An Overview by Susan Wynn
So why feature information about RSD in a newsletter for patients with Multiple Hereditary Exostoses? When my daughter was diagnosed with RSD in 1999, we thought that hers was a rare, isolated case. Nicole suffered tremendously, and it was a difficult time for our entire family. As her condition improved, we were happy to forget about this experience, and were able to put it behind us until Melanie Barousse posted a plea for help on YahooGroups a year ago. The symptoms that she described were so like what Nicole had been through that the memories of that terrible time came flooding back. I called Melanie immediately to let her know what Nicole had been through, and that they might want to present the possibility of RSD to Jake's doctors.
Besides Nicole and Jake, there are three other children and one adult in MHE YahooGroups that we know have been diagnosed with this "very rare" condition during a 2-1/2 year period. While we are not aware of any studies that indicate MHE patients are at a higher risk for developing RSD, we do believe that at very least, the possibility for more cases exists. Perhaps because of the number of surgeries MHE patients go through, or the nature of the surgeries, or the nature of the disease itself, with compression of nerves by exostoses being a fairly common occurrence, we feel that people should at least be aware of RSD. This article is not meant to alarm, but merely to inform. Because early treatment is an important factor in RSD, we are presenting this overview of the causes, symptoms and treatments. If you believe that you or someone in your family may have RSD, please see your doctor for diagnosis and treatment as soon as possible.
What are some of the causes of RSD?
While the causes of RSD are not fully understood, it is believed that RSD is associated with injury to nerves including:
Trauma (even minor injuries, such as a sprain or a fall, can cause RSD); surgery; compression that could cause prolonged pressure on peripheral nerves, such as casting, or swelling due to injury or surgery; heart attack; infection; radiation therapy
What are the symptoms of RSD?
Symptoms and severity may vary from patient to patient and may include the following: Severe, burning pain; muscle spasms; local swelling; softening of the bones; rapid hair and nail growth; restricted or painful mobility; warm, shiny red skin that later becomes cool and "bluish"; and pain out of proportion to the severity of the injury. One of the main characteristics of this disorder is that the pain experienced by the patient is more severe than expected for the type of injury incurred, and that the pain gets worse instead of better. RSD can begin immediately after the injury, or later. Other symptoms may include increased reflexes, tremors, muscle spasms, fatigue, weakness, skin rashes, frequent infections, and more.
What does it feel like to have RSD? We asked the RSD patients in our organization (or their parents) to describe the experience:
Nicole: "What was it like having RSD? Well, at first I thought it was normal but it hurt a lot. It hurt whenever someone touched it. It felt like acid was dripping through their fingers and onto my leg. When I got home it hurt almost all the time. And when I had to walk with the walker, even though no one was touching it, it hurt even more. It felt like a match was lit and someone put it on my leg. The physical therapy helped make it better, but I left there crying from pain everyday. At home I had to do desensitizing exercises. At first I had to put my foot in a bucket of dry rice. I never knew how sharp and pointy rice could be. It hurt but then we moved on to a bucket full of sand. That was much better and after I was done exercising I made little sandcastles. My foot was blue for a while and still hurt but the pain decreased after a couple of months. Now I am feeling much better."
Jake: "After surgery, when we got home the pain started getting worse. It was rrrrrrreally painful! The wind from somebody passing by it and any covering touching it was like somebody taking a pick and stabbing me in that place, but whenever I moved the ankle, it didn't hurt it. When my mom started rubbing lotion on it, even lightly, it felt worse than ever! It felt like an axe instead of a pick and it was bad! Water didn't hurt it, but the washcloth made it hurt like the pick. After a few days of the rubbing and the medicine, it got better. Then the spray really helped. Now it only feels like someone touching it with a spoon. It's not a pain, just a feeling now."
Alida: "I am 39 and have had 27 surgeries related to MHE and removing the exostoses. I never had many problems after surgeries until last year. I had about five exostoses removed from my left knee. The nerves were wrapped around the exostoses. It was not right after surgery that all the problems started, but a few weeks later. I was still using the wheelchair. I had gotten the stitches out and went to take a shower. The water hit my knee and I almost fell to the ground, it hurt so bad. It was so bad that I could not stand anything to touch my knee. I was sent for an EMG. I kicked the guy that was poking my leg, and passed out. It was then that they realized how much pain I was in. Even when the sheets touched it, I would almost pass out in pain. I finally got in to see Dr. Patt, a very good pain doctor. He treats mostly patients with cancer, so he knows about pain. He has helped so much with medicine and desensitizing it with rice bags and other things being rubbed on it. I can stand to be touched there now without such pain. It still hurts, and I'm just getting used to wearing pants on it. So we are learning more and more about it and it makes it easier to deal with."
Robert: "It's a burning, icy, metal pain, and it feels like acid coming out of my foot. It also feels like when you hit your funny bone…"
Brandon: "I had surgery on November 23, 2000. I had surgery because there was a bone in my leg that was making my right foot have a big arch. It also made my foot weaker. Something that happened after the surgery was my leg was very touchy and it hurt sometimes when someone touches it. I never like it being touched because it tickles and feels very odd now. I don't like how it feels. It's like feeling something that is fuzzy, rough, and gentle at the same time. I'm supposed to have my mom or dad rub it but I don't like the idea. But it is alright now."
Linda: "Johan Olav got RSD after a surgery in September last year. The first day after surgery was "normal" but already the 2nd day I understood something wasn't right. He had severe pain and heavy cramps, his leg was shaking and was "out" of control. We couldn't touch him or the bed, just small things would trigger the "attacks". I've never seen him in such pain ever before or later, and he has had surgeries both before and after this one. He was really sick for a week or so and then it sort of settled. But the recovery was slow and he still had lots of pain. After 3-4 weeks it took a turn to the worse and he couldn't take it if we touched his leg between the knee and ankle. It seems that soft, gentle touch is worse than firm and things like getting dressed or having something close to the leg is troublesome. His PT worked with him for a couple of weeks, but we could see none or little change. It's more difficult for me to work with it, cause he won't let me. I think he's a bit better now, but he still wakes up if I touch it when he's asleep and he won't let me come near it. Sometimes the pain can just come while he's not doing "anything" and then it looks like he gets cramps and he says it burns."
How is RSD Diagnosed?
The diagnosis is often made through observation of symptoms and based on a thorough history. Other diagnostic tests that may be used include thermography and nerve conduction studies. Other tests may be used, as required.
How is RSD Treated?
There are many forms and combinations of treatment. What works for one patient may not work for another. A variety of drugs, including Neurontin, are used to treat RSD. Corticosteroids, vasodilators, and alpha- or beta-adrenergic-blocking compounds are among the types of drugs that may be used. Injection of a local anesthetic may also be used, and a prescription spray, Guanethedine, has been shown to be effective in some cases. Other treatments include: Physical therapy, desensitization of the affected area, Transcutaneous Electrical Stimulation (TENS), Nerve Blocks, psychological support, and in some severe cases, sympathectomy may be required to relieve pain. In this surgical procedure, cutting the nerve or nerves and interrupting the affected portion of the sympathetic nervous system destroys the pain almost instantly, but it may destroy other sensations as well.
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