AMANDA'S PAGE

Hi, my name is Amanda and I go to the University of Pittsburgh at Bradford in which I am majoring in the Nursing Program.  I hope to complete five years and end up with my Master's Degree. 

A Little About Me:

Favorite thing about college: Definitely the freedom

Favorite band/artist:  I like a lot of music and don't really have one favorite. I listen to many different bands such as Lifehouse, Finger11, Staind, Cold, Three Days Grace, Live, etc...it's a long list.

What I am most proud of : I am really proud of all my accomplishments.  I'm proud to say that I have a lot of academic accomplishments, along with music accomplishments. This however, is also with the help of my parents.

What I would like my life to be like 10 years from today: I think a lot about the future and what I would be doing in 5 or 10 years. I would really like to have at least my Master's Degree in Nursing, or I may decide to go on to Medical School. At any rate, I would like to be working in the medical field.  This type of occupation has interested me for quite some time and I think it really makes an impact on other people's lives, along with my own.  I love to help people, and this is one great way to use this part of me.  I would also like to have my own family.

My Favorite Quotation: "A man once asked, 'Jesus, how much do you love me?' And Jesus replied, 'This much!' and He stretched out his arms and died."

The most important thing that needs changing in the world: I have a strong Christian background, and have been brought up as a believer in Christ.  I think that one important thing that needs changing is the involvement of God in our society.  If you look, you always hear about bad things that is happening in the world. Well, God is about goodness, and I think that if you have more of Him in people's lives, there would be a definite change in today's world. 

AMYOTROPHIC LATERAL SCLEROSIS

(Lou Gehrig's Disease)

            Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease, is one type of motor neuron disorder that affects the Upper Motor Neurons (responsible for descending supraspinal innervation) and Lower Motor Neurons (responsible for the movements of skeletal muscles).  The symptoms begin due to the loss of these selective motor neurons, and as the cells continue to die the affected muscles cannot be stimulated, and they gradually waste away and that part of the body becomes increasingly weak.  

SYMPTOMS OF ALS INCLUDE:

• 

• Stiffening or weakening of the upper and lower limbs

•  Loss of finger dexterity

• Wasting hand muscles

• Severe muscle cramps

• Difficulty swallowing, walking, or any other muscle-powered activity

These symptoms begin to gradually take over the body’s many different functions, and by the time ALS is clinically diagnosed, there are malfunctions at the cellular level that include alterations in the cytoskeleton, mitochondrial function, microglial activation, and metabolism of reactive oxygenating species and glutamate.  The symptoms and defects gradually increase and the patient will usually die within five years from respiratory muscle paralysis.  However some people can live many years with ALS, such as the famous astronomer/author, Steven Hawking. 

This neurological disorder was once thought to be rare, but it affects between 1.2 and 1.8 out of 100,000 people.  It is also an age-dependent disorder and usually occurs in adults over 40 years of age. 

There are three main variants that are usually believed to explain ALS, which include the Western Pacific Type (often associated with dementia), Familial ALS (with mutations in gene coding for the antioxidant protein, copper/zinc superoxide dismustase), and Sporadic ALS.  

CAUSES OF AMYOTROPHIC LATERAL SCLEROSIS: 

Unfortunately, there is no known specific cause for ALS, but researchers have found many different explanations for the cause of this nerve-degeneration disease.

Researchers have studied 10% of ALS patients who had inherited the disease (Familial ALS) and have found that it is caused by an abnormal form of superoxide dismutase, an enzyme that would normally break down oxygen free radicals, which are toxic by-products of metabolism.  If these oxygen free radicals are not broken down, they build up and the motor neurons begin to degenerate in the cerebral cortex, the brainstem and the spinal cord and are then replaced by fibrous tissue. 

Other theories include: 

GLUTIMATE EXCITOTOXICITY:

            This theory suggests that there is an excess amount of glutamate in the brain.  This chemical occurs naturally, and plays a role in 30% of neurotransmission involved in quick responses to stimuli, memory, cognition, movement and sensation.  An excessive amount of this chemical occurs when it isn’t removed after nerve signals.  This excess then becomes neurotoxic because it over stimulates neuronal metabolic functions. This causes the motor neurons to take in too much calcium, which then leads to the disruption of other cellular functions, and thus causes cell death.  

PROTEIN AGGREGATES:

            Patients with familial and sporadic ALS have been known to have abnormal aggregates (clumps) of proteins in the motor neurons.  Although the significance is not truly known, some researchers believe that these aggregates are toxic to nerve cells.  Others state that they are not the main causes of nerve cell death but the aggregates may be byproducts from overwhelmed cells that are trying to repair incorrectly folded proteins.  The situation may also occur faster because of age-related changes or environmental factors.  

AXONAL STRANGULATION:

            This focuses on the neurofilaments, which are clumps of proteins that provide structural support for the axon of nerve cells.  But these can become tangled and the transports of nutrients are blocked from the cell body and cannot reach the axon, the long process of a nerve cell.  Without the proper nutrients, the nerve cells begin to slowly die from “strangulation”.  

OTHER FACTORS:

            There are other possible hypotheses of the causes of ALS that include viral infections, deficiency of nerve growth factor, apoptosis (programmed cell death) and trauma, along with environmental or occupational toxins.  Some of these environmental/occupational factors include trauma in the brain, spinal cord, and peripheral nerves, stress related illnesses, environmental lead and manganese, along with selenium in drinking water, the use of pneumatic tools, excess amounts or deficiencies of dietary nutrients, damage to DNA, and exposure to electric shocks.  This, however, is still questionable as there have been conflicting results and it has been hard to reproduce. 

As stated before, the specific cause of ALS is still unknown and it may be a combination of all these factors, or ones not yet discovered.  Therefore, because there is no known cause, there is no real cure.  There are treatments available that are designed to ease the symptoms and help the patient remain relatively stable and mobile and thus independent.  The treatments include non-invasive positive pressure ventilation, aggressive nutritional intervention, physical, occupational, and respiratory therapy, which all contribute to an

.

References:

ALS Survival Guide.  "Causes of ALS." 22 Aug 2003. 14 Oct 03.

     <http://www.lougehrigsdisease.net/als_causes_of_als.htm>.

Butler, Jackie, Ricki Lewis, and David Shier. Hole's Human Anatomy and Physiology. 10th

     edition.  New York: McGraw Hill, 2004. pg 379.

Finkel, Asher J. MD and Jeffrey R.M. Kunz, MD. The American Medical Association Family

     Medical Guide.   New York: Random House, 1987. pg 295.

Strong, Michael and Jeffrey Rosenfeld.  "Amyotrophic Lateral Sclerosis: A Review of

     Current Concepts."  Taylor and Francis Health Sciences. Vol. 4 #3. Sept 2003,

     pg 136-143.