MHAUS Newsletter, April 2008 Special Edition
In this issue:

Death of 18-Year-Old Calls Into Question MH Preparedness in Ambulatory Surgery Centers

Stephanie Kuleba, a healthy 18-year-old Florida cheerleader and popular student died on March 22 from complications of malignant hyperthermia. What made front page news was that the death occurred in a physician’s operating room suite. Malignant Hyperthermia (MH) has attracted national attention as a result of this tragedy.

MHAUS extends deepest condolences to the family and friends on their loss.

 

The death has raised numerous questions in the media and in state capitols.

Was the center prepared for such an event?  Was MH detected early enough and dantrolene administered promptly and in sufficient doses? Were the EMTs and emergency room physicians prepared to handle a case of MH?

How many other deaths from MH are occurring in outpatient centers?

Should regulations be passed to restrict the use of general anesthesia or general anesthesia with MH trigger agents in the freestanding outpatient centers?

 

These questions are difficult and will require much time and consideration to answer.

For now the heightened awareness of MH by the public is leading to numerous inquiries and web “hits” at MHAUS.

 

“The popularity of cosmetic surgery and the fact that more and more surgeries are being pushed out of the hospital setting reasons and into office-based surgery suites and ambulatory surgery centers has raised concerns by many” says MHAUS President Dr. Henry Rosenberg.

 

The Ambulatory Surgery Center Association reports that last year about half of 43 million procedures were done outside hospitals, about 10 million each in doctors’ offices and free-standing outpatient centers.

 

“Ambulatory surgery centers are subject to different rules and regulations depending on the state and accrediting agency, which may not be as strict as those in a hospital setting,” says Dr. Rosenberg.  “Because there tend to be fewer staff at such centers, when there is an unexpected complication, there must be prompt, well thought-out, and practiced responses.”

 

Indeed, MHAUS expressed concerns about the problems of an MH event in such settings even before learning of the Florida death.  The spring issue of The Communicator features the article “Planning for the Unexpected MH Crisis: Special Concerns for the Ambulatory Surgery Center.” This resulted from the death of a 21 year old patient in a surgeon’s private operating suite in Beverly Hills in February 2007 under almost identical circumstances to the current death.

 

MHAUS provides many services and products that can be used by ambulatory surgery centers to help them identify, prepare for, and treat an MH event.  Services such as the “Speakers Bureau” and products such as the “In-Service Kit” and the “MH Protocol Poster” can be accessed through the medical professionals section of the MHAUS website at http://medical.mhaus.org/

 

The death of Stephanie Kuleba has garnered not only regional but national attention, as her parents appeared on the NBC Today Show to talk about their daughter and the unfortunate events that led to her death.  The full story can be accessed at http://orlandosentinel.com/news/local/state/orl-office3108mar31,0,1504021.story.

 

The publicity has refocused attention that MH still remains a very real and present danger, especially in ambulatory surgery centers.

 

In what might be the first step in a broader regulation of anesthesia used in office-based surgery, one state, Arizona, has adopted rules governing the use of sedation in the office setting.  The American Society of Anesthesiologists Newsletter reports in its March 2008 issue that “physicians who use general anesthesia in the office or outpatient setting that is not part of a licensed hospital or ambulatory surgical center must obtain a health care institution license as required by the Arizona Department of Health Services.”

 

The rules, which were adopted in January, address: administrative procedures; procedure and patient selection; sedation monitoring standards; perioperative and patient discharge; and emergency and transfer provisions.  The full article can be accessed at http://www.asahq.org/Newsletters/2008/03-08/stateBeat03-08.html

 

Stephanie’s family is determined to raise awareness of MH among the public and the health care industry. They are also determined to make sure that the quality of care in the outpatient setting is equivalent to that in a hospital. They have established a fund in her name that can be accessed via her website at http://www.stephaniejudekuleba.org/Donations.html.

Donations will be placed in a special fund at MHAUS and used to raise awareness of the dangers of MH.

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Study Links MH to Enhanced Susceptibility to Heat Stroke

A mutation in a gene controlling calcium flux in skeletal muscle cells links the rare condition malignant hyperthermia to enhanced susceptibility to heat stroke and sudden death, said a consortium of researchers led by Baylor College of Medicine in a report in the current issue of the journal Cell.

"We created our mouse to be a model for malignant hyperthermia," said Dr. Susan Hamilton, chair of molecular physiology and biophysics at BCM and senior author of the report. Coincidentally, they found that the mouse model demonstrated a link to a condition similar to heat stroke – when the mice were exposed to warm conditions and stressed.

When they initially tested the mouse, they found that the animal did not respond negatively to the anesthetics, probably because the body temperatures of mice regularly drop when they are under anesthesia. However, if they tried to control the animal’s temperature to more normal levels under the anesthesia, its muscles contracted and it had malignant hyperthermic episodes. Later, they found that if the animal was frightened or stressed in a warm room, it had the same response.

"One day, we were in a closed off room that was really warm. All of the animals seized while running on the treadmill," she said. "We found that they were vulnerable to increased temperature. After careful analysis, we realized that the muscle was contracting at temperatures that are normally well tolerated."

Hamilton said there are human medical implications from the study. "There are documented cases of temperature sensitivity in some families with histories of malignant hyperthermia. I think this is telling us something really important."

"Malignant hyperthermia syndrome a potentially fatal inherited disorder is most often ‘triggered’ by certain gas anesthetics and the paralyzing drug succinylcholine. In the naturally occurring animal model, certain breeds of swine, the syndrome is also precipitated by environmental conditions, said MHAUS President Dr. Henry Rosenberg.

"It has long been debated as to whether some cases of heat stroke and exercise-induced muscle breakdown in humans are related to malignant hyperthermia as well,” he said. “This study defines a biochemical pathway that might very well clarify the relationship between anesthesia-induced malignant hyperthermia and heat stroke. This elegant study, using modern molecular techniques, opens new avenues for the study of the not-uncommon problem of heat stroke and exercise-induced muscle breakdown and the risk for malignant hyperthermia."

The full article regarding this study can be accessed at www.cell.com.

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Anesthesiology Publishes “Cardiac Arrests and Deaths Associated with MH”

Researchers from the North American Malignant Hyperthermia Registry (NAMHR) of the Malignant Hyperthermia Association of the U.S. (MHAUS) have published a report in the April 2008 issue of Anesthesiology on “Cardiac Arrests and Deaths Associated with Malignant Hyperthermia in North America from 1987 to 2006.”

 

Drs. Marilyn Larach, Barbara Brandom, Gregory Allen, Gerald Gronert, and Erik Lehman, MS, determined associated cardiac arrest and death rates in cases from Canada and the United States as reported to NAMHR and analyzed factors associated with a higher risk of poor outcomes.  The authors conclude modern U.S. anesthetic practice did not prevent MH-associated cardiac arrest and death in predominately young, healthy patients undergoing low-to intermediate-risk surgical procedures.

 

“Some young healthy patients unfortunately continue to die during MH events,” the authors write.  “To improve our understanding of this potentially lethal condition, we encourage healthcare providers to obtain blood specimens appropriate for molecular genetic analysis on all patients suffering a possible MH-related arrest or death.  We urge anesthesiologists, nurse anesthetists, and intensivists to continue to anonymously report adverse anesthetic events to the Registry.”

 

Contact the North American MH Registry at 1-888-274-7899 or visit www.mhreg.org to obtain appropriate consent forms.  Blood preserved in 5-ml anti-coagulated (purple top/EDTA-preferred) tubes will be suitable for genetic analysis.  If storage is necessary, refrigerate at 4 degrees C.  Results from RYR1 analysis can be linked to clinical event date in the Registry database.

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President’s Blog Goes Online

MHAUS has added a new edition to our website with the President’s Blog now available at http://www.mhaus.org/presidentsblog.  The April blog is titled “The (Re) Discovery of Malignant Hyperthermia.”  Post a comment or check out some of the favorite links.

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