by Henry Rosenberg, MD President, MHAUS
The Congress is the meeting of the World Federation of Societies of Anesthesiologists (www.anaesthesiologists.org), an organization that represents scores of anesthesia societies around the world. In March of this year the meeting was held in Buenos Aires, Argentina. I was invited to put together a four-hour segment of the meeting related to MH. With the able assistance of Dr. Sheila Muldoon, Dr. Kumar Belani, Dr. Jerry Parness, and Dr. Tae Kim, all of the US, and Dr. Robyn Gillies from Australia, we covered topics related to the clinical presentations of MH, pathophysiology of MH, genetic testing for MH, treatment and management of MH, management of MH in the outpatient setting as well as anesthesia machine preparation for the MH susceptible. Even though the session took place on the last day of the meeting, the room was filled. The attendees asked good questions and were very complimentary of the presentations. It was a great event and I believe we provided important information concerning MH for anesthesiologists who ordinarily do not have the opportunity to hear in depth presentations on the subject. We learned that there are still many countries that do not have dantrolene and as a result people still die from MH in many parts of the world. In particular, Dr. Belani has learned of deaths and near misses from MH in India, where most hospitals do not have dantrolene. He has been in active communication with anesthesiologists in India who are now interested in obtaining the drug and educating anesthesiologists about the management of the disorder. India has a population of over 1 billion people! In addition several of hospitals in India have been established by highly trained physicians who gained their experience in the US, UK and other European countries. A brisk â??medical tourismâ?? business has developed in India. There is no doubt that there are many cases of MH that are not reported regularly. India is not an exception. Many other countries also lack adequate supplies of dantrolene. Additionally, education on recognition and management of MH patients is also limited. MHAUS will do whatever we can to assist health care providers in any country to recognize, treat and prevent MH.
At the end of the meeting, I met with Ms. Laura Vitcop from Buenos Aires who has a history of MH in her family and is interested in providing more in depth education about MH to patients and families who are affected by MH in Argentina. She and a lawyer from Buenos Aires, Alfredo Mayol, along with others have organized a patient advocacy umbrella group, APAC (www.apac.org.ar) which stands for the Civil Association Help for Critical Patients. The organization provides information for patients with liver diseases, rheumatoid arthritis, Crohnâ??s disease and MH. It is the intent of the organizers to develop an MH organization in Argentina patterned after MHAUS. They have secured support from Astra Zeneca Company and are seeking additional funding to develop the MH component of the organization. I offered that MHAUS would be pleased to provide guidance, copies of our educational material and assistance in whatever way we can. I look forward to continued communication with Ms. Vitcop and the organization. I will keep you informed of our progress, or you may follow the conversations on the MHAUS Facebook site.
Yet another important event took place shortly after the World Congress Meeting. The Society for Inherited Metabolic Disorders (www.simd.org) is a society of scientists and clinicians that focuses on inhertited disorders of metabolism. Many of them are included in mandatory screening in the newborn period by blood tests. Generally the disorders are genetically determined enzyme deficiencies that require special diets, medications, or avoidance of triggers to reduce the occurrence of significant problems that may have implications for growth and development and malfunction of a variety of important organs. These include amino acidopathies (e.g., phenylketonuria.) Other examples of inborn errors of metabolism include lysosomal storage diseases, organic aciopathies, and metabolic muscle diseases, to name a few. Through communication and collaboration with Dr. Georgirene Vladutiu at the NY State University at Buffalo, MHAUS was invited to provide a lecture at the Society's annual meeting on April 1, 2012. Dr. Cynthia Wong graciously accepted the offer to present an overview of MH for the group. This was the first time MH was discussed in a formal way with members of the Society. I am told that Dr. Wong's talk was a great success and there is a lot of interest in furthering communication and possible collaboration between the two societies. This makes a good deal of sense to me because MH is indeed an inherited metabolic disorder, even though the manifestations are generally episodic. Common ground between the two societies is based primarily on an interest in genetic changes that affect organ system function. We are indebted to Dr. Vladutiu for making us aware of the Society. Although Dr. Vladutiu is in charge of a laboratory that provides testing for inborn errors of metabolism (the Guthrie lab at the University of Buffalo), she is also interested in the effects of cholesterol lowering drugs (statins) on muscle function. A significant percentage of patients taking such statins complain of muscle pain and some have significant muscle pain, muscle destruction and even life-threatening symptoms related to these medications. Her investigations have shown that some of the patients with severe muscle pain from statin agents also harbor mutations associated with MH susceptibility! The implications are obvious. She is now forming collaborations with various MH testing laboratories to further investigate the relation between muscle destruction, statin ingestion and MH.
I would also like to provide a follow up to our ongoing efforts to create consensus statements on topics of importance related to MH. To date there are two such statements, one concerning anesthesia machine preparation and the other temperature monitoring during anesthesia. These statements are now posted on the MHAUS web site. We have also just concluded work on a statement related to the association of heat and exercise with MH susceptibility. It is important to point out that the information is not intended to be a guideline, which has a very specific meaning in medicine, but a statement of best evidence, together with the rationale for the statement. We intend for these recommendations to assist patients and clinicians in the care of the MH susceptible or potentially susceptible patient in a straightforward, direct and succinct manner. Other statements are in process, including some on the relation of various muscle disorders to MH. We have previously made recommendations on management of the pregnant woman who is not MH susceptible but whose husband is MH susceptible; the risk for MH-susceptibles to work in an operating room where they might be exposed to anesthetic gases (it is safe). We have followed a rigorous process in the development of such statements including review by our hotline consultant and professional advisory committee members along with a period of public comment and revision based on feedback from the various groups.
Here is the most recent statement:
Our MHAUS Scientific Officer, Sharon H. Dirsken, PhD has brilliantly coordinated these statements. Sadly, Sharon has recently advised us she will be leaving her position at MHAUS to take on new challenges incorporating her strong writing talents in order to teach courses on science writing at the University of Rochester, her home town, as of May 11, 2012. We will miss Sharon's drive and enthusiasm and the strong bridge she provided between the clinical and patient perspective, and her role as a conduit to bring educational projects to fruition. We wish her all the best in her new path and know that she will be a positive influence to those she interacts with in the future.
This is just a sample of some of the activities and concerns of MHAUS more projects are in the pipeline. Our dedicated hotline consultants and professional advisory council members contribute their time and expertise to the development and implementation of our panoply of educational and patient care activities.
Of course our Board of Directors and the MHAUS staff are fully engaged in the mission of MHAUS.
Your encouragement and support in word and deed is essential as well. We always want to hear of your comments.