Question: Many of us have a lot of pain and wonder if it is due to CMT. At our San Diego support group I asked a doctor who does electrical testing for CMT about an aching/burning pain I have from my neck to my right hand on a nerve path. I want to know the exact source of this pain since it could be emanating from my neck, my shoulder, wrist or elbow. His response was it's not CMT causing the pain since it is not symmetrically expressed, and as far as locating it, the electrical noise generated by the CMT condition will make it very difficult to locate the source. Is there a procedure for CMT patients to identify the source of a long nerve pain that is non CMT based?
Dr. Greg Carter, Medical Director of Providence Rehabilitation Hospital in Chehalis, Washington, answers: I agree to an extent with the doctor that spoke at your support group. It sounds like you may have a pinched nerve in your neck, which can be diagnosed with needle electromyography and nerve conduction studies. The "background noise" from CMT will make that a harder, but not impossible study to do. I would also recommend getting some X-rays or possibly an MRI of your neck to assess for a herniated cervical disc.

Question: Has anyone ever found that nerve muscle stimulation does us any long-term good?
Answer from Dr. Thomas Bird: Nerve stimulators are sometimes used to reduce chronic pain. They are moderately helpful in some individuals. They can be tried for pain relief under supervision of a physician. There is no evidence that they treat or prevent the underlying biologic process causing CMT.
Answer from Dr. Orest Hurko: Neither I nor any of my colleagues at the Department of Neurology at the Johns Hopkins Hospital use such stimulators for treatment of CMT. I know of no published studies evaluating the use of such devices in this group of disorder.

Dr. Harvey Rose on pain
Linda here - I asked Dr. Rose if he would answer questions on pain and he agreed. Because he is so familiar with pain medications, he rattles them off at a speed that can boggle a layman's mind. However, if you read the questions and answers carefully, and discuss what he has said with your doctor, chances are you'll find alternative ways to treat your CMT pain.
Question: In the last 18 months I have been bothered by a nagging and sometimes severe pain that runs along my lower rib on the left side. I evidently had shingles during the time that my mother was ill and dying. I remember a lot of burning and itching as well as pain that I thought might be the start of an ulcer. I was broken out in a couple of spots but attributed that to allergies and nerves. Over the months, the pain has increased and the skin is extremely tender and sensitive. It was finally diagnosed as shingles neuralgia (damage to the nerve left by the herpes zoster virus, the same virus that causes chickenpox; the virus tends to stay dormant in the body until a time of severe stress or a reduced immune system). The pain has been very resistant to topical creams or medications.
My neurologist sent me to Dr. Susan Powers of the Institute for Rehabilitation and Pain Management. Dr. Powers is a practising anesthesiologist who took special training under Dr. Gabor Razz at Texas Tech University. Dr. Razz is known throughout the world for his innovations in the field. Dr. Powers is outlining a pain treatment plan for me in order to relieve some of the pain that is most intrusive in my life.
I had a nerve block in my spine to calm the nerves and to interfere with the pain signals. Unfortunately, the first procedure did not have an effect on the pain. It often takes two to six days for the medication to take hold. I did not find the procedure painful but I was disappointed that it didn't work and hope that further treatment can be done. Dr. Powers is giving me pain medication but I prefer not to add the tiredness caused by the medication to the lack of energy I feel on a normal, daily basis.
I am very thankful for the availability of such clinics for I feel that I will receive help which will allow a more normal life.

Answer: Regarding the question, it is obvious the writer has post herpetic neuralgia, which is a residual, painful condition following, of course, the shingles. She had a nerve block and that didn't work, and frequently nerve blocks do not work very well once the post herpetic neuralgia is established. There are some people who feel that sympathetic nerve blocks done early on during shingles may help cut down the instances of post herpetic neuralgia. Also, there are new antiviral agents like Valcyclovir, that, when started at the first onset of the shingles, may help prevent the post herpetic neuralgia. Unfortunately, patients may not come to doctors until four or five days after the onset and then these medicines are not as effective in treating the acute condition as well as preventing the chronic pain.
The writer states that the pain medicine she is getting makes her very, very tired and gives her a lack of energy. I'm willing to bet that what she is getting is a tricyclic antidepressant like Elavil (the generic name is amitriptyline) whose main effect is severe fatigue, although it does help improve sleep at night. I'm also willing to bet that she's not getting the opioid analgesics, because some people do not believe in giving opioid analgesics for chronic neuropathic pain like post herpetic neuralgia, but many times that is the only thing that will work.
It was interesting that at the most recent American Pain Society meeting that I attended in Los Angeles, there was a half day symposium discussing post herpetic neuralgia, and finally, there were some papers presented that opioids have been used for this condition and, if given enough, they will work. In the past, they said opioids did not work in neuropathic pain, but that was probably because it was given in "usual PDR (as needed) doses" and these were not effective, but larger doses of opioids can give relief of post herpetic neuralgia. Using small amounts of Elavil can also work as an adjunct.
Other medications used for post herpetic neuralgia neuropathic pain are the anticonvulsants like Tegretol, and also some of the cardiac antiarrhythmic drugs like Mexilitene, but these really have very limited use and they have many more side effects than, of course, the opioids whose main side effect is only constipation. Some people get very, very tired and fatigued with large amounts of opioids, which I'm sure the writer is not getting, than for instance our cancer patients. We then give them stimulants like Ritalin or Dexedrine to counteract the effect of the fatigue, and these stimulant medicines also have some analgesic enhancing effect with the opioids.

Question: We would like any and all information that would help us find something for the pain, burning, aching, stinging of the feet. Also, the legs are affected. The disease has also started to move into my husband's arms and hands. He has suffered with the feet and legs about 15 to 20 years but has gotten much worse in the past year and a half. He is still working but by night is suffering terribly and also he still has this pain in the mornings after resting. Can you help us at all? Is there help for this pain anywhere by anyone? He has been seen by our regular doctor and two other specialists regularly for the past two years. Does staying off the feet and legs with time help with this pain?
Answer: This problem is probably the same thing. This gentleman probably also has neuropathic pain…the burning, stinging in the feet. Again, these are difficult conditions to treat but low doses of the tricyclic antidepressants combined with opioid analgesics can give some comfort and relief, since we do not have any cure for this condition. Typically, neuropathic pain tends to be worse at night and early morning and may be a little bit better when they are up and about during the day. Staying off the feet does not help.
Again, these are difficult conditions, but by using all the modalities one can think of, particularly the hot chili pepper creams, tricyclic antidepressants in lower doses that don't knock you out, or some of the less sedating tricyclic antidepressants, and sometimes the anticonvulsants, one can find help but, again, in my experience, the mainstay for these are opioid analgesics given in generous dosages until the pain is sufficiently controlled. As I mentioned, it was a paper that was given by doctors at Johns Hopkins where they are finally realizing that opioid maintenance does have a place for the relief of chronic intractable neuropathic pain.
It would be very interesting to find out if it's the tricyclic antidepressants that are making the previous writer very tired; I doubt if the opioids are.

Linda here: Another reader wrote to say he has had burning pain in his thumb and two fingers for nearly two years. His doctor wrote him a prescription that he says really works. The ointment is called Phenomenthoeucerin and I asked Dr. Rose to comment on it.
Dr. Rose replies: Regarding that prescription, eucerin is just a moisturizing cream, but it's probably used as a base. The phenol and menthol are called counterirritants and appear in things like mineral ice, that type of thing, so it may relieve some burning pain.
Again, whenever you hear about burning pain, similar to the two cases we have discussed, one thinks about neuropathic pain which is due to nerve injury. Other items that are used topically for neuropathic pain are the hot chili pepper creams like Zostrix or Capsaicin. These do not work immediately but when used for prolonged periods of time can help. For instance, we have people who use it four times a day for about a month at the .025 strength; if that doesn't work, we try the .075 strength for about a month; and if that doesn't work, we forget it. It works locally getting rid of the substance p in the area, which is a substance that the body produces in injured tissue that tends to aggravate pain. Some people do get relief, and that's nice."
Linda here - The ingredients for the phenomenthoeucerin used here are: eucerin with phenol 1 gm. and menthol 0.25 gm. Apply as needed. This makes 120 grams. We have also found that creams like Zostrix can be torturous for people with burning pain, so be careful.

Question to Dr. Charles K.N. Chan, a specialist in pulmonary and internal medicine.
Can shoulder pain be associated with diaphragm problems in CMT?
Answer: It is reasonable to say that any disease process that affects the proximity of the diaphragm can potentially create shoulder pain, and that is what we call referred pain. In other words, pain actually occurred elsewhere in the body but was referred to some other parts of the body at the time you perceived it.
My experience in dealing with people with established diaphragm problems is that they usually have a bit of pain, and this is usually more to the chest wall because of the overuse of the muscles, but whether they have frank pain to the shoulder is less likely but possible. The reason I say it is possible is because the muscles around both shoulders that are connected to the neck are often utilized when the patients' diaphragms are weak, but frequently it occurs in people with advanced diaphragm problems.
In terms of early sign of diaphragm involvement, I would recommend symptoms along the line of breathing difficulty or shortness of breath while lying down as the very first indication. This happens months or even years before more profound symptoms follow and usually transpires to shortness of breath on minimal exertion.

Ask The Doctor
with Dr. Lowell Williams

Question: Although my upper extremity has moderate involvement I am now experiencing radiating pain and decreased range of motion in my left arm. I use heat, ice, anti-inflammatories, immobilization and pain medication during acute flare-ups. It feels more like a neuralgia or nerve entrapment. What is the incidence of neuralgia with CMT? What suggestions for preventing the flare-ups? If the pain becomes chronic what treatments are indicated? This type of pain has travelled from my peroneal nerve right leg, sciatica left arm. What can I do to inhibit the progression of the pain to other nerves?
Answer: The symptoms you describe do sound like neuralgia or nerve entrapment. Although shooting pain may be a symptom of CMT since the nerves are undergoing demyelination, there should not be signs of "inflammation" such as heat, swelling and redness of the limb. I suggest you have your doctor examine your arm when you have symptoms to see what anti-inflammatory drug might by indicated. It is possible that you have a second condition that might respond to treatment, as well as CMT which goes its own way. We do not yet know a way to prevent its progression.

Question: I would like to know about blocking nerves that cause a great deal of pain. Is it effective and does it last?
Answer: Nerves that cause pain can be blocked by injections. The effect depends on the drug used, the amount injected, and the nerve involved. Some nerves are more resistant to pain relief. Although our understanding of CMT does not include a reason for painful nerves, I know from personal experience and many patients' testimonials that it exists. Blocking of the painful nerve by a doctor trained in this area could be helpful.