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June 24, 2008

Comments

Michael Wesolowski

Dr. Thompson, do you know about the MHAUS Facebook Cause Page of the Malignant Hyperthermia Facebook Group page? If not, I invite you to visit.

Joe Tobin

Hi Dr. Thompson,
I am an anesthesiologist who runs a Malignant Hyperthermia biopsy center. I agree that we do not have a sufficient number of well trained pediatric neurologists or muscle pathology specialists to help patients with muscle disorders. My cautions from your blog include the following: We do perform muscle biopsies for patients at risk for Malignant Hyperthermia with general anesthesia using what is called the safe technique, where the agents known to trigger the illness are not used. Also, if a biopsy specimen is to be obtained for Malignant Hyperthermia testing, it can only be performed in one of 5 centers in the U.S., the test is on live muscle and local anesthetic cannot be used as it prevents the muscle from contracting and invalidates the test. Certainly when there is a concern about a muscle disease which may be associated with Malignant Hyperthermia, a patient should be referred to a center which is capable of examining the tissue for Malignant Hyperthermia and other diseases. The procedure is done on an outpatient basis and usually only takes about an hour to an hour and a half.

Charlotte E. Thompson, M.D.

Dr. Tobin,
I am aware of the biopsy centers for MH and the technique used to do the study. The problem is that many parents don't want a biopsy done at all, even to make a diagnosis. Deaths still occur from MH, as you know. Many muscle disease patients are either undiagnosed or improperly diagnosed. Thanks for your reply.

Kindy

I am a "lifetime" orthopedic patient. I was never diagnosed as a child, but when I became an adult the doctors insisted I had arthrogrypsosis...as does my first born son (and he has it much more severely). Throughout my childhood I was told I did NOT have AMC, but something that mimicked it due to the fact that I had normal strength in my limbs. I was never told what it was that mimicked it. It was frustrating. My mother took Bendectin when she was pregnant with me in 1969 and we had a lawsuit at the time, but it has since been dropped for a myriad of reasons. I had genetic testing, which came back normal. So it is possible to live in the US and still not have an accurate diagnosis.

My son had a spinal fusion in April 2008 and due to an anesthesia error, aspirated after surgery and nearly died with ARDS and septic shock. He has always had complications from anesthesia.

Charlotte E. Thompson, M.D.

Kindy,
I am so sorry to hear of your difficulty in getting a diagnosis for a neuromuscular disorder. In the United States, this is unfortunately a common problem. There are few pediatric neuromuscular training programs in the U.S. Since I am no longer seeing pediatric neuromuscular paients, the only place in the U.S. that I am comfortable sending a child for a diagnosis is the Mayo Clinic. They do their muscle biopsies using a local anesthetic and have facilities to do all the newest studies.

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