You know the importance of following your doctor’s guidelines about medication, exercise, and rest; reducing stress; and eating a healthy diet. But if you’re like many people with lupus, at one time or another you’ve come across a dietary or herbal supplement that really sounds as though it could help you. So, what do you do?
Safeguarding Our Youth:
In the 1950s and ’60s, the long-term prognosis for people with lupus was not encouraging. The disease was under-diagnosed and misdiagnosed, and research was underfunded. Children, in particular, did not fare well. Delays in diagnosis led to organ damage. Doctors were reluctant to use medications and treatment regimens developed for adults, and the long-term effects of lupus and its treatments were not well understood. But in 2010, children with lupus have a much better prognosis, both short- and long-term, thanks to the persistence and ongoing commitment of the lupus community.
Bringing Down the Barriers
For more than 30 years, the Lupus Foundation of America (LFA) has supported the work of pediatric rheumatologists who are unlocking the mysteries of childhood lupus. In 2006, the LFA was granted $1 million from the Wallace H. Coulter Foundation in memory of Michael Jon Barlin, who lost his battle with lupus at age 24. The LFA used a portion of these funds to launch a pediatric lupus research program. In doing so, the LFA became the first patient advocacy organization to establish a research program dedicated specifically to the effects of lupus on children and adolescents.
Today, the Michael Jon Barlin Pediatric Lupus Research Program has become a mainstay in the LFA research portfolio as part of its prestigious national research program, Bringing Down the Barriers. In recent years, LFA’s research program has funded an increasing number of grants for pediatric lupus research.
Federal Recognition of Need for Pediatric Lupus Research
Statistics show that 15 percent to 20 percent of those with lupus are diagnosed before age 18. In the majority of these young people, lupus begins around puberty, between ages 12 and 15. As with adults, the vast majority of children affected are female. Girls are diagnosed four times as often as boys if the disease begins before age 10 and nine times more frequently when the disease starts after puberty. The signs and symptoms of lupus in childhood and adolescence are virtually identical to those of lupus in adults. Also, as with adults, the disease course is variable, with flare-ups and periods of relative disease inactivity.
In 2007, the National Institutes of Health (NIH) released the report “The Future Directions of Lupus Research.” A section dedicated to pediatric lupus states, “Because of their disease severity and young age, lupus is a particular burden for children. Thus, any research that leads to improved understanding, treatment, and quality of life in children has significant, lifelong implications.”
The report cites research highlights, which include:
- Recognition of the role that type I interferon signature and abnormal myeloid cells play in active pediatric lupus.
- The establishment of the Childhood Arthritis and Rheumatology Research Alliance (CARRA), a national network of clinician-researchers who are studying and treating young people with lupus and other childhood rheumatic diseases.
- The establishment of the APPLE trial (Atherosclerosis Prevention in Pediatric Lupus Erythematosus), in which statins—drugs used to lower low-density lipoprotein (LDL, or “bad” cholesterol levels)—are being tested for their ability to lower fat buildup (atherosclerosis) in the blood vessels of children with lupus.
A more recent CARRA effort has aimed to develop “best-care protocols” for physicians caring for children with class III/IV lupus nephritis. The LFA staff participated in an advisory capacity, providing input on quality-of-life issues from both the parents’ and children’s point of view.
Diagnosis, Treatments, and Side Effects
The time it takes to diagnose lupus in children is one of the most pressing issues in caring for children with lupus, says Thomas J. A. Lehman, M.D., professor of clinical pediatrics at Weill Cornell Medical Center in New York, NY, and a member of the LFA’s Medical-Scientific Advisory Council. “Too many children are allowed to have lupus for too long before their disease is recognized and appropriate referral is made,” he says.
Another concern is that the medications used to treat symptoms of lupus were developed for adults. “Pediatric rheumatologists have traditionally had to rely on research conducted in adults to make tough decisions about treatment for children with lupus. However, we don’t know whether the same drugs will work the same or as effectively in children as in adults,” says Norman T. Ilowite, M.D., professor of pediatrics at Albert Einstein College of Medicine in Bronx, NY.
At the same time, the multifaceted nature of lupus means no single treatment regimen is likely to be universally effective in children and adolescents.
Deborah K. McCurdy, M.D., professor of pediatrics at the University of California-Los Angeles, believes the future is brighter now for lupus physicians and patients, thanks in part to the development of medications that do not suppress the immune system, but instead interrupt the mechanisms of disease. “With the more advanced understanding of the immune system and the advent of biologic agents, pediatric rheumatologists of the 21st century will likely begin to treat children with powerful, but less toxic agents,” she says. McCurdy’s pediatric lupus research has been funded by the LFA for the past two years.
Still, knowing which medications will work best will not ensure that a young person with lupus will be able to successfully live with a chronic illness. “More children with lupus do poorly today because they do not follow through with their treatment regimen appropriately, rather than because we lack an appropriate regimen for them to follow,” cautions Lehman.
Growing Up With Lupus
Emily von Scheven, M.D., professor of clinical pediatrics at the University of California, San Francisco (UCSF) Medical Center, directs the multidisciplinary Lupus and Antiphospholipid Syndrome clinics at UCSF, where physicians, nurses, social workers, nutritionists, and physical therapists offer care to young people up to age 21. Many of their patients participate in clinical research studies investigating compliance and disease management strategies.
Critical issues include learning to balance work and/or school with the limitations imposed by active lupus, recognizing the warning signs of a lupus flare, and continuing to take prescribed medications even when feeling well. Von Scheven recently received an LFA grant for work in this area.
Von Scheven believes the transition period for young people—from a pediatric clinic setting in which the parents and a team of health care providers are very involved, to health care in an adult clinic, in which all decisions are left up to the patient—must be carefully managed. “A combination of patient education providing short- and long-term strategies for disease management, and adult supervision to help adolescents stay on track, can help ensure treatment regimens are followed,” she says. “It is essential that young adults are prepared.”
Less toxic medications; treatment regimens specifically for children; improved time to diagnosis; counter-measures to ensure better long-term outcomes; strategies to help young people help themselves—these are some of the many ways research is improving the lives of children and adolescents with lupus. The LFA looks forward to expanding its pediatric research grant program through Bringing Down the Barriers and to continuing to provide support and hope to children with lupus and those who care for them. You can help! Go to lupus.org and click on “Research” to find out how your contribution can make a difference in the lives of children and adolescents with lupus.
LFA-Funded Research in Pediatric Lupus
Over the years, the Lupus Foundation of America has funded many individuals seeking answers to the complexities of lupus in children and young adults. These are some of the projects funded over the past 10 years:
Marisa Klein-Gitelman, M.D., M.P.H., Children’s Memorial Hospital, Chicago
Analysis of neurocognitive function in children with SLE
M. Virginia Pascual, M.D., University of Texas, Southwestern Medical Center at Dallas
B lymphocytes in pediatric SLE
Emily von Scheven, M.D., University of California, San Francisco
Improving transition readiness and quality of life with a pediatric health passport
(The pediatric passport is an adaptation of the adult version, which was developed by Paul R. Fortin, M.D., M.P.H., senior scientist at Toronto Western Hospital.)
Deborah K. McCurdy, M.D., UCLA David Geffen School of Medicine, Los Angeles
Pathways of autoimmunity linking polymorphisms in genes regulating the IFN-a pathway, IFN-a induced gene expression, and regulatory T-cell expression of FoxP3 in pediatric/adolescent lupus
Joseph Ahearn, M.D., University of Pittsburgh, Pittsburgh
Biomarkers for diagnosis, monitoring and prognosis in pediatric lupus
Hermine I. Brunner, M.D., Cincinnati Children’s Hospital Medical Center, Cincinnati
Long-term outcomes and gonadal function in childhood-onset lupus
Anne Stevens, M.D., Children’s Hospital and Regional Medical Center, Seattle
PDLI as a marker of disease activity in pediatric lupus
Eyal Muscal, M.D., Texas Children’s Hospital, Baylor College of Medicine, Houston
Cognitive impairment and white matter changes in pediatric lupus
In addition to medications and other medical care from doctors, a large and growing number of people turn to other healing practices to try to improve their health.