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Myasthenia Gravis

Myasthenic crisis

02/17/2010

Question:

what type of care do u give for myasthenic crisis and what is it

Answer:

Crisis is severe exacerbation of myasthenic symptoms, and is differentiated from normal myasthenic symptoms by the severity, the continued downward trend, and a heavy emphasis on the worsening respiratory function. Signs of crisis can be worsening limb and trunk weakness, neck extensor weakness, increasing difficulties with swallowing and chewing, worsening double vision and droopy eyelids, and increasing shortness of breath.

Acute treatment: Admission I admit patients to the hospital when they begin to have myasthenic crisis when they start having breathing problems or problems swallowing. I teach my myasthenic patients to pay attention to their normal respiratory capacity. Most of my patients know how to do a simple test, I call the counting test, which we do over the phone if they call me at night. I ask them to count as high as they can on one breath. If this number starts to worsen, and worsen dramatically, this is a sign for admission for me.

Acute Treatment: Nursing In a hospital setting, where crisis is usually treated, I look to nursing as my frontline and expect nursing to watch for swallowing and chewing difficulties that might warn of aspiration; work with respiratory therapy to follow breathing tests to make sure a patient is not in danger of respiratory failure. Patients often end up with a feeding tube as it becomes less safe to take food by mouth and much higher risk for food to go down the wrong tube and end up in the lung rather than the stomach. This can provide problems with pneumonia and inflammation of the lungs.

Acute treatment: Respiratory Care I have respiratory therapists follow my patients at regular intervals (e.g. every 2 hours, every 4 hours), depending on how the patient is looking. If a patient starts reaching certain respiratory thresholds, like an FVC (Forced Vital Capacity), we have to discuss respiratory safety first. If the FVC becomes less than 20 mL/kg body weight, we begin discussing intubation and the ventilator (going on the breathing machine). At 12-15 mL/kg, I usually insist. I can follow these numbers and make sure they are trending up or down, and I tailor my treatment to that.

Acute treatment: Immune system treatment The next big question is how to turn the immune system back down. Depending on the patient's past medical history and the availability of drug or operators for the treatment, I either do IVIG (Intravenous Immunoglobulin) or plasmapheresis. Pheresis is more invasive and you need a surgeon to put a dialysis catheter in, and a technician to operate the pheresis, which is essentially filtering of the blood. But IVIG can be no picnic either, as it can cause kidney damage, allergic reactions, cause clots, cause headache and meningitis, and can cause heart failure from the fluid volume. The treatment must be tailored again to the individual and the situation. In addition, decisions about other immunosuppression, such as steroids and other steroid-sparing immunosuppressants, must be made. Remember that steroids can acutely worsen respiratory failure, so this is usually not my first action unless I already have the patient on a ventilator.

Identify the trigger: Crisis can have many triggers, from an upper respiratory infection, to a medication, to a change in lifestyle. Sometimes we can't find a good correlating reason for why myasthenia gets worse. If you can find a trigger, remove it. But you will still need aggressive supportive and medical care.

The important thing is to pay attention to safety throughout the entire process.

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Response by:

Robert W Neel, IV, MD Robert W Neel, IV, MD
Assistant Professor of Neurology
College of Medicine
University of Cincinnati