Amyotrophic Lateral Sclerosis

Introduction

Amyotrophic lateral sclerosis or, in other words, ALS is one of the most widespread and dangerous neurological diseases that may affect all people, regardless their race as well as ethnic background. In general, ALS is associated with the death of neurons that results into numerous health disorders. The given paper aims at determining the nature of this lethal disease, its pathophysiology, manifestations, effective traditional methods of treatment as well as alternative therapies.

Systems Affected by ALS and the Pathophysiology of the Disease

It is rather important to note that ALS may be also referred to as Lou Gehrig’s disease. Considering its nature, it attacks neurons that are responsible for control of voluntary muscles controlled by the so-called person’s will, including arms, legs, and even face. ALS is included in the list of health-threatening motor neuron health problems that is characterized by such dangerous consequences as gradual degeneration and, finally, the death of neurons that are primarily located in the human brain and the spinal cord. In other words, motor neurons serve as effective communication links that set a connection among the nervous system of humans and various types of voluntary muscles. ALS causes the death of upper and lower motor neurons because when these neurons die, they stop transmitting the so-called messages to different muscles. Unable to function in an appropriate way, these muscles start to weaken and atrophy. As a result, the human brain loses the natural ability to control and operate voluntary muscles.

Taking into account the pathophysiology of this progressive disease, it is important to note that before a full destruction of motor neurons, they may develop numerous protein-rich inclusions that include in their structure a regulatory protein that is commonly referred to as ubiquitin. Besides, people who suffer from ALS have increased amounts of intracellular calcium that influences the extra release of neurotransmitters.

According to the research results, the life expectancy of people diagnosed with ALS is equal to three years as a rule. However, about 5 % of them may live with this disease either several months or even several decades. ALS causes such severe cellular processes as mitochondrial dysfunction as well as the generation and transmittal of free radicals. Despite the fact that this disease is classified as comparatively rare, two per one hundred thousand people face it each year. Researchers claim that adult individuals between 55 and 65 are the most vulnerable to this disorder. In addition, unhealthy ways of living, harmful habits, addictions, stresses, and athleticism may be referred to as possible contributors to the disease. Furthermore, professionals in the sphere of medicine claim that approximately 5-10 % of adult individuals inherit ALS. Unfortunately, clinical trials are ineffective, as no biomarkers can show the progression of the disease. The National ALS Registry provides data that ALS has proven to be more common among the whites.

Manifestations of the Disorder and Diagnosis

Researchers have estimated that the limb weakness is one of the most widespread and obvious manifestations of ALS. Besides, such symptoms as foot drop, difficulties in the process of walking, and, finally, shoulder weakness may affirm the early diagnosis of this lethal disease. Besides, such common symptoms as permanent weakness, eclipse as well as atrophy may caution about the ALS disease too. ALS may also have such typical manifestations as limb dysfunction and falls. That is why, people that are diagnosed with this disorder feel the need in caregivers that can help them to stand or walk.

Furthermore, with time, some people diagnosed with ALS may suffer from anarthria. Swallowing problems are typical of this kind of disease. They may result into malnutrition and dehydration that are usually followed by the loss of weight or aspiration. Permanent weakness and dysfunction of axial musculature may lead to the head drop that deprives patients with ALS of the opportunity to eat or drive a car on their own. In addition, the amount of patients that suffer from cognitive impairment because of ALS is equal to 25%. Scientists emphasize that various cognitive dysfunctions and abnormalities may result even into such health-threatening consequences as personality changes and the inability to speak, judge, or make decisions. These symptoms reduce chances of patients for survival and normal living.

The results of statistics prove that patients with ALS find it extremely difficult to control emotions and facial expressions. Besides, at the primary stages of this disease such symptoms as long-term depression and anxiety may be witnessed as well. However, anxiety, fear, and depression may also shorten the life expectancy of patients as these symptoms cause a reduced appetite, troubled sleep, feeling of hopelessness, and a failure to make decisions. In most cases, ALS makes patients diagnosed with ALS feel frustrated and helpless because of the loss of mobility. Psychological as well as physical discomfort always pursues people with ALS.

Such manifestations as shortness of breath, headaches, and, finally, weekend cough indicate intensification or, in other words, an acute condition of the disease. Such dangerous symptoms as respiratory failure or aspiration pneumonia are considered the most threatening ones that result into death of patients. As a result, having analyzed the main manifestations of this incurable disease, it is possible to summarize that they usually involve such phases as initial signs, progression of the disease, and the late stages of ALS. The initial stage of this lethal disease involves such basic symptoms as weakness and atrophy of muscles. Besides, other typical signs include troubled swallowing, awkwardness in the process of walking, and difficulties while formulating speech. At the stage of progression, amyotrophic lateral sclerosis is characterized by varied symptoms. However, the research results prove that many patients are deprived of the opportunity to control their muscles. They cannot walk on their own and perform handwork because their arms and hands do not function appropriately. Besides, survey-based clinician studies prove that the weakness and atrophy of muscles usually spreads to other parts of the body, including the face and head. This process is called the progression of the disease. However, patients under 40 usually feel slower progression of ALS. Finally, the late stages of the disease involve problems with breathing and the inability to perform such vital functions as eating, chewing, drinking, and swallowing. According to the investigations of researchers in the sphere of medicine, the majority of patients who are diagnosed with ALS die because of either pneumonia or respiratory failures. However, regardless of the incurable nature of this disorder, the life of patients may be prolonged with the help of numerous effective nursing interventions and alternative therapies.

Nursing Interventions that Treat ALS

Unfortunately, no effective cure for ALS has been found or developed by scientist. However, there are special drugs that can slow down the progression of this incurable disease. Riluzole is considered the only drug that is approved by specialized medical agencies. It is believed to be prescribed to people with ALS to slow down the course of the disease. According to the results of the first trial, the drug contributes to the slowed deterioration of the strength of muscles. However, the quality of life of patients with ALS is not increased even with the help of Riluzole. Moreover, the results of medical experiments provide efficient data that Riluzole that may be commonly referred to as Rilutek lengthens the life expectancy of clients for the period of three or four months. However, the Food and Drug Administration has approved Riluzole as this drug extends the period of time when patients need ventilation support in order to survive. Regardless numerous attempts of drug administrations to create a drug that will be able to renew damaged motor neurons, Riluzole does not perform this function. Besides, doctors usually recommend patients with ALS other types of effective medications that are created to control spasticity, lessen fatigue, reduce pain in muscles, and prevent excess saliva. In order to cope with depression, sleep disturbances, anxiety, and constipation, many doctors prescribe specialized pills. For instance, baclofen and diazepam are effective enough for those ALS patients that have problems with spasticity control. Trihexyphenidyl is recommended to those who are unable to swallow and chew. More than a half of the U.S. patients and approximately 90% of people with ALS in Europe take Riluzole.

Because of the progression of the disease, many patients feel the need in additional multidisciplinary care. It should be noted that the latter prolongs the life of patients with ALS. A care plan is as a rule centered on decisions of patients. Moreover, it involves such interconnected features as care and education of people. In general, neurologists bear the responsibility for the care of people diagnosed with amyotrophic lateral sclerosis. The role of ALS nurses is connected with providing usual nursing care to patients. However, it is estimated that the performance of such professionals as physical therapists, occupational therapists, psychologists, psychiatrists, and even dietitians is not less important as they contribute to physical, psychological, and emotional well-being of people with ALS. Numerous non-pharmacological therapies, including gaze communication devises, qualitative voice amplifiers, walkers or wheelchairs are important as they improve the quality of life of ALS patients. It should be stated that such medical procedure as NIPPV or, in simple words, positive-pressure ventilation is important for those people who feel the weakening of respiratory muscles. Special equipment that resembles an intermittent ventilator is required for those with respiratory insufficiency in order to imitate vitally important physiological functions. NIPPV does not only extend the survival process, but increases the quality of life and improves cognition.

Alternative Therapies

Despite the fact that the diagnosis of ALS involves devastating consequences because there is no medicine that can cure this disease, patients can choose alternative treatment in order to prolong their life. Being disadvantageous because of its price and side effects, Rilutek may be replaced by alternative medications and complementary therapies. Researchers do not recommend ALS patients to use “off-label” drugs or, in other words, medicine that has not been approved by legal bodies. The use of “off-label” medicines can cause numerous problems. For example, these drugs can accelerate the progression of the disease. Moreover, they are usually not rigorously tested. That is why a physician cannot prescribe the “correct” dose of these medications. The majority of physicians oppose the idea of the so-called poly-therapy or the use of several drugs at one time.

Non-conventional or “natural” therapies are beneficial for patients with ALS. For example, appropriate nutrition, healthy supplements, and detoxification can serve as effective “cures” for such people. These alternative therapies may “beat the system” and renew the natural state of health as well as vibrancy. Numerous herbal supplements slow down the development of ALS. Scientists prove that many herbs include similar active molecules as drugs that are produced in a synthetic way. For example, the Canadian government has introduced special legislation policies that control the processes of production, labeling, distribution, and sale of herbal medicines to patients with ALS. Manufacturers, importers, exporters, packagers, and, finally, labelers should follow strict regulations in order to have the authority to distribute products of alternative medications. Besides, herbal medicines are subjected to dozens of observational studies, experimentations, and reports of expert committees in order to be supplied to the world markets.

Some researchers recommend stem cell therapy as one of the effective treatments of ALS. However, an expensive cost of this procedure is one of the biggest disadvantages. Besides, complementary medicine may be used together with conventional medications in order to strengthen the positive effect of treatment. For instance, such complementary therapies as mediation, acupuncture, and hypnosis have proven their efficiency. Moreover, clinical and trial data prove that these therapies reduce stress, anxiety, fear, and discomfort. In general, acupuncture and herbs are considered the most effective alternative treatment methods of ALS. Acupuncture is an extremely efficient procedure because it influences the activities of the nervous system. Besides, it slows down the atrophy of muscles due to nourishing and invigorating them. Moreover, appropriate herbs are beneficial to the human brain, spinal cord, and various muscles. Herbs may not only affect the regeneration of damaged neurons, but also soften sclerosis. Doctors believe that traditional drugs together with acupuncture, massage, herbal medicine, and an appropriate diet, that includes vitamins, are aimed to reduce detoxification, repair damaged nerves, stimulate the work of the nervous system, and counteract the progression of the disease.

Current Research on ALS

Unfortunately, professionals in the sphere of medicine have not succeeded in founding a cure for this degenerative and lethal disease. However, researchers have managed to determine the factors that contribute to the development and progression of ALS. The results of experiments prove that heavy metal toxicity, complicated head or neck trauma, some genetic predispositions, extremely low amounts of calcium, vitamins, and magnesium, decreased levels of fatty acids, and dangerous pesticides are among such factors.

At present, enormous amounts of clinical trials are conducted in various countries around the globe. Currently, approximately twenty-five hundreds of people are diagnosed with ALS on the territory of the USA. An average age of patients is equal to 50-55 years. The project called MinE that is initiated by people with ALS is one of the most effective genetic studies that contributes to the research on the disease. Besides, there is one more international and placebo-controlled study named “BENAFIT-ALS” that involves approximately 700 hundreds of participants. Finally, it is important to note that hundreds of effective laboratory tests, electrodiagnostic investigations, and genetic studies are conducted in various countries, especially in Europe and the USA.

Conclusion

As a result, having analyzed and evaluated the nature of amyotrophic lateral sclerosis, its initial manifestations, progression, final stages, and traditional as well as alternative methods of treatment, it is possible to sum up that unfortunately researchers have not found a cure for this progressive neurological disease. Amyotrophic lateral sclerosis kills motor neurons that are located in the human brain, brain stem, and the spinal cord. This disease results into gradual degradation and death of motor neurons. In simple words, ALS patients lose the ability to walk, speak, eat, and even swallow. The most common symptoms include muscle weakening, atrophy, disability to perform voluntary movements by hands, legs, and face, depression, failure to breathe without the so-called ventilatory support, and drastic changes in cognitive functions that negatively influence the memory and processes of decision-making.