Doc: Myelin damage of CIDP compromises nerves

Dear Dr. Roach: Eight years ago, I was diagnosed with CIDP (chronic inflammatory demyelinating polyneuropathy). After several tests, it finally was diagnosed by a spinal tap. One of the effects of this disease is that one’s toes feel as if they are asleep, and in my case, it made my legs very weak and affected my balance. About two years ago I began having that same tingling in my fingers.

The doctor assigned my case really didn’t know what to do to treat this relatively rare disease, so he started me on steroids intravenously for a few weeks and then prescribed CellCept. As I am in an area where there seems to be no doctor that is really familiar with the disease, I wonder if I should still be taking that medication. Also, is there any progress being made toward finding a cure for this disease? I am now 86 years old and have learned to cope, but I am more concerned with younger people who get this disease, since it is very debilitating. If there is a cure for CIDP, I was not made aware of it.

D.M.

Dear D.M.: CIDP is a condition where nerves are compromised by damage to the myelin, a protein that envelops and protects nerves and improves nerve conduction. Damage to the myelin causes weakness (your legs are weak) and sensory abnormalities (the toes feel asleep). Balance problems, like you have, occur less commonly. The nerves to bladder and bowel may be affected in more-severe cases.

So far, I could be describing Guillain-Barre syndrome. The difference between Guillain-Barre syndrome and CIDP is that while GBS gets better in a few weeks, CIDP lasts longer than eight weeks. Some with CIDP, mostly younger, have intermittent episodes. Some progress over time; that includes mostly those in the older age group. CIDP is most common in older males. Its cause is unknown. Nerve-conduction studies, nerve biopsy and spinal fluid analysis are used to make the diagnosis.

Treatment is done most commonly with prednisone (a medicine that has many side effects if given long term), immune globulin (expensive and often limited in supply) or plasma exchange (expensive and available only at referral centers). Only about 25 percent of patients will have long-term complete remission from the condition. There will be some response in about 60 percent, and about 15 percent will have severe disability (unable to walk).

CellCept (mycophenolate) has been used for this condition, but it concerns me that you are using this second-line treatment and lack confidence that your doctor is expert in treatment of this condition. Rare and serious diseases require experience for best outcomes. You can read much more about the condition at gbs-cidp.org.

Email questions to ToYourGoodHealth@med.cornell.edu.