From the Archives: Summer 2004 Lupus Now magazine
LUPUS AT A YOUNG AGE: A child’s needs and concerns are different and the same
by Nancy Duncan
He’s a published author and a Phillies fan. His favorite food is popcorn. He lives in a small town in Pennsylvania with his parents, a brother and a sister, and two black-and-white cats named Rex and Rascal. He plays baseball, basketball, hockey, football and lacrosse and has lots of friends. Yes, Scott Exler is really just a regular 12-year-old boy, except that he was diagnosed with lupus when he was 8.
"When I was little, I seemed to be just fine," he writes in his book, Loopy Lupus Helps Tell Scott’s Story. "But one day I came home from school and everything was different. My knees and ankles hurt. I had trouble running and playing with my friends. I was tired and couldn’t do my homework because I just didn’t feel well. I had a fever and I ached all over. Mom took me to the doctor’s office. She and the doctors knew right away that I was sick, but they didn’t know what was wrong until they did some special tests."
Lupus affects children in much the same way it affects adults, although some researchers think that more organ systems are involved in pediatric lupus. Roughly 5,000 to 10,000 kids under 18 in the United States have lupus, according to Thomas J.A. Lehman, M.D., chief of pediatric rheumatology at the Hospital for Special Surgery in New York City, but he notes that precise figures are hard to come by. Most kids are diagnosed between the ages of 11 and 15, but lupus can strike younger children too. Although 90 percent are girls, Lehman says it’s important to remember that pediatric lupus does occur in boys and is often overlooked. And, like lupus in adults, pediatric lupus cuts across racial and ethnic lines.
Because lupus can express itself so differently in each person -- and kids are no exception -- treatment can vary greatly. "Children with mild lupus often take only steroids and Plaquenil, an antimalarial drug, in the hope that they may not need very much for very long," Lehman explains. "Children with moderate lupus may be offered the same regimen, but they often require the steroids in higher doses. If they require a significant dose of steroids, they may be offered immunosuppressives as well, which allow us to minimize the steroids."
In addition to steroids, which have anti-inflammatory and immunoregulatory properties, treatment can include other anti-inflammatory drugs, such as aspirin, ibuprofen or naproxen.
Some children have obvious disease symptoms: fever, the facial "butterfly" rash, kidney involvement. “The most common symptoms of lupus in childhood are fatigue and achiness,” says Lehman, although he advises parents to look for signs beyond the butterfly rash. "Most cases of pediatric lupus are diagnosed when physicians remember to include an ANA blood test in the evaluation of a child who is not doing well or has unusual findings in the urine test." (Interestingly, although a positive ANA is generally required to make a lupus diagnosis, many children with positive ANAs do not have lupus.)
Knowledge and Patience
On a warm spring day in Nashville, TN, Linda Davis was leaving for church with her three children when she noticed that one of her daughters was having problems walking. Tiffany, then 9, was moving slowly, stumbling as though her legs were simply not working.
"One day she could walk, open the car door, do all the normal things, and the next day she couldn’t," Davis remembers two decades later. "She’d try to open the refrigerator or turn on a sink faucet, but she was unable to use her hands."
Davis took Tiffany to the family doctor, who thought that she might have pulled a muscle during dance class or while playing. The pediatrician advised rest. It didn’t sound right to Davis, but she wasn’t sure what to do. The next day, the symptoms worsened. Baffled and scared, Davis took Tiffany to the emergency room. After a battery of tests -- including blood work, a kidney biopsy, urinalysis and a neurological exam -- she was misdiagnosed with Guillain-Barré syndrome, a common misdiagnosis in lupus cases. Finally, a week later, the diagnosis was corrected: Tiffany had pediatric lupus. Having never heard of lupus, Davis had no idea what the diagnosis would mean for her daughter and for the family. Tiffany began a regimen of prednisone and Cytoxan.
And then, a year later, Tiffany’s sister Shaunté was diagnosed wih lupus as well -- in her case, cutaneous lupus. Davis learned firsthand that every case of lupus is different: Tiffany has undergone two kidney transplants (the second organ was donated by her brother, Dewayne) and has had central nervous system involvement plus joint and kidney problems. Shaunté’s symptoms have included a severe facial rash and extreme sun-sensitivity.
Looking back, Davis says that knowledge and patience were the keys to surviving the double lupus gantlet. Knowledge gave her a better understanding of the disease, and patience helped her deal with unforeseen changes in her daughters’ lupus, endless rounds of doctors’ appointments and multiple medication side effects. "You have to have faith," Davis says. "And you can’t be nervous. The kids are nervous enough -- you have to be strong."
Wants vs. Needs
When a child is first diagnosed, parents are likely go through a period of mourning. This is entirely normal, says Robert H. Phillips, Ph.D., founder and director of the Center for Coping in Hicksville, NY. "Parents are grieving the loss of their child’s previous healthy state," he explains. On top of this, parents may have feelings of guilt, especially if they or another relative has lupus. "They may feel that they have ‘contributed’ to the diagnosis," Phillips says, even though heredity plays but a small role in lupus. Only 10 percent of adults diagnosed with lupus have a parent or sibling who has the disease or will develop it; a mere 5 percent of the children of people with lupus will develop the disease.
Naturally, children and parents will have very different concerns. Parents worry about the big issues -- medication side effects, the course of the disease, what will happen to their children after they themselves are gone. "I worry about everything, from how the disease has affected his ability to have close friends, to whether he’ll ever be able to hold a job with benefits, to the side effects of the Cytoxan he’s taking," says Karen Jackson of Overland Park, KS, whose son Casey, 15, was diagnosed when he was 11.
But Casey’s chief worry is getting outdoors to play his beloved sports. Andy Marshall, also 15, of Hopkins, MN, agrees. "The worst part of lupus is the sun factor," he says. "I can’t ski; play golf, tennis or baseball; or go fishing without putting on sunscreen. Plus I have to wear special SPF-65 sunscreen with zinc oxide and put on my SPF-rated swim shirt before I even go into the pool."
Another big anxiety for kids is appearance. "In middle school I was pudgy from the prednisone," says Tiffany Davis. "It made me hungry all the time, and I gained a lot of weight." Although her dosage was later reduced and she lost the weight, the social stigma is still vivid. "Kids asked if I was taking steroids, but I didn’t want to tell anyone about lupus. Some people even thought lupus was contagious."
When Jaclyn Law of Toronto, Ontario, Canada, was diagnosed 11 years ago at age 13, there was a shortage of lupus information aimed at children and addressing their particular worries. "A book written for a middle-aged woman," she says, "is hardly going to answer a kid’s most urgent questions: Should you tell your boyfriend? How can I tell my teachers?" Law felt so strongly about the lack of support that she started a webzine called LGirl for young people who have lupus.
Many parents will feel a strong -- and understandable -- desire to protect their child. But be careful not to be overly protective, says Laura Schanberg, M.D., associate professor of pediatrics at Duke University Medical Center in Durham, N.C. Her research has shown that parents with the most fears and worries, regardless of the severity of their child’s illness, had the kids with the most social and emotional problems. Far better is simply offering the information, understanding and support your child needs.
"A parent who has a recently diagnosed child will probably be overwhelmed," says Betty Ann Exler, Scott’s mother. "We try the best we can to treat Scott as if none of this was a factor. We keep high expectations, play with him, give him chores and laugh with him."
Side Effects and Complications
As any parent of a child with lupus can tell you, prednisone -- one of the corticosteroids most commonly prescribed for lupus -- causes side effects ranging from weight gain and acne to thinning skin and easy bruising. And prolonged steroid use is even worse: It’s the major cause of treatment-related illness for children with lupus. Corticosteroids also cause stunted growth, Lehman notes, adding: "This is why I’m a major advocate of immunosuppressive medications, which permit a lower dose of steroids."
Since children in the Wonder Years still have lots of growing to do, it stands to reason that growth delay is a central issue for many parents and kids. Casey Jackson had grown less than an inch in two years when his rheumatologist referred him to an endocrine specialist to talk about growth hormone. "His bone age was 11, even though he was 15 years old,” says his mother, Karen. And although Casey doesn’t love the hormone treatment, he loves what it’s done for him -- he’s grown five inches. “He’s so excited," his mom exults.
But kids and parents should approach growth hormone treatment with care, cautions Christy Sandborg, M.D., associate professor of pediatrics at the Stanford School of Medicine in Stanford, CA. "The disease has to be under good control, and children need to be on low doses of steroids, or the growth hormone won’t work," she says. And, she adds, families should be aware that there is some anecdotal evidence that growth hormone can cause flares of autoimmune diseases.
The long-term use of steroids can also cause early-onset osteoporosis. In addition, "There may be other effects that are less well quantified," Sandborg says, "such as problems with tissue integrity, evidenced by more fragile skin and connective tissues."
Sandborg and Schanberg are in the early stages of studying a complication of increasing concern to lupus experts: heart disease in children with lupus erythematosus. Co-chairs of the Childhood Arthritis and Rheumatology Research Alliance, or CARRA, they are examining the use of cholesterol-lowering drugs to combat this type of heart disease; their drug trial includes 280 pediatric lupus patients at 20 sites throughout the United States and Canada.
Kids Just Want to Have Fun
But no matter what the side effects and the complications, the drug regimens and the flares, families should keep in mind that kids with lupus can have a normal childhood. After an initial adjustment period, many children adapt smoothly to life with lupus. Marissa Reyes, 11, of Merced, CA, was diagnosed last year, when she was 10. "I played soccer, but there was too much running. So now I’ve switched to basketball -- and I take lots of breaks. That way, my legs don’t get so tired," she explains matter-of-factly. "At first a kid might think, ‘Oh, poor me.’ But you can’t think that way -- you just have to think good thoughts."
And Tiffany Davis, now 29, is a long way from being that little girl who couldn’t open the refrigerator. She leads a full and busy life, working as a pharmacological research assistant at Vanderbilt University in Nashville, leading a lupus support group and thinking about going back to school for a degree in social work. "Lupus is just a new chapter in your life," she says, "with challenges you have to face -- that’s all. Sure, you have to keep an eye on the disease, but you also have to go ahead and live."
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