Sample Letter to Family Members

Dear

I am writing to let you know about the potential susceptibility to malignant hyperthermia in our family.  We have discovered a genetic trait, which could have a potentially tragic effect on even distant relatives if they require surgery under general anesthesia.

We learned about this when ___________________ was given a commonly used general anesthetic and then had a life-threatening reaction.  Although we didn’t realize it, _______________________ was at risk for developing this reaction because of an unusual hereditary muscle disorder.

This disorder makes a person susceptible to developing a chain reaction of symptoms (a syndrome) called malignant hyperthermia (MH).  (Don’t be over concerned about the word “malignant” – MH has nothing to do with cancer.)  MH occurs when a susceptible individual is given the drugs that “trigger” MH.  The triggering drugs are:  volatile inhalation anesthetics and succinylcholine, a muscle relaxant.

The reaction, which may (but does not always) occur, includes a variety of symptoms:  muscle rigidity; dramatic changes in the acid content of the blood (which can cause irritation to the heart and possible cardiac arrest); increased amounts of carbon dioxide expelled by rapid and deep breathing (hyperventilation); breakdown of muscle, as indicated by very dark urine; and high body temperature (a late sign).

This syndrome is reversible if the operating team is prepared to act quickly.  And, the severe problems associated with the syndrome are preventable if doctors know in advance of their patient’s potential MH susceptibility and take certain precautions.

Although MH is uncommon in the general population, the odds are quite high for family members of known susceptible patients to also have the trait.  Most susceptible parents transmit the trait to about half of their offspring.  It’s very important to note that more than 50% of MH-susceptible patients had general anesthesia on at least one occasion (uneventfully) before they even knew they were susceptible.  You should not assume you are safe because you have had uneventful anesthetics.

The enclosed pamphlets are published by the Malignant Hyperthermia Association of the United States (MHAUS), a non-profit educational organization that provides a variety of information materials about MH and what you can do. 

Right now there are several steps you should take:

Ø      Please let me know about any anesthetic reactions you or others in the family have experienced.  Has anyone in the family died “mysteriously” during or following surgery?  This information can help determine which family line carries the MH trait.

Ø      Notify your own doctors about your potential susceptibility.  MHAUS can provide you with literature to give them.

Ø      Notify school nurses and teachers and make a note in your children’s medical records.

Ø      Contact hospitals in your area to be sure that they stock the antidote drug, dantrolene sodium for injection, and that they are prepared to care for your family in an emergency.  MHAUS has a form letter you can use for this purpose.

Ø      Consider obtaining a medical ID tag such as a bracelet or necklace.  MHAUS has a Medical ID Program of its own with the MH Hotline phone number already engraved on each tag.

At present, there is only one accurate diagnostic test for MH, and it involves having a muscle biopsy.  MHAUS can send you more information about the test and a list of the testing sites in the United States which perform it by contacting:  MHAUS, PO Box 1069, Sherburne NY 13460-1069 / 1-800-986-4287.

If you establish that you are, indeed, carrying the MH gene, you should talk with your doctor about registering yourself with the North American MH Registry of MHAUS as an MH-susceptible individual.  You may contact the Registry at the University of Pittsburgh Children’s Hospital, Anesthesiology Dept, Room 7499, 3705 Fifth Ave at DeSoto St, Pittsburgh, PA 15213-2583 / 412-692-5464.

                                                            Sincerely,

Enclosures