DWQA QuestionsAnesthesia protocols for CPT II patients
Susan wishnick asked 5 years ago
there seems to be some confusion or disagreement among patients and different metabolic physicians over the issue of which general anesthesia agents, separately or in combination, are the safest for patients with CPT II. There also appears to be no settled opinion or expertise among anesthesiologists. Is there a recommended protocol for patients? This is a particular concern in the event of an emergency in which surgery is necessary immediately without time to consult or evaluate the pros and cons of different approaches. 

1 Answers
INFORM Physician answered 5 years ago
I actually don’t advocate any special anesthetic regimen for my patients with FAODs in general and CPT2 specifically. The literature on anesthesia in fatty acid oxidation defects is minimal there isn’t anything specific to CPT2 deficiency. While there might be a temptation to be restrictive and follow guidelines for mitochondrial respiratory chain deficiencies due to the functional relationships between the two disorders, I think this is probably a mistake. In discussing the issue with my anesthesiologists, they argue that going to a second choice anesthetic agent on the basis on an unsubstituted theory (especially one that is rather indirect) exposes the patients to increased real anesthetic risks. This makes sense to me and thus I let the anesthesiologist dictate their therapy and just make sure they are aware of the risks related to metabolic stress in a patient with chain fatty acid oxidation defects. Specifically, I admit the night before to and give IV glucose when the patient is made NPO, use glucose throughout the procedure and recovery, and monitor carefully until PO intake is reestablished. Of course the anesthesiologist should have dantrolene available regardless of an FAO diagnosis in case of malignant hyperthermia. I resist any requests to perform procedures on an outpatient basis. I’ve not noticed an increased incidence of adverse anesthetic events in my patients. Hope this helps. Dr. Vockley