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Lupus in Childhood

LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDE
From the Lupus Foundation of Delaware Valley, Inc.
Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert
S. Rosenthal, M.D.  Page Logo

Lupus in Childhood
Phyllis Slutsky, MEd, RN
Barbara 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.