Regular updates by our President, MHAUS staff, and guest contributors.
A few months ago, I received an unusual call from the MH Hotline who connected me to an anesthesiologist and OR team who were desperately trying to manage a Malignant Hyperthermia crisis. They were frantic and clearly trying to stabilize a patient experiencing a severe MH episode. The patient was an athletic older teenager undergoing an elective orthopedic surgical procedure on the ankle. Anesthesia was being conducted with the commonly used gas anesthetic sevoflurane and Succinylcholine had not been used.
In the episode, the young patient undergoing emergency surgery develops elevated levels of carbon dioxide, increased heart rate and elevated temperature. The anesthesiologist recognizes the signs and informs the team of the problem. One of the OR personnel is dispatched to obtain ice and the anesthesiologist whips out a vial of dantrolene, injects it and waits for the signs of MH to respond.
At this joyous time of the year, I would like to wish all the friends and supporters of MHAUS best wishes for the holidays and the New Year. Your support and encouragement of our activities and programs motivates the entire team to continue to bring the latest information and educational programs to those with an interest in this complex, potentially fatal disorder first described over 50 years ago.
From the beginning descriptions of the MH syndrome in the 1960s it was noted that the animal model for MH, i.e. certain pig breeds would develop muscle breakdown, acidosis, and high body temperature with stress alone (“awake MH”, i.e. MH signs without exposure to anesthetic agents).
1981 was a rather important year in my professional life. On July first I assumed the Chairmanship of Anesthesiology at Hahnemann University in Philadelphia. In October 1981 the first meeting of what would turn out to be the founding Board of MHAUS met in my unfinished offices at Hahnemann. It is hard to believe that thirty years have gone by which witnessed the evolution of a patient advocacy organization from an unknown entity to one where anyone seeking information and guidance on MH turns first.
Surprising revelations concerning the association of MH with Drug induced muscle pain; muscle weakness with age; and an inherited syndrome of muscle weakness.
In this month’s blog, I have asked Dr. Stanley Caroff, Professor of Psychiatry at the University of Pennsylvania, a long time member of MHAUS and prolific author on the subject of Neuroleptic Malignant Syndrome (NMS) to summarize several drug related reactions that resemble MH in some ways but are generally thought to be unrelated to the mechanisms that lead to MH.
I was privileged to attend the 30th Annual European MH Group (EMHG) meeting in the town of Nijmegen, Holland from June 8-10 this year. The EMHG was developed in the early 1980s by a group of clinical anesthesiologists and researchers interested in advancing the understanding of MH and providing optimum care to MH susceptibles and their families. The EMHG has focused on testing for MH susceptibility, muscle biopsy contracture tests as well as genetic tests and their relation to biochemical and physiologic changes that underlie the MH syndrome.
On May 14, 2011 MHAUS sponsored the first ever day long retreat for our hotline and professional advisory consultants. 30 consultants, including one board member (Debra Merritt), a genetic counselor associated with the University of Pittsburgh MH DNA testing center (Deanna Steele) and Dr. Albert Urwyler, head of the MH testing center in Basel, Switzerland and member of the European MH group, met in a hotel near the Chicago O’Hare airport to discuss many perplexing issues related to MH.
As part of my “day job” I am in charge of graduate medical education (i.e. residency programs) as well as medical student education at a large academic medical center. In that capacity I and my colleagues who are supervising educational programs are required to keep up with the evolving standards that govern the training of the physicians of the future.
Last month I discussed the difficulty of understanding how MH susceptibility relates to heat stroke, muscle breakdown with exercise and MH without anesthesia. This is an exceedingly complex and murky topic.
On behalf of MHAUS, its Board, Staff, Professional Advisory Council, and Hotline Consultants I would like to wish all of you a Happy and Healthy New Year. Our hope for the future is to fulfill the mission of eliminating death and disability related to MH and MH-related syndromes.
First, I would like to apologize for the somewhat technical blog I posted for October, 2010. I realize that not everyone would be familiar enough with the science of MH and cellular physiology to understand some of the information behind my reasoning. However, in basic terms what I was trying to do was separate MH into subcategories based on what is known of the molecular biology and biochemistry of the disorder. I realize too that not everyone would agree with my reasoning, but that is what a blog is for, i.e., theorizing and proposing some new ways of looking at things.
Malignant Hyperthermia (MH) is a life threatening syndrome characterized by hypermetabolism, hyperthermia, acidosis, muscle contraction, muscle membrane breakdown, hyperkalemia and rhabdomyolysis. The syndrome occurs as a result of exposure to certain drugs and /or environmental conditions.