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Myasthenia Gravis

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| Research Paper Part 1 – Introduction & Body Paragraphs | EN 1320 | | Anthony Brown | Week 8 |

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Myasthenia Gravis (pronounced My-as-theen-a Grav-us) derived from the Greek and Latin words and it means “grave muscle weakness.” Myasthenia Gravis (MG) is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body ("National institute of," 2012). The prevalence of MG in the United States is estimated to be about 20/100,000 people ("What is myasthenia," 2010). MG is a rare but very serious disease and many people who have been diagnosed do not recognize the symptoms. If detected early, MG can be treated successfully with, if any, very little side effects in the future. Once diagnosed with MG, that person must go through a drastic life change; Enduring rigorous treatments depending on how aggressive the disease has become, possibly undergoing a life threatening surgery, and living with myasthenia gravis for the duration of their life.
“Muscle weakness caused by MG progresses as the effected muscle is used repeatedly. Since symptoms typically improve with rest, but your muscle weakness may come and go” (Staff, 2010). The first signs and symptoms of MG are ptosis and diplopia. “Ptosis is a drooping of the upper eyelid. The lid may droop only slightly, or it may cover the pupil entirely” (What is ptosis?, N.d.). “Diplopia (double vision) is a common subjective complaint, or diplopia may be elicited during the course of an eye examination. Diplopia is often the first manifestation of many systemic disorders, especially muscular or neurologic processes” (Diplopia, N.d.). During the beginning stages of MG some people may show no concern of their muscles becoming fatigued faster because with adequate rest, the muscles will regain their strength back. What the person may not know is even though the muscles are gaining their strength with rest, the muscles are not gaining 100% of their strength back. This happens because the symptoms of MG will progress over time, eventually rendering a person helpless.
After a diagnosis has been made, medication and therapy happens extremely fast. Your neurologist has to move fast and aggressively with this disease because the longer a person goes without treatment, the harder it becomes to treat. Neurologist will prescribe a series of medication at the beginning to see how a person’s body will respond. The two most common medications are Mestinon (pyridostigmine) and CellCept (mycophenolate). Pyridostigmine affects chemicals in the body that are involved in the communication between nerve impulses and muscle movement. Pyridostigmine is used to treat the symptoms of myasthenia gravis ("Mestinon," 2012). Pyridostigmine sends signals to the nerve receptors within the muscles telling them to function. Pyridostigmine is prescribed 60mg (one pill) four times daily. Pyridostigmine does work short term, but works best when used in conjunction with a chemotherapy (CellCept) medication. CellCept (mycophenolate) lowers the body's immune system. The immune system helps the body fight infections. The immune system can also fight or "reject" a transplanted organ such as a liver or kidney. This is because the immune system treats the new organ as an invader. CellCept is used to prevent the body from rejecting any foreign organ ("Cellcept," 2012). Both, pyridostigmine and CellCept work together to fight the symptoms of Myasthenia Gravis. These drugs do not cure the underlying problem, but they do improve muscle contraction and muscle strength (Staff, 2010).
Another form of therapy for MG patients is Intravenous Immune Globulin (IVIG). IVIG provides the body with normal antibodies, which alters the immune systems response. It has a lower risk of side effects than does plasmapheresis and immune-suppressing therapy, but it can take a week or two to start working, and the benefits usually last no more than a month or two (Staff, 2010). IVIG is an infusion of hemoglobin, which is collected from people who donate blood. It is then put through a filtration process where the hemoglobin is separated from the blood. Myasthenia Gravis patients then go through an eight hour five day infusion process. One of the main side effects of IVIG is intense migraines that can last upwards to two weeks. Depending on how a person’s body will respond to the medications and the IVIG infusions, the neurologist will dictate how much further to go in the treatment process.
Considered to be the most critical treatment of the MG is the removal of the thymus gland (thymectomy). This is a life threatening surgery to remove the thymus gland. It is located in the upper portion of the chest behind the sternum. The surgery is very painful because the doctors cut through the sternum with a saw and then pry the chest open. A person can be in surgery for upwards to 5 hours depending how enlarged the thymus gland is. The larger the thymus gland, the larger it is to remove because it can wrap itself around the esophagus.
The thymus gland, which lies in the chest area beneath the breastbone, plays an important role in the development of the immune system in early life. Its cells form a part of the body's normal immune system. In adults with MG, the thymus gland remains large and is abnormal. It contains certain clusters of immune cells indicative of lymphoid hyperplasia—a condition usually found only in the spleen and lymph nodes during an active immune response (National institute of, 2012).
The thymus gland is considered to be the base for the antibodies within the human body. When a person gets ill, the thymus gland will release antibodies to fight off the illness. For a person with MG, rather than attack the disease, the thymus gland will attack the body. After going through different types of treatment medications and therapy, living with MG for the rest of a person’s life can become quite overwhelming. Once the neurologist is able to stabilize the MG, in order to keep it at bay and not encounter a myasthenic crisis, one must go through multiple sessions of chemotherapy. The chemotherapy drug is a combination of Cytoxan and Solu-Medrol. While it is not directly stated how the two drugs work with MG, I am living proof that they do indeed work. I have been receiving chemotherapy for thirteen months now and this time last year (November, 2011) I could not walk without a walker. Receiving monthly treatments of chemotherapy is overwhelming because I have to plan my schedule around the treatment days. Myself, I do not encounter the usually side effects of nausea, vomiting, loss of appetite or weight, abdominal pain, diarrhea, hair loss, sore on the mouth or tongue, changes in skin color, or changes in color or growth of finger or toe nails. Actually the only side effect that I do encounter is fatigue. Constantly I am thinking “what if I fall, who would help me up” or “are my shoulders getting weak?” These thoughts play in my mind every day because to the average person I look completely healthy, so if I was to fall and not be able to get up, who would help me and who would just stare at me. Nevertheless I was diagnosed with Myasthenia Gravis on February 21, 2009. I had no prior knowledge to what this disease was or what it could do to a person. After my diagnosis, I suffered a myasthenic crises and I would not wish that upon anyone. My neurologist prescribed pyridostigmine and mycophenolate to see where my level of MG was at. The pyridostigmine worked but only for a short period time throughout the day. I would take it every four hours on the dot. If I forgot or was even five minutes late, my body would definitely let me know. I wouldn’t see any progress from the mycophenolate until two years after consistently taking it. I remember the treatment stages of my disease really well because I couldn’t understand why this disease was not made public. By writing this paper I hope to bring awareness about this disease to the public because I don’t want anyone to have to suffer the way I did.

Annotated Bibliography
What is myasthenia gravis (mg)?. (2010). Retrieved from http://www.myasthenia.org/WhatisMG.aspx
The Myasthenia Gravis Foundation of America (MGFA) is the only national volunteer health agency that is dedicated solely to the fight against myasthenia gravis in the United States. MGFA is committed to finding a cure for MG, improving treatment therapies.
National institute of neurological disorders and stroke. (2012, October 15). Retrieved from http://www.ninds.nih.gov/disorders/myasthenia_gravis/detail_myasthenia_gravis.htm
The National Institute of Neurological Disorders and Stroke (NINDS) is one of the National Institutes of Health (NIH). The NINDS has the primary responsibility for conducting and supporting research on brain and nervous system disorders, including myasthenia gravis.

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