For most of us, when we hear the word “arthritis,” we tend to think of frail senior citizens hunched over canes. But that depiction relates only to osteoarthritis, the degenerative condition caused by a wearing out of the cushioning layer between a person’s joints, usually as a result of advanced age or, in the case of professional athletes, overuse.
And while 92 percent of the 50 million Americans suffering with arthritis do have the osteo-variety, there is another form of the illness with an entirely different cause and a much younger victim.
Four years ago, Mindy Gaemi’s daughter began experiencing extreme stiffness and pain in her hips and lower limbs that soon extended to her knees, lower back and neck. Gaemi knew something was amiss (“When a teenager can’t bend over enough to put on their own shoes or tilt their head up enough to see the ceiling, it is evident that something is wrong,” she says), but repeated doctor visits offered no solution. The Gaemis tried everything, including physical therapy, pain relievers, muscle relaxers and chiropractic treatments. But it wasn’t until Gaemi requested an appointment with an expert in pediatric rheumatology at the University of California, San Francisco Medical Center that Gaemi and her daughter finally received some news they could use.
At 16, Gaemi’s teen daughter was diagnosed with ERA, short for enthesitis-related arthritis, which fits under the larger umbrella of juvenile idiopathic arthritis (JIA). As in Gaemi’s daughter’s case, JIA is often marked by swollen, painful joints and immobility, but unlike osteoarthritis, it is caused by a malfunctioning of the immune system that causes the body to attack healthy tissue as if it were a virus, bacteria or other foreign invader.
Considering that only 8 percent of arthritis sufferers fall into the JIA set would likely cause most people to write off the disease as a rare anomaly, but that statistic doesn’t tell the full story. According to Joel Rothman, Chair of the Juvenile Arthritis Association (and father of a seven-year-old JIA patient), the 300,000 children diagnosed with JIA each year is twice the number of kids diagnosed with juvenile diabetes and more children than are diagnosed with juvenile diabetes, muscular dystrophy and cystic fibrosis combined.
As a parent, here’s what you need to know about this debilitating condition:
Early diagnosis is critical.
It took four years for Gaemi’s daughter to receive a diagnosis, and after another month of wrestling with the pharmacy and insurance company for medication authorization, she finally was able to begin treatment. But Gaemi’s mother does have some concerns: “We suspect her growth may have slowed down, and she’s certainly missed out on a lot of physical activities that were difficult or impossible for her to do,” she says, adding that if she could go back in time, she would have “pushed sooner and harder to see a specialist.”
Though Gaemi’s daughter appears to be on the road to health, Rothman warns that permanent damage can result if diagnosis or treatment is delayed too long. Because JIA attacks children, it is particularly dangerous because the child’s bones and joints are still growing. “If there is unchecked inflammation present during this growth, the joints will develop incorrectly,” says Rothman. “This can lead to permanent loss of mobility in the affected joints. If the joints are in the legs, this can mean that the child is confined to a wheelchair or must walk with a cane. If it’s in the hands or arms, it can mean inability to hold things or do tasks with hands. I know a girl who has had both hips replaced by the age of 12.”
But even the most dire consequences in those cases pale in comparison to the threat that accompanies systemic arthritis, which can affect the organs as well as the joints. “In these cases,” Rothman says, “death is a potential outcome if treatment is delayed too long.”
Eye health must be closely monitored.
Though seemingly unrelated, the same genetic predisposition that triggers attacks against the joints of JIA patients can also cause ocular inflammation, also known as uveitis, says Dr. Benjamin Ticho, M.D., a pediatric ophthalmologist at The Eye Specialists Center in Chicago Ridge, Illinois. For this reason, JIA treatment often involves regular eye screenings, usually performed every three months for those at high risk (typically young girls).
“Frequently, [uveitis] results in red eyes, light sensitivity, blurred vision and asymmetric or irregularly shaped pupils,” explains Ticho. “Occasionally, however, there may be no outward signs of the disease, and the child may not complain of eye problems. This asymptomatic uveitis can cause irreversible damage before it is detected, including both cataracts and glaucoma. Fortunately, most JIA uveitis can be successfully treated with eyedrops, but the diagnosis needs to be made before the damage goes too far.”
Treatment must be constantly evaluated and/or adjusted.
Unfortunately, unlike with some childhood conditions that tend to disappear or minimize with age, JIA must be treated for a lifetime. And while available options are highly effective, there is some evidence that specific treatments may lose efficacy over time.
“In general, a combination therapy of a biologic agent, like Enbrel and Methotrexate (a chemotherapy agent used in low-doses for rheumatology patients) proves effective for the majority of patients,” says Rothman. “But it is not uncommon for a particular biologic medication to work wonderfully for some period of time and then stop working altogether, necessitating a change to a different one. As there are only a limited number of these medications, it can be difficult for some patients to find a medication regimen that works consistently over a long period of time.”
Certainly, the facts related to JIA can be sobering, but the good news is that help is available and organizations like the Juvenile Arthritis Association provide support and assistance for families impacted by the condition. Most important, though, is the power that each parent has to ensure that their child is diagnosed and treated in a timely fashion, should JIA strike.
Adds Rothman, “Parents often instinctively know when something is wrong with their child. If a parent has reason to believe that their child has a health issue, they should not stop until they get a diagnosis and a treatment plan. Sometimes, this can be difficult, but a parent must always be their child’s best advocate.”