- Diseases, Disorders & Conditions
In March 2008, a Florida teenager died during corrective breast surgery due to a rare anesthesia complication called Malignant Hyperthermia. Stephanie Kuleba, varsity cheerleading captain and high school senior, was known by all as a person who loved to do things for others. She planned to attend the University of Florida in the fall of 2008 to pursue a degree in medicine. Her death was a shock to her family, friends, and the medical team entrusted with her care. The event left many people wondering how something like this could happen.
The Kuleba family talks with Matt Lauer
What is Malignant Hyperthermia?
Malignant Hyperthermia (MH), also known as hyperpyrexia, is an inherited metabolic disorder of the skeletal muscles that presents itself when a person undergoes general anesthesia. Some call it an allergic reaction to anesthesia. It happens so rarely that most anesthesiologists will never see a case throughout their entire careers. MH was first identified in the 1960s, and at that time, the death rate from MH was 80%. In 1975, a medication called dantrolene sodium was discovered and used for the treatment of MH. The mortality rate dove from 80% to just 10% with the introduction of this invaluable drug.
MH happens so infrequently that most people have never heard of it. In Stephanie Kuleba's case, even the 911 dispatcher seemed uncertain when the nurse on the other line explained the situation they were dealing with. MH is estimated to occur in 1 in every 15,000 pediatric anesthetics and 1 in every 50,000 adult anesthetics. MH occurs when a person with the inherited trait is exposed to certain triggering agents. These agents include inhaled anesthetic gases and a medication called succinylcholine that is often given through the patient's IV during general anesthesia.
The body reacts to these triggers by releasing calcium inside muscle cells. This leads to continuous muscle contraction, which increases energy utilization and heat production. The muscle cells eventually run out of energy and die. When these cells die, they release large amounts of potassium into the bloodstream, which can cause irregular heart rhythms that can lead to death.
Signs and Treatment
The anesthesiologist is typically the first person on the surgical team to notice signs of MH. Signs of MH include:
- Rigid contracted muscles
- Abnormally fast heart rate
- Increase in exhaled carbon dioxide
- Decreased oxygen levels in the blood
- Extreme fever, often as high as 113 degrees
- Rapid breathing
- Dark brown urine
- Flushed skin
As the episode progresses, muscles deteriorate and blood stops clotting. According to Dr. Henry Rosenberg, president of the Malignant Hyperthermia Association of the United States, Kuleba's fever reached 108 degrees, her blood could no longer clot and the bleeding could not be controlled.
While the anesthesia provider is often the first to notice signs of MH, all members of the surgical team work together to treat the patient during a crisis. The anesthesiologist immediately stops the use of the anesthetic gases that caused the reaction. The nurses work with the anesthesiologist to prepare and administer dantrolene as quickly as possible. Nurses use ice packs, cooling blankets, and cold irrigating solutions to cool the body down. The anesthesiologist continues to administer oxygen and other medications. Meanwhile, the surgeon and scrub tech work quickly to complete the surgery as best they can and close the patient's surgical incision.
Even if the patient is successfully treated during the MH episode, organ damage can occur. Complications that can result from MH include brain damage, muscle destruction, kidney failure, and impaired function of the heart, lungs, liver, and pancreas. Damage to the central nervous system can also occur, leading to paralysis and/or blindness. 25% of patients successfully treated in the operating room will have another episode in the hours following the first crisis. The patient must be closely monitored during the recovery period.
Operating Room Preparedness
People who work in surgery, such as surgeons, anesthesiologists, nurses, and scrub technicians, are supposed to be educated at least once a year on the dangers of MH and how to react during an MH episode. If a patient is known to have the MH trait, there are certain steps that can be taken to prevent an episode. All disposable pieces on the anesthesia machine are replaced with new parts. The existing soda lime, which absorbs the exhaled CO2, is exchanged for new. Replacing these parts lessens the chance of a reaction due to residual or "left-over" gases in the anesthesia machine. Anesthesia should be performed without the use of triggering agents. The anesthesia provider should administer alternative anesthetic agents if the patient has a family history of an unexplained anesthetic death or complications that may indicate a susceptibility to MH. Administering a non-triggering agent to a patient who has the MH trait can prevent MH and save the patient's life.
Every operating room should have an MH cart. This cart should be stocked with medications and supplies that are necessary for treating an MH episode. The cart should be wheeled into the room prior to surgery if there is any indication that a patient may be susceptible to MH. Time is critical during an MH crisis, so it is important to have necessary medications and supplies ready. The MH cart should be checked regularly by OR staff to make sure it is fully stocked and none of the medications have expired.
Dantrolene is the most critical medication that should be stocked on the MH cart. Dantrolene relaxes the skeletal muscle contractions that are caused by the triggering anesthetic agents. By relaxing the muscles, further damage to other organs can be prevented. A patient weighing 70 kg requires 36 vials of dantrolene during a crisis. Each vial of dantrolene must be reconstituted with 60ml of sterile water before being injected into the patient's IV.
Dantrolene has its obstacles. First, the medication is physically very difficult to reconstitute, so several nurses are needed to prepare the medication during a crisis. In addition, dantrolene is very expensive, so most facilities stock only 36 vials, which is the current standard in healthcare facilities. Because MH happens so infrequently, dantrolene is rarely used before its expiration date when it must be thrown away and replaced. Some facilities opt to share their dantrolene supply. Two hospitals may each keep 18 vials, and in a crisis, they can call on the other for their supply. This saves money for the facility, but it is not in the best interests of the patient.
The Kuleba family's lawyer has suggested that there was not enough dantrolene on hand at the surgicenter where Stephanie's surgery was performed. Medical records show she did not receive an adequate amount of the drug. He also claims the surgicenter was not prepared for an MH crisis and did not have enough people available to help during Stephanie's MH crisis. The outcome of Stephanie's MH episode may have been entirely different if the staff at the surgicenter had been more prepared.
Inheritance of the MH trait cannot be prevented, nor can it be easily detected. There is no blood test for MH. The most reliable test for MH is a muscle biopsy from the thigh. This is usually only performed for patients that have a confirmed family or personal history. The biopsy is only performed at eight locations in the United States and Canada. If you know you have a family or personal history of MH, make sure to notify the anesthesia provider, surgeon, and surgical nurse so measures can be taken to prevent an MH crisis. Patients who have a known MH trait should ask how much dantrolene the facility has on hand in case of a crisis. It is also a good idea to wear a medical alert bracelet that alerts others of a person's MH history. Educate family members that this is a genetic disorder. If one person in the family had an MH crisis, the entire family is at risk.
When I read about Stephanie Kuleba's death, I was shocked and saddened. As a surgical nurse, I have been educated regularly about how to recognize and treat MH. Fortunately, I have never seen a case myself, and most of the people I work with have never seen a case either. It is something that we must be prepared for everyday. Many hospitals run MH drills to make sure the staff knows what to do when an MH crisis arises. I hope that Stephanie's death raises some flags in the surgical world and prompts surgical facilities to make sure they are prepared to save the life of a patient with MH.