Anesthesia can trigger malignant hyperthermia, a rare but deadly disorder that has received attention following the recent death of Stephanie Kuleba, a Boca Raton, Florida teenager who underwent elective breast surgery in a local surgical center.
Anesthesia administered in hospital settings is generally considered safe because trained anesthesiology professionals are always available. And, although malignant hyperthermia can occur in any health-care setting, doctors offices and surgical centers may not always have an anesthesiologist or nurse anesthetist with graduate training who can recognize and respond to danger signs, problematic given the rise in surgical procedures occurring outside of hospitals.
Malignant hyperthermia is caused by an inherited genetic mutation that triggers an uncontrolled rise in calcium levels in muscle cells when a patient inhales some common anesthetics. A patient’s body temperature can jump to 108 degrees in minutes, followed by cardiac arrest and death. Dr. Rosenberg, director of medical education at St. Barnabas Medical Center in Livingston, New Jersey, says incidents are likely underreported and estimates that there are about 1,000 cases occurring annually. Researchers are currently studying malignant hyperthermia—which was discovered three decades ago—and recent studies indicate that as many as 1 in 3,000 people may have the genetic mutation that predisposes them to a malignant hyperthermia reaction under inhaled anesthesia.
Those with muscular dystrophy in the family are one such group of patients that may be at risk. Researchers also are studying whether there is a relationship between malignant hyperthermia and sudden deaths in young athletes and soldiers linked to heat stroke. Teens and young adults—especially those who are highly athletic—tend to be more vulnerable, perhaps because of their stage in muscle development or their unique hormone levels. The most accurate test to determine predisposition involves a muscle biopsy and is only available in a handful of centers and can cost over $6,000. A genetic blood test is available, but is not as accurate. Rosenberg says susceptible patients may have no problem in one surgery but may be at risk in a future procedure; patients can also experience a reaction in the recovery room, so close monitoring is critical even after the surgery is complete.
The muscle relaxant dantrolene can quickly stop the uncontrolled release of calcium cells; however, while most hospitals generally carry adequate supplies of dantrolene, doctors’ offices and surgical centers often do not, generally because of the drug’s cost. The cost of an adequate supply to treat an episode is about $2,500, considered relatively inexpensive given the possibilities. But, dantrolene can require preparing and administering as many as 36 vials in quick succession. Recently, Arizona’s state medical board banned physicians in office-based surgery centers from using any drug that could trigger malignant hyperthermia.
Stephanie’s father, Tom, says he never heard of malignant hyperthermia and knew of no family history of anesthesia problems. Stephanie’s surgery was performed by a board-certified plastic surgeon with an anesthesiologist present. Dantrolene was administered; however, dosing has been questioned and Stephanie’s death is under investigation by the state medical board.
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