a cane followed by a walker and then a wheelchair.  As the legs get weaker, so do the arms and hands.  The patient loses the ability to write, type, and feed themselves.  As the muscles of the limbs become weaker, difficulties in speaking and swallowing begin to occur.  This may result in the need for an augmentative communication system in order to communicate and a feeding tube in order to get adequate nutrition.  Typically, the ALS patient has a gradual decrease in lung vital capacity as the muscles of the chest and the diaphragm begin to weaken.  When lung vital capacity drops below 50%, the ALS patient needs to consider using a ventilator in order to stay alive.

This progression may be as quick as six months.

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Does exercise help slow ALS progression?


Exercise does not slow down the wasting of muscles due to ALS.

Heavy exercise is not recommended because of the fatigue it causes on already weakened muscles.  Light exercise such as walking, swimming, and stretching can help maintain strength in the muscles which are not yet affected by ALS.  When muscle atrophy becomes severe, the only recommended exercise is range of motion to preserve joint flexibility.

   

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Does ALS affect speech?


ALS does affect speech and swallowing when the disease involves the corticobulbar area of the brainstem.

As the muscles of the mouth and tongue weaken, speaking becomes increasingly difficult. Speech therapy may be helpful to an ALS patient and can enable the person to be understood even with a weak voice. Eventually, ALS patients lose the ability to speak and must rely on an augmentative communication device in order to communicate.  

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What is Bulbar ALS?

 
Bulbar refers to the brain stem where motor neurons are concentrated.

Bulbar ALS is the type of ALS where speaking and swallowing difficulties are the first symptoms.  Many patients with bulbar ALS are unable to speak or eat but are able to walk and write.  Bulbar-onset ALS patients generally have a faster disease progression than do limb-onset patients. Limb-onset patients eventually develop bulbar symptoms as their disease progresses.

 

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What is the average age of ALS onset?

The average age of ALS onset is 55.

ALS can affect people at any age, and cases have been found in persons as young as 12 and as old as 98.

80% of ALS cases begin between the ages of 40 to 70.

There appears to be a trend of more younger patients in there 20's and 30's being diagnosed with ALS  .

 

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Is ALS caused by environmental factors?

The cause of ALS in most cases is unknown, although environmental factors are strongly suspected.

A higher incidence of ALS is correlated with exposure to agricultural chemicals and solvents.  

 

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Why is ALS called Lou Gehrig’s disease?


In the U.S., ALS is known as Lou Gehrig’s disease because the famous baseball player, Lou Gehrig, died from the disease in 1941. Lou Gehrig played for the New York Yankees and was one of the best baseball players ever. He got ALS in 1939 at the age of 38.  

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How do ALS patients eat when they lose their ability to chew and swallow?


As muscle weakness makes eating more difficult, dietary consistency changes are needed.

Gradually the diet includes softer foods and eventually may consist of puréed foods. When eating becomes too tiring or causes problems with choking, many ALS patients opt for a feeding tube. This is a relatively simple procedure, which can greatly extend the quality and quantity of an ALS patient.

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Is there equipment available to help ALS patients breathe?

Daily exercise with a spirometer helps to keep the chest muscles from tightening. Non-invasive ventilation such as bi-pap can greatly extend the time before an ALS patient will require a ventilator.

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Do all ALS patients become bedridden?

 
Many ALS patients do not remain bedridden even though they may be totally paralyzed. Specially designed wheelchairs and lightweight, portable ventilators allow the patient to be mobile. Improvements in technology allow paralyzed ALS patients much more freedom of movement than in years past.

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What other health problems are caused by ALS?

 
Several health problems can occur because of the immobility caused by ALS. All of these health problems are treatable. They include: skin breakdown, which can lead to bedsores; problems with constipation and painful joint contractions resulting from lack of movement.

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Is depression common among ALS patients?

 
Depression is common among ALS patients and may be more common among caregivers. Depression can be minimized through the use of antidepressant medication, counseling, and a strong network of supportive family and friends.

 

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Is ALS an expensive disease?

ALS is a very expensive disease.

The only FDA approved medication, Rilutek, costs approximately $700 a month. Other medications to treat ALS symptoms are also expensive. Adaptive equipment is expensive. A power wheelchair with tilt and recline features may cost $17,000. A home health aid for 10 hours a day may cost more than $30,000 a year. When an ALS patient goes on a ventilator, he or she will require skilled nursing care. This may cost in excess of $250,000 a year. Insurance companies may pay some of these expenses.

Medicare and Medicaid may pay some of these expenses.  

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This information was initially put together by Steve Weekes to help his family, friends, and other patients understand more about the disease.

 

 

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Rockin' For A Cure
April 12th, 2014

 

 

All Proceeds Benefit 
The ALS Association Wisconsin Chapter for Research and Patient Services

 

 



All Proceeds Benefit 
The ALS Association Wisconsin Chapter for Research and Patient Services

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Rockin' For A Cure
April 12th, 2014

        

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