The Lupus Group of WA

Childhood Lupus

Thomas J A Lehman MD FAAP FACR
Chief, Division of Pediatric Rheumatology, Hospital for Special Surgery, New York
Professor of Pediatrics, Weill Medical College of Cornell University
A reprint from the Lupus Foundation of America Article Library 2000

Caring for a child with lupus is one of the greatest challenges a family can face. After the diagnosis, the first step for every parent is to learn as much as possible about the disease and the special needs of a child with lupus.
Parents will need to educate the child with lupus and their other children as well as their child's teachers, and family friends. This article will address some of the medical considerations of which the family must be aware. It also will address the social and psychological concerns which can have a major impact on the outcome for children with lupus and their families.

Is lupus the same in children and adults ?
At one time lupus was thought to be more severe in children than in adults, but most physicians no longer believe this. It is true that children diagnosed with lupus often have been ill for a longer period and are more likely to have significant internal organ involvement, than most adults with lupus. This may be because many children are not recognized as having early lupus until the disease has become worse. As a result children with lupus often are required to begin aggressive therapy soon after diagnosis.

The diagnosis
The first step for every family is to be sure the diagnosis is correct. Lupus is a highly variable disease.

Some children have obvious disease symptoms with fever, rash, and kidney involvement.
Others may only complain of not feeling well, being tired, and weight loss.
Some children may look fine, but may have blood in their urine or other unseen problems that require additional tests that will help the physician to make the diagnosis.
Although a positive ANA is generally required to make the diagnosis, there are many children with positive ANAs who do not have lupus.

The prognosis
Once the diagnosis is established, parents are immediately concerned about what will happen to their child. While lupus can be a severe and life-threatening disease, many children with lupus will do very well.
The prognosis of lupus in childhood depends on the severity of the internal organ involvement :

Children with significant kidney or other internal organ disease require aggressive treatment.
Children with mild rash and arthritis may be easily controlled.

However, lupus is unpredictable and no one will be able to predict with certainty the long-term outcome for a specific child. Success in every endeavor is a combination of hard work and good luck. None of us can control good luck. This article is intended to help you get the best outcome for your child by explaining to you the importance and nature of the hard work.

Following doctor's orders
Compliance with physician instructions has been shown to be one of the most important factors in determining outcome for people with SLE.
Research suggests the severity of a child's lupus is determined by many factors which science has not been able to control. However, some very important aspects of treatment that require special attention can make a difference in the outcome of this disease‹for example :

taking medications as directed
having blood or urine tests when requested
keeping scheduled doctor appointments
following instructions about avoiding too much sun.

Children have special needs
It is important that those caring for the child, i.e., parents and medical professionals, understand these needs. Very important factors in the successful treatment of this disease include :

the parents' understanding about lupus
the reasons for the prescribed medications
the expected outcomes
any problems to be aware of.

Everybody with lupus is different
Much of the information and advice for children with lupus is the same as given to adults. This article emphasizes only those aspects of lupus and its treatment which are peculiar to children and young adults.

The treatment
Treatment for childhood lupus is dependent on the degree of severity of the disease. The most important subject for parents of children with lupus is dealing with the side effects of these drugs.

Lupus medications
Corticosteroids (eg. prednisone)
Many children with lupus require dosages of corticosteroids that produce side effects. The most obvious changes that occur are :

weight gain
a Cushingoid (rounded, puffy face) appearance
acne
unwanted hair growth
stretch marks

These changes are easily visible to other children and may upset you and your child. Over time corticosteroids can cause other more serious problems:

weak bones
poor growth in height
atherosclerosis (fatty deposits in blood vessels that can affect blood flow)

No one wants your child to have these side effects. Side effects can be minimized by keeping the dosage as low as possible, while still maintaining effective control of the disease.
Cytotoxic immunosuppressants
Cytotoxic drugs are one alternative to high doses of corticosteroids. These drugs allow a dramatic reduction in the corticosteroid dosage, often to a level without obvious or severe side effects. This is often a major medical and psychological benefit to children. However, cytotoxic immunosuppressants carry several risks :

Short term, cytotoxic drugs carry the risk of damage to the bone marrow that can cause bleeding or make the child unable to fight off an infection. Careful monitoring usually reduces these risks.
Cytotoxic drugs may increase a child's risk of developing certain forms of cancer.
There is also evidence that cytotoxic drugs may interfere with the ability to have children in the future.

These are scary problems. Doctors must balance the risk of these future problems against the benefits of better control of the lupus and less corticosteroids. These are complicated decisions no one is happy facing. However, many more people are greatly helped than hurt by these drugs.

Alternative treatments
Due to fear and frustrations, parents may turn to other treatments, such as herbal "remedies" or "miracle cures." However, these alternative methods of treatment have not been properly studied for their effects on the human body. They must be subjected to rigorous research studies before they are used, so children are not put in jeopardy. Everyone caring for people with lupus is committed to finding better alternatives to current therapies.

Behavior and responsibility
One of the most difficult problems for every parent is how to let their children "grow up" without letting them take unnecessary risks. This is even harder for parents of children with lupus. The key concept must be, "as normal as possible." Every decision you face with your child (school trip permissions, friends, a sleep over, etc.), ask yourself, would you let them go if they didn't have lupus?
The only big exception is exposure to excessive sunlight, which is known in some instances to make lupus worse. A child with lupus may not be able to go to the beach for the day, or sit out in the sun by the pool. If you are unsure, ask your physician.

Special steps parents should take
If your child is participating in short activities, e.g. visiting friends or going to a movie, usually no special arrangements are necessary.
For longer activities, where a child may be away overnight, the situation can be more complicated.

If they are old enough you can simply send them with their medications.
- In general, children over the age of 10 should know their medications and when to take them.
- If the child is under age 10, be sure there is a reliable chaperone who can take responsibility for the medication and its administration.
No matter where your child goes, it is important that they carry your doctor's emergency phone number where someone will find it easily if the child is injured or becomes ill.
For longer trips you should also make sure the chaperone has your doctor's emergency phone number.
A "medic alert" bracelet should be considered for any child or teenager who is on chronic corticosteroid therapy.
- Some children resent wearing a bracelet, but it can be worn around the neck or even on an ankle, where it is less obvious.
- Unpredictable events do occur, and these "alert tags" provide crucial information and can speed appropriate treatment when a child doesn't know or can't give the necessary information.
- If your teenager resents these as an "emblem" of the disease, let them pick the style and color. Remind them that lots of adults wear such bracelets.
- If necessary you may choose to compromise on a wallet card with their diagnosis and emergency contact information.

Educating your child
The feeling of being overwhelmed can be a very real feeling for parent and child. However, educating yourself and your child can help to alleviate some of the worries and concerns. How to approach your child is dependent on your child's age. Of course no two children are the same, but these general guidelines can help.

For children younger than age 10, excessive explanations aren't very helpful.
- During this period children rely on their parents to "fix everything" and have little sense of responsibility.
- While your child should know that they have lupus, you don't have to spend a lot of time trying to explain it all.
- They will need to understand that lupus is a disease, and that it's not their fault they have it.
- They need to know they didn't get lupus from doing something wrong, that it is not something they caught from someone, and that they cannot give lupus to anyone else.
It is important that your child understands the need to take medicine.
- For older children you can begin to explain the names of the medicines and the doses.
- For smaller children this isn't helpful. Sometimes well-intentioned parents go to great lengths to explain every detail to their children as if they were small adults. Under the age of 10 this information is often misunderstood and despite the best intentions it may do more harm than good.
For children 10 and older it is important to begin explaining the nature of lupus.
- The transition from a small child who must simply do what they are told, to a mature self-reliant adult, may be a difficult one under the best circumstances. Thus it is important that children with lupus must be allowed to assume a progressively greater role in taking their medications and making choices about their care.

Educating others
In addition to coping with your feelings and those of your spouse, and your child, you will rapidly become aware that you have to :

Educate the school faculty and leaders of organized activities in which your child participates. On behalf of your child, you will have to explain :
- absences for doctor's visits
- fatigue
- changed appearance
- restrictions in activities.
- Your doctor and the local Lupus Association can be very helpful in this regard.
You may also wish to educate your neighbors and friends. Not everyone will be aware of what lupus is or how a person gets lupus. You may help them understand that it is not contagious, and that a person who has lupus is genetically predisposed.
- Sometimes this can be done by sharing information available in pamphlets supplied and requesting information about the disease.
Lupus support groups are a good way to help the parent and child meet other children who have lupus, as well as their families, to learn more about their disease and explore ways to cope.

You can ask your doctor or the Lupus Association for assistance in answering additional questions.

Is your child angry ?
It is important to recognize that your child will have days when they are upset about having lupus- just as you are upset that they have it! They will be upset about the side effects of their medications and upset about the limitations placed on them because they have lupus. The fact that it's no one's fault doesn't make it better.
You must treat your child just like you would if they were angry about something else. Occasional problems are normal. If the problems become prolonged you may need professional help. No one has a single solution that will work for everyone.

Is your child depressed ?
It is very important that parents be aware of this problem. Children who become depressed about having lupus may stop taking their medication without telling their parents. This can be disastrous.

Watch the medicine bottles.
- Are they getting empty?
- Do you need refills as often as you used to?
- Even if you are the one administering the medication everyday, stop sometimes to watch and make sure the pills are being swallowed, not spit out when you turn around.
- You might be complying with the all the doctor's advice while your child is not.
If you discover that your child hasn't been taking their medication, talk about it with them, and be sure to let your doctor know.

No one likes being sick
It is important to remember that most children hate being sick. Often this hatred will be expressed as :

hatred of the pills
hatred of the blood tests
hatred of the doctor's visits
hatred of advice from parents.

Frequently this can be dealt with by reasoned discussion and making sure the child knows why they have to do unpleasant things. Few children in the pre-teen and early teenage group realize their actions may have permanent consequences-that if they do the wrong thing they can't simply "be forgiven and start over." At some point they need to understand that doing the wrong thing could make them very sick.

If you and your child can't work it out, get help
Don't be afraid to ask your doctor for help if you are having trouble getting through to your child. Some children require psychological intervention. Improperly dealt with, a child or young adult's resentment about having lupus may result in risky behavior that could affect their well-being.
Not only does having lupus cause psychological problems, but lupus itself may cause problems with thinking. Sometimes what a child who is refusing their medicine needs is more medicine. It is important to understand the psychological problems, as well as the effects that lupus can have on cognitive (thinking) impairment.

Asking, "What if . . . ?"
Even children who appear to be doing well with their lupus can have questions they may be afraid to ask. In the middle and late teenage years, "children" are beginning to think about their life as adults. Children with lupus will wonder:

Am I going to be able to get married ?
Will I be able to have kids ?
Will I be able to earn a living ?

The answer should always be that you expect them to lead productive lives. Since most children with lupus will live well into adulthood, it is important to make sure they understand the importance of doing all the things that can medically help them achieve this goal.

Could anyone else in my family develop lupus ?
Studies have shown that close relatives of adults and children with lupus are more likely to get lupus than are individuals randomly selected in the general population. However, the risk is still very small ( about 1/50 ).
As many as one-third of the sisters of a child with SLE may be ANA positive. Physicians have now had the opportunity to follow groups of sisters for many years. Roughly one in 10 have gone on to develop SLE over a 10-year period. However, this also means that nine out of 10 did not.
If one of your other children has symptoms which make you think they might have lupus, have them evaluated by your physician.

There is no specific test for lupus
Remember, a positive ANA does not make the diagnosis of lupus. In the absence of symptoms sufficient to warrant treatment with prednisone, it is appropriate just to carefully monitor the ANA-positive sibling and/or mother of a child with lupus.

Drug-induced lupus
One special situation is when a relative of a child with SLE is placed on one of the drugs associated with drug-induced SLE. Often this can be done safely, but these relatives may develop lupus-like symptoms when placed on certain anti-seizure drugs and anti-tubercular drugs.

Lupus: a combination of genetics and environment
Family studies of children with SLE also have shown an increased frequency of serologic abnormalities in the mothers. Because usually these are healthy mothers without evidence of disease, this was surprising. This finding strongly supports the hypothesis that SLE results from the combination of a genetic predisposition and an environmental exposure.
Serologic abnormalities in the fathers were much less common, but that doesn't mean they don't contribute to the genetics of SLE. It only suggests that expression of those genetics as a serologic abnormality may be influenced by sex hormones.

Disclaimer :
The Lupus Group of WA (Inc) does not recommend or endorse any products, drugs, treatments, procedures, medical or health professional in this article. We suggest you discuss this information with your doctor or specialist.