Diagnosis to Recovery

When we started out to develop our CSS patient advocacy site, it struck me that I might be in a unique position to help communicate important ideas and issues to other people about this little known disease. My focus with these series of articles is to talk about many of the things that can occur along the way after the diagnosis of Churg Strauss Syndrome, creating stumbling blocks in getting back to "healthy" again.

March, 2006- a note from Carol: I have been under the weather the last month or so and hence have asked a friend and previous associate of mine to write this month's article on the Road to Recovery. Dr. Phyllis Cullen is a board certified anesthesiologist and pain management physician and I thought she would be an excellent person to write on the topic of peripheral neuropathy. She is very knowledgeable about the latest treatments of pn and is also a very bright and caring person. She was nice enough to put some of her knowledge down on 'paper' for us this month. Carol

Peripheral Neuropathy by Dr. Phyllis Cullen

Peripheral neuropathy, which means damage to the nerves outside the brain and spinal cord, is especially common in the areas furthest from the central nervous system, such as the legs and feet.. About 60-70% of all patients with vasculitis, an inflammation of the blood vessels, which is common in CSS, will experience peripheral neuropathy. In fact, about 34% of all patients with vasculitis will have symptoms of peripheral neuropathy as the only evidence of their vasculitis.

Vasculitis can prevent sufficient blood flow from reaching various organs and tissues of the body. Because adequate blood flow is required to provide the organs and tissues with oxygen, vasculitis causes damage to oxygen-deprived organs and tissues. When peripheral nerves are oxygen-deprived due to vasculitis, vasculitic neuropathy ensues.

Neuropathies come in many patterns. A mononeuropathy is damage to a single nerve. Polyneuropathy is several nerves involved at the same time. A common polyneuropathy is the one that diabetics get, where nerves in toes, feet, ankles, and legs below the knees are involved.

CSS is most commonly associated with a type called mononeuritis multiplex which means that one nerve after another gets damaged . Usually the next mononeuropathy occurs after the first one resolves.

Most people with vasculitic neuropathy develop pain, and then weakness in a random, nonsymmetric distribution throughout their limbs; a smaller number (about one-third of all sufferers) notice pain and weakness that progress in a symmetric fashion, beginning with the feet or hands and progressing up the limbs. The pain of vasculitic neuropathy can include shooting, sharp pain, tingling, numbness, burning, and stinging.

Once the cause of the neuropathy is determined, the most effective treatment is to treat the underlying disease. For CSS vasculitis, that means that steroids are most likely to help. Physical therapy and certain medications, including anti-seizure drugs antidepressants, pain medications may be needed. Weakness may indicate the need for foot brace, walkers, etc.If there is decreased sensation, or numbness, special care must be taken to prevent injury, which could be slow to heal.

Time honored medications for neuropathic pain include, besides narcotics, various types of antidepressants, older ones like amitryptiline (Elavil), doxepin etc to newer ones, like Effexor, and the very newest, Cymbalta (duloxetine) which was basically developed specifically for neuropathy. (The fact that it helps depression as well is certainly a bonus when chronic illness likely really does have you depressed!) The SSRI types of antidepressants, like Prozac, Zoloft, Paxil , Lexapro, might help the depression but don't do much for the neuropathy per se.

The other major category of drugs for treating neuropathy is anticonvulsants, or anti-epileptic drugs(AEDs). Although they were developed to treat seizures, it soon became apparent that they were also very useful for neuropathic pain. Neurontin is the one with the most experience and probably greatest reliability, but every one (there are about 20 of them) have been used. They each have certain chemical pathways and targets in the nervous system, so if one doesn't work, or the side effects, like fatigue, confusion, weight gain or loss, rashes etc, are intolerable, try a different one. The newest one is Lyrica (pregabalin), which was developed specifically for neuropathy. It hasn't been out all that long but the preliminary results suggest that it seems to work a little better, with side effects that aren't too bad. All of these anticonvulsants should be started at a low dose, and gradually increased to avoid being hit by intolerable side effects.

Some other medications come from special compounding pharmacies, rather than the usual drug companies, and provide medications in a form different from the usual way to take the drug. For example, Ketamine has been used for years by injection, but can be obtained as a pill or acream and can sometimes have real benefit on neuropathic pain. Other medications available for topical use include Lidocaine , a numbing medication, available commercially in patches (Lidoderm) which are often very helpful, or in combination with other medications often tried for neuropathic pain, such as clonidine, antidepressants, anticonvulsants, ketamine, antiinflammatory medicines, muscle relaxants, et.) The only trouble with topical medications is that you have to put them right on the painful area, so if your whole body is burning, they won't work. However, the side effects of using them this way are less than taking them by mouth.
New research is going on like crazy for new medications, treatments, and ways of helping the nervous system repair itself. New ideas using hormones, growth factors, cell building enzymes, stem cells, etc are being revealed virtually daily. More effective treatments and ultimately cures are really, truly on the horizon, and it is important to keep a positive attitude, because it is going to get better. More doctors understand these conditions and are prepared to believe and help their patients, more psychologists and other therapists are available to help with ways to use the mind's ability to generate its own anti pain chemicals, and support systems, like this group, offer information, resources, and well, support!

If you have specific questions regarding what I've discussed here, and your doctor can't help, email me here on this website and I'll try to help you.