Weeks after having shoulder surgery in 2009, police officer Nicole Chiarella should have been well on the road to recovery. Instead, the Albany, NY, resident endured debilitating pain her doctors couldn't explain or treat.
It took three more years of dealing with worsening, life-altering pain before she learned she had complex regional pain syndrome (CRPS), a neurovascular condition that can develop when trauma leads to damaged nerves and blood vessels that, in turn, cause pain and other symptoms that seem disproportionate to the original injury. While research shows that most patients with CRPS end up pain-free after one year as injured nerve cells regenerate, a small group of people like Chiarella wind up with a grueling, hard-to-diagnose condition that can last for decades.
In the eight years between her injury and the surgery, Chiarella did physical therapy and lived with manageable complications, such as numb fingers. Things spiraled after surgery, though, as the new pain went unabated.
“Doctors said the surgery went fine, and they couldn't find anything wrong with me,” recalls Chiarella, now 52 and living in Westin, FL. “I felt they thought it was in my head, but the pain kept getting worse. I had pins and needles in my neck, couldn't turn my neck or lift my arm, and my fingers were purple.”
Chiarella had been diagnosed with brachial plexus neuropathy in 2010 and reflex sympathetic dystrophy (RSD)—a now-obsolete term for a form of what today is known as CRPS—the following year. She tried treatments including an implanted spinal cord stimulator and an intrathecal pain pump, which delivers medication directly to the patient's spinal fluid, letting the drugs interrupt irregular signals in nerves. A pain management doctor prescribed narcotics and other medications that caused hallucinations as Chiarella's mental health worsened. She gained over 100 pounds, relied on her teen sons for help, and lost her job.
When hallucinations led her family to take her to a hospital in 2012, a neurologist who fully understood CRPS admitted her. Under care, she reduced her medications, her hallucinations ceased, and she lost about 30 pounds.
“I was finally thinking clearly, and from there my life finally started turning back around.… I began to feel more like myself,” Chiarella says.
She still has daily pain she manages with a low dose of a non-opioid pain reliever through her intrathecal pump. Retired from law enforcement and now a professional bodybuilder, she has rebuilt her life and embraces all it offers.
Like Chiarella, Carolyn McNoldy struggled to get an accurate diagnosis and treatment after developing CRPS 15 years ago following surgery to both elbows. When a doctor told the paralegal from Marshall, VA, to “just deal with it,” she felt despondent but cast a wide net for advice, seeing a neurologist, nerve and hand surgeon, pain management specialist, and others. Her tenacity led to numerous surgeries and treatments. While these “all took away small pieces of my pain pie,” she says, she still had “aching and burning pain as if someone were drilling into my elbow.”
After two years, a nerve surgeon recognized what she described as CRPS, which let her establish the best management routine possible. However, McNoldy's pain spread to her shoulder and neck and is more troublesome than her original issue. “I live every day at 7 on a 1-to-10 pain scale,” she says.
Today, McNoldy manages her CRPS with many tools, including a peripheral nerve stimulator, a small electrical device implanted next to a peripheral nerve that replaces pain with mild tingles; monthly trigger point injections; massage; and medications including hydrocodone, oxycontin, gabapentin, and a muscle relaxant. Getting to this point hasn't been easy, and finding the right providers was key. “I tried so many doctors who weren't a good fit or who thought it was all in my head,” she says.
An Often-missed Condition
Anne Louise Oaklander, MD, PhD, FAAN, a peripheral nerve specialist at Massachusetts General Hospital, says Chiarella and McNoldy's experiences are not unique for patients with this rare form of CRPS. “It's poorly understood, partly because chronic pain conditions are stigmatized and CRPS more often affects females,” she says. Most trauma and pain specialists also are not neurologists, she adds, and “invisible internal sensory nerve injuries are notoriously difficult to detect. In CRPS, the hidden nerve injuries are more severe than the clearly visible other trauma.”
Still, Dr. Oaklander says, clinicians do understand a lot about CRPS. It is known to occur after injuries such as wrist fractures and other medical trauma, like surgery, heart attack, stroke, or having a too-tight cast. Occasionally, it happens with no known trauma, though in those cases, she says, an “invisible trauma,” like a blood clot, may be the culprit.
Symptoms vary among patients and may even fluctuate within one person, Dr. Oaklander says. Common symptoms include abnormal temperature in the affected limb; allodynia, in which normal touch becomes painful; weakness and muscle atrophy; changes in sweating; and nail and hair growth in the area linked to the damaged nerve.
Two types of CRPS exist. CRPS-I occurs without confirmed nerve damage, which means the nerve injury is more subtle and tough to diagnose. CRPS-II occurs after a confirmed nerve injury, so it's easier to identify. Nerve damage in CRPS mostly centers on C fibers, a nerve fiber that carries pain sensations and controls blood vessels, immune and inflammatory responses, sweating, and the health of nearby non-neural tissues, including bone and skin.
CRPS only affects one or maybe a few nerves, typically in the limbs, and symptoms are often worse in the hand or foot. Pain can travel far beyond the original injury site, however, even “mirroring” to the other side of the body.
People with neurologic pain may undergo electromyography or a nerve conduction study, both of which assess damage to much larger nerve cells but do not measure C fiber function. Tests that can detect C fiber injuries include neurography (an MRI of the nerves), neurodiagnostic skin biopsies to count C fibers, and microneurography. However, many patients can't access this type of testing, which typically has long delays.
What isn't understood is why some people, like Chiarella and McNoldy, end up with lifelong CRPS. Dr. Oaklander says some research suggests it may be an autoimmune issue, though more study is needed. (In autoimmune diseases, the body's immune system mistakenly attacks healthy tissues and organs.)
Limited Treatment Options
Early intervention is key to preventing CRPS from turning into a chronic condition. “The longer it goes on, the harder it is to get rid of because, if the central nervous system, and primarily the brain, gets involved, it is almost like the system learns how to keep doing it,” says Charles Argoff, MD, a professor of neurology at Albany Medical College in New York who help Chiarella manage her condition.
Patients have many treatment options, he says, but finding the right combination may require trial and error. In addition to physical and occupational therapies, many patients try neuromodulation, a technology that delivers electrical or pharmaceutical agents to modify nerve activity in order to reduce pain.
Pharmacological therapies such as duloxetine, an antidepressant medication often used to treat pain, and gabapentin, the antiseizure medication McNoldy takes, also could help control neuropathic pain. Some patients may opt for a nerve block, in which a doctor injects a medication near nerves with the aim of temporarily blocking pain signals from reaching the brain.
Dr. Argoff says patients with CRPS must approach pain management like Rocky Balboa: “You don't have to go down, but it's important to work with someone who has the collective skill set to put all of these things together in a methodical way to see what is working.”
The journey to diagnose and treat CRPS can take a toll. For McNoldy, finding a community of others with CRPS —and seeing them enjoy life and find happiness—has been a huge boon. “I have [CRPS], and it's not going to go away,” she says, “so I'm making the most of the life I was given.”
Resources for People with CRPS
