When Pain Is Persistent, Mysterious — and Unbearable

Eight years ago, Peggie Riechard’s future looked promising: She loved her job as a computer programmer, and was moving into a new apartment in St. Louis, Missouri. When she injured her left foot during the move, it seemed like a minor setback — a stress fracture at worst. But her foot continued to swell and ache, and even brushing it against the bedsheets produced excruciating pain. After five puzzling months, doctors diagnosed her with complex regional pain syndrome, or CRPS.

Unfortunately, putting a name to the pain did little to ease it. Attempted treatments were largely futile. Working through the pain grew more and more trying. People around her didn’t understand. “But you don’t look sick,” they would say. When the pain spread to her spine and made sitting or standing difficult, she was forced to scale back her hours. As a result of her disability, she was eventually fired.

Confusion and controversy

Pain is considered “adaptive” when it helps you heal. For example, your shoulder hurts after an injury because your body is telling you it needs rest and treatment. In this sense, pain is good. But for someone suffering from CRPS, the pain doesn’t fade as the shoulder heals. In fact, it grows worse.

Symptoms may include edema (swelling), discoloration, excessive sweating, and of course persistent pain, often at the slightest touch, such as a light breeze. “Imagine you have the worst sunburn of your life, and someone comes along with a piece of sandpaper and scrapes back and forth all day long,” says Tricia Skiba, who was diagnosed with CRPS after knee surgery to correct a broken tibia. “It doesn’t relent. It never stops.”

The syndrome appears to be more prevalent in women, and it sometimes shows up after an infection, heart attack, or stroke. But beyond that, there is little evidence to determine who is at particular risk. Nor does any type of trauma appear more likely to cause CRPS. While you might expect an invasive event like surgery to be a common trigger, it appears that sudden and sometimes minor traumas — banging a knee, a hard fall on concrete — are just as likely culprits.

Historically, the International Association for the Study of Pain has identified two types of CRPS: CRPS-I, previously called reflex sympathetic dystrophy (RSD), and CRPS-II, previously known as causalgia. A diagnosis of CRPS-II was reserved for cases definitively linked to a specific nerve injury. However, new research suggests that patients with CRPS-I as well as CRPS-II also have nerve damage. According to Dr. Anne Louise Oaklander, director of the nerve injury unit at Massachusetts General Hospital, it’s a “no-brainer” that patients with CRPS-I also have specific nerve damage (known as neuralgia). In her 2006 study (published in the journal Pain), skin biopsies revealed “small-fiber” nerve damage in 17 out of 18 subjects with CRPS-I. Significantly, small-fiber nerves are involved in our pain response.

Some nerve damage in CRPS can change blood flow to the affected area, which may eventually damage the nails, bones, and skin. Until recently, doctors thought all cases of CRPS involved damage to the sympathetic nervous system. For this reason, pain from the syndrome was often treated with a sympathetic nerve block, in which a local anesthetic was injected into some part of the body. This can provide temporary dramatic relief if the pain does involve the sympathetic nervous system.

Doctors now believe that in the majority of CRPS cases, however, the pain stems from a combination of other factors — such as stress, failing to use a painful body part, and damage to the brain and spinal cord. New studies are tracking harm right down to the cellular level: A recent small German study found impaired flat cells that line internal body cavities in the area affected by CRPS I. Nonetheless, the condition is still so mysterious that it led authors of an article in the Clinical Journal of Pain to make this sweeping conclusion: “No other chronic pain syndrome is as shrouded in confusion and controversy.”

Once the condition starts, skeptics abound, and finding relief and treatment can be an overwhelming challenge. Forty-five-year-old JoAnne Maluchnik of Blairstown, New Jersey, visited a well-known surgeon after hurting her knee when a wooden stair gave way. The surgeon performed two operations (her second and third overall) on the knee. When neither surgery eased her ceaseless pain, he labeled Maluchnik a “hysterical woman looking for attention.”

“He was one of those surgeons who could not believe I wasn’t ‘fixed’ by him,” Maluchnik says. “Therefore, my pain was imaginary, in my head.” After her third surgery, a radiologist suggested CRPS, which the dubious surgeon considered a “made-up” diagnosis.

‘I feel like I’ve let everyone down’

Tricia Skiba met similar skepticism when she applied for a sticker for handicapped parking in Massachusetts. “We do not issue placards for pain issues,” read the state’s official reply. Her doctor had listed “complex regional pain syndrome” on the application and, according to Skiba, “They saw the word ‘pain’ in it, and thought I just wanted the placard because I was in pain.” When she reapplied, her doctor listed the inscrutable “reflex sympathetic dystrophy” on the application instead. It was approved.

“I think this spells out how little people know about [CRPS],” she says. “Every time I go out, there’s at least one person who gives me a dirty look because I walk with a walker. I don’t look my age. I’m 31, but look 18. ‘Why are you walking with that then? You’re too young.'”

“You can’t really quantify pain, you can’t really measure it,” Skiba explains. “It’s not emphysema, where you have an oxygen tank. That’s why it’s so difficult for other people to sympathize.” She often hears the advice: “You should just get over it. Get on with your life.” If only it were that easy, wrote Skiba about her CRPS saga: “I am stating emphatically that there is nothing we would not do, no experimental treatment we would not try, no hard work we would not be willing to put in, if only to live up to the standards of ‘life’ that we all hold as dear to us.”

As Sally Lucker, who worked with CRPS for over three years before having to quit her job as a legal assistant, puts it: “Trust me, if you aren’t in this kind of pain, you just don’t get it. I feel like people don’t believe me. Shoot, I don’t know that I would believe me. The pain makes absolutely no sense and never goes away. I feel like I’ve let myself down, let my husband down, let everyone down.”

In fact, the mounting isolation and emotional duress that comes with losing jobs, hobbies, and friends may be more damaging than the pain itself. In her days as a nurse (her CRPS was triggered by an injury sustained while restraining a patient), 47-year-old Karen Nelson “was always on the go, reaching out to those in need. Now, I spend my time taking meds, trying to relieve my pain, and focus on medical improvements. My life, as it was, is gone.”

Peggie Riechard echoes this sentiment. After being fired as a result of her disability, she was forced to leave her apartment and friends in St. Louis and move in with her parents in Hannibal, Missouri. She spent two and half years convincing the government to grant her social security disability insurance. Today, at age 33, CRPS prevents Riechard from sitting up for more than an hour at a time, so she spends most of the day in a reclined position. “My job was going well,” she reflects. “Who knows what could have happened. Then, all of a sudden, pain happened.”

Pinpointing the cause and treatment

Doctors often diagnose CPRS without lab tests. Instead, they use a physical exam to look for swelling, mottled or purplish-blue skin, abnormal skin temperature, changes in hair and nail growth, and too little or excess sweating in the affected area. Other key symptoms include severe pain that’s triggered by things that don’t normally cause pain, (such as wind or clothing), and severe pain when only slight pain would be normal (such as a doctor pricking the skin with a pin).

A tool known as quantitative sensory testing (QST) that tests for abnormal responses to heat, cold, and vibration has long been the gold standard of diagnosis, but, as Oaklander points out, it’s subjective. Skin biopsy, she says, “is a more sensitive way to identify damage to the kind of nerve fibers we now know are damaged in CRPS.”

Definitively linking CRPS to nerve damage is important, Oaklander says, because it should end speculation that the condition is psychosomatic or motivated by patients looking for a “high” from painkillers. Presumptions like this have made it challenging for patients to receive effective treatment. “Most are shunted from doctor to doctor like a hot potato,” Oaklander explains. “No one wants to take the responsibility.”

Compounding the problem, few clinical trials have been dedicated to CRPS. Pharmaceutical companies are hesitant to put money behind trials for historically confounding conditions like CRPS: It’s hard to design drugs to treat a condition in which the underlying cause is uncertain. But according to Oaklander, now that CRPS can be tied to specific nerve damage and classified as a neuralgia, doctors can base their treatment for CRPS on the numerous clinical trials for neuralgias.

Manhattan’s Beth Israel hospital recommends that most patients with CRPS try at least one nerve block, which — depending on the cause — may immediately result in pain relief. The relief is temporary, however, and doesn’t deal with the underlying cause, so it’s not a long-term solution. As with all types of neuropathic pain, one has to be willing to do some trial and error with a variety of approaches.

“If I could have only one drug to treat this condition,” says Oaklander, “I would choose nortriptyline because it has the highest efficacy in many trials for other kinds of neuralgia.” Originally developed to treat depression, nortriptyline and other tricyclic antidepressants (amitriptyline is another) were later tested and found to be effective in treating neuralgia. Antiepileptic drugs were also found to be helpful for various types of neuralgia, and some studies suggest that the side effects may be milder than that of the antidepressants. However, according to Oaklander, tricyclic antidepressants tend to be more affordable.

If tricyclics afford no relief, Oaklander says she might try something like an antiepileptic medication, or low doses of opioids combined with other drugs if necessary. Topical local anesthetics are also low-risk, inexpensive, and often effective.

A pharmaceutical revolving door

For many struggling with CRPS, the medication lists are long and subject to rapid turnover. Nelson currently takes 20 different medications, including Valium (diazepam), amitriptyline, morphine, atomoxetine, and lidocaine. Experts say that some people find that rotating their pain medications, or switching from one drug to another periodically may be helpful.

Not every drug targets the actual pain. Some are prescribed to treat the painkillers’ side effects. For example, people taking opioids might need other drugs to treat side effects such as constipation and/or nausea. It’s a delicate balancing act, to say the least, and side effects can be debilitating. “I’ve not had complete pain relief from any of my medications,” says Lucker. She’s tried zonisamide (made her “psychotic”), oxycodone (she nodded off at the wheel), and methadone (had nausea that never passed) along the way. “Most of them have given me side effects that haven’t outweighed the benefits yet.”

Maluchnik, who moderates an online support board for people with CRPS and runs a mortgage business with her husband, says that her prescriptions all but incapacitated her: “I could not form a complete sentence. It affected our business greatly — who wants a mortgage from someone who doesn’t make any sense?”

Physical therapy and rehabilitation are critical as well, says Oaklander. “If patients don’t use the painful area — a natural tendency — they get atrophy and stiffening of connective tissues and loss of range of motion, which worsen pain and disability.” In short, she says, “If you don’t use it, you lose it. Even if [my patients] can only walk 100 hundred yards, they should get out there every day, and maybe in a month, they’ll walk 110 yards. If they don’t, they may find themselves unable to walk at all.”

Physical therapy gets a vote of confidence from Dr. Steven J. Weisman as well. His pediatric pain clinic at the Children’s Hospital of Wisconsin sees about 30 CRPS patients a year, and he says they’ve had “a nearly 100 percent success rate” with a treatment plan that revolves around physical therapy. “We follow patients until their pain and symptoms are completely resolved,” he says, noting that most had improved or normal function within three to six months.

Also essential to most CRPS cases is mental health care, especially because people who are depressed often have a lower threshold for experiencing pain. “Many patients become discouraged, depressed, anxious, and fearful,” explains Oaklander. “That compounds their disabilities.” Psychological problems related to the condition should be diagnosed and treated, according to Oaklander: A depressed patient, for example, may be less likely to do her physical therapy. Of course, medicating CRPS becomes more complex when prescribing drugs to treat its equally taxing emotional and mental effects.

Some complementary treatments have also brought relief. Oaklander has seen some success with cognitive-behavioral therapy and biofeedback. Although a review of studies on acupuncture and neuropathic pain suggests that the tiny needles may have limited benefit for CRPS, some patients report relief from the practice. Weisman’s clinic also incorporates gentle massage in order to desensitize the body parts affected by CRPS.

Surgery is the treatment of last resort, says Oaklander, but “surgical exploration of damaged nerves can sometimes give patients relief where drugs have refused to work.” Electrodes surgically implanted in the spinal cord and used to stimulate nerves there sometimes prove effective, as do low-risk hand-held nerve stimulators that patients can operate themselves.

While these strategies are fairly straightforward, the all-encompassing pain of CRPS has led some patients to seek extreme treatments. Dr. Robert Schwartzman induces a coma in CRPS patients by administering a heavy dose of the anesthetic ketamine. The patient remains unconscious for five to seven days, with the hope that during this time her pain sensors will essentially reset themselves. Since it is considered risky and highly experimental, Schwartzman conducts the procedure outside the U.S. in Germany. Both Oaklander and Weisman have major reservations about the treatment.

“It’s a radical treatment, and it has three strikes against it,” asserts Oaklander. It’s dangerous, unproven, and extremely expensive, she says. “Only after you’ve tried treatments that have proven cheap and safe and effective can you start going for the Hail Mary pass.”

Weisman concurs. “I cannot imagine using this potentially dangerous methodology to treat CRPS,” he says.

Baby docs, early diagnosis, and momentum

Potentially good news for CRPS sufferers, however, is that chronic pain has caught the government’s attention: Congress declared this the Decade of Pain Control and Research, and National Institutes of Health (NIH) investment in pain research grew from $82 million in 1997 to $224 million in 2007. In 2004, $1.5 million was dedicated to CRPS research led by the NIH’s National Institute of Neurological Disorders and Stroke (NINDS). In fact, Oaklander’s promising nerve damage study was funded by the NINDS.

And patients have seen encouraging signs from the next generation of doctors. Skiba fondly refers to the “baby docs,” usually still residents, who she believes are far more aware of CRPS than the old guard. When Riechard’s general practitioner retired, her new one was a doctor just out of residency who came in knowing all about CRPS.

If CRPS is on the radar, we might not see the winding paths to diagnosis so many have endured. Oaklander says early diagnosis lends itself to a more positive prognosis. Weisman’s success in treating young patients bears this out as well — his clinic boasts complete recovery in most of its young patients.

And, according to Oaklander, now that a distinct cause has been found, new research into more definitive diagnostic tests and innovative treatments are likely to follow, with more pieces of the puzzle falling into place.

Where some years ago support may have been virtually impossible to find, several Internet support boards have sprung up in recent years. Maluchnik started the RSD-CRPS of America group in 2000. It’s become a place where people can, above all, be understood. “It’s not a misery loves company thing,” Riechard explains. “It’s that no one can understand what you’ve lost until they’ve lost it too.”

Says Maluchnik: “People feel safe there, safe to share their feelings and frustrations. We have, I am sure, saved people from the desperation of suicide.”

The Web community can be a practical resource as well, especially with a little-known condition like CRPS. According to Maluchnik, members of her board helped newly diagnosed patients find doctors specializing in CRPS treatment.

“I believe that you can control how you deal with this, she says, “and I choose to deal with it in a positive way. I have [CRPS], it does not have me. I have to believe that they will find a cure or an effective treatment. It’s my lifeline, and everyone with CRPS needs that. I could never continue to live if I thought that this was forever.”


Interviews with Dr. Anne Louise Oaklander, director of the nerve injury unit at Massachusetts General Hospital

Interview with Dr. Steven J. Weisman, pediatric pain clinic at the Children’s Hospital of Wisconsin

Interviews with JoAnne Maluchnik, Tricia Skiba, Peggie Riechard, Sally Lucker, and Karen Nelson, pain sufferers

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