Funeral services were held for 18-year-old Stephanie Kuleba on Wednesday. Family friends and mourners from Boca Raton, Florida said goodbye to the high school cheerleader known as "Sunshine" who was supposed to be attending the University of Florida this fall on her way to becoming an MD of plastic surgery.
Kuleba had a rare reaction to anesthesia in the plastic surgeon's office where she was having reconstructive surgery on her breasts. By the time Malaignant Hyperthermia (MH) was identified, she was on her way to a respiratory crisis and increased body temperature characteristic of this genetic allergic reaction to anesthesia.
Just how common is MH and how would you know if you had it?
IB News Interviews Dr. Henry Rosenberg, President Malignant Hyperthermia Association of the United States
The Malignant Hyperthermia Association was created by the family of the family members of a patient who died of MH. The family was amazed how little information was available to the public on MH. They hired Dr. Henry Rosenberg a practicing clinical anesthesiologist until 2002 to be president of the organization.
The number of the MH Association of America is 1-800-MH HYPER (1-800-644-9737)
IB: What triggers MH?
HR: "99% of the time it’s related to anesthesia. There is some evidence that if you are pre-disposed to heat stroke that might indicate you have a problem with MH, but there are not a lot of studies with those patients."
How do you know if you have it?
“So far as a general statement it’s based on anesthesia-related history. For example, my family member had routine surgery and died or woke up packed in ice or had tremendous pains or peed brown urine. Or maybe under anesthesia you’ve been unstable and they didn’t know what it was. Those are unexplained tip offs.
“Screening will be based on the history of the patient. The question usually asked is whether anyone in your family had an unexpected problem related to anesthesia. MH is related to muscle disease in some and it can predispose them to this problem, so if there is a history in the family, someone in the family had a problem with surgery or a reaction under anesthesia could be an indicator."
What percent of the population is affected?
"There are several answers to that but the best known number of cases is in the one in 10,000 to one in 30,000 range. The mutation association with MH doesn’t manifest itself each time because an IV and a local anesthesia don't trigger MH. If you don’t get anesthesia you won’t know.
"In the early 1970s people began to realize it was a problem - 80 percent of patients died who had MH because they got a high body temperature. It’s a frightening disorder , just ask any anesthesiologist and he or she will remember that patient - it's very frightening."
Is there a test?
“There is a unique biopsy testing the contraction response in a lab setting that is only available in a number of centers. There has been developed a DNA test since it is an inherited disorder genes are identifier for MH. But it’s an evolving test and not always accurate."
If there’s an antidote why not have it on hand during every surgery?
“The drug comes in vials of 20 milligrams each and has to be reconstituted with water. You may need 10 to 12 vials and it takes time to mix it up draw it and inject it. When you are dealing with MH every minute counts because you can go rapidly downhill in 15 to 20 minutes. So it’s a complicated because things can go awry in minutes."
Isn’t a hospital a safer place to have surgery?
"My opinion is based on hospital work. If there is a problem in a hospital likely in the adjacent room will be an expert who can help you. Whereas in the smaller office based surgical centers there is no one, you have to call EMS and they don’t know MH." #