“Within a month, things snowballed,” says Christina. “Sam had many symptoms, including difficulty breathing. He made a strange grunting sound when he took in air.” That’s when the doctor noticed that Sam had some inflammation in the lining surrounding his heart – a condition called pericarditis. Because there’s no test for pericarditis, the doctors had to first rule out cancer before administering steroids to reduce the inflammation. “After his biopsy came back negative, Sam started heavy doses of steroids,” Christina says.

Barely six months later – when Sam was about a year-and-a-half old and had just learned to walk – he had to be hospitalized again, this time for pneumonia. “Sam wasn’t responding to the antibiotics and [his health-care team] began to suspect a condition called pneumocystis, which is a lung infection that can affect those with suppressed immune systems. It’s very hard to treat,” says Christina. 

While Sam endured a three-week-long hospital stint, his muscles atrophied and he lost the ability to walk. With physical therapy, Sam learned to walk a second time. “But then he and his dad took a little tumble down the stairs,” says Christina. Travis was carrying Sam, and he missed a couple steps, she explains. Her husband fell back on the stairs to stop himself. “It was such a minor fall,” she says. “Sam’s left leg got wedged between Travis’s back and a step. He limped a little afterward, but his leg didn’t swell.” But because Sam’s bones were so brittle from steroids, the slight fall had actually fractured Sam’s fibula and tibia. “Sam was in a cast for six weeks and, needless to say, he stopped walking again.” For a third time, Sam had to relearn to walk.

Inevitable ‘Second Guessing’

Christina frets over whether she and Travis are making the right decisions regarding Sam’s care. “Everything comes with a price,” says Christina, referring to the medicine Sam takes. The Lincolns know all too well the toll steroids can take on a child’s growth and bone strength. Potential side effects from biologic agents alarm them, too. “The Remicade comes with an increased risk of tumors and other illnesses,” she says. 

What’s more, Sam’s daily naproxen can cause stomach complications among other problems, and, if continued long-term, his weekly dose of methotrexate may affect his liver. Even the iron supplements Sam must take constipate him, making daily laxatives necessary.

Causing further angst for the Lincolns and other parents of kids with juvenile rheumatoid arthritis, most of the available treatments are not officially indicated for use in children. Because cases of juvenile arthritis are limited compared with cases of adult rheumatoid arthritis, drug companies usually haven’t been willing to fund the studies necessary to get their medicines FDA-approved for children. Now, however, every new or existing drug that might be given to children must be tested in children, thanks to the Pediatric Research Equity Act of 2003. And if the drug is approved specifically for pediatric use, child-size doses must be included in the prescribing information.

Early on, Sam took the biologic agent etanercept (Enbrel), which is one of the few arthritis treatments approved for children with JRA. However, it stopped working for Sam after a period of time. That’s when he switched to Remicade, which is being tested in children, but has not received FDA approval.

Another tough decision the Lincolns have contemplated is whether to allow Sam’s health care team to insert a port into one of his veins. “Sam’s veins have been tapped into so many times that they’re very thin,” explains Christina. “I’ve had to hold him down when [caregivers] have attempted eight times to start an IV. He’ll ask, ‘How many more times, Mommy?’ And I’ll say, ‘one more time, one more time.’ It breaks my heart.”