Pete's July 1998 Article.
Help Prevent a Tragedy by Pete Bennett
* Our son Brian at 11 months and at 5 years old.
On September 29, 1972 an event happened that would change our lives forever. Im speaking of the lives of the members of my immediate family, myself, my wife Doris, son Ben and daughter Brenda. It was on that day in 1972 that we lost our son and brother Brian Brooks Bennett to the dreaded medical phenomenon known as Malignant Hyperthermia. Remember the name, Malignant Hyperthermia.
Brian was born on 2 Dec 1966 in the St. Marys Hospital in St.Marys, PA. He was a perfect baby, although the doctor informed us immediately after his birth that he had been born with a birth defect. He had a full cleft pallet of his right lip and face. We, as young parents were devastated, yet determined from the very beginning, to see that everything possible would be done for Brian and that he should have the corrective surgeries that would be necessary to ensure that he could and would be raised as a normal child.
Brian was about as normal as our other two children and required no special help when he began to bottle feed, although his cleft was completely open from his lip to his soft pallet, which was split into two at the back of his throat. He had his first surgery to close his lip, at the Williamsport General Hospital, when he was six months old. Everything went perfect and the operation was deemed a complete success, although we knew it was only the first of several surgeries he would need to complete the reconstruction of his face.
There would be other operations to close the cleft and in the meantime he would wear a prosthesis while he had his speech therapy to correct any speech problems that might develop. He did very well at this and for other minor surgeries to guarantee that his teeth would be correctly aligned when they began to come in and these operations all went well, too. He also had surgery at four years old for a hernia repair that also was uneventful.
Brians final surgery, to close and re-construct his soft pallet, was a new and innovative type of surgery, a method that his surgeon recommended and we elected to proceed with, so that he would be completely normal. The surgery was scheduled for September 29, 1972 at 8:00 a. m., also in Williamsport General Hospital where the others all had taken place. Little did we suspect that this would be no normal operation.
As I recall it, the operation was long in duration, planned for about five hours. At about four and one half hours into the procedure, Brians body became very ridged and there was a sudden elevation in his body temperature. These incidents were the first signs, or warnings of the onset of the medical syndrome, malignant hyperthermia. They went unnoticed for a period of time. His temperature reached 110 degrees, the procedure was stopped. Although efforts were eventually made, nothing could be done to revive him. Brian was just 5 years 9 months and 23 days old. No words can convey our feelings at the great loss we had suffered. We grieved his loss immensely and probably never will be free of the terrible remembrance of this profound tragedy. Although his life was a short one, he will never be forgotten by us, his family.
Later, in our searching for a means of acceptance and understanding we learned that MH susceptibility is inherited through a gene passed from a parent and is known as malignant hyperthermia (MH). Of those who develop it, up to 10 percent may die. In severe cases, survivors may be left with brain damage, failed kidneys or impaired functions of other major organs. We had to learn more about it, for as I mentioned earlier, very little was know about it, at the time of his death in 1972.
In 1981 we learned of a group know as The Malignant Hyperthermia Association of the United States (MHAUS) which was founded by a small group of concerned individuals with a personal interest in malignant hyperthermia (MH), together with Henry Rosenberg, MD, an anesthesiologist with extensive experience in treating MH. In 1995, MHAUS merged with the North American MH Registry. The Registry had been established in 1987 to compile a list of know cases of the syndrome for study. The merger between MHAUS and the Registry brought together all the MH data into one organization and allowed for greater focus on critical MH research and clinical initiatives.
MHAUS is the only association in the United States dedicated to the control of MH. Today the membership of the association numbers in the thousands and includes medical professionals from many disciplines, as well as MH-susceptible individuals and their families.
Many MHAUS members have had firsthand experience with the devastating impact of an MH episode. The mission of MHAUS is to educate medical professionals and MH susceptible individuals before MH strikes.
MHAUS is dedicated to reducing the morbidity and mortality of MH by (1) improving medical care related to MH, (2) providing support and information for patients and ( 3) improving the scientific understanding and research related to MH.
Doris and I became dedicated members of the Association shortly after it's inception and are today still dedicated to educating others of MH's existence by directing people to and urging them to support MHAUS. Weve dropped off many brochures and much educational material to hospitals in our area along with attending educational seminars throughout the eastern United States sponsored by the Association. These seminars helped us understand the rarity of this syndrome and directed us to the wheres and hows of understanding that all our relatives, immediate and future, may suffer a similar tragedy while undergoing surgery. Anyone in our family may carry the gene that may be sparked by anesthetics that would send them into a similar episode. As you read on you will find that there is no simple test to determine susceptibility. Although there are tests that can be conducted, they just are not accurate enough. We, did have one family member tested using one of the methods available and it is believed this member would be 100% positive if exposed to conditions in a similar situation.
Here are some of the questions we had prior to our discovery of the Association and the answers they provided us back then and that they still provide today for those interested in preventing a great tragedy such as the one my family suffered. Read them and feel free to contact MHAUS ANYTIME.
WHAT IS MALIGNANT HYPERTHERMIA?
MH is a chain reaction of abnormalities (a syndrome) triggered in susceptible individuals by commonly used general anesthetics. The signs of MH include a greatly increased body metabolism, muscle rigidity and eventual hyperthermia which may exceed 110 degrees F. Death can result from cardiac arrest, brain damage, internal hemorrhaging or failure of other body systems.
WHO IS SUSCEPTIBLE TO MH?
MH susceptibility is inherited. The basis for the underlying problem for many families is a single defective gene, usually inherited from one parent. In other families the genetic pattern is not yet identified.
Those who are susceptible may be completely unaware of this risk unless exposed to anesthetics leading to a life-threatening crisis. And, it should be noted that not everyone who has the MH gene develops an MH episode upon each exposure to triggering anesthetics.
WHAT DRUGS TRIGGER MH?
The volatile gaseous inhalation anaesthetics (e.g., halothane, enflurane, isoflurane, sevoflurane and desflurane) are MH triggers, as is the muscle relaxant, succinylcholine.
ARE OTHER ANESTHETICS SAFE?
Yes. Barbiturates, narcotics (opioids) and tranquilizers, along with the inhaled gas nitrous oxide, are safe for MH-susceptible persons. Local anesthetics (both amide and ester) have also been found to be safe. Drugs routinely used for sedation and intravenous aesthetic induction agents (which are injected to induce loss of consciousness prior to the administration of a more potent drug) also do not induce MH.
Safe muscle relaxants include pancuronium, atracurium, vecuronium, pipecuronium, mivacurium, doxacurium and rocuronium.
WHAT IS THE INCIDENCE OF MH?
The exact incidence of MH is unknown. The rate of occurrence has been geographically estimated to be as frequent as one in 5,000 or as rare as one in 65,000 administrations of triggering general anesthesia.
WHAT CAUSES AN MH EPISODE?
Although the cause of MH is not yet known with certainty in all inherited forms, research evidence points to a generalized derangement of the processes which regulate muscle contraction.
The triggering agents induce increased concentrations of calcium in the muscle cells. High calcium levels cause the muscles to contract and become rigid, leading to greatly increased metabolism. This process results in heat production (hyperthermia) and muscle cell breakdown.
WHERE CAN A PATIENT OR HEALTH CARE PROFESSIONAL GET MORE DETAILED INFORMATION ABOUT MH?
Most of the information presented here was copied in part from the MHAUS internet site at: http://www.mhaus.org/. Visit the site for all the information provided there, for it is the best source of information and educational materials for health care professionals and MH-susceptible individuals. Call or write today to help prevent a tragedy:
MHAUS also operates a 24 hour HOT LINE that responds to questions concerning MH episodes. Names and phone numbers of on-call anesthesiologists available to consult in MH emergencies may be obtained 24 hours a day:
1-(800) MH HYPER
Outside the US call: 001-1-315-464-7079
Thanks everyone for your indulgence. This article is only intended to provide you with an awareness of the existence of this medical phenomenon, MH. Till next time.....
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