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Rare Pain Syndrome Tricks Immune System and Doctors

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Judy Hopkins recently drove down to Virginia to vacation in Colonial Williamsburg. Not only was it her first time there, it was her first non-medical trip in four years and the first vacation she has taken without her parents in 10 years.

This is one of a few quiet victories the 27-year-old New Jersey native has celebrated since she came out of her second ketamine-induced coma to relieve the excruciating pain she has been in since she was 16.

It all started in her junior year of high school when the varsity basketball player had surgery to remove a tumor in her right heel. No one realized it at the time, but the surgery resulted in a severed nerve and vein. It would be two years before the surgical error was diagnosed, and five years until anyone realized that it had also triggered Reflex Sympathetic Dystrophy (RSD) – a chronic pain syndrome that is as unpredictable as it is severe, and has terrorized her body non-stop over the past 10 years.

“There is just not enough known about the disease,” Hopkins told FoxNews.com. “And no one knows why one specific trauma triggers it. But it can be any major or minor trauma. I know some people, it's been something as simple as stubbing your toe that triggers it.”

Dr. Richard Sultan, a pediatric neurologist at Jersey Shore Neurology in Neptune, N.J., who diagnosed Hopkins in her sophomore year of college, described RSD – also known as CRPS (complex regional pain syndrome) – as a malfunction of the immune system.

“It’s most likely some disconnect between the nervous system and the sensory nerves… What happens is some type of stimulus triggers your immune system in exaggerated spots,” said Sultan, who added that the most common symptom is pain felt by RSD patients that is disproportionate to the degree of injury.

THE TROUBLE WITH DIAGNOSING RSD

RSD affects between 200,000 and 1.2 million Americans, according to the Reflex Sympathetic Dystrophy Syndrome Association website.

It is highly manageable if caught early, but that rarely happens, Sultan said. RSD patients will typically see five doctors before getting an accurate diagnosis because there is still so much that isn’t known about the condition. And on top of all that — many doctors don’t recognize RSD because they haven’t seen it.

In Hopkins' case, two years had passed from the time of her heel surgery to when a doctor at the Hospital for Special Surgery in New York City diagnosed her severed vein and nerve. During that time, her condition deteriorated due to multiple misdiagnoses, in addition to another tumor found in her left breast and underarm, also requiring surgery. By her sophomore year of college, she had endured 18 surgeries and was taking 30 different medications.

“Unfortunately, many surgeries and many traumas had occurred in-between that caused the RSD to spread before anyone realized there was anything neuropathic,” Hopkins said. “They thought it was just a localized nerve issue.”

This is where things get tricky for physicians like Sultan.

“A lot of people believe that it can then switch to another unaffected part of the body,” he said. “I think that’s a little controversial. Not everyone agrees upon that.”

Hopkins says she has stage four RSD, which some describe as the rarest and most aggressive form of the disease, where your entire body is afflicted with pain, and the disease begins to affect internal organs.

Sultan is cautious about this definition of stage four RSD, and specifically rejects the idea that it can ever cause problems with someone’s organ system.

He described two different systems of classification. The first separates it into Type 1, which is when the triggering nerve injury can’t be identified, and Type 2, which is when it can be identified.

He acknowledges that RSD patients also can be classified into stages, beginning with the acute stage, which he said occurs in the first three months. The second and third stages – the dystrophic stage and the atrophic stage, respectively – share many of the same symptoms with the acute stage, but are more severe.

In addition to pain being disproportionate to your injury and severe burning, which Hopkins says can “feel as if you've been doused with gasoline and lit up,” other symptoms include spasms, changes in skin color and temperature due to blood flow, joint restriction, tissue swelling and hypersensitivity.

“A breeze can set you off with excruciating pain. Someone just brushing up against you can be worse than someone squeezing you,” said Hopkins, describing how hypersensitivity can affect an RSD patient.

Patients with stage two RSD can begin to see “wasting of the muscle,” Sultan added, and when a patient gets stage three RSD, the affected body part becomes “just a painful appendage and is really not being used.”

TREATMENTS FOR RSD

Sultan strongly disagrees with the polypharmacy approach to managing RSD pain that patients like Hopkins are put on. Instead of prescribing multiple pain medications like Lyrica, Norontin and ketamine, he favors the use of an aggressive physical therapy program.

“I do believe, which some people don’t like to hear, is that when it’s treated with multiple medications… that, itself, puts a patient’s life at risk,” he said. “While patients know that it runs their lives, they need to adopt a posture of, ‘it can be managed, and it can be treated, and, if you’re aggressive in terms of your therapy, it can improve, and your life can improve.’”

Hopkins, who left school after her RSD diagnosis in the summer of 2003, agrees that physical therapy can play an important role is treating RSD and wasn’t happy with her polypharmacy regiment either.

“I literally lived in a recliner in the downstairs of my parent’s house. I only left the house to go to doctor’s appointments and treatments. I was in excruciating, level 10 and above pain all the time,” she said.

After diagnosing Hopkins, Sultan sent her to James Smith, a physical therapist with Crest Physical Therapy in Manasquan, N.J. Hopkins’ left arm was half the size of her right arm when she began seeing him, and even though physical therapy hasn’t turned out to be the solution to managing her pain, it has had some positive effects.

“He has prevented me from getting a single, solitary frozen muscle in my entire body in the time that I've been with him, as well as preventing my muscles from atrophying,” said Hopkins, who has seen Smith for more than seven years. “I spend more than 20 hours a week in the gym. But that alone has not prevented the RSD from progressing.”

Dr. Richard Schwartzman, Neurology Department chair at Drexel University in Philadelphia, also agrees with Sultan’s point about physical therapy. But, he reached a different conclusion on how to treat Hopkins’ advanced pain syndrome when she was referred to him.

He put her on an active waiting list to receive a ketamine coma — a dangerous and controversial procedure that intentionally puts you into a five-to-seven day coma by administering an exorbitant amount of ketamine. The patient has to be put on life support to prevent the lethal dosage from killing them.

Ketamine is a central nervous system depressant that produces a rapid-acting dissociative effect, the U.S. Department of Health and Human Services said on its website. It was developed in the 1970s as a medical anesthetic for both humans and animals.

“The point of giving you that much ketamine is to literally shock your nervous system,” Hopkins said. “And they compare it to rebooting your computer. So, what they're doing is rebooting your nervous system, as if giving you a clean slate… as if starting anew.”

In October 2007, Hopkins got her affairs in order and signed over her medical power of attorney to her parents in preparation for the possibility that she wouldn’t return. Then she flew to Germany, one of the few places where the procedure is allowed.

“My parents understood and I understood that when you go to Germany, you sign your life away,” Hopkins said. “But, at that point, you literally are at death's door anyway. So, this is an opportunity to actually get a life. Whereas where you are now, you have no opportunity for a future.”

Hopkins would get that clean slate when she woke up in complete remission, despite suffering an infection that prolonged the coma two more weeks. Unfortunately, her new lease on life only lasted one month.

By November 2008, her symptoms had returned and were worse than ever, leading her to opt for a second ketamine coma. She said that she became the fifth person in the world to have a second ketamine coma in November 2008.

This time, she was in the coma for the minimum amount of time with no complications. Although she didn’t fly home with full remission, the results were positive.

“I was perfect. I came home and I had the most amazing four months of my life. I rode my bike for the first time in eight years, I dribbled a basketball for the first time in eight years,” Hopkins said.

But that bliss was short-lived. A few months later, doctors found another tumor in the meniscus of her knee requiring surgery to remove the bone, which triggered a complete relapse.

WHAT THE FUTURE HOLDS

Although Hopkins has recovered from the knee surgery, she is still considering another round of ketamine coma. At this point, she views it as the only thing that can get her back to remission. If she did opt for a third round, she said she would only be the second person in the world to do so.

Sultan doesn’t agree with the use of the ketamine coma to treat severe RSD, and while he said he understands why people who have exhausted all other options would turn to such a dangerous alternative, he wouldn’t order it for a patient.

Schwartzman, who helped Hopkins get on the waiting list for her first ketamine coma, could not be reached for comment.

“I think that it is extreme,” said Sultan, regarding the controversial treatment. “The risks in it are so high and we can do other things here in the U.S., such as RSD-directed physical therapy programs.”

People with RSD can suffer from atrophy in their affected body parts, which Sultan believes may be best helped by intensive physical therapy. The idea is that by challenging the muscle to function normally, it will increase blood flow, which is lacking in some RSD patients, and therefore, reduce the painful symptoms and strengthen the muscles.

“You could probably come off medications. A lot of people don’t believe it. I believe it, I’ve seen it. It’s much, much more to the patient’s advantage than polypharmacy,” said Sultan, who added that it would have to be done at a qualified physical therapy or university center to be effective.

While different doctors approach treatment differently, the one thing everyone can agree with is that getting RSD diagnosed early is crucial. The first step in achieving that is to raise awareness about RSD in the medical community.

“Years ago, it had different names and no specific diagnosis, so a lot of doctors weren’t trained to diagnose RSD,” Sultan said. “Now it’s a lot more recognizable early.”

The problem, though, remains that many doctors don’t know what to look for with RSD. There’s not one specific test to diagnose it, which leaves it up to the doctor to be able to use the entire clinical picture, including how the patient looks, the exam and the history, to recognize it.

For those who are faced with situations similar to that of Hopkins, she has some simple advice: Be persistent.

“If your doctor doesn't listen to you, go to another doctor,” Hopkins said. “And keep going until someone finally listens to you. Because one thing you'll learn is that you are your best advocate. And that's really the most important thing to know.”

While Hopkins considers a third ketamine coma, for the immediate future, the former theater and dance major hopes to get her feet wet with a creative writing class this fall.

“For right now, I'm feeling good, I’m staying positive, and I have the actual possibility of a future and that's more than enough for me right now,” she said.