Circulation 80,350 • Volume 21, No. 2 • Summer 2006   Issue PDF

Malignant Hyperthermia Death Holds Many Lessons

Henry Rosenberg, MD; Al Rothstein

The death of a young, vigorous, healthy individual during routine surgery is an emotionally devastating event. The family and friends of the person are shocked, angry, depressed, beset by guilt, frustration, and thousands of questions. Was there something they could have done to prevent the death? Should the surgery have occurred? Did the physicians and nurses miss something or act inappropriately?

These were the feelings and emotions experienced by the family and friends of Steven Nook, a 20-year-old athletic, affable, young man who died following surgery to repair an injury to his shoulder experienced during a skiing accident on January 5, 2005.

Steven had so much to live for. He was a popular sophomore at University of Wisconsin-LaCrosse and aspired to be a physical education teacher and football coach. He made friends easily and was outgoing.

What happened and why did it happen? Steven died as a result of malignant hyperthermia (MH) syndrome, a rare reaction to commonly used anesthetic agents. This syndrome, first recognized in 1960, results from alterations to a specific gene. Unlike many other inherited (genetic) disorders though, these rarely produce symptoms or signs until the patient receives a general anesthetic agent. It is like a viper lying in wait for the right circumstances to strike.

In the 1960s and ’70s the diagnosis of MH during surgery was a virtual death sentence; 80% of patients died after experiencing MH. However, thanks to the efforts of many anesthesiologists and other physicians and scientists around the world, deaths from this disorder now occur infrequently during or following the 30 million or so anesthetics administered in the US every year. Anesthesiologists and nurse anesthetists have so many sensitive devices to monitor vital functions—respiration, oxygenation, cardiac function, temperature, and kidney function—that deaths related to anesthesia have become very uncommon, perhaps 1 in 250,000 healthy patients. Therefore, when a death occurs it is a shock and trauma to the entire anesthesia, operating room, and surgical teams. This story focuses on the short- and long-term reactions of the caregivers when a healthy patient succumbs despite the best treatments available.

Where did the term MH come from? When the disorder was first formally described, the dramatic and unusual feature of the reaction was an elevation of body temperature to levels incompatible with survival—107-109°F or higher—in medical terms, hyperthermia. Since 80% of patients diagnosed with the disorder died, it was a malignant disorder, hence the name. The reaction was especially impressive because, as a rule, a patient’s body temperature has a tendency to drop during anesthesia.

The basic problem in MH is an increase in metabolic rate in response to most gas anesthetic agents, such as sevoflurane and desflurane, and a particular paralyzing drug called succinylcholine. Further details concerning the mechanism of the increase in metabolism may be found on the website of the Malignant Hyperthermia Association of the United States (MHAUS), a not-for-profit patient advocacy group formed by a relative of a young man who died from MH in 1981 (www.mhaus.org).

What has made death from MH a rarity at this time? Three main factors: education of the anesthesia community to screen patients for family histories of MH susceptibility and to recognize the early signs of MH, the routine measurement of exhaled carbon dioxide (CO2 rises rapidly when metabolism increases) and body temperature, and the US FDA approval of dantrolene sodium IV for the treatment of MH in 1979.

Dantrolene was indeed present in the OR when Steven developed MH, and it was administered to him rapidly and in sufficient doses; however, his case was one of those very unusual circumstances because the development of full-blown MH occurred very late, more than 3 hours into the surgery. Carbon dioxide levels rose very slowly initially, and then raced ahead explosively when the syndrome took hold. Elevation of body temperature, always a later sign of MH, was especially late in his case. So, by the time the anesthesia team determined that MH was occurring and dantrolene should be given, the train had left the station and was racing down the tracks. In fact, the antidote drug did retard the metabolic changes, but unfortunately damage to vital body functions had taken place and over the ensuing 2 days, and despite heroic efforts, Steven’s coagulation system went awry and led to massive, uncontrolled hemorrhage.

Steven was not the stereotypical physical education major. He was not afraid to express himself in poetry. For example,


People say, “I’m sorry” when I say I was born on 9/11…

But today is a day to be proud of…
Today, my birthday, America united
—“9/11” by Steven Nook


“The most shocking things were the subtle clues that this might be malignant hyperthermia (MH),” says Tami Ulatowski, MD, an anesthesiologist with Summit Anesthesiology group who was quickly called in when the staff began to suspect that Steven was displaying signs of MH.

“The younger nurses thought of him as a peer, the older nurses as their son,” remembers Kathy Delleman, Intensive Care Unit Manager for Aurora Sinai Medical Center in Milwaukee, WI. Delleman says that it is unusual to identify so closely with a patient, but because of his age, personality, and skiing accident, Steven had qualities that the staff could identify with.

One of the first steps that was taken once the diagnosis was made was to call a special “hotline” established in 1982 by MHAUS in order to help anesthesia caregivers deal with the emergency management of this complex disorder. This free service is staffed by approximately 30 anesthesiologists expert in the management of MH. Three are available at any one time on a 24/7 basis. Andrew Herlich, DMD, MD, was the MH Hotline consultant at the time. Herlich is Professor of Anesthesiology, Otolaryngology, and Pediatrics, and Medical Director of the Human Simulation Center at the Temple University School of Medicine. Herlich was reassuring to Steven’s medical team and was on the phone with them several times a day, having given them his personal home number and beeper number. Dr. Herlich’s message was one of support, advice, and reality.

“He told us not to be discouraged even though Steven’s temperature reached 109°,” says Dr. Wilfrido Castillo, chief of the anesthesiology department at Aurora Sinai. “We knew what we were up against, but because of Dr. Herlich’s guidance we knew that we were doing everything we could, and this prepared us in case of a bad outcome.”

“I tried to be strong and reassure Steven each time I was with him that he would get through this,” recalls Steven’s mother, Jacque Nook. “I believe he heard my voice and it gave him some comfort. He must have been so scared. It helped me to know that the monitor on his forehead (that measures the level of consciousness) indicated that Steven was most likely aware of my voice. I rejoiced each time the numbers on the monitor suggested that Steven was hearing me. He knows his mother’s voice! So I held it together as best I could, but I was screaming inside.”

The hospital staff was still hopeful that Steven would survive, but he wasn’t showing signs of improvement. “We felt helpless,” says Delleman. “We wanted our interventions to work and they weren’t.”

A Devastated Medical Staff

As the situation worsened for Steven, it did the same for the medical staff. Despite their professionalism and disciplined approach from years of medical training, an unfortunate MH death can have a devastating, personal effect. An effect that takes time to surmount—one that motivates staff to recheck every move, and in the end, look for new ways to prepare for another MH case.

Stephanie Kassulke was the anesthesia recovery room nurse and because she could identify so well with Steven, she was starting to have a difficult personal time when she saw that Steven was not improving. “When I went home that night, when Steven was still alive, I was crying so hard in the car that I almost had an accident. My kids are that age. They are young healthy kids. You put all of your effort into it to help him make it, and you hope you will get a miracle.”

Two days after Steven entered the hospital, he passed away.

Dr. Herlich summarizes, “When Steven’s temperature precipitously rose in the OR, the anesthesiologist immediately called for help. He proceeded to treat him by discontinuing the volatile anesthetic, cooling him by all means possible, administering intravenous dantrolene, and correcting the metabolic abnormalities revealed by blood tests. However, over the ensuing hours after transfer to the intensive care unit, Steven developed a severe bleeding problem as well as a problem with heart function.”

“As a result of the stress of the MH, Steven developed severe heart and kidney failure over the course of the next 2 days. The bleeding disorder worsened and was unresponsive to even the most advanced of therapies, including dialysis and the administration of many medications and blood products that stabilize blood clotting. During this period, dantrolene was continuously administered to maintain Steven’s temperature and blood chemistries as close to normal as possible, which was obviously impossible.”

“They (the hospital staff) made every effort to tell us what they were doing, how he was responding, and give us any little hope they could muster,” remembers Mrs. Nook, herself a nurse. “However, I could see the frustration in their eyes, when Steven kept getting worse despite their efforts. They wanted to see improvement as much as we did, and it didn’t come.”

“When they called the code on Friday I knew it was him,” says Kassulke. “When he died, I felt a lot of anger.”

“This came out of nowhere,” says Delleman. “No warning.”

Before the procedure, his anesthesiologist had questioned Steven about whether there had been any family history of adverse reactions to anesthesia. This question is part of the screening process for MH and should be asked of all patients about to receive a general anesthetic. In Steven’s case, the answer to the question was no known family history. Many MH susceptibles have a benign family history for the disorder because MH does not manifest with every exposure to anesthesia. This fact made the medical staff feel even more shocked, because they had followed the proper screening procedure with Steven.

“The day he passed, there were a lot of tears,” says Delleman. “I don’t think there were a lot of words. There were more hugs and putting arms around shoulders. Not everybody recovered in the same way. Some were able to move on immediately. For others, it was foremost in their mind. Some are still dealing with it several months later.”

“That following Sunday I went to church and cried through the sermon. I would be in the grocery store and the thoughts would infiltrate. It happened during routine things.”

Delleman and Kassulke say that the medical staff who have children close to Steven’s age had the toughest time recovering. Delleman added, “I myself have a son a couple of years older. When I go home, I look at him in a different light. He had surgery a year ago and could have responded that way.”

Kassulke remembers, “I went home that night and I hugged both of my kids and told them I loved them. There were about 2 weeks when you sit down and cry right away just by thinking about it. What I have had to deal with is the lack of control, watching this young man slip through our fingers.

“I’m still having a hard time getting over it. You know in certain circumstances that there is nothing you can do, but this wasn’t cancer or hemorrhaging. There was no previously known disease process to help you prepare for what might happen. I went back to work on Monday, but people were asking me what was wrong because I was down, subdued for awhile.”


In Remembrance, a child does not age

And so they never leave,
They’re in your heart at every stage
Of their life they weave.
—“Remembrance” by Steven Nook, December 2004


Dr. Castillo says that his main concern was with Steven’s anesthesiologist: “It was obvious he was having a hard time. There is a decompression time, just like for a policeman or fireman involved in a crisis in the line of duty. He took some time off to be with his family.”

Dr. Ulatowski says, “It’s the one thing anesthesiologists fear, the MH episode. I think it is every anesthesiologist’s worst nightmare. To lose a young, healthy patient in the OR in this day and age is virtually unimaginable, but that is what can happen with MH.”

Dr. Herlich says that it can be much more shocking to a medical staff when a young, healthy man walks into a hospital and does not walk out, than if a 90-year-old with pre-existing conditions succumbs. Although saddened by that death as well, he says medical professionals are not as emotionally burdened because the patient has lived a full life, Herlich states.

Dr. Castillo believes that most doctors feel they can carry on after a situation like this, “but we need to admit to ourselves that we are human, and we need to realize that we may not respond in the normal way in the immediate future. We need to give ourselves time to recover. Physicians should be honest with themselves.” The same may be said for all those who care for such patients.

Dr. Castillo initially had to push his own emotions aside, and let part of his recovery process include preparing for the next time this might happen. “There are a lot of technical questions: Why did this happen? What did we do? What could we have done? Although I was not emotionally involved because Steven was not my patient, I became involved emotionally with the family later. I shed a lot of tears when I sat down with the family, but one way I dealt with this was to be super prepared for such an event the next time.”

The close-knit Nook family itself was a source of support for the hospital staff. Dr. Castillo remembers that the staff and the family supported each other a lot, the first time he had ever seen a family support a staff so strongly. The Nook family even e-mailed poetry and anecdotes to the staff. “My strength comes from inside me and my love for my sons, from my strong faith, and from my wonderful family and friends,” Mrs. Nook says. “I attribute my ability to help the staff afterwards, to the fact that we shared this awful, gut-wrenching experience with them, and bonded with them,” says Mrs. Nook. “I felt a camaraderie with them I can’t explain. They had all the best critical care skills, but saving Steven was out of everyone’s reach. We were all in this horrible pain together. I KNEW they were doing the best they could. They used all the best resources available to them.”


So when you’re lost, or

don’t know what to do,
Remember…
Life brings with it, what you want it to.
—Untitled poem by Steven Nook


For the family and hospital staff, there was discourse about sadness, support from the hospital chaplain, psychologists, and the Crisis Intervention Department. The medical staff found that going to Steven’s funeral and giving a creative memorial gift helped them recover. “There were several of us who went,” says Delleman. “Steven’s favorite color was blue, so we purchased a blue candle a foot high in a hurricane glass, with a ring of blue flowers. A surgery nurse and I drove to Steven’s aunt’s house to present it.”

“When we talk about crisis events, for some of the medical staff the sharp professional edge is dulled until the recovery is over,” says Marcia Williams, LPC (Licensed Professional Counselor) and Clinical Nurse Specialist in Crisis Intervention and Traumatic Grief at Aurora St. Luke’s Medical Center. “One measure of the recovery process is how much the crisis continues to intrude into their daily lives. The disruption is real. So with most traumatic events the recovery time may be 4–6 weeks, but for some it can actually get better within a week.”

Ed Foster, the Chaplain Supervisor at Aurora-Sinai, points out that even when a medical professional thinks they have recovered, the sad feelings can be triggered again. “It could be that another person resembling Steven comes in and it brings up all of those feelings again. The trouble is that folks want to judge themselves harshly and think that there is something wrong with them. In that case they may really need to talk about it with another medical professional or even consider therapy. They don’t want to take the approach that they have talked about it enough.”

Preparing for Next Time

Part of the recovery process involves preparation for another possible MH episode. For example, the staff is planning to have a mock MH drill at least once per year, reinforcing all of the proper steps needed to respond to an MH crisis. These mock drills are emphasized and covered thoroughly in the MH procedure manuals for hospitals, ambulatory surgery centers, and surgical offices produced by MHAUS.

Steps include

  • assign specific tasks to staff
  • provide checklists and worksheets
  • emphasize frequent mock drills.

A slide show called “Managing Malignant Hyperthermia Risk in Today’s Surgical Environment” is also available through MHAUS. The slide show assists in developing standard of care practice guidelines and algorithms to ensure that patients will have access to appropriate interventions for treating MH.

Dr. Herlich suggests that MH drills should include failure to control the syndrome with the first lines of therapy. “For instance you are giving your first or second dose of dantrolene and the temperature is still rising, the calcium values are decreasing, the potassium values are rising, and the patient’s coagulation is starting to deteriorate. Don’t go through a drill as if giving 1 or 2 doses of dantrolene means everything is hunky-dory and the patient walks out the door 1 or 2 days later.”

Recovery room nurse Kassulke advises hospitals to increase awareness within all of the inpatient units. For example, if a pregnant woman is about to undergo general anesthesia, ask the father about MH as well to see whether it might affect the baby. She also emphasizes the importance of the immediate availability of an adequate supply of dantrolene. In Steven’s case, 90 bottles (450 milligrams) were used in the initial 2 hours of the crisis. “We are fortunate at Sinai because we could pull dantrolene from 5 different hospitals. One of the reasons Steven made it out of OR and into critical care for 2 days is because we were able to get all of that dantrolene. We wiped out just about all of the supply in the Milwaukee region.”

Dr. Ulatowski’s message from Steven’s death is that, “The work of educating and understanding the presentation and optimal treatment of MH is not yet done. Not until there are no more deaths from the disorder.”

Dr. Herlich advises the OR team to call the MH Hotline as early as possible. “It is analogous to many other emergency situations. If you think it, you call. Don’t wait. If in your mind you have a situation that you can’t explain and MH might be occurring, even though the likelihood is low, you should call the MH Hotline.”

Steven Nook – A victim of MH

Mrs. Nook advises patients who are unfamiliar with anesthetics to be aware of any family history of anesthesia problems, and not to take the experience of undergoing general anesthesia lightly. “Little did Steven or anyone else know that this would be an issue. But, just maybe, knowing MH could happen would make the OR team feel the patient’s skin a little more often, or maybe, just maybe, catch something going wrong a little sooner.”

 

Conclusion

Over the past 3 decades, the mortality rate for MH has dropped dramatically. However, as we see from Steven’s case, death from MH can still occur, even in the best of hands. When it does, it can have a dramatic, traumatic effect on the entire medical staff, one that can bring with it a long recovery time. However, something positive can result. For example a scholarship in Steven’s name has been established at his high school, Wauwatosa West, which already has garnered more than $10,000 of community support. Also, the Nook family is participating in the newly available molecular genetics testing procedure to help determine MH susceptibility in other family members. Information as to the diagnosis of MH by laboratory testing may be found at

http://www.mhaus.org/index.cfm/fuseaction/Content.Display/PagePK/MolecularGenetics.cfm.

If the experience of Aurora-Sinai results in more awareness of and preparation for other medical facilities, the MH mortality rate should continue to drop. It is hoped that the experience of the Nook family and the Aurora Sinai staff will perhaps save others from experiencing the same emotional devastation.


So do children, seem to grow

Pretty soon they’re on their way
And so you see them pack and go
It may seem a most lonesome day.
But know this is not the case
For they really never leave
Because memories have their place
And leave no room to grieve.
–“Remembrance” by Steven Nook, December 2004

Henry Rosenberg is the President of the Malignant Hyperthermia Association of the United States and Director of Medical Education and Clinical Research at Saint Barnabas Medical Center, Livingston, NJ. Al Rothstein is the public relations consultant for the Malignant Hyperthermia Association of the United States.