One severe side effect of myasthenia gravis is a myasthenic crisis. It results in respiratory failure, a potentially fatal illness.
An autoimmune disease called myasthenia gravis results in voluntary muscular weakening. These are the muscles that are attached to your bones and regulate every bodily movement, including breathing.
According to the National Institute of Neurological Disorders and Stroke, 15–20% of individuals with myasthenia gravis are expected to experience at least one myasthenic crisis.
Continue reading to find out more about this issue, including how to identify, avoid, and treat it.
What is a myasthenic crisis?
One potentially fatal side effect of myasthenia gravis is a myasthenic crisis. It occurs when the respiratory muscles weaken to the point where you require medical attention to recover.
A myasthenic crisis is more common in older persons with myasthenia gravis than in younger adults.
A myasthenic crisis could be more likely to occur if you:
- have severe or untreated myasthenia gravis
- possess had a myasthenic crisis previously
- possess a weakness affecting your oropharynx or throat.
- test positive for antibodies against muscle-specific kinase (MuSK).
- own a thymoma, a kind of tumour that can grow in the chest
Some patients have myasthenic crises before realising they have myasthenia gravis.
Myasthenic crisis symptoms
Usually, a myasthenic crisis takes several hours, days, or weeks to develop. But on occasion, it progresses more swiftly.
The following are possible indications of a myasthenic crisis:
- difficulty breathing
- general weakness
- weakness in your limbs
- shallow breathing
- weak cough
greater bulbar weakness, which could result in:
- drooling
- lip trembling
- difficulty swallowing or talking
- nasal or hoarse voice
- loss of gag reflex
- tongue or jaw weakness
If you believe you may be going through or developing a myasthenic crisis, get medical attention immediately. It's critical to start treatment early to improve your results.
What causes a myasthenic crisis?
Acetylcholine is a substance that your nervous system utilises to communicate with voluntary muscles. Your immune system alters, inhibits, or eliminates proteins that facilitate acetylcholine transmission between neurons and muscle fibres if you have myasthenia gravis. This is the reason for muscle weakness because it prevents the muscle from contracting correctly.
The muscles in your neck, chest, and diaphragm that assist in controlling your airway and breathing might be impacted by myasthenia gravis. Breathing problems and a myasthenic crisis might result from weakness in these muscles.
A myasthenic crisis can be brought on by a few factors, such as respiratory infections.
Other possible causes are as follows:
- a negative drug response
- surgery
- childbirth
- stress
- injury
- pregnancy
The cause of anywhere from 30% to 40% of myasthenic crises is unknown.
Can you avoid or prevent a myasthenic crisis?
You can lower your risk of experiencing a myasthenic crisis by adhering to your myasthenia gravis treatment plan. To treat myasthenia gravis, your doctor might recommend medication in addition to other therapies.
You may be less likely to experience a myasthenic crisis if you take precautions to prevent or control possible triggers. As an illustration:
- By receiving the required vaccines, avoiding direct contact with ill people, and routinely washing your hands with soap and water or alcohol hand gel, you can reduce your chance of developing respiratory infections.
- Before you start a new drug, go through a planned surgery, become pregnant, or give birth, find out from your doctor how to minimise the chance of a myasthenic crisis.
- Limit stress and injuries that can be avoided by taking action.
Treating your myasthenic crisis symptoms as soon as possible may help lessen their intensity and speed up your recovery.
How is a myasthenic crisis diagnosed?
A myasthenic crisis will be identified by your doctor by:
- considering your symptoms
- conducting a physical exam
- requesting examinations to assess your respiratory health
To rule out additional possible causes of your symptoms, such as heart failure, acute respiratory distress syndrome, or a blood clot in your lungs, he or she will also request testing (ARDS).
The majority of patients with myasthenic crises are already aware that they have myasthenia gravis. However, some persons do not have a diagnosis of myasthenia gravis before they have a myasthenic crisis.
By requesting blood tests and electrical tests of your muscles and nerves, your doctor can make the diagnosis of myasthenia gravis. To rule out other diagnoses or look for indicators of myasthenia gravis, they could also request additional testing.
How is a myasthenic crisis treated?
If you have a myasthenic crisis, you will probably be treated in an intensive care unit (ICU) of a hospital.
Any of the following could be part of your treatment:
- Intubation is the process of inserting a tube into your windpipe.
- breathing assistance from a ventilator machine
- Breathing techniques to enhance lung health
- Using airway suction to clear your airways of mucus and other substances
- medication to control symptoms, change your immune system or open your airways
- Intravenous immunoglobulin or plasma exchange, which translocates unhealthy immune proteins with healthy donor proteins to momentarily modify your immune system
- drinks and dietary supplements to maintain your hydration and meet your demands for nutrition
Your medical team will also keep an eye out for any indications of problems that might call for additional therapies. They can provide information about your prognosis and available treatments to you and your loved ones.
After you leave the hospital, your doctor can recommend rehabilitation therapy or other therapies to help you recuperate. They could suggest that you see a respiratory therapist or other experts in rehabilitation. Psychological counselling could also help you deal with the emotional strain that a myasthenic crisis might bring.
The takeaway
A severe respiratory problem brought on by a myasthenic crisis is a complication of myasthenia gravis.
A respiratory infection, a drug side effect, or any other type of physical or mental stress may cause it. It's not always clear what sets it off.
You can lower your risk of experiencing a myasthenic crisis by adhering to your myasthenia gravis treatment plan. For myasthenia gravis, your doctor might recommend medication in addition to other therapies. They might also provide advice on how to deal with any myasthenic crisis triggers.
You must get medical attention as soon as you have breathing problems or other myasthenic crisis symptoms. To aid in your recuperation, you might require hospital therapy.
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