By ConditionComplex Regional Pain Syndrome (CRPS)
Complex regional pain syndrome (formerly known as reflex sympathetic dystrophy or RSD) is a debilitating painful disorder of a part of the body, often one or both of the extremities (although it can be anywhere on the body). Pain may occur after a minor injury or surgery but also may occur without an obvious prior event. The pain is often described as a burning, squeezing, or stabbing/shooting pain. The hallmark of CRPS is that the affected area is super-sensitive to even light touch, has the type of pain noted above, and often interferes with the use of the leg or arm if that is the part affected. Sometimes, there is swelling and color changes (red/blue). There may be skin and hair changes from lack of touch to that body part. The muscles may weaken and shrink from non-use. Below, two children describe what CRPS feels like to them:
I sometimes feel like someone is taking a torch of fire to my ankle when I walk on it; it feels like a lightning bolt is going up my leg. Sometimes when I bend or move it feels like it is going to explode. It is so sensitive that I can’t wear my jeans and I have to wear baggy pants all of the time. If someone tries to touch my leg during physical therapy I go through the roof in sharp excruciating pain. Or even if someone accidentally bangs my knee, it seems to be worse for the rest of the day.
If I had to describe the pain in my leg, I might describe it as numb, but I really don’t think that’s quite right. It’s not numb like you get with epidurals. First, there is the lack of it feeling stable, because of a pins and needles feeling, which is really disturbing, and it doesn’t want to move normally, and won’t take my weight, and then there is a circulation being-cut-off kind of numbness. I get shooting pains but not all the time. Most of the time my leg seriously aches, feels like it’s being crushed and generally being eaten up from the inside (I know that sounds strange but it’s a description I developed a while ago for this cold burning kind of feeling).
The painful body part is often sensitive to light touch; even a brush of clothing, water, or a light breeze can be excruciatingly painful, and any contact with that sensitive area (such as someone accidentally bumping it) can cause more severe pain. A child with CRPS-1 may not want to wear shoes or have the sheets touch that part of her body at night, which may lead to sleep problems. Because the child is often afraid of someone touching the painful body part, she may attempt to avoid school or other social events. Additionally, the painful body part might become sensitive to temperature (either heat or cold). As the pain worsens, patients have difficulty moving the limb, which can lead to stiffness and more pain. These symptoms can also come and go.
The pain started suddenly when I was nine as a slight pain in my knee, which kept getting worse and worse. I went to an orthopedic surgeon near where I lived and had an MRI. They found a slight tear in the cartilage and the doctor decided to operate. It was successful at first. It was a slight arthroscopic surgery, and I thought I would be back on my feet in no time. But as I went to physical therapy, the pain started getting worse. I kept pushing myself. My parents didn’t know what to think. T he doctor implied that I had a low pain tolerance and that I didn’t want to work hard to get better.
The pain was getting worse and was affecting my sleep. I was having trouble walking. The pain was a burning sensation, like my leg was on fire. I couldn’t straighten my leg because the pain was so intense. It finally stayed like that. The muscles in my ankle had atrophied and couldn’t hold me up. I was starting the 5th grade, trying to do normal things, but the pain was so great that I couldn’t concentrate. I was going to school only half days because of physical therapy. I was becoming really depressed. I was losing hope. I was in so much pain, I didn’t know what to do about it. I wasn’t going to school normally, I didn’t have any friends. It was so horrible at that age to see everyone else run around, and I couldn’t. I was taking a lot of pain medication. I was also taking anti-anxiety pills. Nothing was really helping.
And then, I don’t know how to describe it, but It’s like you’re traveling up a hill and you don’t think you are ever going to get to the top and then you finally just reach the peak and things start happening. By the middle of sixth grade I was out of the wheelchair and back on crutches. It took a long process to get to that point. It started by taking a step on the crutches and then I’d sit back in the wheel chair. Once I was vertical again on the crutches that really helped. I don’t know how I originally damaged my knee. It started out as just an ache and then grew worse and worse. I was limping. There had been some torn cartilage, but I probably could have done without surgery. Today, my right leg is curved and the calf is smaller and sometimes walking long distances is difficult. I have arthritis in my leg and hips. But I am in college now and am able to live a full and active life.Headaches and Anxiety
Danny is a 12-year-old boy who developed headaches that got worse and worse and did not respond to any pain medication. He was unable to sleep or go to school. He was hospitalized and, following a normal MRI, Danny underwent multiple tests including several lumbar punctures (LP’s) (needles placed in his back to get cerebral spinal fluid to check for meningitis and to check the pressure of the fluid). Because the doctors had technical difficulty during the first two LP’s, they performed a third. The tests all came back normal, but Danny’s pain was no better. Frustrated, the doctors began to wonder if Danny was increasing his pain complaints to “get attention” and decided to refer him to our pain clinic because the referring physicians knew we had psychologists as part of our program. During Danny’s evaluation, we discovered symptoms of a generalized anxiety disorder and other factors that had initiated the headaches which were then made significantly worse by the medical traumas Danny underwent in the attempt to “make a diagnosis.” These medical traumas actually led to post-traumatic stress disorder (PTSD), further aggravating Danny’s pain problem.
This story illustrates that a detailed medical history at the start could have revealed factors that were causing Danny’s headaches or at least have indicated what was making them worse. Invasive medical tests should not be the first approach since many medical tests have risks. Doctors often forget to ask about risk factors, such as past traumas, or don’t evaluate children for anxiety, learning disabilities, and other problems that affect pain.Irritable Bowel Syndrome (IBS)
JoAnn is a 13-year-old girl who, about six months before she came to see me, had developed belly pain associated with some intermittent constipation, diarrhea, bloating, and nausea. She had had gastroesophageal reflux disease (GERD) early on but this had stopped. These symptoms continued and worsened until she was having significant abdominal cramping and diarrhea every morning. Soon she was regularly missing the entire morning at school. She began to restrict what she ate and developed rigid food preferences because she feared that certain foods would cause her diarrhea.
JoAnn also began having trouble falling asleep at night, although once she was asleep, she remained asleep. She started limiting her social activities because of the diarrhea and because she was not feeling well. She said that nothing helped and eating made her belly pain worse. JoAnn originally saw a pediatric gastroenterologist who performed an endoscopy (a procedure where a tube is used to examine and take biopsies of the esophagus, stomach, and small intestine) which was normal. He recommended that JoAnn see a psychiatrist, explaining that this was a “stress-related condition.”
A few months after the belly pains began JoAnn had seen a psychologist for three visits for grief counseling after her grandmother’s sudden death. Four months later, she was evaluated by a second pediatric gastroenterologist and she was placed on Elavil. She was referred by that gastroenterologist to the Pediatric Pain Program after the Elavil failed to relieve her pain.
JoAnn described her belly pain as “throbbing, stabbing, cramping,” and often associated with sore abdominal muscles. She rated the pain on a scale of “0-10” (in which “10” is the worst pain possible) as averaging a “6” with a range from “2-8.” She reported intermittent diarrhea and constipation, and said that the problems related to reflux and bloating were gone but she still had occasional nausea. She was no longer vomiting. She had previously been given a number of medications, including Tigan for nausea, Elavil, Bentyl, Immodium, stool softeners, fiber, and Mylanta.
Dr. Zeltzer discusses JoAnn.
Reviewing JoAnn’s symptoms with her, I learned that she had general fatigue. She had headaches at least once a week which were helped by Tylenol. She had occasional trouble breathing, heart palpitations, and tightness in the throat from panic attacks. She had some dizziness when standing and occasional hot and cold flashes. The pain attacks occurred about once a week. JoAnn’s mom reported no significant medical history other than as described above. JoAnn still got A’s and B’s, even though she had been missing every morning of school for about 6 months. JoAnn’s physical examination was normal except for some slight belly tenderness near the bottom at the right. S he reported having trouble concentrating, feeling lonely, and crying often. She said that she had been having panic attacks for the last 3-4 months, although she was “used to them” and recognized that they were “stress-related.” She said that she was most stressed out by homework and by pressure to be at school when she was sick. She admitted that she was a “worrier.” As a result of what I learned about JoAnn and my physical exam, I diagnosed her with the following: 1) Irritable bowel syndrome with constipation and diarrhea, abdominal pain and nausea; 2) Myofascial (muscle-spasm) headaches; 3) Major Depressive Disorder, in partial remission; and 4) Recent onset of panic attacks.
One thing that made JoAnn anxious was that she might have an attack during school and might not be able to get to the bathroom. This is a common concern for children with IBS. I recommended a medication to treat JoAnn’s IBS, a drug that would quiet down her nervous system and help ease the panic and belly pain. This was an SSRI that has been shown to be effective in children with IBS. I told JoAnn that she could use Immodium for diarrhea and Miralax for constipation. We talked at length about cognitive therapies such as hypnosis or biofeedback and physical therapies such as yoga or massage. Also, I encouraged her to get more exercise and recommended swimming and walking. We discussed her diet and reviewed what a healthy diet was and I recommended that she try to eat fewer greasy and fatty foods. Finally I referred her to a psychologist who was skilled in cognitive-behavioral therapy (CBT) so that JoAnn could learn some skills to reduce her central nervous system sensitivity, calm her body and her mind, and reduce her feelings of panic and her pain. I asked her to come back in a month to see how this plan was working. When I saw JoAnn again, she was doing much better and, after six months of treatment, she was taking only the SSRI, no longer had belly pain or panic attacks, was attending school fulltime, was active again with her friends, and was finding it much easier to concentrate on her schoolwork.