Q & A for Children & Young Adults with SLE

By Sandra Watcher, RN BSN, Laura Garcia, MSW, Katherine Marzan, MD, Bram Bernstein, MD, Bracha Shaham, MD, Wendy DeLaP ena, MD, Andreas Reiff, MD
Childrens Hospital Los Angeles, Division of Rheumatology
What is Systemic Lupus Erythematosus(SLE)? 
SLE is a disease in which your own immune system(defense system) attacks your body and creates inflammation in the skin, joints, blood vessels(veins and arteries) and internal organs.
What is my immune system? 
This is the part of your body that normally defends against infections, such as viruses and bacteria.  When you have SLE your defense system is over active and begins to attack your own body. 
What is inflammation? 
When you fall and scrape your knee, sometimes it becomes swollen, red and has fluid.  This is a type of inflammation similar to what happens in the blood vessels of your body when you have SLE, except that there is no fall, trauma or infection that causes it.  Blood vessels become dilated, white blood cells and other proteins of the immune system invade the area and cause damage.
How do doctors know I have SLE?
There are a number of symptoms of SLE. However, there are only 11 criteria that are used to diagnose SLE and you must have at least 4 that are not explained by other medical reasons.  These include: 
Malar Rash Sometime called a butterfly rash over the checks and nose
Discoid Rash Red/raised patches that may look like quarters 
Photosensitivity A rash brought on by and worsened by sunlight 
Oral ulcers Sores in the mouth or nose
Arthritis Swelling and pain in the joints 
Serositis Inflammation around the lung or heart (pleurtitis or pericarditis)
Renal Disease Shown by the amount of protein, blood, and/or casts in the urine and/or abnormal renal biopsy 
Neurologic Seizures and/or psychosis not explained by other medications or causes. 
Hematologic Hemolytic anemia(anemia from a breakdown of red blood cells), leukopenia(decreased white blood cells), lymphopenia(decrease in a specific type of white blood cell), or thrombocytopenia (decrease in platelets)
Antinuclear Antibody A positive test by IFA (immunoflorescent assay). 
Immunologic A positive anti-double stranded anti-DNA test, positive anti-Sm test, positive antiphospholipid antibody such as cardiolipin, or false positive syphilis test (VDRL) 
Why me? 
We do not know the specific cause of SLE.  We believe it is a combination of multiple factors.  These include the environment(virus, sun, drug reactions), hormones(puberty), and/or a genetic predisposition to have the immune system be overactive.  It is not contagious, it is not something you ate, and you did not do anything to cause it. 
What about my brothers and/or sisters? 
The risk of your brothers and sisters is small but slightly higher than in other healthy families. 
Can I be cured? 
No.  Since we do not know the specific cause of SLE we cannot cure it.  However, treatment can bring it under control or remission.
What is remission? 
This is when the disease is not currently active in your body. When you are off all your medication and the disease is under control you are in remission.
Will I be in remission forever? 
No, you will most likely have flares at some point during your life and you will need to restart medication. However, the length of time in between and the treatment needed varies from person to person and flare to flare. 
What is a flare?
This is a period of time when the lupus is active in your body.  Lupus is known as a disease that has quiet periods called remissions and actives periods called flares. Your Pediatric Rheumatologist may be able to identify  a flare early and treat them earlier to avoid damage.
How is my disease different than adults? 
It is less common in children but usually more aggressive.  It is more likely to attack your body organs like kidney’s and/or brain.  This is why children and young adults are treated more aggressively than adults.
Can I die? 
If you take all of your medications regularly and follow up closely with your doctors appointments the risk is much lower than it was a few decades a go.  However, some patients with SLE can die.
What can I do? 
Your best outcome can be achieved by having your care provided by a Pediatric Rheumatologist.  In addition, eat a good balanced diet, do regular exercise, wear sunblock at all times, and take your medication regularly. These are your best controls.  It is very important to speak with your doctors honestly and openly.   
Why should I see a Pediatric Rheumatologist? 
A Pediatric Rheumatologist is a qualified pediatrician with additional training to become a Rheumatologist.  Since SLE is more aggressive in children than in adults we cannot treat them the same.  Since you are growing and your lupus may be more aggressive than an adult it is important to seek out medical care from people who specialize in your treatment.
What are the treatments? 
There are several medications that are “first line” medications. What the doctor will use depends on your symptoms. Since each of us are unique individuals, the treatment depends on your bodies response. Some common medications are NSAIDS(ibuprofen or naprosyn), prednisone, plaquenil, methotrexate, immuran, cellcept and cytoxan.  
How do these medications work? 
Each medication takes care of the lupus inflammation in its own way.  Often they are combined for a better effect and less side effects.  For example,  NSAIDS are an anti-inflammatory so it works against inflammation.  Prednisone is a stronger anti-inflammatory.  Plaquenil is an anti-malarial which has been found to help with skin related symptoms. Methotrexate alters the immune-system and sends a message to your immune system to “quiet down” and it's an anti-inflammatory in low doses.  Immuran, cellcept, and cytoxan all work to suppress the immune systems response to stop “attacking” your blood vessels and connective tissue.   
Do these medications have side effects? 
Yes, each of these medications have possible side effects. The most common medication prescribed that has the most noticeable side effects is prednisone. Ask your doctor about side effects before starting the treatment.  
What are the possible side effects of prednisone? 
Since prednisone is a glucocorticosteroid it actually “builds fat” not muscle.  Some of the possible side effects are weight gain, puffy face (cushingoid effect), problems with blood pressure, elevated blood sugars, mood swings, altered growth, stretch marks, and thinning bones.
What can I do about the side effects of prednisone? 
Watching what you eat is important, since your body can rapidly build fat while taking this medication.  Eating a low fat, low sugar, low salt, and balanced diet is important. Cut down fast food, sugary snacks, soda pop, fatty foods and chips and increase your fruit and vegetables. Continue to exercise as tolerated. Activities such as walking, swimming and bicycling are all low impact ways to keep from having an excessive weight gain and will actually increase your energy.  Work closely with your doctor and ask them any questions or concerns that you have. .
Is there anything else I should know about prednisone? 
Yes, NEVER suddenly stop prednisone.  Your body actually makes cortisone (like prednisone) for normal energy.  When you are taking steroids your body will make less on it’s own, when we slowly decrease the dose your body starts making it again.  However, if you stop the medication quickly your body can not respond to the stress and this is a life threatening condition that can lead to death. 
Should I tell my friends? 
This is a personal choice.  Who you tell is up to you. You may chose to tell some friends or classmates, or you may decide to confide in only a few close friends.  For some patients the support of their closest friends will be crucial.  For others, choosing who to tell, what to tell, and when to tell will vary.  Each child and young adult should work with their family and social worker in order to decide what feels comfortable.  However, if a friend expresses an interest in learning more about your illness, explain it to them.  Sometimes being honest about the illness may be easier than trying to pretend that it didn’t exist.  The best plan of action in telling your friends is to do so in a way that feels comfortable and honest.
Can I go in the sun?
Yes, but you must wear sun screen, at least 45-70 SPF. Also, limit direct sun exposure in the afternoon to 20-30minute intervals, wear a hat and light long sleeve coverings to protect your skin.
What about school? 
You should attend school but you will need to make some adjustments.  Allow your medical care team, your parents and your school to work together to make your school experience a good one.  This includes having morning or indoor physical education (PE).  Depending on your symptoms, you should discuss other recommendations with your doctor.
What about physical activities? 
You can participate in anything that your body will tolerate, and fits with your doctor’s recommendations. 
Will I be able to have a family? 
Yes, most patients with SLE live normal lives.  This includes attending college, having a career, relationships and children.  
Will my children have Lupus? 
The chances are small but slightly higher than in families where none of the parents have SLE. 
What other kind of help might I need? 
At your pediatric rheumatologist's office you can ask what other services are available to support you.  Medical care teams may be comprised of a registered nurse, social worker, physical therapist, occupational therapist, child life specialist, and/or a nutritionist.  Because adjustments to your lifestyle will become necessary, individual and family counseling may also be helpful.  Support groups can also be helpful in meeting other children/teens who have lupus and provide an outlet to share your feelings. Support groups are different than therapy they are meant to share ideas and meet others living with lupus.  Ultimately, patient care based around a good support system such as family, medical care team, friends and/or religious groups tend to cope best.  
What about when I become an adult? 
Anywhere from age 18 to 21 you will begin to transition to an adult Rheumatologist.  This can be a difficult time when you are preparing to go to college or move away from home.  Work with your Pediatric Rheumatologist and his/her office to help you with this transition. 
Where else can I get more information about lupus? 
Be careful of the information you find on the internet. If you have any questions about anything you read ASK! Never hesitate to ASK questions of your healthcare team. Your doctors, nurses, social worker, therapists, nutritionists, child life specialist are all available to help you.  There are many support networks and lupus groups to help you obtain accurate information.