Anesthesia and the CMT Patient
by Joseph F. Antognini, M.D. University of California Davis Medical Center

Anesthesia is safer now than it has ever been. This is particularly true for individuals who have medical problems, including patients with Charcot-Marie-Tooth disease. Two important reasons for this improved safety are better monitoring devices and shorter-acting anesthetics. How do these improvements specifically impact patients with CMT?

First, the last decade has seen the introduction of pulse-oximetry and capnography into routine anesthesia practice. Pulse-oximetry can measure the amount of oxygen in your bloodstream...a small "Band-aid" is placed around a finger, earlobe or toe, and a special light is passed through the finger and a receiver on the other side captures the light and is able to tell your anesthesiologist how much oxygen is in your bloodstream. This technology allows your doctor to watch how well your lungs are working to deliver oxygen to the rest of your body.

Capnography measures the amount of carbon dioxide that your lungs are producing, and this is an important way of detecting changes in your breathing while you are asleep for your surgery. Because breathing problems are an important cause of anesthetic complications, this monitor has improved our ability to detect problems early, thus decreasing serious complications. These monitors are important for CMT patients, since they may have lung problems which they don't know about.

New anesthetic drugs have the advantage of being shorter acting, so that patients wake up faster. This may be important for the CMT patient, since anesthetics depress breathing, and in the light of the potential lung problems in CMT patients, these newer drugs have an obvious advantage. These new drugs include propofol and desflurane.
Since many patients with CMT have orthopedic surgery, usually on their legs or feet, the best choice for anesthesia would be a regional anesthetic such as a spinal or epidural. Both of these "numb" your body from the waist down and last for 1-3 hours or longer, depending on what type of anesthetic drug is used. These anesthetics avoid general anesthesia, which depresses the whole body. If your surgery is very minor, you may on need a local injection of anesthetic around where your doctor is going to do the surgery...this is the best choice, if possible, since you can recover quickly and go home.

Your anesthesiologist may speak to you the night before surgery, or, quite often, just prior to your surgery. This sometimes prevents you from having a lengthy and informative talk about your anesthetic. It is important that you have every question answered, so as to allay any fears. Several days before your operation, ask your surgeon who your anesthesiologist is going to be, and call him or her. Tell him/her about your CMT and any problems that you may have had with anesthetics in the past. In this way, you can make sure that they are aware of your condition, and they can develop an anesthetic plan that is safest and best for you.

Remember, the type of anesthetic you receive is a decision made by you and your anesthesiologist. There may be reasons why your anesthesiologist might recommend an anesthetic different from what you might be thinking about...every case has to be individualized. But rest assured that no matter what choice is ultimately made, anesthesia is safer than it has ever been!

Editor's note: - You have every right to know who your anesthetist is and to talk to him or her. If you do your planning ahead of time and let your doctor know you want to talk to your anesthetist, it can be easily arranged. Most people just leave everything in their doctor's hands and then they worry and stew. Have CMT information for him, make sure he has it and you have the answers you need.