Looking Back and Looking Forward
2019 marks the 38th year since the formation of the Malignant Hyperthermia Association of the United States. The vision of the founders of MHAUS seemed grandiose at the time, but the accomplishments have been truly remarkable. To enumerate all the activities and programs that have been established even in the past several years would take too many pages, but I urge you to peruse the web site for more information. None of MHAUS’ “signature” programs could have come to fruition without the generous contributions of the members (past and present) of the Board of Directors, the Hotline consultants, Professional Advisory Council members, the members of the North American MH Registry, the staff of MHAUS, and the supporters of MHAUS. Without all these individuals, our initiatives to promote patient safety and advance knowledge related to MH and allied disorders would not be possible.
Thanks to a better understanding of the molecular genetics of MH and the collection of data in the North American MH Registry, as well as the investigations by leading scientists at MH Diagnostic Centers, we continue to see the far-reaching consequences of MH outside the world of the operating room. The insights gained are shared through publications in scientific journals, the publications of MHAUS such as The Communicator, presentations at scientific meetings including MHAUS sponsored conferences, webinars, and discussions with other physicians and scientists at meetings – just to name a few avenues of dissemination of information. Education of clinicians and patients are a core goal of MHAUS, and the members of the Professional Advisory Council and Hotline Consultants are often called on to publish their findings in journals and often invited as visiting speakers at Departments of Anesthesiology, Associations of OR and PACU nurses, and other groups focused on patient safety.
A recent example of how MH experts work together to answer difficult questions is a publication in January 2019 (Anesthesiology 2019, Vol.130, 41-54). The article examines the rationale for stocking dantrolene in ambulatory care centers that do not regularly use MH trigger agents but use the paralyzing drug succinylcholine (an MH trigger) for airway rescue. Using data from the North American MH Registry (2013-2016), the Anesthesia Closed Claims project (1970-2014), the Multicenter Perioperative Outcomes Group (2005-2016), and a comprehensive literature review, it was demonstrated that succinylcholine without volatile anesthetics triggered 24 MH events, thirteen requiring dantrolene. There were over 6 million cases included in the analysis. Without the availability of dantrolene within 10 minutes, the chance of patient harm was increased. The authors of the publication include Drs. Marilyn Larach, Barbara Brandom, Sheila Riazi, Darlene Mashman, Andrew Herlich, Kumar Belani, Erica Sivak, Tae Kim, Janine Limoncelli, and John Capacchione, all MH expert members of the MH Hotline and/or the Professional Advisory Council. Drs. Tom Klumpner, Michelle Vaughn, and Sachin Kheterpal participated from the University of Michigan-based Multicenter Perioperative Outcomes Group. The citation for the article abstract may be found at anesthesiology.org, January 2019, along with an editorial by Philip Hopkins, of the European MH Group. Even though MH is an infrequent event, it does occur without warning and all anesthesia providers need rapid access to dantrolene.
Another important topic that is receiving more attention is the relation between susceptibility to MH and predisposition to exertional heat stroke. We intend to explore this issue in more depth during this year. We have begun a search for an expert in exercise physiology and sports medicine to serve on our Professional Advisory Council.
Our experts are also involved in helping to develop a molecular genetic test with the same accuracy as the muscle biopsy contracture test. Our colleagues in the European MH Group are also focused on these issues. They sponsor an annual meeting to share their information with MH experts around the world (www.emhg.org) and several of our experts attend the meeting regularly.
Genetic changes in the principal gene associated with MH also appear in several congenital myopathies marked by weakness and disability even without drug intervention. This set of disorders is the focus of the RYR-1 foundation (RYR1.org).
The Neuroleptic Malignant Syndrome Information Service (NMSIS) is a subsidiary organization of MHAUS. Neuroleptic Malignant Syndrome is a reaction resembling MH, but is related to certain drugs used in the management of psychiatric disorders. Dr. Stan Caroff, one of our long-standing Board members has developed a series of conferences to bring attention to the disorder.
Several of MHAUS’ programs bring an expert to a hospital or ambulatory center to assist with the development of drills to prepare for an MH episode. (See MH Prep Check)
This past year the North American MH Registry moved from Pittsburgh to University of Florida in Gainesville. We owe a special debt of gratitude to Drs. Barbara Brandom, Greg Allen, and Marilyn Larach for their leadership of the Registry over many years. The team in Gainesville, led by Dr. Nick Gravenstein, is developing an updated MH mock drill training program and reorganizing the data in the Registry to ensure greater accuracy and accessibility.
We continue to learn that MH related to anesthesia is just one aspect, although an important one, of this disorder. We are determined to provide education, information and guidance for clinicians, scientists, and patients to make death from MH a “never event” both in the US and other countries. I look forward to collaborating with all of you in furthering our understanding of malignant hyperthermia in all its various manifestations.
Finally, I would like to wish you a Happy and Healthy New Year and thank you for your continued support.
Henry Rosenberg, MD