15 Questions – Pediatric Lupus
(October 2011) While lupus most often affects women in their childbearing years, it can occur in either gender at any age, including children and teens. This month, the LFA invites you to join us for the "Pediatric Lupus" Q&A with guest expert Dr. Norman Ilowite. This is your opportunity to ask questions and learn from an expert.
1. Does pediatric lupus potentially take a more aggressive course?
There is some evidence that pediatric lupus takes a more aggressive course. Disease activity at presentation and throughout the course of the disease is higher in children than in adults. There is a higher degree of kidney involvement in children as compared to adults as well. Therefore, children are more likely to be treated with immunosuppressive therapies. Because children have the disease for longer periods of time than adults, rheumatologists need to attend to preventing damage from the disease or its therapy including premature atherosclerosis and osteoporosis.
2. Are there any special tests that need to be done to diagnosis lupus in a child?
There are no special blood tests needed to be done to diagnose a child with lupus. The tests are essentially identical to those used to diagnose adults.
3. How does the treatment of lupus in children differ than in adults with lupus?
Because sometimes the disease is more aggressive in children, pediatric rheumatologists are more aggressive in the treatment of the disease. We use innovative ways of delivering corticosteroid therapy while minimizing toxicity, and we more often use potent immunosuppressives. Additionally, we pay a lot of attention to preventing premature atherosclerosis. We also pay a lot of attention to insuring normal growth and development. Growth, especially in patients who are on chronic steroid therapy should be monitored carefully and if patients are not growing normally they can be referred to endocrinologists for possible hormonal therapy. Bone health is also very important in children with lupus. Bone density measurements need to be performed and if patients are osteopenic or osteoporotic, supplemental vitamin D, calcium and sometimes bisphosphonates are given.
4. My son was 16 when he was diagnosed with Lupus and Marfan's. He is now 17. I myself have lupus. How common is it for teens to develop more than one autoimmune disease, and what can I expect? I have a hard time knowing what to pay attention to for myself, with him I have no idea and it scares me.
Marfan’s disease is a genetic disease and not an autoimmune disease. Certainly, every child or adolescent’s lupus is different and you should speak to the geneticist who takes care of your son’s Marfan’s syndrome and your rheumatologist about what to expect, and what can be done.
5. My daughter Sofia had a biopsy aged 3 and was diagnosed with Discoid lupus. She is using at present a skin steroid (elecon) and Protopic for her lesions (primarily on her face). Her iron levels remain low and due to less exposure to light, she is tested for Vitamin D every 3 months. Her consultant would like us to consider oral steroids or cortisones - both of which we've refused. My husband & I feel that these drugs will do more damage in the long run and her condition seems to be improving not worsening. There isn't much info that I can find online to justify the need to provide oral steroids / cortisones at this stage, but I also don't want my daughter to suffer due to my medical ignorance. Can you advice, or point us towards additional literature to help us understand more.
Certainly I have prescribed corticosteroids in some patients with severe discoid lupus, but for most patients antimalarials are sufficient (hydroxychloroquine or chloroquine). You should speak to your dermatologist or rheumatologist as to whether lenalidomide might be useful if your daughter is not controlled on antimalarials.
6. At what age do you check for lupus if there is a history of lupus in the family and a child is showing indications of possible symptoms?
I don’t routinely “check” for lupus by doing laboratory tests in well children with a family history of lupus. Your pediatrician should go over the symptoms of lupus with you so you can bring the child to medical attention if they occur.
7. My 17 year old daughter was diagnosed with connective tissue disease and Hashimoto's. We noticed a behavior change in her before we found out she was sick. She went from a wonderful child to a nightmare. The Hashimoto's is not active and connective tissue disease involves lupus and Raynaud’s. They don't think the lupus has affected her brain. Should we be looking for anything special?
Seventeen year old adolescents can have any number of reasons for behavior change from their diseases, or from going through adolescence, per se. It is difficult for them to accept the fact that they don’t feel well, as they often consider themselves invulnerable. Your daughter may be having difficulty adjusting to the idea of being ill and may need to talk to someone about these feelings. If you feel your child is having difficulty adjusting to her diagnosis you may want to speak to the rheumatology team about available support systems such as support groups, counseling, etc. Certainly high or low levels of thyroid hormone can also contribute, but you say your daughter’s disease is inactive. I would speak to your doctors about all of these possibilities.
8. What are the statistics for a 4 yr old boy having Lupus? I understand it can affect anyone but I can't find specific information with regards to our possible diagnosis.
The younger you are when you get lupus, the more likely you are to be a boy. In adults the ratio is 9:1 (females to males), in children 4:1. The ratio approaches 1:1 in very young children under 2. Unfortunately, young boys who have lupus seem to get significant internal organ disease more than older girls. Response to treatment is usually good, however.
9. My 8 year old daughter has SLE. What are the chances of sisters being diagnosed?
The risk of a sibling of someone with lupus getting lupus is about 29 times higher than in the “normal” population. The concordance rate in identical twins is between 24-58%; in fraternal twins or siblings it is 2-5%. So, although the risk is higher in siblings of lupus patients than in siblings without lupus in their family, the overall risk is still very low.
10. Are there certain things that may cause a flare in children? And other than medications what can we do to help keep her from having a flare up?
Sun protection is probably the most important thing you can do. Exposure to the sun and other sources of ultraviolet light not only makes the skin disease of lupus worse but also the internal organ disease. White blood cells behave “badly” when exposed to UV light in the skin making systemic disease worse. Additionally, as with any chronic illness, living a healthy lifestyle including low fat diets, exercise and stress reduction, go a long way in promoting overall health and well being.
11. How does puberty affect children with lupus and can medications affect puberty?
Onset and progression of puberty can be delayed in children with lupus. Growth failure also occurs during this time. The delays in puberty and growth are both related to the underlying disease, especially if it is poorly controlled, and the some of the treatments – especially with corticosteroids. There is some evidence that certain hormones which are secreted in high amounts during puberty can cause the first appearance of or exacerbate existing lupus. This is an especially challenging time for the patient, family and health care providers.
12. My 14 year old son has been on steroids for 3 years now. Should I be talking to his doctor about a bone density study?
All children who are on steroids chronically should have a DEXA (bone density study) at baseline (when the diagnosis is made) and yearly to see changes over time as well as to monitor response to therapy.
13. Is the HPV vaccine safe for young girls with lupus?
The HPV vaccine is not a live virus vaccine so it is safe for girls with lupus. If the disease is very active or if the patient on immunosuppressive therapy, the patient may not respond with a vigorous enough immune response, so the vaccine may be less effective, but this doesn’t influence safety of the vaccine, as far as we know.
14. My 16 year old daughter was diagnosed with lupus this past year. She was very active with soccer. Her doctor has told us to keep her out of the sun. How can she continue to participate in sports without causing problems with her lupus?
My philosophy is to try to not interfere with productive activities outdoors if the patient really enjoys them. Ideally she should avoid being in the sun from 10AM to 3PM, wear a hat with a wide brim when possible, and seek the shade when she can, whether it be on the soccer field, the beach, or ski slope. It is key to use a sunscreen that blocks UVA and UVB rays and I usually recommend SPF 45 or higher. Oftentimes the above cannot be achieved. We have to weigh the benefits of exercise in the sun against the risks, and I come down on allowing the patient to participate in sports. If patients continue to have a photosensitive rash despite the sunscreen, I advise them that whatever they are doing is not enough, and we need to intensify UV protection, such as reapplying more often, wider brim hat, etc. Usually patients are able to fully participate in the sports they enjoy.
15. What information should I give my daughter’s school about her systemic lupus? Are there any special precautions the school should take with my child?
It depends on her lupus, as well as you and your child’s personal preferences. In general we encourage full participation in school activities including physical education. For most patients we advise informing the schools that the student has SLE, that she should be excused from any activity she finds too painful or strenuous, and that she should be provided the opportunity to seek shade when necessary. We warn the school that fever or significant change in behavior may represent a flare or serious infection and the rheumatologist or primary doctor should be contacted immediately. If there is significant arthritis, a duplicate set of books is requested to minimize the burden of carrying them back and forth to school. Rarely, elevator passes need to be obtained. Sometimes patients attend school most of the day and you may need the nurse’s help in administering medication. There is a lot more that can be done if the student is not performing well in class or not functioning well socially. Indeed, if the disease or its treatment is interfering with learning, the school is required to develop an individualized education plan that addresses the relevant issues. If she has mild lupus that is well controlled on medications that are not immunosuppressive, you don’t need to notify the school, if you don’t want to, in my opinion, although school personnel are required to maintain your daughter’s privacy, so you shouldn’t worry about that too much.