ALS primarily affects the upper and lower motor neurons, which only have an average survival time of 3-5 years. These neurons are the early indicators of ALS (Doyle, Jr., et al, 2000). Early symptoms of ALS vary from patient to patient, and it depends on the mild and relative intensities with which the pyramidal cells in the motor corte and motor neutrons in the brainstem and spinal cord are. It is often missed diagnosis for cervical spondylitic mylopathies, radiculopathies, various myleopathies and mutifocal motor neuropathies. All of which have similar early symptoms as ALS (Doyle, Jr. et al, 2000). How ALS differs from these diagnoses is the pathphysiological changes in an ALS patient. The pathophysilogical changes in ALS are dictated by which motor neurons are lost. The surviving motor neuron either adapt to the losses of motor neurons or they themselves are affected by the disease (Doyle, Jr., et al, 2000) Other central systems may be affected later as the disease progresses but the loss of motor neutron in the nervous system is the first stage (brain, spinal cord, and nerves).
The loss of motor neurons reduces the numbers of motor units (a single alpha-motor neuron and all of the corresponding muscle fibers it innervates; all of these fibers will be of the same type) and thins out the muscles (see figure 1) (Doyle, Jr., et al, 2000). Different studies of ALS in relation to motor units have agreed that ALS patients all have rapid loss of in motor units. This rapid loss is a lot quicker than regular age -related loss. A study from Dantes and McComas shows that initially that the loss of motor neurons is very rapid, however as the disease progresses this loss begins to slow down. (see figure 2) (Doyle, Jr., et al, 2000).
Due to the loss of motor neurons the surviving few have to adapt. This adaptation includes the development of sprouts from axons of surviving motor neurons, forming new synaptic contacts between the sprouts and the muscle fibers (Doyle, Jr., et al, 2000).
It can also cause increases in muscle fibers, and increases in the twitch tension of muscle units. Nonetheless this adaptation also has benefits such as an increase in the size of motor unit action potentials (Doyle, Jr., et al, 2000). Among the loss of motor neurons it was also found with preservation of the forsal roots that the ventral nerve roots are subject to thinning and discoloration (see figure 3).
To diagnosis ALS, neuroimaging, such as magnetic resonance imaging (MRI) or computed tomography (CT) can be used. These play an essential role because they identify a treatable condition that has the same symptoms as ALS, and point out pathophysiological changes in the central nervous system that are relatively specific for ALS. All of this assists in making the diagnosis for ALS (Doyle, Jr., et al, 2000). Furthermore alternative devices such as neuroimaging, are now being used for research purposes that may find cure or prevention methods.
There are anatomic changes in the brains and spinal cords of patients with ALS. This is due to the neurodegeneration (Doyle, Jr., et al, 2000). MRI is mostly used in identifying ALS because of its ability to reveal parenchyma details. Some of the MRI changes with patients suffering from ALS have found increased signals along the corticospinal tract on T2-weighted in the posterior limbs of the internal capsule and extending into adjacent fronto parietal white matter (see figure 4) (Doyle, Jr., et al, 2000).
Although today, there are no treatments that halt or reverses for ALS, there is one FDA approved drug, riluzole that slows the disease down and gives the patients another 1-3 months to live (ALS site). Recent studies are using gene mutation on mice to see if they some day just alter the gene for some cases.
ALS can be in heredited but only in a small percentage of families, the majority of the patients (90%) have no family history of ALS (ALS site). There are at least 3 different inheritance for family related ALS. The most common is autosomal dominant; it is located on chromosome 21 and called superoide (SOD1) (ALS site). The SOD1 gene is composed of five regions called exons. Its normal role is to detoxify free radicals that can be harmful to motor neurons (ALS site). To locate this genetic factoring ALS, a study did a mutation with mice in Cu/Zn-SOD1 (Eisen, Andrew, and Charles Kreiger, 1998). The mice with this mutation showed similar symptoms to patients with ALS. The only odd result was that posterior horns of the spinal cord remain unaffected, even in the aged mice (Eisen, Andrew, and Charles Kreiger, 1998). Vacuolar changes appear to extend from the rough endoplasmic reticulum with the swelling of the organelle (Eisen, Andrew, and Charles Kreiger, 1998).
In recent studies, they have found that there is a change in the rough endoplasmic reticulum (Eisen, Andrew, and Charles Kreiger, 1998). Impaired ultrastructural changes in the motor neurons associated with changes in the rough endoplasmic reticulum derives from experiments on partial central differentiation where a persistently elevated frequency of C-type synapses is seen (Eisen, Andrew, and Charles Kreiger, 1998). The C-type synapses are characterized by a 15nm wide cistern beneath the postsynaptic membrane which extends over the entire length of the presynaptic terminal (Eisen, Andrew, and Charles Kreiger, 1998).
Symptoms of ALS include muscle weakness, cramping of muscles, twitching of the muscles, impairment of arms or legs, "thick speech" and difficulty in voice projection. In advance stages there would be shortness breath, difficulty in breathing and swallowing (ALS Site). All these symptoms are due to the loss of these life depending motor neurons.
All ALS patients come to a time in their physical degeneration when they are totally dependent, and physically unable to digest and control bowel movements. However ALS patients are still “intelligent enough to reason and are emotionally able to feel despair and indignity” (Doyle, Jr., et al, 2000). At this time, patients will often refuse treatment and suffer through tremendous amounts of pain until their death (paquin, 2003). Physician-assisted suicide (PAS) and active euthanasia (AE) are two choices that could help relieve the suffering of all of these issues. PAS refers to physician providing the means of death in a prescription form. AE means that the physician would act by injecting a lethal drug that would kill the patient immediately (Birnie, 1994).
PAS and AE offer ALS patients the choice to die instead of just refusing treatment. In The State of Oregon PAS and AE have been legal since 1997, helping many ALS patients die peacefully (Dignity Act). In a case study on ALS patients in Oregon, 56% of ALS patients reported they would consider assisted suicide (Ganzini). It was also reported that on average 0.4% of death in terminally ill cancer patients and 33% of ALS patients die with physician-assisted suicide in 1997” (Doyle, Jr., et al, 2000) AE. Furthermore it was found that men with higher education and a higher quality of life were more likely to consider PAS or AE (Ganzini). According to the study most patients who wanted PAS or EA “wanted control over her/his life at a time when every day was a struggle to regain control over a lost function" (Birnie, 1994).
Human autonomy is what makes humans morally superior to other animals. It is the “independence or freedom, as of the will or one's actions: the autonomy of the individual” (Barber, 2001). This is the quality that lets us humans create plans and goals, and set out to do them (Birnie, 1994). Through human autonomy, humans are able to create individuality through cognitive, emotional and physical skills (Wade, 2001). Within law people can not hurt another human’s autonomy but the autonomy itself can cause emotional and physical harm to ALS patient. “It can be intolerable to watch oneself rapidly and increasingly lose control over physical abilities and ordinarily define individual independence and privacy” (Doyle, Jr., et al, 2000). In order for PAS/AE to be performed the patient must first give consent. The reason many ALS patients consent is because is provides the choice to die with dignity and not in pain.
ALS patients go through tremendous amount of physical and emotional pain (Wade, 2001). ALS patients receive pain killers to help reduce pain and will often receive a J-tube which is inserted into the upper small intestine called the jejunum to help them digest (ALS site). Over time ALS patients’ loose control over their throat muscles and food has to be dislodged into to their air ways (Wade, 2001 ). As ALS patients physically deteriorate, they will often go through emotional pain such that most of them ALS “don’t know how to die” (paquin, 2003) or question “who owns my life?” (Niemer). By refusing treatment patients will only suffer more but with PAS and AE patients die quick and pain free (Wade, 2001).
The counter dictating arguments for PAS and AE is that it “demeans the value of human life,” (ALS Website)“it violates doctors’ Hippocratic Oath” (ALS website), and “doctors and families may be prompted to give up on recovery much too early” (ALS Website)If it became legalized in Canada, Canadian laws would be changed, so that a doctor administering AE would not be going against the Hippocratic Oath [the famous phrase;
“Above all, do no harm” (Doyle, Jr., et al, 2000) .
In Oregon, there are specific codes and rules the doctors have to follow (so that doctors can not give up on recovery too early) prior to giving AE or PAS (Ganzini). A Canadian survey shows that in Canada 52% of Canadians said that PAS and AE should be legalized and 48% of Canadians said no. When the survey asked if people have the right to die, 75% of Canadian agreed (Birnie, 1994). What are your thoughts about AE and PAS? Should they be legalized in Canada to relieve patients of pain and suffering? What regulations should be placed if this was to occur?
Bibliography:
Work
Eisen, Andrew, and Charles Kreiger. Amytrophic Lateral Sclerosis: A Synthesis of Research and Clinical Practice. United Kingdom: Cambridge UP, 1998. Print.
This book had information on Physician-assisted suicide (PAS) and active euthanasia (AE)
The ALS Association. Sept. 2008. 9 Mar. 2010
This Website has general facts about ALS
Barber, Katerine, ed. Canadian Oxford dictionary. Don Mills, Ont: Oxford UP, 2001.
Used to help define words.
Birnie, Lisa Hobbs, and Sue Rodriguez. Uncommon Will: the Death and Life of Sue Rodriguez. Toronto: Macmillan Canada, 1994. Print.
Sue Rodriguez was a woman with ALS who went to the supreme court and as for PAS in Canada.
Doyle, Jr., et al. Amyotrophic Lateral Sclerosis. New York: Informa Healthcare, 2000.
This online book had detail information on how ALS is cause and the symtoms
Ganzini, Linda, Predictors and Correlates of Interest in Assisted Suicide in the Final Month of Life Among ALS Patients in Oregon and Washington, Sileria, Maria. Elsevier. 15 Apr 2010
This website explain the case with ALS and PAS/EA in Oregon, Washington
Paquin, Susan L. I Don't Know How to Die. Baltimore: PublishAmerica, 2003.
This book was first person narratored book about a woman in her 40s with ALS
Wade, Mary Dodson. Als-Lou Gehrig's Disease (Diseases and People). New York: Enslow, 2001.
This book gave a bibliography of Lou Gehrig, the baseball player and his fight with the disease
Niemer, Ellen. "Who owns my life?" May 2008. Alt HealthWatch. Greenboro, Ottawa. 7 Apr. 2009
Sue Rodriguez was a woman with ALS who went to the supreme court and as for PAS in Canada.
State of Oregon: Death With Dignity Act." Oregon.gov Home Page. 21 Apr. 2009
This website explain the case with ALS and PAS/EA in Oregon, Washington
Figure 1
The ALS Association. Sept. 2008. 9 Mar. 2010
Figure 2
Doyle, Jr., et al. Amyotrophic Lateral Sclerosis. New York: Informa Healthcare, 2000.
Figure 3
Eisen, Andrew, and Charles Kreiger. Amytrophic Lateral Sclerosis: A Synthesis of Research and Clinical Practice. United Kingdom: Cambridge UP, 1998. Print.
Figure 4
Eisen, Andrew, and Charles Kreiger. Amytrophic Lateral Sclerosis: A Synthesis of Research and Clinical Practice. United Kingdom: Cambridge UP, 1998. Print.
No comments:
Post a Comment