After you sprain your wrist, break an ankle or undergo shoulder surgery, you expect to recover from pain as the injury heals. Unfortunately, for people with complex regional pain syndrome, months go by and pain persists.
“The patient has a pain that seems well out of proportion to what you might expect from the normal tissue healing that should occur after an injury,” says Dr. Sean Mackey, chief of pain medicine and director of the Stanford Systems Neuroscience and Pain Lab at the Stanford University School of Medicine.
An intense, burning pain is what patients describe, Mackey says. It feels like the aftermath of freezing your arm or leg while you’re out in the snow, he says, as numbness passes and unpleasant sensations begin. The difference with CRPS is those sensations may never end.
Seven years ago, Adele hurt her leg in a work-related accident. She expected the injury to heal and pain to subside. Instead, severe pain remained and even spread to other parts of her body over time.
Eventually, Adele, now 45, was diagnosed with CRPS. Ever since, unrelenting pain has marked every aspect of her existence. Work, relationships with family and friends, social life and peace of mind are threatened in the face of ongoing, disabling pain for Adele, who requested that a pseudonym be used to protect her privacy.
“Basically, this diagnosis took away my career and general state of good health,” she says. “The recovery pain from the surgeries was nothing compared to the CRPS pain that is ongoing.” Self-esteem can fade along with a sense of purpose and accomplishment when pain simply makes work too difficult for those with CRPS. “Oftentimes, we have to cancel plans or say no to invitations because we are in unbearable pain.”
Despite many trials, chronic pain management techniques that can help people manage CRPS didn’t benefit Adele. Infusions of the anesthetic drug ketamine were effective, at first, but side effects forced her to stop. “Opioids were not a treatment option for me due to undesirable side effects,” she adds. After several years, she realized CRPS was never going to go away.
Complex regional pain syndrome is classified as a rare disorder of the nervous system. Young adults are most often affected, with women at higher risk. According to a July 2015 analysis published in BMJ, slightly less than 200,000 patients in the U.S. are affected by CRPS at any given time.
In Type I CRPS, the origin of the pain isn’t known. In more-common Type II CRPS, or causalgia, the related nerve injury can be identified.
CRPS usually starts with a localized injury to a single part of the body, often a fracture. However, trauma such as surgery, crush injuries, sprains or immobilization can also lead to CRPS.
Because there’s no single test to identify CRPS, it’s challenging to diagnose. Increased sensitivity to pain, or hyperalgesia, is a major diagnostic criterion, explains Dr. Salim Hayek, chief of pain medicine at University Hospitals Cleveland Medical Center.
Changes in skin color or temperature also occur. An affected lower leg could be hot and red, or bluish and cold in obvious contrast to the healthy leg. Limb swelling and sweating are also signs. Patients may have decreased limb range of motion or function, weakness, tremors or involuntary muscle contractions. Hair, nail and skin changes, such as thinning or thickening, are hallmarks.
Quality of life is also affected. “Frequently, most people with chronic pain — be it CRPS or something different — will describe disruptions in their sleep, their mood, high levels of fatigue,” Mackey says.
Before a CRPS diagnosis is made, all other possible causes with overlapping symptoms must be ruled out. Tests to measure muscle activity and nerve conduction, MRIs and X-rays, testing for deep vein blood clots and other procedures help make the determination.
Pain programs use specialists from a variety of disciplines to address a patient’s medical, mobility, functional, occupational and psychological issues stemming from CRPS. Physical therapy is the mainstay of treatment.
As time goes by, patients with CRPS may get in the habit of “guarding” the affected part of the body. However, “If you do that for a long time with CRPS, it’s counterproductive,” Hayek says. “So the whole thing is about breaking the cycle and getting the patient to reuse the arm.”
Physical and occupational therapists work with patients in an ongoing, structured fashion to help them make gradual progress. Specialists help patients control pain so they can participate in long-term therapy to restore and improve function.
Cognitive behavioral therapy and other psychological treatments help CRPS patients cope. Some patients benefit from medication to treat anxiety and depression.
For Adele, it took a three-month, inpatient hospitalization for a multifaceted treatment approach at Johns Hopkins Hospital in Baltimore to make a real difference. Daily physical therapy, cognitive behavioral therapy, medication for depression and anxiety, group therapy and classes on coping strategies were “life-changing” program aspects.
“With CRPS, you use it or lose it,” Adele says. “Muscle wasting is common with CRPS. I worked as hard as I could to keep my body in good physical shape to combat this atrophy.”
Adele walks with a cane to assist stability and alleviate pressure on her leg. Attending events such as family reunions, holiday parties, weddings or concerts comes at the cost of resting beforehand and spending days recovering after.
To help others, Adele founded a local CRPS support group through RSDSA, a nonprofit association whose mission centers on helping people with CRPS, which is sometimes also referred to as reflex sympathetic dystrophy syndrome. Family members may also attend support groups, which can help them understand what their loved one is going through. “You just never know how someone is suffering by appearance,” she says.
Doctors prescribe a wide range of drugs to treat CRPS, including drugs used off-label. Anticonvulsant drugs, steroids, opioids, nonsteroidal pain relievers, bisphosphonate drugs typically used for osteoporosis and skin patches to reduce pain sensitivity have been used to manage CRPS symptoms.
With the opioid epidemic and increasing awareness of the risks associated with these drugs, doctors are prescribing opioids like OxyContin much more cautiously. That’s hard for CRPS patients who rely on them.
“In general, there’s no good evidence for long-term or lifelong efficacy of opioids for chronic non-cancer pain, whether it’s CRPS or not,” Hayek says. However, he adds, “I never argue with the patient because I’m not the person in pain.”
Mackey takes a middle ground. “I do find there are some patients who have been helped significantly, long-term, being on opioids,” he says. “And I’ve also found people whose lives have been destroyed with opioids, and taken them off them.”
More evidence is needed on long-term effectiveness, Mackey says, although data is improving. He emphasizes that he rarely starts patients on opioids as a first- or even fifth-line treatment.
As CRPS becomes better understood, more effective treatments should come forward, Mackey says. His brain-imaging research includes a February 2014 study in The Journal of Pain, which found CRPS is associated with distinct structural abnormalities in pain-related regions of the brain.
A decade ago, Barbara tore the rotator cuff of her right shoulder. As she relied on her left arm to compensate, she tore that rotator cuff as well. “I herniated my cervical spine in the process of trying to adapt, and then my trouble really started,” she wrote in an email. Impaired nerve conduction resulted in painful, permanent bladder conditions and she developed CRPS.
“Even my clothing hurts on bad days,” says Barbara, who asked that only her first name be used, for privacy. “I hurt from my eyeballs to my feet, and everywhere in between.” Pain keeps her from sleeping and she is chronically exhausted. Barbara’s husband has late-stage kidney disease and she is his caregiver.
When Barbara sees across-the-board policies aimed at limiting opioid prescribing, or reads expert opinions that opioids are ineffective for long-term control of chronic pain, she gets angry. She believes media, regulatory and medical establishment pressures to address the opioid epidemic are stripping away the single effective means to maintain a minimal quality of life for some people with CRPS.
“Telling patients like me who are basically bedridden without that help of pain medication that ‘opiates don’t work’ for chronic pain patients is laughable,” Barbara says. “Without opiates, I wouldn’t be able to bathe myself, cook meals and take care of my husband.”
Without pain medication, she says, “I live my life feeling like somebody is firing phasers at my genitals, bladder, ovaries and colon 24/7. With my medication, I can function, albeit not like I used to. I will never be able to return to my job. All this has rendered me destitute.” Other pain management methods haven’t worked.
Barbara expresses compassion for parents who have lost children to overdoses. She seeks similar recognition for patients with chronic pain. “The stories of chronic pain patients who are dying — either by their own hand because they can no longer live with their pain, or by the way undertreated and untreated pain ravages our bodies to the point where our hearts stop, or we have strokes, or other essential organs fail — well, we’re not hearing reporting about that,” she says.
Heat therapy and mirror therapy , in which patients move their unaffected limb in front of a mirror to reduce neural pain feedback to that limb, may help with CRPS. For patients who don’t respond to more conservative approaches, nerve block injections and intravenous anesthetics like ketamine infusions may reduce pain.
Transcranial magnetic stimulation — a noninvasive method using magnetic fields to stimulate nerve cells in the brain — showed significant pain relief for with upper- and lower-extremity CRPS, in a small pilot study in the June issue of Neuromodulation.
With invasive treatments like implanted spinal cord stimulators, clinicians and patients must weigh benefits and risks. Spinal cord stimulators account for a significant number of medical device injury reports, according to a recent investigation by the Associated Press.
If you’ve been diagnosed with CRPS, don’t suffer in silence, Mackey says. “Seek out good multidisciplinary help — ideally, people who have expertise in this field and who work collaboratively with physical therapists, psychologists and health care clinicians.”
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