This rare genetic disorder triggers a severe reaction to certain anesthesia drugs, causing rigid muscles, high fever, fast heart rate and rapid breathing.
Malignant hyperthermia is a severe reaction to certain drugs used for anesthesia. This severe reaction typically includes a dangerously high body temperature, rigid muscles or spasms, a rapid heart rate, and other symptoms. Without prompt treatment, the complications caused by malignant hyperthermia can be fatal.
In most cases, the defective gene that puts you at risk of malignant hyperthermia is inherited, though sometimes it's the result of a random genetic defect. Genetic testing can reveal whether you have an abnormal gene. This genetic disorder is called malignant hyperthermia susceptibility (MHS).
Treatments for malignant hyperthermia include the medication dantrolene (Dantrium, Ryanodex, Revonto), ice packs and other measures to cool body temperature, as well as supportive care.
In most cases, no signs or symptoms of susceptibility to malignant hyperthermia exist until you're exposed to certain drugs used for anesthesia.
In rare cases, people at risk of malignant hyperthermia have shown signs of a reaction after intense physical activity during excessive heat or humidity, during a viral illness, or when taking statin medication.
If you're at risk of malignant hyperthermia and you don't have a serious reaction during your first exposure to certain anesthesia drugs, you're still at risk if you receive these drugs in the future. Other anesthesia medications that don't trigger a reaction can be used instead.
Signs and symptoms of malignant hyperthermia may vary and can occur during anesthesia or during recovery shortly after surgery. They can include:
If someone in your family is known to be at risk of malignant hyperthermia and you need to have anesthesia, it's important to tell your doctor and anesthesia specialist (anesthesiologist). Other drugs may be used instead.
Malignant hyperthermia susceptibility (MHS) is caused by a genetic defect (mutation). The abnormal gene increases your risk of malignant hyperthermia when you're exposed to certain anesthesia medications that trigger a reaction. The abnormal gene is most commonly inherited, usually from one parent who also has it. Less often, the abnormal gene is not inherited and is the result of a random gene mutation.
Different genes can cause the disorder. The most commonly affected gene is RYR1. More rarely affected genes include CACNA1S and STAC3.
Your risk of having malignant hyperthermia disorder is higher if someone in your family has it.
Your risk of having malignant hyperthermia is also higher if you or a close relative has:
If not treated promptly, malignant hyperthermia can result in major complications, such as:
If you have a family history of malignant hyperthermia or a relative who has problems with anesthesia, tell your doctor or anesthesiologist before surgery or any procedure that requires anesthesia.
Evaluating your risk of malignant hyperthermia allows your anesthesiologist to avoid certain anesthesia drugs.
Malignant hyperthermia is diagnosed based on signs and symptoms, monitoring during and immediately after anesthesia, and lab tests to identify complications.
Testing to find out if you're at increased risk of malignant hyperthermia (susceptibility testing) may be recommended if you have risk factors. Testing may include genetic testing or a muscle biopsy test.
If you or someone in your family has malignant hyperthermia and you need to have anesthesia, it's important to tell your doctor and anesthesiologist. Drugs that do not trigger malignant hyperthermia may be used as part of your anesthesia.
Immediate treatment of malignant hyperthermia includes:
Malignant hyperthermia usually resolves within a few days with treatment.
If you've experienced malignant hyperthermia due to certain anesthesia drugs, exercising during excessive heat and humidity could trigger another reaction. Talk to your doctor about any precautions you should take.
Also, check with your doctor to see if you should have genetic testing to determine if you have an abnormal gene that puts you at risk of malignant hyperthermia. Ask if close family members should also consider genetic testing.
If you have the abnormal gene that puts you at risk of malignant hyperthermia, wear a medical alert bracelet or necklace. This lets health care providers know of your risk, especially in an emergency, when you may not be able to talk.
December 24th, 2020