An autopsy has confirmed that the South Florida teenager, Stephanie Kuleba, who died this Spring after corrective breast surgery (reportedly for significant asymmetry and inverted nipples) suffered from a rare genetic disease that had been speculated to have causes her death. Genetic testing at the University of Pittsburgh shows she possessed the genetic mutation RYR-1 which is responsible for most cases of the malignant hyperthermia (MH) response to certain inhaled anesthetics. However, over 80 genetic defects have now been potentially associated with MH. As these mutations are inheritable, they will vary in rates among the population and some increased clusters of MH mutation carriers have been suggested in states like Wisconsin, Nebraska, West Virginia and Michigan.
The exact incidence of Malignant hyperthermia is unknown, but the rate of occurrence has been estimated to be as frequent as one in 10,000 or as rare as one in 100,000 patients who undergo general anesthesia. (A range that big suggests they have no idea to me) There is no practical screening test to determine if a patient has the rare condition so you rely on family history or consultation questions to identify high risk patients. Again, the incidence is so rare there is no way to prevent these MH events from happening. The signs that develop are usually suble (ie. a tense jaw) before they're not (ie. 104 degree temp and cadiovasular collapse).
Despite her doctors efforts to treat the Ms. Kuleba during the event with the medication Dantrolene, her parents claim her Plastic Surgeon's office was not prepared to care for their daughter once they had figured out that she was suffering from the hidden hereditary condition and have (in the great American tradition) announced their intention to file a lawsuit.
When MH is identified or suspected, time becomes valuable for salvage treatment. As soon as the malignant hyperthermia reaction is recognized, all anesthetic agents are discontinued and the administration of 100% oxygen is recommended. Dantrolene should be administered by continuous rapid IV "push" beginning at a minimum dose of 1 mg/kg, and continuing until symptoms resolve or the maximum cumulative dose of 10 mg/kg has been reached.
Kulebas' family attorney Roberto Stanziale, has said the teenager should have received as many as seven vials of the drug as an initial dose. On medical records Stanziale obtained following her death, one doctor noted she received one vial of the antidote. The other doctor wrote she received two. It's not known at what time the drug was administered or whether there was enough Dantrolene available at Dr. Schuster's Boca Raton clinic, Schuster Plastic Surgery. Both doctors have defended their actions, saying the situation was handled appropriately and that Kuleba received the Dantrolene dose needed once they consulted with the Malignant Hyperthermia Association (MHA) hotline and called an expert at the Mayo Clinic in Minnesota.
This dosing issue and it's timing is going to be a big issue in the lawsuit. You can't really give informed consent for MH as it's so rare so that shouldn't be an issue (although that will likely be claimed by a plaintiff's attorney). According to the brochure for Dantrolene, each vial contains 20 mg of the drug. As it's suggested in her anesthesiologists notes, she received 2 vials initially (40mg) while they called the MHA hotline to confirm treatment (as again it's so rare no one really has a lot of experience with treating it). That 40mg dose is in the ballpark for the recommended range (by weight) for initial treatment for most thin teenagers.
At the end of the day, I'm not sure what's going to be achieved with this lawsuit. It sure seems like reasonable steps were initiated by her doctors after the event to try and save this girls life. There is only so much you can do when unforeseen or extremely rare complications arise and no amount of preparation can prevent some bad outcomes. Contrast the hostile posture of the Kuleba family attorney with this MH tragic event during orthopedic surgery on a 20 year old described by Dr. Henry Rosenberg, President of the Malignant Hyperthermia Association of the United States. The pain of the medical staff and their communion with the deceased's family is moving.
I hope that this event will continue to foster more discussion on oversight for office-based surgery and anesthesia. It's ironic that it's actually been Plastic Surgery that been the most progressive in regulatory oversite in ambulatory surgery. While this case was an anesthesia complication rather then a surgical one, the who's, where's , and how's of who can (or should) be doing surgery is overdue for more scrutiny.
Rob
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