CAUTION WITH ANESTHESIA URGED FOR CHILDREN WITH MITOCHONDRIAL MYOPATHIES

MDA grantee Margaret Sedensky of the Department of Anesthesiology at University Hospitals of Cleveland (affiliated with Case Western Reserve University) was among those who recently found that at least some children with mitochondrial disorders are probably extremely sensitive to gas anesthesia.

Mitochondrial disorders result from flaws in various genes that affect the functions of mitochondria, tiny energy factories that exist in all the body’s cells but are particularly important in cells that need a lot of energy, such as those of the heart, skeletal muscles (including those involved in breathing), heart, liver, kidneys, digestive tract, nervous system and eyes.

Those that affect muscles are covered in MDA’s program under mitochondrial myopathy. (For information about these disorders, see MDA’s Mitochondrial Myopathy Web site and a recently released MDA booklet on the subject.)

MDA’s 2002 Goodwill Ambassador, Mattie Stepanek, and his mother, Jeni, have a mitochondrial disorder. (See Best-Selling Child Poet)

Unfortunately, the study shows, individual reactions to anesthesia in these disorders are hard to predict.

Preliminary results of studies performed at Sedensky's institution have shown that at least some children with mitochondrial disorders become deeply unconscious much more quickly than do other children when exposed to the anesthetic sevoflurane. This was the case for four of 16 children they studied, all of whom received the gas anesthetic sevoflurane. Most of the children were undergoing muscle biopsy procedures.

"All in all, I think clinicians will be interested in this study," said Sedensky, an anesthesiologist with an interest in basic genetics, "as it would caution them to proceed very slowly with very low doses of gaseous anesthetic for children with mitochondrial diseases. Whether this same caution is necessary for other sorts of anesthetic drugs is not clear, but I think the prudent practitioner will view all the drugs in his armamentarium a little more cautiously for these children."

Results of a completed study are scheduled to appear in the journal Anesthesiology spring of 2002.