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Overview and Pathophysiology of Myasthenia gravis (MG)
Myasthenia gravis (MG) is considered as a very common neuromuscular transmission disorder. The most causal cause behind this disorder is the acquired immunological abnormality. In the some of the cases it has also been reported that genetic abnormalities at the neuromuscular junction can also lead to this disorder (Anom Arie et al, 2013). The facts related to Myasthenia gravis are often under-diagnosed, and women are more tend to suffer from this disorder. This disease is now understood as an autoimmune disease and neuromuscular disorder, which results in weakness of the skeletal muscles. These muscles are very important for different movements in body. This disorder hampers the communication between the nerve cells and muscle cells (Hickey, 2013).
The main reason behind the occurrence of this disorder is due to autoimmune problems. Autoimmune disorders are caused, when the immune system mistakenly affect the healthy tissues (Jayam Trouth et al, 2012). In such situation that antibodies inside the body, which are proteins and normally attack foreign bodies, attacks the neurotransmitter substance acetylcholine (Ach) (Howard, 2016). This substance is very essential for communication between muscles and nerve cells. With every single impulse the amount of the released Ach from the presynaptic motor neuron is generally decreased (Farrugia, & Vincent, 2010). This happened due to the temporary reduction in the presynaptic ACh stores. This phenomenon is also called as the presynaptic rundown.
It is observed that in MG, there is a decrease in the number of AChRs, which are found at the muscle endplate, which also leads to flattening of the postsynaptic folds (Howard, 2016). Even if the normal amount of the ACh is released, it could only generate potential in few end plates and threshold value for potential generation could not be reached. Normally present phenomenon of presynaptic rundown and inefficient neuromuscular transmission results in the potential reduction in the amount of the muscle fibers, which are being activated by the nerve fiber impulses. This phenomenon explains the tiredness, less energy and fatigue in MG patients (Engel, 2012).
The indications are seen in the physical condition of the patient, when the number of Ach is decrease to approximately 30% from the normal level (Howard, 2016). It is also observed that smooth cardiac muscles have cholinergic receptors. These receptors have different antigenicity then skeletal muscles, thus they remain unaffected from the disease. It is observed that “decrease in the number of postsynaptic AChRs is believed to be due to an autoimmune process whereby anti-AChR antibodies are produced and block the target receptors, cause an increase the turnover of the receptors, and damage the postsynaptic membrane in a complement-mediated manner” (Anom Arie et al, 2013).
According to the clinical observations, immunogenic mechanisms play a very significant role in Pathophysiology of MG. This observations states that MG is caused due to presence of other autoimmune disorders such as, autoimmune thyroiditis, rheumatoid arthritis [RA]), and systemic lupus erythematosus [SLE] (Howard, 2016). Such disorders are also found in the patients suffering from Myasthenia gravis (MG). It is also noted that children born to myasthenic mothers can develop myasthenialike syndrome (Jayam Trouth et al, 2012). Such patient responds to various therapeutic treatments such as “immunomodulating therapies, including plasmapheresis, corticosteroids, intravenous immunoglobulin (IVIg), other immunosuppressants, and thymectomy” (Howard, 2016).
Assessment of Myasthenia gravis
History | In 50 % of the patients suffering from MG, it is found that ptosis or Extraocular muscle weaknessis present, and this usually occurs during the 90% course of the illness. Bulbar muscle weakness is commonly identified, along with the weakness of flexion and head extension. The weakness may also encompass limb musculature associated with myopathylike proximal weakness. This kind of weakness is greater than the distal muscle weakness. The identification of the limb muscle weakness is very rare and occurs only less than 10% patients (Anom Arie et al, 2013). Understanding the history of the disease in the patient is very important for the proper diagnosis of the disease. According to the history and its examination, it is observed that patient condition changes from mild to severe over the course of few weeks or even months. The muscle weakness spreads from ocular muscle to facial; from facial to bulbar muscle and then towards truncal and limb muscles. However, on the other hand it has also noticed that symptoms of the disease remain limited to eyelid muscles and extraocular muscles. It is rarely seen that patient with severe condition of this disease may not have associated with weakness of ocular muscles. The reaction of sunlight, immunization, surgery, and menstruation must be determined, as they may often get worse with patient’s condition (Burns, 2010). |
Physical Examination | The patients suffering from MG can display wide range of physical signs and symptoms that often depends upon the severity of the disease. The presence of mild symptoms is related to subtle findings of ptosis, and these are limited to bulbar muscles. The proper findings may not occur or become apparent till the time muscle weakness is not provoked to repeated use of the muscles (Zisimopoulou et al, 2014). The recovery of the muscles is seen after the period of rest, by applying ice to the affected muscles. On the other hand increase in the temperature can worsen the muscles condition. Demonstrable findings could be absent and only weakness may appear. Such condition can also lead to misdiagnosis for example, it could be diagnosed as functional disorder and not as MG. Important aspect of physical assessment in the patients must include the recognition of imminent respiratory failure. The identification of difficulty in breathing requires immediate evaluation and treatment. Physical examination also requires sensory examination as weakness is present in various sensory muscles such as proximal and symmetric (Berrih-Aknin, Frenkian-Cuvelier, & Eymard, 2014). According to the studies it is clear that MG is an autoimmune disorder, thus it is also important to diagnose other autoimmune diseases in the patients. |
Investigations |
The investigation of Myasthenia gravis (MG)can also display other diagnosis in aged people. Due to the unclear symptoms and signs, the diagnosis and investigation of this disease may be delayed by years. Anti–acetylcholine receptor antibody: The first investigation to be carried out is Anti–acetylcholine receptor antibody. This test is considered as very reliable for the diagnosis of autoimmune myasthenia gravis (MG). The results found through this test are 90% positive in generalized MG (Skeie et al, 2010). Anti–striated muscle antibody: Another investigation is Anti–striated muscle antibody. This investigation is important as Anti-Sm AN is present in 84% of the patients, who have thymoma, and these are the patients who are younger than 40 years and less. Anti-MuSK antibody: This investigation is important to identify positive test of muscle-specific kinase (MuSK). This kinase is important for the development of neuromuscular junction (Guptill, Sanders, & Evoli, 2011). Some of the other important investigations are Anti-lipoprotein-related protein 4 (LRP4) antibody, Antistriational antibody, and test for rheumatoid factor and antinuclear antibodies (ANAs). |
Nursing care Goals
Proper Gas Exchange: The first nursing care goal includes assisting the patient in proper gas exchange. Due to the respiratory failure, patient may not be able to exchange gases normally. The impaired gas exchange occurs due to the muscle weakness and ineffective breathing pattern in patient suffering from MG. This also leads to the risk of aspiration, which is related to difficulty swallowing. The expected outcome for this nursing goal is no occurrence of aspiration. The nurses are responsible for providing quality care and thus this goal is very important to be achieved. This goal requires patient to maintain a respiratory rate of 12-20 with ADL’s and oxygen saturation of >92%(Fowler et al, 2013).
Self-Care Deficit: Self care deficit is associated with muscle weakness and general fatigue. This nursing goal is to encourage patients to perform their self-care activities and gain more independence. This is also important so that patient may demonstrate the ability to use the adaptive devices (Fowler et al, 2013). Due to the muscle weakness, patients are often unable to complete their self care tasks accurately, and thus depend on their family members or support team. This goal will help to gain the desired outcome of improving independence of patients. Due to muscle weakness, patient often suffer from impaired physical mobility. That can also be improved through this nursing goal.
Improve Imbalanced Nutrition: Providing balanced nutrition to body is important for better health and movement. The patient suffering from chronic illness of MG faces difficulty in chewing and swallowing food (Nutrition and myasthenia gravis (MG), 2010). Due to this, they could eat properly and often suffer from deficiency of nutrition. To maintain the normal functions of the body, it is important to intake balanced diet with adequate nutrients. The goal is to prevent problems like dysphagia, intubation, or muscle paralysis, which are related to the imbalance of nutrition according to the body requirements (Weeks, 2012).
Nursing Strategies or Care Plan
Ineffective breathing pattern or Impaired Gas Exchange:
The purpose of this strategy is to aid patient in maintaining the proper gas exchange. Interventions may include:
Self-Care Deficit:
The purpose of this intervention is to understand the current capability of the client and encourage them to complete 25% of their tasks by themselves (Gulanick & Myers, 2013).
Nutritional Balance: Nutritional imbalance in patients suffering from MG is very common, this is because patients face many challenges while eating, chewing and swallowing their food.
Purpose of this intervention is to save the patient from nutritional deficiency (Gulanick & Myers, 2013).
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