What about the what ifs
with Linda Crabtree
Growing older is just a fact of life. Welcoming the years is sometimes not easy and can be frightening when you have a progressive syndrome such as CMT. Often, the ‘what ifs' crop up. What if I get worse, how will I manage? What if I have pain, what if I can no longer look after myself?...and on it goes.
I've asked you for questions and I'm taking them to Dr. Greg Carter who will do his best to answer them. I am also printing some wonderful letters from you, our readers, sharing your experiences of living and growing older with CMT.
I, too, fear the future, What if my husband dies before I do? Can I continue to live with this pain?
One of our more experienced older readers said something so sane and comforting that I have never forgotten it. He said something like, "You just do it. Every year you cope and before you know it you are living your what ifs one at a time and getting through it." I guess that's what life is. Conquering one ‘what if' after another. We'll begin with your questions to Dr. Carter. I'll not use names because so many people asked almost the same question that I've bunched them into one, but you'll see yourself. One question asked by many who responded was:
Q: How can we tell the difference between another CMT stage and natural aging. There are different responses we can take if we know which it is. Along the same lines - Can aging in any way cause our CMT to progress faster because as we grow older our bones, joints, muscles, etc. get weaker?
Dr. Carter: That is an excellent question but somewhat difficult to answer. There has been an explosion of research on aging in the last decade, although not specifically aging with CMT. Aging, it seems, produces some neurological problems in and of itself. Aged people often have sensory (feeling) problems, particularly in their feet, which is why so many old people fall. They also have slowed reaction times and may develop slow movement in general, often resembling a mild form of Parkinson's disease. I'm sure you have seen the "old person shuffle." We all know, and experience, the decline in cognitive (thinking) abilities, that as best as I can tell, starts in the 40s (speaking personally here!). So take all of that and add it to CMT and you can well imagine the problems that older people with CMT have. Does aging actually accelerate the disease process? I doubt that but it certainly can make the symptoms worse for all the reasons I have noted above.

Q: My concern is with the drugs that are prescribed for things like high blood pressure, high cholesterol, arthritis, and all the conditions that are generally related to aging. Sometimes the fine print mentions peripheral neuropathy but most doctors are not aware of these side effects. I know we must be our own advocates, but more information on drugs to be wary of would be helpful. And on the same topic - As we age, there is a tendency for us to look around for various drugs that might help the loss of muscle, pain, and general deterioration of our bodies. Aren't we taking chances if we ask doctors to give us prescriptions for drugs we might see advertised or hear about, which have been approved less than five years? Can doctors really be sure these will help our aging bodies and not be harmful for people like us who have neuromuscular diseases?
Dr. Carter: Again, excellent questions. People with CMT have to be cautious about any drug they take and Linda has done a wonderful job identifying problem drugs for folks with CMT in the newsletter. You have to be your own advocate here. All people, but especially those with a chronic disease like CMT, need to be active participants in their healthcare. If you like your doctor but he or she doesn't know much about CMT then educate them! People with CMT should become experts in this disease since you have to live with it your whole life. Most good doctors would appreciate getting help in understanding the disease. Take the list of drugs published in the CMT Newsletter to your doctor. As for new drugs prescribed to you, ask him or her to run MedLine or MicroMedEx on-line searches to see if there are any contraindications to a particular drug. You always take a certain degree of ‘chance' when taking a new drug but sometimes the potential benefit is worth the risk. There are so many new drugs I can't really go in to specifics (that would take a book). You must look at each new drug you take, run the on-line searches with your doctor or, if you or your doctor are not computer people, ask your pharmacist to do this for you. Pharmacists are often better than doctors about keeping up with drug interactions and side effects because that is their main job and most modern pharmacies are equipped with computers that have programs that specifically look for problematic drug interactions or side effects.

Q: I have a male CMT question for the doctor. Prostate cancer in the western world appears to be on the increase. Having CMT and the neuropathy problems that it encompasses are men with CMT within a predisposed higher risk category than someone without a form of neuropathy?
Dr. Carter: Not that I am aware of, although I do not know of any research looking at that question specifically. Supposedly, all men, if they live long enough, will get prostate cancer but most men will die of something else before then (like heart disease). The big problem is early detection. Prostate cancer is definitely curable if detected early. Once it metastasizes outside of the prostate though, it is a bad disease.

Q: Is CMT degeneration gradual or does it stay on a plateau for a while and then drop? I will seem to be going along at the same state and then suddenly realize I've lost some power in my fingers or some such. Am I just suddenly aware of it or does it suddenly happen? I'm 80.
Dr. Carter: We studied strength and functional abilities in people with CMT over a 10 year period (for those interested the paper was published in the American Journal of Physical Medicine and Rehabilitation in 1995 and is on Medline. Search under CMT). Our conclusion was that the disease itself (i.e. the neuropathy) probably progresses fairly steadily and slowly. However, functional abilities seem to plateau and then drop off suddenly, then plateau again at a lower level, then drop off again after a period of time. We believe it is because a certain "threshold" of strength is needed to do a particular task (i.e. hold a fork). In reality, you are very slowly getting weaker but you don't notice it because you can still hold a fork (for example). Then one day you reach that threshold and you can no longer hold the fork. So all of a sudden you think you have gotten a lot weaker all at once because you are now dropping the fork. Does that make sense? Your particular situation is probably due in part to aging though, at 80 years old.

Q: I watched my aunt and grandfather grow old with CMT (old was mid-70s in their generation). It wasn't the CMT itself, but CMT along with another disease that lowered the quality of life toward the end of their lives. So I'll ask: Is the quality of life of people with CMT lower than other elderly people because they have one more disease than everyone else to contend with?
Dr. Carter: We are studying that question right now through a NIDRR (National Institute on Disability and Rehabilitation Research) grant. Our preliminary data would indicate yes, people with CMT do report a lower quality of life in certain areas. However we haven't fully analyzed the data yet so I can't really get more specific.

Q: We know that if we exercise the wrong way we can cause our CMT to progress. By getting older we won't be able to do as much exercise. What could this do to our bodies having CMT? Could it put us between a rock and a hard place?
Dr. Carter: Let me clarify that exercising the wrong way (overdoing it) could harm your muscles and make you weaker but it does not actually affect the neuropathy per se. Pool exercises (aqua-aerobics, etc.) are a great way to go for older people in general and particularly people with CMT. If you're in the pool you may be able to swim out from under the rock and escape the hard place.

Q: Is there a limit to the amount of exercise regarding the use of my leg that I should do as I get old. I've had heart bypass surgery and am currently undergoing cardiovascular rehab treatment. It includes a stationary bicycle and treadmill. When I do not go to rehab I walk a mile. I am 66.
Dr. Carter: That is a tough question because you also have significant heart disease. Heart disease notwithstanding, for people with CMT that are able to walk reasonably well, I think 20-30 minutes daily or at least every other day of water exercise or stationary bike, along with a good stretching program and maybe some gentle weight lifting would be a good baseline. However, in your case, you need to follow the guidelines of your cardiologist and/or exercise physiologist in cardiac rehab.

Linda here - Remember, in most cases, wrist weights are better for us than hand weights.

Q: My main question is my eyesight. Is it bad because of CMT? If so I'd like to know why. I really have trouble reading. My eyes get tired. And my eyesight, both near and far sighted is more than awful. I'm 48 years old. And on the same topic - I, too, have problems with my eyes. I am 53 and I know that age has something to do with not being able to focus on close things very well. I have bifocals but still have to hold things at arms length to read. When I was first diagnosed I was told my eyesight was affected by CMT because the muscles around the eyes were working too hard. I have never heard anything about this since. So my question is, Is it aging, is it CMT or a combination of both?
Dr. Gareth Parry answers this one: As the literature below shows, there have been occasional families with both neuropathy and optic atrophy but they are rare. Visual loss is not a feature of CMT in general. The symptoms described sound very much like the "normal" deterioration in vision with age. There is a tendency amongst both doctors and patients to attribute all ills to CMT. However, having one fairly common inherited, and therefore lifelong, disease unfortunately does not protect you from getting the normal ravages of age.

Q: What worries me most about growing older is everything. Fears of the unknown. Where will my disease take me? Will I have to bear it alone, that is huge for me. I think about the physical limits I have now, and wonder how much more I will lose as I get older. Who will care enough about me to treat me with respect and dignity to the end of my days? I also have financial fears. I am on SSI now and receiving child support. Will it last? Will it always be available to me? How will I support myself if I didn't have that? I am enrolled in college, beginning January of 2001. I want to find something I can do to support myself if I have to. I am terrified that I will never be able to do it. I am 33 years old. College was impossible for me to do at age18. But, I am older and have learned some ways to deal with my limitations more productively. College is a reach into the unknown for me, but I feel I have to accomplish this for myself. Any suggestions? Thoughts as to how to get through would be wonderful.
Linda here - Dr. Carter tossed this one back to me. I will say that if you have the strength and courage to get this far you'll have no trouble finding the answers to your questions as you come to them. A good dose of fear can be very healthy. It motivates us to really think and put into action things we might not do if we weren't faced with the ‘what ifs'. Dr. Carter suggested that you could use a good case manager to help you tap into federal and or local resources for people with disabilities. They are out there, why not use them to help you solve your problems as you work things through. Good luck.

Q: My grandfather has CMT and is really affected. I was wondering if it is recommended to get braces if you are over 70. It was recommended that he get some but he isn't sure. He uses a walker, cane, scooter and wheelchair.
A member who knows answers: I am 80 and use braces and love them. They help me walk better with a cane or walker and might help him get out of the wheelchair a little more. Age shouldn't keep any of us from trying. I was just sitting here thinking I was sorry I had concert tickets for tonight because I am so tired, but I am going to get dressed up and go early to get the handicapped parking place and leave whenever I feel like it. But I will go.

Q: I was wondering how we get doctors interested and to understand CMT. I seem to be having a hard time even piquing a doctor's interest even when I give them a book to read. For example: I need surgery and I was talking to my GP about it. I was concerned about the anesthetic they would be using. His reply was, "They will put in a tube and it will breathe for you." Then he couldn't even find the report or my X-rays. I then asked him if he had read the book I gave him, and he said, "Well, just part of it!" Not once has he looked at my feet even though I have diabetes as well as CMT. I just want a doctor who cares about me and to whom I'm not just another number! So how does one do that?
Dr. Carter: Get a new doctor now. Any doctor who doesn't inspect the feet of a diabetic with CMT at every office visit is incompetent in my opinion.
Q: My question re aging has to do with pain level. I'm 44 now and am on m.s. contin, amitriptyline, and an anti-inflammatory, naproxen. Assuming my pain level will increase with CMT's progression, how am I going to fight pain without becoming a drugged out zombie? Are there new and better pain killers on the way?
Dr. Carter: Better treatments for neuropathic pain are already here and available. You are taking drugs that are "ancient" and only partly effective. Get your doctor to prescribe drugs like neurontin, topamax, lamictal, Effexor or other newer anti-depressants (that help like amitriptyline but have much fewer side effects), lidocaine patches, custom compounded topical creams. Find a doctor who has joined the 21st century. Get a referral to a pain specialist (often an anesthesiologist, physiatrist, or neurologist).
Q: Do you think the research on myelin regeneration will be useful during our lifetime? Do you think research will be able to come up with something to treat CMT in the next 10 years or so?
Dr. Carter: I certainly hope so, but I am not sure. The best treatment would be to correct the DNA errors that cause the disease. That technology is evolving as we speak. There is much cause for hope but as for the timeline to a cure, I am not sure.
Q: I have carpal tunnel syndrome and had surgery about seven years ago. I got it back again because I type for a living. I also have osteoarthritis in my thumbs. My question is whether or not there have been any updates on tendon transfers? My hand specialist had suggested this back then but after some info in the newsletter I decided not to do it then but I was told as I get older they will only get worse the more I use them. They've gotten to the point where I need to have something done because of the pain all the time.
Dr. Stuart Patterson, our hand surgeon, answers: Four separate issues appear to be at issue in this specific situation.
1) Pain in the thumb
2) Carpal Tunnel Syndrome
3) CMT
4) Osteoarthritis
Pain in the thumb is not due to CMT. This can result from a number of conditions in the thumb area, including osteoarthritis and carpal tunnel syndrome. Tenderness over the base of the thumb, where it meets the wrist, is most commonly due to osteoarthritis of the thumb carpometacarpal joint. Pain in the muscle of the thumb, radiating into the fingers, can be caused by carpal tunnel syndrome(CTS). However, CTS most commonly presents with numbness, tingling or pins and needles in the thumb, index, long and ring fingers. This is most severe at night, or when holding objects, such as a book, telephone receiver or steering wheel.
Tendon transfers are indicated for weak thumbs. The transfer is to assist in positioning the thumb for pinching or grasping activities. A tendon transfer will not help the hand with carpal tunnel syndrome or osteoarthritis. Severe, chronic CTS, that has caused permanent paralysis of the thumb muscles, may require a tendon transfer. This is unusual though.

Someone with severe osteoarthritis of the thumb, in addition to CMT, may be better off with a hand based splint initially. If splinting, anti-inflammatory medications and steroid injections do not help, then surgery may be indicated. At that time a decision would need to be made based on the function of the hand. A joint replacement (with or without a tendon transfer) or fusion may be indicated.

Recurrent CTS is unusual. Surgical treatment for this is not as successful as the first operation. Other causes for numbness in the fingers should be excluded, including neck disorders, diabetes mellitus and the CMT disease itself.
Q: I've seen my dad's legs deteriorate over the last several years. He has a lot of muscle atrophy and can't stand for very long or walk very far. Is this something we all have to look forward to as we get older? I know he could use a scooter or other help but his pride won't let him do it. My legs are also getting worse and I'm only 39.
Dr. Carter: All people with CMT will have some deterioration of function with age but the disease is quite variable. Some people retain the ability to walk their entire lives, others require a wheelchair at a young age. There can be significant variability within the same family line too so I can't give you a more specific answer.

Q: Has there been any evidence that links CMT and heart problems? My dad has had three heart attacks but only one he really knew about. As a result he only has about 20% of his heart muscle left. His doctors couldn't understand how this could happen without his knowing about it. So I'm kind of worried about this happening to me.
Dr. Carter: The heart is not directly affected by CMT. However, a sedentary lifestyle, which many disabled people live, is associated with a higher risk for heart disease.

Q: Aging and having a progressive neuromuscular disorder is a challenge. Can you give us something positive to go on?
Dr. Carter: Exercise if you can. Eat well, keep your weight down. If anything is a magic bullet to aging it is exercise and nutrition. Have faith in a higher power and use prayer and meditation to help you cope with your problems. If you are depressed, get help. There are excellent medications for that now. Seek peers with the same problems you have. Get involved in helping the public and doctors better understand your disease. Find a hobby. Listen to good music. Linda and I prefer jazz. Put on some old classic Sinatra with the big band and have a glass of good Merlot. Life doesn't get any better than that...