The Malignant Hyperthermia Association of the US was formed in 1981 in order to fill an unmeet need concerning awareness of MH and its consequences by physicians, nurses and other health care workers. Although by that time, the mortality from MH had fallen significantly from over 70% to below 20%, nevertheless many patients were inconvenienced, given conflicting information and in some case suffered the consequences of lack of preparedness for this potentially fatal syndrome.
Fast forward to 2009 where all anesthesia providers, surgeons and just about all medical and nursing professionals are aware of MH. MH is now described in varying amount of detail in the medical and nursing literature and the mortality from MH is very much reduced (although there are some circumstances where the fatality rate is still about 20%--see my blog of February 2009). The question is “Why is MHAUS still in existence and what purpose does the organization serve?”
Here are some of the reasons why I think MHAUS is still important today.
1. MHAUS is the only organization specifically dedicated to improving the care of patients with MH and MH-like disorders. As our new mission statement says:
"The mission of MHAUS is to promote optimum care and scientific understanding of MH and related disorders."
2. MH is an acute emergency that is likely to lead to patient injury or death if not recognized and treated promptly. Our hotline is the only one in existence that deals with MH. The hotline connects the practitioner confronted with an MH episode or a problem that resembles MH with an expert in the field promptly at no cost 24/7. Hotline calls are received from both the US and Canada and sometimes other countries. There are approximately 700 acute cases that are handled by our hotline consultants annually. Since MH does not happen often, and since the time course of the syndrome is rapid, practitioners cannot take the time to research optimum treatment.
3. MHAUS brings the most recent findings concerning MH to both patients and medical professionals either at no or very low cost and in a convenient manner. For example, the MHAUS web site contains brochures, printed material, power point slides, videos of topics that bring information as to the basic science as well as the clinical care of MH susceptibles and their families.
4. Through the hotline and the North American Registry MHAUS has helped clarify the varied expressions of the syndrome. For example early on, the relation between jaw muscle rigidity after succinylcholine and MH susceptibility was clarified; the recognition that sudden cardiac arrest during anesthesia in young males might be related to an underlying myopathy led to a change in the wording of the package insert for succinylcholine; the recognition that post operative muscle breakdown might be a sign of MH susceptibility; the relation between muscle disorders and predisposition to MH.
5. MHAUS sponsors face to face conferences for MH susceptible patients as well as providers caring for MH patients. No other organization does that.
6. MHAUS supports research efforts aimed at clarifying the diagnosis of MH as well as the underlying defects in the syndrome. Even though the amount of our funding for research is small, nevertheless the interest in the research and the promotion of research findings encourages others to pursue better understanding of the syndrome.
7. The North American MH Registry, of MHAUS, has the largest collection of confirmed MH cases. This includes specific details as to the presentation of the syndrome, the results of treatment and the results of diagnostic testing. This database has been and continues to be a rich source of information concerning the manifestations of the syndrome. Many publications and presentations at national meetings have resulted from mining this database.
8. The MH experts under the auspices of MHAUS develop guidelines and clarification of how the MH susceptible and his/her family should be cared for, thereby eliminating confusion, and inconvenience. For example, MHAUS has created guidelines for management of the MH susceptible for surgery; developed a statement on whether the MH susceptible should/could work in an operating room environment; and outlined how to manage anesthesia care for the pregnant patient who is not MH susceptible but whose spouse is MH susceptible.
9. MHAUS policies and procedures and guidelines for management of MH have been reproduced and reprinted in other languages and have provided guidance for health care professionals in many other countries. MHAUS serves as an exemplary model of how implementation of how risk management and preventive services can improve patient care.
10. MHAUS also brings information and advice to patients and practitioners dealing with other pharmacologically induced metabolic disorders. This includes Neuroleptic Malignant Syndrome, malignant catatonia and serotonin syndrome. The subsidiary organization is called the Neuroleptic Malignant Information Service (NMSIS—www.nmsis.org)
As testament that our educational efforts are still needed, we still receive feedback from MH susceptible patients describing negative experiences. For example some are given faulty information as to optimum care. Others are told that anesthesia poses no problems despite their MH status.
Finally, there is still much more to learn about MH. This includes the expression of the disorder on exposure to anesthesia as well as identification of which patients may be at risk to environmental stresses such as heat and vigorous exercise.
As I have mentioned previously, although MH is an uncommon disorder almost all deaths from MH are preventable (one never says always and never in medicine) with prompt recognition and treatment. The educational programs and specific risk management techniques that apply to MH also apply to many other situations. Since MH is an inherited disorder related to changes in DNA the lessons learned from understanding how those DNA changes are expressed in patients has meaning for many other genetic diseases.
Perhaps there will be a time in the future where all the questions regarding MH have been answered and where new drugs will be in use that do not “trigger” the syndrome so that the programs offered by MHAUS will no longer be needed. I don’t see that happening in the near future. So long as patients die or suffer significance consequences from MH, then MHAUS and similarly focused organizations are needed.