Linda here - Dr. Stuart Patterson, hand surgeon and Assistant Professor at the University of Western Ontario, London, Ontario, Canada, agreed to talk to me about CMT and hands. Having extremely weak hands myself, I thought I'd ask the questions I'd like to know the answers to and I'm sure he will answer others for us in the future if you send them on to me at CMT International.
CMTI: Thank you, Dr. Patterson, for agreeing to answer questions about CMT hands and surgery. In the last 13 years I've seen all kinds of hands: extremely weak, limp hands; clawed but pliable hands; clawed and rigid hands; hands with the fingers curled right into the palm; rigid, almost normal hands; and hands that appear normal but are so weak when you shake hands they feel like cotton.
Can you please tell me what initial functions you look for when you examine a CMT person's hands and arm?
Dr. Patterson: The most important issue to be determined is the degree of disability the person experiences. If there is no significant disability then issues regarding the potential for future disability arise. If the person has some disability, this needs to be clarified (what can you not do?) and a determination made as to the severity of this. If the loss of function is impairing the individual's ability to care for themselves or prevents them from doing activities that they enjoy, one would be more likely to recommend surgery. In general, the best upper extremity to operate on is the hand that has supple joints, good sensation and strong muscles originating in the forearm. The person who has severe weakness of all muscles in the upper extremity, with or without stiff hand joints is a poor candidate for reconstructive surgery.
CMTI: If the person has lost the ability to pinch forefinger and thumb together, what would they have to have left to repair that and how successful do you think these kinds of operations are?
Dr. Patterson: The loss of ability to pinch normally is commonly seen in the CMT person's hand. Surgical restoration of this ability is often one of the most gratifying procedures to perform. The basic requirements are: 1) A supple hand without contractures or stiffness; 2) Strong forearm muscles, as the tendon of one of these muscles will be used to restore thumb motion. The results are excellent (90% chance of good function) and predictable, in the selected patient.
CMTI: Can anything be done to reduce the clawing of fingers?
Dr. Patterson: Yes! This can be treated either with splinting or tendon transfers. Again, the results are predictably good.
CMTI: People who have lost the feeling in their hands but still have movement would like to know if anything can be done to restore the feeling. What causes the loss of sensation?
Dr. Patterson: The loss of sensation is usually due to the same process that injures the motor nerve to the muscle. The damage to the nerve sheath results in the sensory nerve being unable to send messages back to the brain. This cannot be surgically reversed. However, some CMT patients have compression of the peripheral nerves. For example, the median nerve may be compressed under a ligament at the wrist, causing numbness in the hand. This is called carpal tunnel syndrome. You can also get carpal tunnel syndrome if you have CMT. Unfortunately, carpal tunnel syndrome may be missed, as the loss of feeling may be attributed to the CMT instead. The best way to differentiate the two is by electrical testing. Many people with CMT find this test very distressing though.
CMTI: What can be done for tightly fisted fingers that are stiff? I've seen hands that are so tightly fisted the person uses their wrists to lift cups and hold brushes.
Dr. Patterson: First of all, do not allow them to get this way! Joint stiffness or contractures can be prevented by a combination of splinting, range of motion and reconstructive surgery. Once the joints are stiff, it is extremely difficult to reverse the process. Release of the soft tissue contractures alone often does not work. In general, this sort of hand will require a combination of splinting, therapy and surgery to achieve a functional result. The splinting and therapy is mainly used in the early stages when the tissues can be stretched to allow correction of the deformity. Surgery would include either joint fusions or tendon transfers to correct the muscle imbalance.
CMTI: Do hand exercises help? Personally, the more I use my hands, the weaker they get.
Dr. Patterson: I know of no scientific evidence supporting exercise as an effective treatment for CMT. Given that the muscle bulk has decreased in the hand, one would expect the remaining muscles to fatigue sooner with sustained exercise. As with most things in life, moderate activity to maintain muscle bulk, endurance and strength is probably appropriate. The discomfort or weakness experienced in the hands, following use, should be used as a measure of what you can or cannot tolerate.
CMTI: What about bracing: Is it better than surgery for some?
Dr. Patterson: Individual patients have different perceptions and expectations of bracing. Some find the splints cumbersome, uncomfortable or just totally useless. Some do not like to use a splint because it draws attention to their impairment. Splints are not for everyone, but everyone should be given a trial of splinting. Some deformities are well treated with splints, e.g. clawing of the fingers, while functional losses, such as the loss of pinch function, are not amenable to splinting. In the latter case, surgery is a better option.
CMTI: In the past, we, as people with CMT, have always been told that to put a hand and wrist in a cast is to lose the function of the hand, as no amount of physio will bring back muscles that have atrophied from being held still in a cast for a matter of weeks or months. Is this a myth or are there new techniques? How do you keep the hand strong and not lose muscle through atrophying while it is healing and recovering from the surgery?
Dr. Patterson: Anyone who has an extremity immobilized will notice that their muscles rapidly atrophy. This can be seen, for example, within three days of injuring your knee. The quadricep muscles of the thigh become flabby and lose their bulk and tone. People with CMT have less muscle bulk to start off with and not surprisingly, when they have their extremities immobilized, the muscle atrophy that occurs has a more severe effect on their strength. We all have the ability to regain most of this muscle bulk, but it may take up to a year.
I suspect that the ability of CMT patients to regain their lost muscle is compromised. I am not aware of any research that has been done in this area on patients with CMT. When I do hand surgery, I try to keep the hand and wrist immobilized for as short a time as possible (four weeks) and allow some protected motion of the fingers immediately. This reduces the chance of significant muscle atrophy.
CMTI: Does hand surgery always work in people who have CMT? Who has the best chance of successful surgery?
Dr. Patterson: No, hand surgery does not always work. With any surgical procedure, there is a risk of failure or a poor outcome. It is unrealistic to expect perfection, although that may be our goal. Patients who are well motivated, follow their rehabilitation orders and have an appropriate hand for reconstruction demonstrate the best outcomes.
CMTI: Can any hand surgery be done under local anesthetic: Many of our people are wary of anesthetics, especially the ones with breathing difficulties.
Dr. Patterson: Definitely! Most hand surgery procedures can be done under regional or local anesthesia, where only the hand and forearm are ‘frozen'. In general, we try to use this type of anesthetic for procedures that take less than two hours, as patients tend to get restless if asked to lie still on the operating table for greater lengths of time.
CMTI: We don't hear anything about hand surgery and CMT. There are rarely professional journal articles written on hand surgery and CMT. Why do you think this is?
Dr. Patterson: CMT usually develops and progresses slowly. The functional losses that occur do so over a prolonged period. As a result, people with long-standing CMT compensate very effectively for their loss in different ways. They are very protective of the function they have retained and are extremely cautious about doing anything that may worsen their function. As a consequence, few people with CMT are eager to have their hands operated on. Most surgeons, therefore, have a limited experience in the surgical management of CMT hands. The result is that there is insufficient data available to permit the publication of a scientific journal article. I am very upbeat about surgery in CMT, as my personal results have been excellent. However, I, like others, need greater numbers of patients before I can state unequivocally that there is no reason not to have the surgery.
CMTI: What would you suggest a person with CMT hand problems do if they cannot find a surgeon with any knowledge of CMT but are determined they want to go ahead and try to find help. We have all heard of hand surgery turning out to be a negative and some surgeons promise the world. How do we know if a surgeon knows his/her stuff?
Dr. Patterson: There are a number of well qualified hand surgeons in North America who are very capable of performing this surgery. In your community, you should ask if there is a fellowship trained hand surgeon who is Board certified in plastic or orthopedic surgery. Some of the best surgeons will have a Certificate of Advanced Qualification in hand surgery in addition.
CMTI: Would you be kind enough to answer questions from our readers about CMT and hand surgery in the future.
Dr. Patterson: Absolutely!
CMTI: Can people with CMT come to London, Ontario to see you? What about people from the United Sates and elsewhere? How can that be managed? Do you have colleagues in the U.S. and other countries you can recommend?
Dr. Patterson: London, Ontario is a major university centre of medical excellence. We treat large numbers of patients from the U.S.A. and overseas. A referral can be made to the appropriate physician, just as one would in your home situation. We tend to be a lot busier than our colleagues south of the border, so it does take longer to get an appointment than it would in the U.S.A. I know many excellent hand surgeons in the U.S.A. who wold be able to provide excellent care and would be happy to assist in directing people to them.
CMTI: Thank you, Dr. Patterson. You've shed some light on a topic that isn't often discussed in CMT literature.