Research, Education, and Awareness of Reflex Sympathetic Dystrophy Syndrome

Nearly 142 years have passed since Dr. Silas Weir Mitchell, a Union Army surgeon, first described the excruciating pain he witnessed in David Schiveley, a 17-year-old soldier wounded at Gettysburg.  Dr. Mitchell coined the term causalgia (Greek for burning pain) to describe the soldier's pain. The gunshot wound had healed, yet Schiveley was still suffering with unbearable pain. 

Since then, this syndrome’s name has changed many times: Sudeck’s atrophy, traumatic arthritis, minor causalgia, posttraumatic osteoporosis, posttraumatic pain syndrome, posttraumatic oedema, posttraumatic angiospasm, shoulder hand syndrome, reflex sympathetic dystrophy, and recently, complex regional pain syndrome Type I and II. Scientists and clinicians are still baffled by this little-known, poorly-understood collection of signs and symptoms. What causes it?  Why does it develop in one person and not in another with same injury?  Why does it occur in more females than males?  Why do children and teens with this syndrome generally get better while most adults (80 percent in one prospective study) cannot resume prior activities?  

Complex Regional Pain Syndrome (CRPS) is a chronic neurological syndrome characterized by: severe burning pain, pathological changes in bone and skin, excessive sweating, tissue swelling, and extreme sensitivity to touch.  In our recently completed 2005 Internet survey of 1362 people with CRPS, the average pain score (10 being the worst pain imaginable) was 7.9 and 35% described their pain when CRPS developed as a 10, the worst pain imaginable. (For a complete description of RSD/CRPS symptoms, please read the Diagnosis section of the Clinical Practice Guidelines section of our website, www.rsds.org.) 

CRPS is officially classified as a rare disorder (incidence of less than 200,000). No reliable epidemiological studies have been conducted. Although the syndrome can develop after any trauma, researchers are finding that CRPS/RSD has developed following common orthopedic surgical procedures, such as arthroscopic knee surgery, wrist fractures, total knee arthroplasty, ankle fractures, carpal tunnel surgery, and Dupuytren’s surgery. 

There are Two Types of CRPS - Type I and Type II.

CRPS Type I (also referred to as RSD) - cases in which the nerve injury cannot be immediately identified

CRPS Type II (also referred to as Causalgia) - cases in which a distinct "major" nerve injury has occurred

CRPS is best described in terms of an injury to a nerve or soft tissue (e.g. broken bone) that does not follow the normal healing path.  CRPS’ development does not appear to depend on the magnitude of the injury. Initially, the sympathetic nervous system seems to assume an abnormal function after an injury.  Since there is no single laboratory test to diagnose CRPS, the physician must assess and document both subjective complaints (medical history) and, if present, objective findings (physical examination).

Patient Support

RSDSA does not offer direct patient services per se; however I talk to those patients who call, as does my assistant Gayle Bonavita, and several of our board members who have CRPS communicate with patients via e-mail and on the phone.

We are acutely aware of the devastation caused by the pain of CRPS. People become disabled and marriages fail.  Families break part and lives are financially ruined.  People become socially isolated and house bound. In a 2005 Internet-based survey of 1,362 people with CRPS conducted by Johns Hopkins School of Medicine and funded by RSDSA, 47 percent of the respondents considered suicide and 15 percent had tried. If you read the donations section of our newsletter there are several gifts in memory of individuals who, more often than not, have taken their own lives.

We also asked the question, before you developed RSD, did you have any other pain problems affecting your body, 5.85 percent answered that they had fibromyalgia. Significantly, when we asked the question, after you developed RSD, have you developed any other pain problems affecting your body, the number rose to 16.89 percent of the 1362 respondents. 

Research

RSDSA is committed to encouraging research into the cause and cure of CRPS. Each year, we fund at least two research grants (up to $50,000). Since 1992, RSDSA has funded $732,665 in fellowships and research grants.   Recent RSDSA-funded grants were Treatment of Complex Regional Pain Syndrome Type 1 by nitroglycerine, web-based epidemiological survey of CRPS-1, Identification of CRPS Subtypes and Effective Treatments, Changes in CSF Cytokine levels in Reflex Sympathetic Dystrophy, Validation of Revised Diagnostic Criteria for CRPS Diagnostic Criteria for CRPS/RSD, and Non-invasive Investigation of Human Brain Mechanisms Associated with the Development and Treatment of RSD. Detailed application guidelines are on the website.

Incredibly, medical professionals and the public are still largely unaware of this intensely painful and potentially debilitating syndrome. A 1999 epidemiology study published in PAINreported that the mean number of different physicians that evaluated a CRPS patient prior to being seen at a pain center was 4.8. Our 2005 Internet-based survey reported that 56 percent of the respondents saw more than four physicians prior to being diagnosed with CRPS—not much of an improvement.  

Awareness and Education

With the assistance of our Scientific Advisory Board, we have developed a number of informative educational brochures.  One of the most widely distributed is a screening tool for medical professionals—a laminated, two-sided, wallet-sided card that depicts the signs and symptoms of CRPS Type 1 on one side and a pain rating scale on the other. It was an instant hit.  We have mailed the card with an informative cover letter to members of the American Academy of Family Physicians, American Academy of Physician Assistants, and the American Society of Pain Management Nursing.  The letter began with the prescient sentence: "A minute of your time can prevent a lifetime of suffering."  The response was robust and far-reaching.  Many of the physicians and nurses were on medical and nursing school faculties and requested multiple copies for their students and fellows.  RSDSA members also asked for individual copies for both their wallet and other family members and to distribute to medical professionals in their communities. 

Each year, staff and volunteers exhibit at major medical, insurance, and school nurse conventions and conferences.  Even though RSDSA has been in business for 22 years, it is still vitally important to attend new conferences to inform the attendees about our programs and educational materials, our top-notch website, research funding opportunities, ongoing CRPS clinical trials, and to discuss with medical professionals how we can work together to help their patients and improve their practices.

This year, RSDSA is publishing its third edition of evidencedbased Clinical Practice Guidelines (in press) and The RSDSA Review Digest, a compendium of articles that have appeared in theRSDSA Review on the diagnosis, treatment, and management of CRPS.  Although the articles are archived on our website, 42 percent of Americans do not have access to the Internet and most medical professionals are too busy to thoroughly search our website.  The Digest will be a perfect companion to the Clinical Practice Guidelines.  We expect that medical professionals will also distribute the compendium to their patients with CRPS. 

Government Initiatives

Through grass-roots initiatives, we have helped people with CRPS to get CRPS Awareness legislation passed in Delaware, New York and Pennsylvania; similar legislation has been or will be introduced in New Jersey, Illinois, Oregon, and California. The legislation mandates a State Department of Health conduct medical professional and public education programs to encourage earlier detection and appropriate treatment of CRPS.

In 2003, RSDSA was instrumental in convincing the Society Security Administration to issue a special ruling on how to adjudicate CRPS claims. We continue to receive letters and e-mails from members who have used our information to get their claims approved.

CRPS, Workers’ Compensation, and the Insurance Industry

Injured employees with CRPS do not fare well in the Workers’ Compensation (WC) program. Of the 1,362 people who completed the web-based survey, 41 percent reported being injured at work; however only few of them obtained workers compensation benefits. Too often, relationship between an injured worker and the WC insurance carrier erupts into a war.  Treatment is frequently delayed and denied. 

In 2005, RSDSA exhibited at two major workers’ compensation conferences, the Risk and Insurance Management Society and Case Management Society of America.  We are reaching out to the case managers and risk managers, some of whom still view CRPS as a nebulous, expensive, frequently fraudulent claim.  We launched a special newsletter, Working Together, Ensuring a Brighter Future, at the conferences.  Our goal is to develop a mutually beneficial relationship.  We will distribute our new Clinical Practice Guidelines (in press) to the insurers to encourage early intervention and appropriate treatment enabling individuals with CRPS to recover and return to work.   

Patient Education

Linda Lang, a RSDSA board member and co-author of Living with RSDS, described the tremendous losses that an individual with CRPS experiences. "… in publicizing RSD, we generally focus on the pain, not the disabilities that come with it—the legs and hands that no longer work, the bones that become osteoporitic, the joints that become locked, the muscles that become spastic…There is an awful lot we leave out—how a productive member of society can become too disabled to work or take care of her children. We don’t discuss the tremendous personal losses—families, friends, jobs—that RSD wreaks…"

We address many of these issues in our publications, such as the RSDSA Review (a quarterly newsletter), the Support Group Newsletter (a bi-monthly electronic publication), and In Pain, Out of Work, Can’t Pay the Bills, a resource directory that identifies the governmental and private programs that can help a low-income family obtain treatment, needed medication, pay their bills, and avoid losing their home. Originally published in 2001, the directory is now in its third edition.  We do several brochures as well, such as the one we introduced linking CRPS to sports injuries and the surgeries that traditionally follow them. CRPS/RSD: Prevention is the Name of the Game is written for coaches, trainers, and particularly athletes, a group we believe are at high risk for developing the syndrome.

Website

Most people with CRPS or persistent pain learn about RSDSA by visiting our website, www.rsds.org. In 2005, we had an average 37,000 visits per month. Our highest traffic, 57,000 in April was driven by a People magazine cover story revealing Paula Abdul had been diagnosed with RSD.

The website houses all of our educational brochures (in PDF format), videos or PowerPoint® presentations from past International Conferences, scientific articles on the diagnosis, treatment, and management of CRPS that have appeared in our quarterly newsletter or in peer-reviewed journals, links to other professional and consumer sites, and much more.  We encourage individuals to sign up for free electronic alerts that will tell them about new discoveries, clinical trials, articles in the media, legislative initiatives, and upcoming events.   Srinivasa N. Raja, MD, Director of Pain Research at Johns Hopkins University has created a PowerPoint® presentation On Diagnosis and Treatment Options of RSD/CRPS. This can be downloaded and used for in-service training or personal edification.

RSDSA is a vibrant organization, very sensitive to the needs and concerns of its 6,000 members and the greater CRPS community. In the Fall 2005 RSDSA Review, Norman Harden MD said, "Without RSDSA, progress in fighting the syndrome would likely come to a halt. Even basic and essential information, such as how best to make the diagnosis and what causes the disease, is lacking, and these are traditional topics funded for research by RSDSA."

[1] Veldman H J M , Reyen H M, Arntz R, Goris, J A, Signs and Symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. The Lancet 1993; 342; 1012-1015.