Lupus in Childhood

Lupus in Childhood
Phyllis Slutsky, MEd, RNBarbara E. Ostrov, MD

About 20-25% of all lupus occurs in children. While the exact number of cases is not known, it is not a rare disorder. Girls have lupus about three to seven times more often than boys. Research is being done to study many possible causes of lupus.

Several studies have shown that there is a tendency for lupus to run in families. It is known that there are certain inherited factors called HLA types which make a person more likely to develop lupus. (See Chapter 4.) There is also an inherited lack of immune system proteins (serum complements or immunoglobulins) which increase the risk for developing lupus in some people.

When a susceptible person is exposed to a "trigger" such as a virus or other environmental factor, lupus can develop. A particular kind of lupus may develop in infants of mothers who have SLE. This is called "neonatal lupus" and is associated with an anti-Ro antibody in the mother. An infant with neonatal lupus may have a rash on the face, scalp, or chest. Very rarely, these infants may also be born with an abnormally slow heart rhythm (congenital heart block).

During the first six months to one year, the rash disappears but the slow heart rhythm can be serious and may require the placement of a pacemaker. Systemic lupus erythematosus in childhood can range from mild to severe and affects each individual differently. The usual course of lupus includes periods of "flare-ups" (when symptoms are active) and remissions (when symptoms go away).

Stressors such as infections (which may be more frequent in childhood) and hormonal changes may lead to a flare of symptoms. This may explain why lupus is more common in girls after puberty and may flare up during menstruation and pregnancy. In children, as in adults, sun exposure can bring on symptoms of lupus. Serious complications of lupus are most common when organs such as kidneys, heart or the central nervous system are involved.

The most common time to find out what body systems are involved is at the time that lupus is first diagnosed. As time goes on, the likelihood decreases that new body systems will become involved. Diagnosing lupus involves a thorough physical examination and often many laboratory tests.

There are guidelines which have been developed by the American College of Rheumatology which are used to diagnose lupus. (See Table 1) Four of their eleven selected symptoms and/or laboratory tests (criteria) must be present to make a definite diagnosis. Symptoms of lupus may start in a variety of ways.

A child may be very ill or have only mild symptoms. The most common symptoms in children are rash, fever, fatigue, and joint pain and swelling. Decreased appetite and weight loss are particularly common in children during the active phase of the disease. Inflammation of the kidneys (nephritis), inflammation of the heart covering (pericarditis), enlargement of the liver and spleen, and a low blood count (cytopenia) may be more frequent in children than in adults. Many children have rashes that can appear on the face or anywhere on the body. Ulcers of the mouth and nose are common in children with lupus. Hair loss (alopecia) may range from gradual thinning and change in texture, to loss of large amounts of hair when combing.

Central nervous system symptoms (affecting the brain and spinal column) can occur and may cause headaches, seizures, or a change in memory or thinking ability. Visual changes can also occur, making it important to have frequent checks by an eye doctor. Changes in mood or behavior can occur in children with lupus. These can be an understandable reaction to having an illness. Feelings of sadness or irritability do not necessarily mean there is central nervous system involvement. However, it is important to keep a careful watch on a child's behavior to decide if these things are caused by the lupus or are due to the child's reaction to the illness.

Diagnostic tests such as spinal taps, EEGS, and brain x-rays and scans can be helpful in deciding what is caused by lupus and what is due to psychosocial causes. The kidneys can be affected by lupus. A urinalysis may reveal problems which can then be further investigated with other tests. It is common to collect urine for 24 hours to do further studies. A kidney biopsy is sometimes necessary to determine how seriously the kidney is affected.

This test is used as a guideline for treatment and for monitoring changes over time. The treatment of lupus depends on what part of the body is affected. Being careful about sun exposure is important for all children. Planning the child's schedule to avoid exposure during the peak time of 12:00-3:00 P.M. each day is recommended. Children should be included in working out the schedule so that they understand the importance of any restrictions and will be more willing to cooperate. Another important aspect of treatment is to be alert for any signs of a developing infection.

Families should be aware that fever and increased tiredness or shortness of breath could be a sign of either a "flare-up" of lupus or possibly an infection. The doctor may need to evaluate the child when fever develops. There are a variety of medications used to treat lupus. Many children are treated with a non-steroidal anti-inflammatory drug (NSAID) which helps reduce inflammation and joint pain. Antimalarial drugs such as Plaquenil are sometimes used to treat the skin rashes of lupus. Steroids are sometimes necessary to treat lupus. The goal when using steroids is to use the lowest possible amount needed.

As symptoms improve, smaller and smaller doses are gradually given until the medication can be stopped altogether. When high doses of steroids are used over a long period of time, side effects can occur such as eye problems, and a decrease in the growth of the child. Giving steroids every other day helps to lower the chances of harmful side effects. Some other drugs used mainly when kidneys are affected by lupus are Cytoxan and Imuran. These work to control the reaction of a child's immune system so that it causes less harmful effects on the body. Recent studies have shown that these drugs can be used safely in children and can control the more serious effects of lupus on internal organs. A chronic illness like lupus will of course have a large effect on a child's life.

Often a child misses school during "flares". It is important to make contact with the school as soon as possible and stay in contact during the absent period. A school counselor or nurse can make arrangements to help the child. Some children may need home or hospital tutoring while others may be able to do make-up work on their own. This depends on how sick they are and how long they will be out of school. Because lupus symptoms come and go, arrangements may need to be made before a child is sick so that tutoring can start right away if needed. Many rheumatology centers that treat lupus have a health care team that includes a social worker and a nurse who can help the family with getting services needed for their child. Often children with lupus (like those with other chronic conditions) should have an Individualized Education Plan (IEP) .

This is an evaluation of all of the child's educational, psychosocial and physical needs which is done by a "study team" in each school district. The IEP identifies what each child needs in order to function well at school. Every child in the USA is entitled to this plan due to a government law (PL#94 -142). Children with lupus are also entitled to vocational planning services in order to prepare for school or job training for their future employment.

Each state has an Office of Vocational Rehabilitation (OVR) which offers career counseling that takes into account a child's physical abilities. Any illness in a family member is likely to cause reactions in other members of the family. These can help or hurt successful coping. There is often an increased burden on the family when caring for a sick child. This can also mean lost work time for parents and added financial problems due to medical expenses. Studies have been done to measure the effects on a family of a child with chronic illness. The families that coped best were those that did not place their child in a sick role, and so did not limit the child's activities in daily life.

Families based around a good quality marriage, and with good support from family, friends and/or religious groups cope better with a chronic illness. Support groups for parents of children with lupus exist through the AJAO (American juvenile Arthritis Organization) which is a part of the Arthritis Foundation. The large number of teenage and young adult women with lupus raises some special concerns. A major problem for teenagers is the change in appearance, often on the face, caused by lupus.

The characteristic rash and puffiness of lupus, or the acne caused by steroids cause the child to wonder, "How do I look to the world?" It is important to discuss this as openly as possible with the teenager, as these issues are a major source of stress and worry. Concerns about sexuality and pregnancy are also often uppermost in teenagers' minds when they become ill. Standard oral contraceptives which contain the hormone estrogen may cause an increase in symptoms of lupus and therefore are not recommended.

Sometimes the mini-pill, which contains only the hormone progesterone, is safe in lupus patients. Diaphragms and condoms are fine, and all of these options should be reviewed and discussed. It is recommended that a woman not become pregnant during an active period of lupus. Lupus has not been found to affect the ability to get pregnant, but miscarriages are more common when the disease is active. It is very possible to become pregnant and have a healthy baby, but careful planning with the doctor for the best time to get pregnant is advised. There are many obstetricians available who specialize in "high risk" pregnancies. Because of the many issues and adjustments necessary for a teenager with lupus, individual counseling may be necessary and can be helpful to some adolescents.

Support groups are an ideal way for teens to get the peer support and acceptance they need. Teens who attend these groups report a decrease in feelings of isolation and are better able to accept their illness. There has been a remarkable improvement in the prognosis of children with lupus during the past thirty years. This has happened due to improvements in diagnosis and treatment. Most importantly, children and their families need to seek careful and competent medical treatment and to cultivate the support of their health care team, their school and their community.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.