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Remarks made at the MHAUS reception, December 14, 2014

I would like to welcome you to the first MHAUS reception at the NY Post Graduate Assembly. Thank you all for coming.

Particular thanks go to Eagle Pharmaceuticals, the company that developed and introduced Ryanodex, a new formulation of dantrolene introduced into clinical practice this past summer which is perhaps the most significant advance in treatment of MH in over 30 years.

I also wish to acknowledge the support from companies who have or who are currently marketing the Dantrium formulation over the past several decades, first Procter and Gamble Pharmaceuticals, then JHP Pharmaceuticals, and most recently, Par Sterile Products.

In addition to support from commercial organizations, I would like to recognize the contributions from medical and nursing societies such as the ASA, AANA, NAPA, Dynasthetics, US WorldMeds, Prevention Genetics, numerous state societies of Anesthesiologists, individual physicians and nurses, and many, many patients and their families. Without their support, the work of MHAUS could not occur. 

MHAUS was formed in 1981 by four patients and their families; Suellen Gallamore, George Massik, Owen Davidson, Robert Luckritz; and I was invited to be the physician representative to the new organization.

MHAUS is overseen by a Board of Directors, made up of   anesthesiologists, nurse anesthetists, patients and physicians from other specialties, whose names you can find on our web site (mhaus.org) and is run by a small, but dedicated staff of five employees located in Sherburne, NY.  As one of the visitors to the booth stated, when I told him that we only have five employees, he thought the organization was much bigger. The impact of MHAUS is much bigger than could be expected by a small staff.  Dianne Daugherty, our executive director, and Gloria Artist are here tonight.  Fay Kovak, Sarah Prosser, and Michael Wesolowski are the full time staff.  In addition, MHAUS supports the North American MH Registry based at the University of Pittsburgh Medical Center.  Barbara Brandom, MD is the director of the Registry.

One of the most gratifying aspects of my involvement with MHAUS has been the volunteer efforts of the Board, the thirty plus hotline consultants, the members of the professional advisory committee and members a subsidiary organization, called the Neuroleptic Malignant  Syndrome Information Service.  These experts devote their time to enhancing all of our efforts to fulfill the mission of MHAUS:The mission of MHAUS is to promote optimum care and scientific understanding of MH and related disorders.”   There simply is not enough time to review and describe all the activities related to MHAUS.  However, here are a few highlights:

THE MHAUS HOTLINE.  The hotline has been in operation since 1982 and staffed by a group of dedicated MH experts. Through a partnership with the Medic Alert Foundation, we receive several thousand calls to the hotline each year. Following triage, a volunteer expert hotline consultant is contacted and speaks/ advises the calling clinician about 700 times a year!  The information gathered about MH has been crucial for better understanding. Last year, Dr. Ron Litman, who is an anesthesiologist at Children’s Hospital of Philadelphia, took on the directorship of the hotline in order to better catalog and follow up on calls.

MHAUS WEBSITE AND FACEBOOK PAGE.  This is how people keep up with the latest information in this day and age.  The web site is maintained by the staff at MHAUS with input from a Web Oversight Committee and our Professional Advisory Committee. The website is accessed several million times every year from all over the world. Keeping up with the flow of information consumes enormous amount of time, particularly updating existing information.

PUBLICATIONS AND NEWSLETTERS.  The Communicator, our major newsletter, was first published in 1981 and is still published four times per year! A few years ago we supplemented this publication with an   e-Newsletter.  We continue to revise paper and electronic manuals, brochures and guidance documents.  Most recently Dianne, with the help of patients and consultants, developed a guide for clinicians and patients related to testing for MH.  It is on the web site and called the Road Map for Testing

CONFERENCES.  Last year we held our second very successful Scientific Conference on MH.  The focus was on the ryanodine receptor and its implications for MH and other related disorders. The meeting was held at the University of Toronto, who cosponsored the meeting.  Thanks are due to the MHAUS diagnostic unit director, Dr. Sheila Riazi.

For several years we have sponsored Mini Conferences. Developed for locales who want to host a meeting for patients and providers concerning MH. The MHAUS office works with the local organizers to make that happen.

Meetings of the hotline consultants. The most recent meeting of the hotline consultants and professional advisory council members took place in Sept 2014. The meeting was held to develop consensus-based recommendations on topics that bear on the clinical management of MH, such as disorders that predispose to MH; and the relation between MH and heat stroke.              

Exhibits at meetings of anesthesia providers such as this one and the Association of OR nurses. Did you know that OR and PACU nurses are some of the major supporters of MHAUS? 

THE NORTH AMERICAN MH REGISTRY OF MHAUS.  Originally developed as an offshoot from a series of consensus conferences on creating standards for diagnostic testing for MH in the 1980s, it now is a database of highly specific information concerning 2,500 or so actual cases of MH.   A series of scholarly publications have emanated from the Registry, most recently one documenting the critical importance of intraoperative temperature monitoring in assuring successful outcome from MH.  Particular thanks for extracting valuable information from the Registry go to Dr. Brandom and Dr. Marilyn Larach.

THE MH PREP CHECK. This is a new program that enables a hotline consultant to review a facilities' preparations for an MH crisis, to attend the facility’s mock drill and provide feedback to the facility.  A certificate documenting the review is provided and facility’s participation posted on the MHAUS web site.

RESPONDING to hundreds of queries from patients and providers each year about MH and summarizing the responses on our web site.

Thanks to the programs I have mentioned and others that I have not included, MHAUS has become the “go to” organization when questions arise about MH or related disorders from all over the world.

Since the founding of MHAUS in 1981, great advances have been made in the understanding of MH, prevention of MH, recognition of the syndrome in and out of the OR, treatment of MH, laboratory diagnosis of MH and the basic science of the disorder. There has also been a tremendous growth in knowledge and recognition of the importance of this potentially fatal disorder by many specialties. Chapters in textbooks on MH did not exist in 1981! Now just about every medical and nursing textbook contain chapters or extensive information on MH.

Thanks to an understanding of the DNA changes that underlie MH and the relation to clinical manifestations of those changes, MH is clearly recognized as a prototypical pharmacogenetic disorder. Pharmacogenetics, also referred to as personalized medicine, is the wave of the future in medical care. Taking advantage of the increasing knowledge of molecular genetics, we are slowly moving from the cumbersome muscle biopsy contracture test to a genetic based test. However, we are not there yet.  The sensitivity of genetic testing is limited.  That means there are many who have experienced an MH crisis who do not display changes in the known genes associated with MH.  But then again, there are genetic changes and other genes that influence the expression of MH that have yet to be discovered.  The muscle biopsy contracture test is still needed.

Through studies of the molecular genetics of MH, investigators have determined that the molecular genetic changes found in the genes related to MH are also found in a wide variety of uncommon, but nevertheless significant muscle disorders such as Central Core Disease and Nemaline Myopathy.

In addition, we have learned that some patients who develop heat stroke or exercise-induced rhabdo-myolysis also manifest MH causal mutations.  However, the treatment of heat stroke is still based on surface and intravenous cooling.  Each year about 4,000 people are admitted to an ER with heat stroke and about 160 die.  Although most are elderly and have other medical conditions, nevertheless some are young and fit.  You read about those deaths every year in the press, however, I am sorry to say that the research related to the connection between MH and heat stroke is mostly anecdotal and scattered.  There is a tremendous need for more information.   There is now promise that Ryanodex, because it is so easy to get into solution and a small volume can deliver a large dose of dantrolene, will be effective in the acute treatment of heat stroke. 

It is the mission of MHAUS to bring the latest information, the best evidence based suggestions for diagnosis and treatment concerning MH to clinicians all over the world.  Besides the website, a few of our consultants present information each year at the European MH Group meeting.  In addition, recently, one of our hotline consultants, Dr. Kumar Belani, organized the first ever conference on MH in India.  A few years ago, Drs. Tae Kim and Barbara Brandom went to Indonesia to speak on MH.  Several of our experts organized an educational workshop and seminar on MH at the World Federation of Societies of Anesthesia in Buenos Aires in 2012.

Although  mortality from anesthesia induced MH has been reduced  from about 70% in the early 1970s to probably less than 10% in recent years (we are not even sure of the exact percentage), the morbidity from MH is much higher and even one death from MH, especially because it is mostly young people who succumb is unacceptable to us.

As much as there is more work to be done in the advanced countries of the world, the need is even greater in less privileged parts of the world.  Many countries do not have dantrolene and even some who do, have it in a location that may be many minutes away from a hospital.  We don’t even know how many people die from MH in countries such as the Philippines, China and India, but we do know that there are MH-related deaths there.

In conclusion, I would like to thank all of you who are here this evening for your support and continued interest in MHAUS. I also want to thank all our supporters, both members and non members who enable us to continue to advance the understanding, diagnosis and treatment of MH and related disorders.  There is still a lot more to know and understand about this fatal disorder.

Now it is my privilege to bestow special recognition to one of our most devoted MH experts.   He has been a member of the hotline for many years and is a regular contributor to our discussions and analysis of cases of MH. He has been the chairman of our hotline quality improvement committee where random cases are selected and analyzed by a group of consultants to make sure that we are giving the best advice at all times.  The information from the review is then compiled and distributed to all the hotline consultants.  He has been the Chairman of Anesthesia at Bridgeport hospital for many years and a devoted member of MHAUS. Dr. Charlie Watson is not only an expert in MH related topics, but also has expertise in critical care medicine and in airway management and serves on the advisory board of Anesthesiology News.

Thank you Charlie. You truly make a difference.

As you plan your year end giving, please keep MHAUS in mind.  Best wishes to all for a healthy, happy holiday and New Year.

[IMAGE: Henry Rosenberg, MD presenting award to Charles Watson, MD]
Henry Rosenberg, MD presenting award to Charles Watson, MD

This item filed in the following categories:
  • General
The mission of MHAUS is to promote optimum care and
scientific understanding of MH and related disorders.