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Rare Case of Malignant Hyperthermia Treated at UMC

BY JANIS QUINN

All the anesthesia staff at the University of Mississippi Medical Center knew about malignant hyperthermia. They knew it from textbooks. They heard about it in lectures. They even had a crash cart, inspected weekly, dedicated to this uncommon, but potentially deadly condition — anesthesiology's worst nightmare.
Luckily for Evan Rhodus, UMC nurse anesthetist Ann Hobson had paid close attention in class. She recognized the first signs even though neither she nor any other member of the team had ever seen malignant hyperthermia in a patient. She called in the anesthesiology team that began treatment immediately and saved the life of the 15-month old. January 19, 2007, was the first time the cart had been used in more than a decade.

Malignant hyperthermia is a hereditary condition triggered by certain types of anesthesia. The reaction causes the body to go into metabolic overdrive inducing extremely high fever, muscle rigidity and acidosis. It can be fatal if not treated quickly.

Dr. Claude Brunson, chairman of anesthesiology, said he was very proud of his department for the work they did that day. During his career, which has spanned several decades, he has seen one, perhaps two, cases of malignant hyperthermia. Dr. David Pennington, Evan's grandfather, an anesthesiologist in McComb, has never seen a case.

Chris and Tori Rhodus, Evan's parents, had already become well acquainted with the Blair E. Batson Hospital for Children, part of the University Hospitals and Clinics in Jackson, where Evan had spent all but three weeks of his life. Born two months prematurely at another local hospital, Evan was transferred to the Batson Hospital as a patient in the pediatric ICU. Evan was born with achondroplasia, a birth defect characterized by dwarfism and other physical problems.

Evan had already undergone surgery twice: to put in a tracheotomy tube so the ventilator could breathe for Evan and to put in a feeding tube. Both times, the anesthesia was administered without a problem.

Gail Pennington, Evan's grandmother, a surgical nurse in McComb, credits the anesthesiology staff for saving her only grandson. "Thank goodness Ann (Hobson) called it when she did," she remarked.

According to Hobson, Evan "had some of the classic signs (of malignant hyperthermia), but other indicators weren't there. I was a little worried that people would think I had overreacted."

For one thing, most cases occur with the very first administration of general anesthesia. This was Evan's third for a relatively minor procedure, the insertion of ear tubes, which took the surgeon about 10 minutes.

But Hobson saw Evan go from normal to a temperature of 103.4 in fewer than two minutes. Just that quickly, he went from being dry to looking as though he'd been drenched with water. His heart rate increased rapidly, and there was a dramatic increase in the amount of carbon dioxide he was exhaling.

Dantrolene is the antidote for malignant hyperthermia. Discovered in the 1970s, it is largely responsible for the steady drop in cases of death from malignant hyperthermia — from 80 percent in the 1960s to 10 percent.

But malignant hyperthermia remains a very serious life threatening condition that requires a rapid, multifaceted response, according to Brunson. While Hobson maintained Evan's airway after calling in the team, Sara Beth Brasier, another nurse anesthetist on staff, mixed the dantrolene as it was needed. Treatment also requires the continuous infusion of saline to wash out the acids that the body builds up when it's in this heightened metabolic state, the irrigation of body cavities with iced saline and the administration of sterile bicarbonate soda to neutralize the acids.

Dr. Stan Musick, instructor in anesthesiology and chief resident at the time, praised Hobson, Brasier and nurse anesthetist Catie Burton for their work. "This case was anesthesia care at its best." And Hobson echoed his praise of the team. "Everyone just came together and did what they needed to do. It was real teamwork." Dr. Sanja Raucher, assistant professor of anesthesiology, was the attending physician the day of the crisis and managed Evan's care.

Even when survival is assured, many patients have residual effects from the cascade of biochemical events. Survivors may have brain damage, muscle damage, failed kidneys or impaired function of other major organs. Evan, so far, appears to have none of these.

Brunson estimates that one in 30,000 people have the susceptibility. Before the condition was identified, it was generally said that the patient "didn't tolerate anesthesia."

According to the Malignant Hyperthermia Association of the United States, (www.mhaus.org), any family with a history of anesthetic deaths or complications should make this known to the anesthesiologist before undergoing surgery. Then anesthesiologists will know which agents to avoid. They should also register with the North American MH Registry of MHAUS, Pittsburgh, Penn., by calling (888) 274-7899.

Evan is now with his parents at the University of Alabama in Birmingham, where they are learning to manage the ventilator so they can bring him home. Gail Pennington said chances are good that he'll eventually come off the vent to live his life as it was meant to be lived.

"Because I work in a hospital, I know that many times you only hear from families when stuff goes wrong," she said. "I wanted to make sure everybody knows that this was a case of something that went exactly right. We wouldn't have Evan with us today if everybody on that team didn't know what they were doing."


April 2007

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