TEAMWORK, TRAINING HELP ANESTHESIOLOGISTS TREAT RARE CONDITION

All the anesthesiology staff at the University of Mississippi Medical Center knew about malignant hyperthermia.

They knew 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. Jan. 19 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, says he was very proud of his department for the work they did that day. He has seen one, perhaps two, cases of malignant hyperthermia during his career that spans several decades.

Dr. Stan Musick, the attending anesthesiologist the day of Evan’s case, had never seen a case before. Dr. David Pennington, Evan’s grandfather, an anesthesiologist in McComb, has never seen a case.

Chris and Tori Rhodus are Evan’s parents. They had become well acquainted with the Blair E. Batson Hospital for Children, 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, is a surgical nurse in McComb. She credits the anesthesiology staff for saving her only grandson.

“Thank goodness Ann (Hobson) called it when she did,” Pennington said.

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 that took the surgeon about 10 minutes to insert Evan’s ear tubes.

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.

Musick 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 was needed. It was real teamwork.”

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 also should register with the North American MH Registry of MHAUS, Pittsburgh, Pa., by calling 888-274-7899.

Evan is 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 says 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. 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.”

— Janis Quinn (4-30-07) 

2007-04-30 00:00:00 16865