Once called juvenile rheumatoid arthritis, JIA is the umbrella term used today to describe the most common form of juvenile arthritis. JIA has several subtypes.
JIA is a not the “child version” of adult rheumatoid arthritis (RA). The disease, particularly in young children, is often missed by pediatricians and family physicians. JIA differs “in the way the joint damage is happening; the prognosis is different; the diagnosis criteria are different, as well,” said Dr. Stacey Tarvin, a pediatric rheumatologist at Riley Hospital for Children in Indianapolis, affiliated with IU Health. Tarvin also teaches at the IU School of Medicine in Indianapolis and is Aydra's rheumatologist.
“The earlier you make the diagnosis, the more likely you are to have a good outcome and the more likely you are to go into remission,” Tarvin said. But just six pediatric rheumatologists, all of them at Riley, practice in Indiana, which adds to the challenge of informing and educating primary care doctors and for ensuring specialty care is available.
It took the Anthonys months to get an accurate diagnosis, and they hope other families will learn from their experience to make note of even small changes in mobility, flexibility and other symptoms that can include unexplained fevers or a rash.
Abbey Anthony thought her daughter's gait was a little abnormal when she first started walking, and recalled, “At night, when I'd go to put on her PJs, I would have to bend one leg to get it in (the zip-up sleeper) and she would cry out like something was hurting her. She would sometimes limp when she walked, so I took her to the pediatrician. He checked her and said she was fine.”
Aydra had numerous ear infections, and at various check-ups Anthony mentioned the periodic symptoms she had never seen in Aydra's older sister.
But as often happens, at doctor's visits Aydra walked fine. Then one day, when Aydra was about 17 months, Anthony lifted her off her lap to stand her on the floor.
“Her leg gave out, and she fell down and started crying. I told my husband, 'That's it. We're going to take her to another doctor and figure out what's wrong.' ”
An appointment was made with an orthopedist. X-rays and an ultrasound were normal. Anthony wisely videotaped Aydra at home to show the doctor one of her limping spells. Blood tests were ordered. When the nurse called to say something was abnormal and more blood tests were needed, “I was freaking out,” she said. When the doctor referred Aydra to a rheumatologist, some relief was felt. Still, unanswered questions remained.
Answers finally came after a two-hour exam at Riley. The rheumatologist noted Aydra had swelling in her right knee and the first toe of her left foot. With just one to four joints affected, her subtype of JIA is called oligoarthritis; 40 percent of all new JIA cases are oligoarthritis. JIA diagnostic criteria also include onset of symptoms before age 16 and symptoms lasting at least six weeks.
When the rheumatologist showed Anthony her daughter's “sausage toe,” Anthony said, “How could I not have noticed that?” The doctor reassured her it is common for parents – and many physicians – to fail to notice such symptoms, in part because symptoms are most often worse in the morning or after naptime and improve as the day goes on. Also, kids have a way of compensating for such physical changes, so busy parents and doctors may overlook them. A rheumatologist is trained to detect even slight swelling or warmth in a joint and notice even a hint of a limp.
Inflammatory markers that are present in the blood of adults with arthritis are rarely seen in children. Thus the physical exam is crucial to diagnosis.
JIA can also affect the eyes. A positive ANA, or antinuclear antibody, test presents increased risk of eye inflammation due to the arthritis. Untreated, prolonged inflammation can lead to blindness.
Aydra's ANA is high so every three months she has an eye exam by an ophthalmologist and also uses eye drops. On the up side of things, she no longer must take the non-steroidal anti-inflammatory drug she once took twice day.
Today's drug options are “increasing the likelihood of having very good outcomes,” Tarvin said, but noted, “We can do a better job of educating pediatricians and family practitioners to get early diagnosis.”
Tarvin comes quarterly to Lutheran Children's Hospital for an outpatient pediatric rheumatology clinic. Recently a colleague joined her due to the number of children in the region needing care.
“We've made great strides in treating these kids,” Tarvin said. “It's night and day, compared to what arthritis treatment was 20-25 years ago.”
For Aydra, that's something to dance about!