Four things pediatricians should know about juvenile arthritis

“Arthritis is something elderly people get,” is something Dr. Andrew Shulman, CHOC Children’s pediatric rheumatologist, has heard countless times. People are often surprised to learn that kids — even toddlers — can develop arthritis.

Joint pain can be a bit of a mystery to most people, especially when it comes to young children. With August being Juvenile Arthritis Awareness Month, we looked to Dr. Shulman to learn more about this and other common juvenile joint pain myths he finds most important for pediatricians to recognize and communicate with patients.

Myth 1: All joint pain is arthritis.

False. If joint pain is the primary symptom, chances are it is not arthritis. The most common initial symptoms of arthritis are persistent swelling, redness, stiffness and limited range of motion.

The type of joint pain that is associated with juvenile idiopathic arthritis (or JIA — formerly called juvenile rheumatoid arthritis) is pain in an articular location that is dull, throbbing or sharp and that improves throughout the day with movement. This pain is commonly exacerbated by prolonged sitting, naps and specific tasks, and improves with activity and non-steroidal anti-inflammatory drugs (NSAIDs). Sometimes skin redness is involved.

Myth 2: Arthritis is a precise diagnosis.

False. It is a symptom and a finding, much like a cough is not an official diagnosis. More testing is needed to determine whether or not joint pain is caused by a specific type of arthritis or another rheumatic condition, such as pain from overuse or a neuropathic disorder.

Myth 3: Arthritis is diagnosed with laboratory tests.

False. Labs can be used to characterize arthritis when it is present, but lab work can be totally normal in kids who do have arthritis. Using lab tests alone as a diagnostic tool can lead to a missed diagnosis.

Dr. Shulman urges pediatricians to keep in mind that routine lab work is more useful initially than specialized tests are. “A complete blood count (CBC) with differential, chemistry panel, erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP) tests present more information and are better initial screening tests than specialized tests such as antibody tests and complement studies,” he says.

Myth 4: Only elderly people get arthritis.

False. The incidence rate of JIA is one to 10 out of 10,000 per year, making it a more common condition among young people than cystic fibrosis, juvenile diabetes and muscular dystrophy combined.

Learn how to refer to a CHOC rheumatologist.

Causes and Treatment for Pain Amplification Syndrome

Andrew Shulman, MD, PhD, pediatric rheumatologist at CHOC Children’s, discusses the causes and treatment for this common yet complex condition.

1) What is pain amplification syndrome?

Pain amplification syndrome is a condition (not a disease) in which patients develop an abnormal pain sensitivity. The nervous system registers and processes normal sensations from movement and environmental experience as pain signals. Think of the nervous system as an electronic sensor: The sensor has a detection threshold. All signals with an intensity level below the threshold are perceived as “normal” and all signals above the threshold are “pain.” In children with pain amplification syndrome, the threshold is too low. Normal sensations and movements cause the nervous system to experience pain.

2) What are the causes?

We need to understand much more about what causes pain syndromes, experience over time with many patients with this condition reveals some common triggers. Joint flexibility is the most common cause of musculoskeletal pain in children, and is often responsible for what is known as “growing pains.” Patients with pain amplification syndrome often have flexible joints and have previously experienced joint and muscle discomfort. Some patients have had numerous orthopedic injuries in the past, or a particular traumatic injury that causes pain sensitivity in one part of the body.

In some patients, emotional trauma, psychological difficulties or psychiatric conditions can trigger pain sensitivity. In most patients, sleep is affected and is not restful. Even when psychological factors are not an initial cause, the stress from experiencing pain and the resulting disruption in school and activities can amplify pain sensitivity. We don’t know enough about the genetics of pain perception, but children with pain amplification syndrome commonly have family members with pain difficulties.

3) What are the symptoms?

Symptoms include body aches, joint and muscle pain, abnormal sensations of burning, tingling, numbness or pressure, headache, and chronic abdominal pain. In some cases, light touch to the skin causes pain sensations. Accompanying features include “dysautonomia,” wherein the autonomic nervous system does not perfectly regulate blood flow throughout the body. This results in symptoms of Raynaud’s phenomenon (cold sensitivity of the hands and feet with color changes, numbness, tingling and burning) and dizziness, particularly when standing up. These symptoms are not dangerous but can be uncomfortable and worrisome to patients.

4) What are the treatments?

A number of therapies work together to reset the abnormal pain sensitivity. Physical activity, particularly aerobic exercise, is very important in normalizing the pain threshold. Therapeutic massage can desensitize the nervous system and help the patient feel touch and movement as normal and not painful. Developing a regular, restful sleep schedule is important.

Finally, psychological therapy is often a critical aspect of the therapeutic program. This therapy is focused on stress management and coping techniques. In some cases, medications that decrease nerve signal transmission can be used. These medical treatments only assist the other therapies and are generally not effective in children when used alone.

5) What kind of testing is involved?

No laboratory or imaging tests can confirm the diagnosis of pain amplification syndrome. The diagnosis can be difficult to establish because numerous other medical conditions could potentially cause the symptoms. It’s important to take a step back and look at the “big picture” of a patient’s history and symptoms. Patients commonly have extensive investigations with many specialists before reaching a pain amplification syndrome diagnosis. Physicians with experience in pain amplification syndrome can help recognize the condition. Rheumatologists often help because they are familiar with symptoms and diagnostic challenges posed by autoimmune and inflammatory diseases.

6) Is there a cure?

Pain amplification syndrome is not a disease, so no cure is needed. This is a condition that patients learn to manage and control.

7) What can patients and families expect when treating pain amplification syndrome?

Families can expect more good days and fewer bad days as patients work through the abnormal pain sensitivity. The overall trend is gradual recovery, which can be measured by regular school attendance and activity participation. Reassurance that the patient does not have a serious disease often provides a big boost as the therapeutic program moves forward.

8) How bad does pain have to get before you see a pediatric rheumatologist?

Patients should be evaluated when symptoms become a significant concern and affect school attendance or participation in sports and activities. Pediatricians and subspecialty colleagues should seek rheumatologists’ expertise for help evaluating a pain amplification syndrome diagnosis and/or making treatment recommendations.

For questions or more information, please contact Andrew Shulman, MD, PhD, pediatric rheumatologist at CHOC Children’s, at 714-509-8617. 

Living with arthritis: Carson’s story

Seventeen-year-old Carson comes from a close-knit family of athletes and had been playing baseball for a decade when consistent, unexplained pain left his family stumped and looking for answers. The next 18 months were filled with countless physical therapy and orthopedic appointments. When an MRI of his spine lead the radiologist to assume they were from a middle-aged person who’d played a lifetime of contact sports, rather than a 15-year-old boy, finally the family had a clue that Carson’s pain was not just an overuse injury.

At the time, pre-diagnosis, Carson was traveling a couple hours roundtrip every few days for orthopedic appointments related to wrist, elbow and shoulder pain.

He was ultimately referred to Dr. Andrew Shulman, a CHOC Children’s pediatric rheumatologist, and finally Carson’s family had the answers they’d been seeking for years. Carson was diagnosed with Juvenile Ankylosing Spondylitis, a form of arthritis that affects the spine and sites where muscles, tendons and ligaments attach to bones in the body. Juvenile Ankylosing Spondylitis is diagnosed in 1 in every 50-100,000 children per year.

He was also diagnosed with Pain Amplification Syndrome, a condition in which patients develop abnormal pain sensitivity. The nervous system processes normal sensations from movement and environmental experience as pain signals.

Andrew Shulman, M.D.
Andrew Shulman, M.D.

Looking back as far as middle school, a lot of Carson’s issues were tied to this condition. He had tried everything from water therapy to hypnosis, but nothing worked. Then Dr. Shulman recommended acupuncture, and Carson’s mom Andrea explains how Ruth McCarty, director of Chinese medicine and acupuncture at CHOC, was ‘unbelievable in getting Carson to a good place.’

Additionally, Carson’s multidisciplinary care team includes physical therapist Robin Beauregard and pediatric gastroenterologist Dr. Mitchell Katz. Carson’s family is also a key component of his care team.

“Dealing with these diagnoses is a team effort,” says Carson. “My dad carries me up the stairs when my Pain Amplification Syndrome flares up and I can’t walk, and I squeeze his hand during Humira injections. My mom helps me mentally, and she’s made this so much easier to manage. Dr. Shulman has given us a path forward.”

Despite still making his school’s baseball team while fighting a dual diagnosis, Carson could no longer cope with the week or two of pain that came with playing a single inning. Now he works out daily in a gym whether he’s experiencing a flare up or not, which helps with both diagnoses. To fill the void that competitive baseball left, he often plays catch with his friends and former teammates.

“Carson’s journey with arthritis is a powerful one. It speaks to the importance of diagnosing arthritis, and the outcomes we can achieve with therapy,” says Dr. Shulman. “His experience shows that multidisciplinary care has been transformative.”

Dr. Shulman offers the following guidelines on when to refer for further evaluation:

  • Persistent joint swelling, stiffness, limited range of motion.
  • Musculoskeletal pain not responding to rest, supportive care, modification of activity and exercise.

For a full list of referral guidelines, click here.  To contact Dr. Shulman, please call 714-509-8617. 

Learn more about rheumatology services at CHOC Children’s.

 

Dr. Andrew Shulman Addresses Pain Amplification Syndrome

Pain amplification syndrome is a condition where a patient’s sensitivity to pain is abnormal, Dr. Andrew Shulman, a pediatric rheumatologist at CHOC Children’s, tells “American Health Journal.”

This problem is more common in older school-age children and adolescents, Dr. Shulman says. As much as 15 to 20 percent of these children have experienced significant musculoskeletal pain, he adds.

Learn more about pain amplification syndrome in “American Health Journal,” a television program that airs on PBS and other national network affiliates that reach more than 30 million households.

Each 30-minute episode features six segments with a diverse range of medical specialists discussing a full spectrum of health topics. For more information, visit www.discoverhealth.tv.

Andrew Shulman, M.D., Ph.D., received medical scientist training at UT Southwestern in Dallas. He served as an intern and resident in the combined residency program of Children’s Hospital Boston and Boston Medical Center. He was a pediatric rheumatology fellow in the Children’s Hospital Boston division of allergy, immunology and rheumatology.

Get more information about referring patients to CHOC, including referral information directory, services directory and referral guidelines.