Day in The Life_ Myasthenia Gravis

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Day in The Life: Myasthenia Gravis Pascale Galarneau-Haley Queen's University PHGY 215: Mammalian Physiology I Dr. Chris Ward November 17, 2023
Morning : For someone with MG, even opening their eyes in the morning can be a problem. Because the disease affects muscle strength, even the muscles in the eyes controlled by the autonomic nervous system that allow for eye movement and depth perception are impacted, thus leading to droopy eyelids, double vision and trouble focusing. Double vision can sometimes be managed by wearing an eye patch or putting an icepack on the eyes (Trouth et al., 2012). Getting out of bed and moving also poses a challenge for patients due to muscle weakness generated by altered communication at the neuromuscular junction (NMJ). Typically, electrical impulses from α -motor neurons of the ventral horns of the spinal cord and brain stem stimulate the release of a neurotransmitter called acetylcholine from the synaptic button into the synaptic cleft. Acetylcholine then binds to postsynaptic membrane receptors on the muscle triggering off the end plate potential (EPP) stimulating a contraction. However, in patients with MG, antibodies block or destroy the acetylcholine muscle receptors thus preventing muscle contraction (NINDS, n.d). This reduced amount of acetylcholine molecules at the NMJ diminishes EPP. This EPP may be sufficient at rest, but it may fall below the threshold required to initiate the action potential during repetitive activity. This leads to clinical muscular weakness, which becomes chronic when resting EPP is continuously below the action potential threshold. To help mediate this muscle weakness and allow patients to start their day, medication that enhances neuromuscular transmissions can be taken. One such medication is called Mestion. It’s an acetylcholinesterase inhibitor that works to inhibit acetylcholinesterase, the enzyme that breaks down acetylcholine in the synapse. This reduces the destruction of acetylcholine, thus enhancing the amount of neurotransmitters that can bind to receptors on the muscles and increase neuromuscular communication and strength (Figueiredo, 2023). Only once this medication has started to take effect, around 30 minutes, can the patient get out of bed and start their day. For personal hygiene, electric toothbrushes, electric shavers, and low maintenance hairstyles are preferred
to avoid muscle fatigue (NINDS, n.d). In terms of breakfast, soft moist foods are ideal as they are easier to chew considering the weakened masseter jaw muscle of some patients. Extra precaution must also be taken as there is a higher risk of asphyxiation. Touch receptors in the pharynx trigger the autonomic nervous system and muscles propel the food back into the esophagus. Simultaneously, the epiglottis moves to cover the trachea and prevent food from traveling down towards the lungs. In patients with MG, the muscles responsible for this mechanism weaken and fatigue much faster, thus increasing the risk of choking. This risk can be reduced by eating slowly, eating softer foods, and cutting food into smaller pieces (Melensky, 2022). At this stage, patients might consider taking anti diuretics such as loperamide to combat the side effects of Mestinon. The increased levels of acetylcholine created by the drug act in the gut to increase gastrointestinal motility, often leading to diarrhea (Bird, 2023). Afternoon : As the day goes on, muscle weakness and fatigue tends to worsen. This is why scheduled rest and naps are often recommended to allow time for recuperation. This relates to the pathophysiology of MG, as the continued presence of acetylcholine is required for muscle contraction. The autoimmune attack disrupts this process, leading to fatigue during sustained muscle activity (Maggi et al., 2012). Myasthenics on steroid based medications are prone to weight gain, hence, it can be advantageous to include light exercise throughout the day. During physical activity, it can be helpful to use assistive equipment, ask for help and avoid hot temperatures (Gutmann, 1980). Hot temperatures can actually exacerbate symptoms by affecting the efficiency of nerve impulses. Therefore, it can be beneficial to keep a cold towel nearby, exercise in air conditioned spaces, and wear breathable clothing while exercising. Additionally, exercise might cause breathing to become shallow and ineffective because of extreme weakness in the diphragm and chest muscles that support breathing. In some cases, the airway may become obstructed because of the
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weakened muscles and a buildup of secretions. This is why it's crucial for patients to listen to their body and take breaks when they feel they need it (De Wet, 2022). In the early afternoon, a second dose of medication must be taken to keep adequate levels of acetylcholine in the NMJ and combat muscle weakness. Mestinon is typically taken as a 30mg tablet 3 times a day with a meal (Maggi et al., 2012). In terms of lunch, soft and easily chewed foods are still preferred. At times, the muscle fatigue can be so grave that patients are unable to swallow their food. In these situations, feeding tubes are often used to ensure proper nutrition(Cereda et al., 2009). Night: Muscle weariness associated with MG is generally at its worst by evening. Certain patients may find it difficult to perform simple actions like walking up stairs or bathing, and they may require outside help. Writing, using a computer, and even texting on a smartphone all require fine motor abilities. This is a result of the autoimmune attack on neuromuscular transmissions, which can impair precise movement by causing weak and irregular muscle contractions (Figueiredo, 2023). Any activities done outside the house like shopping, eating out or going on walks may be difficult for patients at this stage in the day. Muscle weakness also impacts facial expressions and speaking which can make interactions with others difficult (Ruiter et al., 2022) For dinner, soft, easily chewable foods should be consumed with caution to avoid choking. Along with this final meal, patients should take their final dose of Mestinon to reinforce acetylcholine levels and maintain a certain level of muscle strength (Maggi et al., 2011). Sleep can present particular challenges for patients with MG. Many have sleep disorders that prevent them from getting a restful and regenerative night of sleep. Some have sleep apnea due to weakness in muscles responsible for breathing. If this is the case, patients may have to wear a Bi-PAP machine that forces regulated puffs of air into the lungs (Conquer MG, n.d). Some commonly prescribed medications for MG
patients may also disturb sleep. This is why sleep aids such as benzodiazepines are sometimes prescribed to help patients fall asleep (Madhav, 2021) . References Bird, S. (2023). Overview of the treatment of myasthenia gravis . UpToDate. https://www.uptodate.com/contents/overview-of-the-treatment-of-myasthenia-gravis Cereda, E., Beltramolli, D., Pedrolli, C., & Costa, A. (2009a). Refractory myasthenia gravis, dysphagia and malnutrition: A case report to suggest disease-specific nutritional issues. Nutrition , 25 (10), 1067–1072. https://doi.org/10.1016/j.nut.2008.12.016 De Wet, R. (2019, January 9). A day in the life of a person with Mg . Myasthenia Gravis News. https://myastheniagravisnews.com/columns/day-life-person-mg-muscle- weakness-walking-eating-fatigue/ Figueiredo, M. (2023, October 10). Mestinon (pyridostigmine bromide) . Myasthenia Gravis News. https://myastheniagravisnews.com/mestinon-pyridostigmine/ Gutmann, L. (1980, October 1). Heat-induced myasthenic crisis . Archives of Neurology. https://jamanetwork.com/journals/jamaneurology/article-abstract/579075 Living Day to day with MG . Conquer Myasthenia Gravis. (2022, January 11). https://www.myastheniagravis.org/life-with-mg/save-your-strength/#:~:text=You
%20may%20need%20more%20sleep,Learn%20what%20triggers%20your %20myasthenia Madhav, K. (2021). Sleep right, sleep tight. Myasthenia Gravis Foundation of America. https://myasthenia.org/MG-Community/Blog/sleep-right-sleep-tight Maggi, L., & Mantegazza, R. (2011). Treatment of myasthenia gravis. Clinical Drug Investigation , 31 (10), 691–701. https://doi.org/10.2165/11593300-000000000-00000 Melinosky, C. (2022). Myasthenia Gravis: What daily life looks like . WebMD. https://www.webmd.com/brain/features/myasthenia-gravis-life-with Phillips, W. D., & Vincent, A. (2016). Pathogenesis of myasthenia gravis: Update on disease types, models, and Mechanisms. F1000Research , 5 , 1513. https://doi.org/10.12688/f1000research.8206.1 Ruiter, A. M., Naber, W. C., Verschuuren, J. J. G. M., & Tannemaat, M. R. (2020, July 14). The face of Myasthenia Gravis . Neurology. https://n.neurology.org/content/95/2/89 Trouth, A., Dabi, A., Solieman, N., Kurukumbi, M., & Kalyanam, J. (2012). Myasthenia Gravis: A Review. Autoimmune Diseases , 2012 , 1–10. https://doi.org/10.1155/2012/874680 U.S. Department of Health and Human Services. (n.d.). Myasthenia Gravis . National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/health-information/disorders/myasthenia-gravis
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