“Hey, ladies – I’m here to get poked,” says five-year-old Sam Lincoln of Remington, Va., greeting nurses at the Lombardi Center for Children at Georgetown University Hospital. Sam goes there for monthly infliximab (Remicade) infusions to treat his juvenile rheumatoid arthritis (JRA), which he’s been battling since age one.

Sam’s had a rough go of it. He’s faced four years of inflammation and pain. He’s never known remission. His wrists are usually swollen and some of his fingers and toes are curled inward where the disease has damaged his joints. He walks with visible stiffness, sometimes throwing his legs out to the side and then forward to move. He frequently runs fevers and he’s covered in rashes daily. Steroid treatments have kept him the same size for about three years now. “Sam only weighs about 33 pounds,” says his mother, Christina Lincoln. “He hasn’t gained any weight since he was two.”

Lucky for Sam, he’s got the unwavering love and support of a close-knit family – his mom and dad, his grandparents, aunts, uncles, cousins and a younger brother. Lucky for the Lincolns, they have this inspiring little man, as they affectionately call him, in their lives.

“He’s an amazing kid,” says Christina. “From the beginning, he just adapted. When he couldn’t crawl, he would use his elbows to move. When that didn’t work, he’d scoot around on his bottom. There’s never been a time through all of this that Sam has lain in bed all day. He always gets up. He always gets dressed. And he always has a big smile,” she says.

Learning to Walk Again and Again

The onset of Sam’s arthritis was quick and chaotic. Around his first birthday, Sam developed a rash on his left arm at the site of a recent vaccination. “It was red and raised about the size of a quarter – it was very angry looking,” says Christina. Persistent high fevers prompted Christina and her husband Travis to take Sam to the emergency room.

For the next couple of weeks, Sam experienced symptoms every afternoon. “The rash flared and his fever spiked to 104 degrees, but by the next morning he was fine,” recalls Christina. “Sam was seen by numerous doctors, including an infectious disease team. After 12 days, everything was ruled out except for rheumatoid arthritis,” she says. 

Because there are so few pediatric rheumatologists in this country, most children have to travel long distances for their diagnosis and care, but the Lincolns were fortunate. One of the sub-specialists worked nearby, and Sam was soon diagnosed with systemic-onset JRA. 

Although JRA is the most prevalent kind of childhood arthritis – some 30,000 to 50,000 kids in the United States are affected – only 10 percent of this population has systemic-onset juvenile rheumatoid arthritis, or Still’s disease. This sub-type disturbs many bodily systems and can be more severe than more common forms of JRA. In addition to inflamed joints, it can cause enlargement of the spleen and lymph nodes; inflammation of the liver, heart and surrounding tissues; and anemia.

“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.”

If Sam gets the vein port, he no longer has to endure countless needle sticks for an IV or a shot. But if Sam runs a fever, he’ll need testing to determine whether an infection got into his blood through the port. So far the Lincolns have decided against it.

Like an Old Soul

Sam’s grandmother Jeanette, who until recently lived a few doors down from the Lincolns and served as Sam’s day-care provider, tells heart-warming stories about her grandson’s spirit.

Jeanette says she and Sam recently went to a baseball game to watch his Uncle Jeff play. “There were a bunch of children running around as we were walking along. A few kids ran by him and Sammy grabbed my hand, looked up at me and said, ‘Nanny, one of these days, I’m going to run just like those kids.’”

Another time, one of her friends visited while she was babysitting Sam. “She sat down with me at the kitchen table and Sammy climbed into her lap. She had just had some tests run, and so she had band-aids in the crook of her elbow from having blood drawn. Sam saw them, but he didn’t say a word to her,” says Jeanette. “He just reached out for my friend and rubbed the back of her hand with his own. She looked at me with tears in her eyes and I said, ‘He understands what you’ve been through.’ He didn’t have to say a word, he just held her hand. He was probably three years old at the time.”

“Sam’s like a little man,” says Christina. “He has such insight. He’s like an old soul. He just gets it, and he has from the beginning. There’s never a day that goes by that Sam doesn’t strive to accomplish something, whether it’s walking up the stairs rather than crawling up them, or climbing up on something.”

A few weeks ago, Sam walked across his grandmother’s yard. “For the rest of the kids [it’s easy], but for Sam it was a big deal,” says Jeanette. “The ground is uneven. There are rocks and sticks to climb over. He said, ‘Nanny, this is going to be hard work.’ And I said, ‘Yes, it is, but you can hold my hand and we can do it together.’”

“I wouldn’t change anything about my son,” says Christina. “I wish he wasn’t in pain every day, but I have to take comfort in knowing there’s a greater plan for him. All of this is teaching him something or it’s molding him into the man he’s going to become. “Sure I hope there’s a cure for arthritis down the road. But right now I’m just thankful that I had yesterday with Sam, and that I get today and tomorrow with him,” she says. “He’s a gift.”