JUVENILE DERMATOMYOSITIS (JDMS)The Arthritis Foundation
acknowledges
with appreciation Suzanne L. Bowyer MD,
JUVENILE DERMATOMYOSITIS (JDMS) Back to padeh.net
The Arthritis Foundation acknowledges
with appreciation Suzanne
L. Bowyer MD,
Pediatric rheumatologist at the
Children's
Pediatric rheumatologist at the Children's
Hospital of Los Angeles, Los Angeles,
California and Charles H. Spencer, MD, pediatric rheumatologist, at the
National
Institutes of Health, Bethesda,
Maryland,
for their assistance in the preparation
of
this booklet.
@
1988 by the Arthritis Foundation
WHAT IS JUVENILE DERMATOMYOSITIS?
WHAT ARE THE POSSIBLE
SYMPTOMS OF JDMS
· a rash on the face, knuckles, elbows,
WHAT IS TH E TREATMENT FOR JDMS?
.
This
booklet is about juvenile dermatomyositis
(JDMS), a disease that affects 3,000 - 5,000 children in the United
States. It is written for parents
or other adults who know a child who has JDMS, or for young adults who have
JDMS. This booklet is not meant to take the place of treatment and teaching
provided by a doctor and other health professionals, but it should help you
understand JDMS in your child. The Arthritis Foundation has other booklets
about rheumatic diseases * in children-Contact your local chapter for more
details.
JDMS
affects every child differently. The disease can be mild in some children and
serious in others. This booklet describes the general symptoms of and treatment
for JDMS, but it is important for you to discuss your child’s particular case
with your doctor. If you have questions as you read the booklet, write them
down and take the written list to your doctor.
.
Juvenile dermatomyositis (der-MA-toe-MY-o-SY-tis)
(}JDMS) is a disease that causes a skin rash and weak muscles in children. }JDMS is different from dermatomyositis
in adults, and from polymyositis * (PAH-lee-MY-o-SY-tis), a disease that
usually affects adults.
The skin rash and weak
muscles of JDMS are caused by inflammation in the blood vessels that lie under
the skin and in the muscles. This is called vasculitis. Since blood vessels run
throughout the body, JDMS can also affect other areas such as the digestive
tract. Usually, the worst symptoms are in the skin and muscles. JDMS is often called an autoimmune disease.
The immune system is a group of cells which normally protects the body from
infections. In autoimmune diseases, these cells are signaled to “turn on” their
infection-fighting process, but then can’t turn it off. The process then ends
up damaging the body rather than protecting it. One way the immune system cells fight infection is by a
process called inflammation (in-flah-MAY-shun). But when the cells can’t “turn
off” the inflammation process, tissues are damaged. In JDMS, blood vessels in
the skin and muscles are inflamed.
An inflamed muscle will feel weak or sore, while inflamed skin will have
a rash.
We don’t yet know what causes JDMS and we don’t
know what causes the immune system to damage the body. Many researchers now
think the autoimmune reaction may be caused by the immune system’s abnormal
response to a virus. That is, immune system cells attack an invading virus, but
then also attack the body’s own tissue. We now think the tendency for the
immune system to react in this way may be at least partially inherited.
JDMS is not contagious. Your
child didn’t catch it from another child, and nothing you could have done would
have prevented your child from developing this disease.
There
are three main symptoms of JDMS: weak muscles, skin rash, and fever.
The skin rash and weak muscles are usually the first
signs of the illness. Some children may also have other symptoms such as
sore joints or
less-common symptoms that occur later in the illness.
The
muscles in and near the trunk, such as the neck, shoulders, back, abdomen, and
hips will slowly become very weak. The muscles used for swallowing and
breathing may also become weak.
What you may notice: Your child is likely to have trouble with common
movements such as c1imbing stairs, riding a bicycle, or getting out of a
bathtub. It may also be difficult to get up from the floor or from a low chair.
Your child will probably have a hard time keeping up physically with children
the same age, may fall more often than usual, and may often feel tired.
Children with JDMS may also complain that their muscles are sore to touch.
In very severe cases of JDMS, even the muscles used
for swallowing can be affected. Your child may have trouble swallowing chunks
of food such as meat, and may choke on some foods. These same muscles are used
for speaking, so your child's voice may become weak, and she may sound as if
she is talking through her nose. If you or your child notice any of these
symptoms, contact the doctor immediately
Major
muscle groups most often affected By JDMS
The skin rash usually occurs
on the face; knuckles; elbows; knees; and ankles. In some children; the rash
may cover the whole body. The rash may appear before; after; or at the same
time as the muscle weakness. The rash may be made worse by sunlight.
What you may notice:
Your child’s eyelids and cheeks may appear red or purplish, and the eyelids may
become puffy. Red patches that look like dry skin often appear over the
knuckles, elbows, and knees. The skin around the fingernails may appear red.
Because of the puffy eyelids and skin rash, this
part of the illness can look very much like an allergy. Since the rash may be
made worse by sunlight, children should wear sunscreen when they are in the
sun-
At the start of JDMS, some children have a fever
along with the other symptoms described above.
What you may notice: Your child’s temperature will
rise one or two degrees.
The following symptoms may also occur in JDMS.
Some are less common, but since they can occur you should know about them.
In JDMS, one or several
joints may become stiff or inflamed (swollen). The joints feel stiff or sore
because the muscles around them are inflamed, or because the joint itself is
inflamed. Unlike other forms of childhood arthritis, the inflammation usually
doesn’t last long or cause severe joint damage.
What you may notice:
Your child may complain of stiff and sore joints. The joint may also feel a bit
warm when you touch it.
Calcinosis (kal-sih-NO-sis) is a process that
causes small lumps of calcium to form under the skin or in the muscle. The
lumps may feel like little rocks under the skin and can range from the size of
a period on this page to the size of a flat pebble. Sometimes, the smaller
lumps join together to form larger calcium lumps. When this happens, it can
impair muscle movement. Too much calcium in your child’s diet does not cause
the lumps - they are a late result of the illness and occur in more than half
of all children with JDMS.
If the calcium lumps are
bumped a lot, they can become sore. This is most likely to happen if they are
on your child’s knee, elbow, buttocks, or other pressure point. Small pieces of creamy white calcium
may break through the skin and drain from the hole. These draining areas will
usually heal, but they can become infected. Con-tact your doctor if this
happens. After a while, the
calcium lumps are usually absorbed back into the body.
A vasculitic ulcer is a hole in the tissue that
surrounds an inflamed blood vessel. In JDMS, vasculitic ulcers can occur in the
skin and in the bowels (the pathway of organs through which food travels from
the esophagus to the rectum).
In the skin. These ulcers happen when the
blood vessels under the skin are severely inflamed. They look like sores in the
skin rash, and can be as small as a pen-ell point or larger than a silver
dollar. Some ulcers are very deep, while others occur only on the surface- they
can be very painful, and can last anywhere from a few days to several months-
the ulcers usually go away with treatment.
In the bowels:
These ulcers happen when the blood vessels in the protective lining around the
bowels are inflamed. This causes a
break, or hole, in the lining.
Strong digestive juices can then leak through that hole and damage
digestive organs. If this happens to your child, you may notice these symptoms:
·
. severe pain in the abdomen
·
. coal-black stools
·
. change in pattern of bowel
movement
·
. passing blood in stools
This happens only rarely, but if you or your child
notice any of these symptoms contact the doctor immediately.
A contracture is a shortened muscle that causes a
joint to remain in a bent position.
In JDMS, there are two ways a contracture may occur:
Through the healing process:
As the muscle heals, it may scar. These scars can shorten the muscle, and pull
the joint into a bent position.
Through a lack of exercise:
At times, your child’s muscles might feel too sore to exercise. Without
exercise, the muscles become weak. Because a weak muscle cannot hold the joint
in its correct, extended position, a contracture will occur. You and your child can help prevent
serious joint contractures with daily exercises and proper physical therapy
(see p. 15).
When the body is fighting a
disease, it uses a lot of the energy, which it would normally use for growth.
Because your child may not feel well, she may not feel like eating. These
factors, along with some of the medicines used in treatment, may cause your
child to grow and mature more slowly than normally. Usually, your child will
begin to grow again once the disease is under control.
In some children, the
signs of JDMS develop over a long period of time. In others, the signs develop
quickly. To find out if your child has JDMS, the doctor will ask about your
child’s health history, perform a complete physical examination, and obtain
lab-oratory tests.
The doctor will ask many questions about your
child’s symptoms, such as:
·
When did the symptoms start?
·
What parts of the body are
involved?
·
Are the symptoms always
present, or present only at certain times?
·
Are the symptoms getting
better or worse?
The doctor may also want to know if other members
of the family have had arthritis or a muscle disease since, in rare instances,
some forms of these illnesses may run in families.
The doctor will examine your child, paying special
attention to skin and muscles. In
particular, the doctor will look for these signs:
knees, and/or
fingertips
·
swelling around the eyes
·
nasal speech pattern
·
sore and/ or weak muscles
·
sore and/or swollen joints
The doctor will also check your child’s strength
and will check routine areas, such as the eyes, ears, nose, and throat.
Laboratory tests help determine if your child has
JDMS. These tests show if there is inflammation in the muscles or in other
areas of the body. Many of these tests may be abnormal early in the course of
the illness, even if your child has only mild muscle weakness. The most common
tests are:
When the muscles are inflamed or dam-aged, certain
muscle proteins (called enzymes) leak into the blood stream. The doctor will
look for enzymes such as CPK and aldolase. If large amounts of these enzymes
are found in the blood, then the doctor knows that some muscles have been
damaged.
Antinuclear Antibodies (ANA)
This test shows whether or not your child’s immune
system is producing anti-bodies against the body’s cells.
Electromyogram (EMG)
This test measures electrical
activity in the muscles. In this test, a thin needle is placed through the skin
and into the muscle. A wire runs from the needle to a machine that records
electrical activity in the muscle. Children with JDMS have a specific
electrical pattern that shows the muscle is inflamed.
This test shows how much the
muscles or blood vessels have been affected by the disease. If your child needs
a biopsy, the doctor will remove a small piece of muscle and look at it and its
surrounding blood vessels under a microscope to see if there is any
inflammation. Children with damaged blood vessels tend to have more problems
during their illness.
By putting together the story of how the illness
started with the results of the physical examination and the laboratory tests,
the doctor can usually make the proper diagnosis.
There is no cure for JDMS,
but treatment can help prevent or control most serious problems. Your child’s
treatment plan will be based on the severity of the illness. The goals of any
treatment program for JDMS are to :
·
control muscle inflammation and damage
·
maintain and improve muscle strength and function
·
relieve pain
·
control or prevent other symptoms
·
help child and family learn to live with the
illness
To reach these goals,
treatment usually includes:
·
medication
·
exercise
·
a balanced diet
·
education
As your child’s symptoms
change, the treatment plan may also be changed.
There
are several medications your doctor may use to treat JDMS. The specific drug or
combination of drugs your doctor uses depends on the type and severity of your
child’s symptoms. Since only common side effects of each drug are listed here,
you should discuss your child’s medications with the doctor.
Prednisone slows down the
immune system and helps control inflammation in the muscles and joints. This
drug is similar to cortisone, a natural body hormone.
Prednisone is used to:
·
relieve muscle and joint pain
·
improve muscle strength
·
control fever
·
Control skin rash Dose: At first, high doses of this drug are
given until your child’s muscle enzyme tests improve. As your child gets
better, the dose will gradually be lowered.
Common side effects: The side
effects your child may have depend on how much medicine the child takes, and
how long she has taken it. Common side effects include :
·
increased appetite
·
weight gain
·
stretch marks on the skin
·
round, full cheeks
·
high blood pressure
·
mood changes
Over a long period of time,
the drug can also cause these problems:
·
weak or damaged bones
·
small cataracts
·
slowed growth rate
·
greater chance of developing
diabetes, if it runs in the family
Prednisone
may cause other side effects, depending on the dose and length of time your
child takes it. Discuss the side effects with your child’s doctor. This drug is
given because the benefits of controlling the JDMS outweigh the risks of side
effects. The doctor will try to lower the dose as soon as possible to lessen
the side effects, while keeping the disease under control. A word about
relapses: as the prednisone dose is lowered, your child’s earlier symptoms of
JDMS may return. This is called a relapse. These symptoms will usually go away
with a mild increase of prednisone.
Although some children may have more than one relapse, a relapse may not
necessarily slow your child’s recovery from JDMS.
If prednisone alone can’t
control your child’s illness, a stronger drug such as methotrexate or
cyclophosphamide (Cytoxan) is often used. These drugs also slow down the body’s
immune system. Dose: The doctor
will use as little of these drugs as possible and will usually use them in
combination with prednisone. The drugs are given as a pill or by injection. As
your child gets better, the doctor will slowly reduce the dose of one or both
of the drugs. Common side effects:
Because these drugs can cause serious side effects, they are used only if other
medicines have not controlled the disease well enough. Talk to your doctor to
decide whether the benefits of the drug outweigh the risk of its side effects.
Side effects may include:
·
nausea
and vomiting
·
diarrhea
·
mouth
sores
·
skin
rash
·
damage
to some internal organs
·
decreased
number of blood cells
These side effects will go away when the dose is
lowered or stopped completely.
These drugs may cause other, more serious side effects. If your child is
taking any of these drugs, the doctor will take regular blood and urine tests
to monitor the drug’s effect on your child.
Hydroxychloroquine (Plaquenil)
Hydroxychloroquine (hi-DROCKS-ee-CLOR-o-kwine) is
a drug sometimes used to treat a severe rash in JDMS. The brand name of this
drug is Plaquenil (PLA_kweh-nill).
Dose: This drug is given as a pill. Since it
simply controls the rash, your child will
will also take other medicines for other symptoms.
Common side effects: Side effects include:
·
upset stomach
·
eye problems
A small dose of Plaquenil is given for JDMS, so eye
problems don’t happen very often. Plaquenil may cause problems such as trouble
focusing sharply on an object, or seeing a “halo” around lights. In the
earliest stages of eye damage, the damage is still reversible. At this early stage,
your child may not notice any symptoms. That is why an ophthalmologist (eye
doctor who is an MD) should check your child’s eyes every three to six months
while your child is on Plaquenil. If eye problems are detected early, they
should go away when the medicine is stopped.
Exercise is a very important part of JDMS
treatment: in fact, it is nearly as important as medication. The purpose of
regular exercise is to:
·
keep joints flexible
A physical therapist will
teach your child exercises to do at home. These exercises will be designed
specifically for your child and will change depending on the activity of the
disease. At first, your child’s muscles may be weak and she may need help with
the exercises. Later on, when your child won’t need as much help, the exercises
will be changed.
Exercises usually take about one hour per day. It
is important for your child to do the exercises regularly. But not to overwork
and tire her muscles. The therapist will try to combine some exercises with
your child’s normal play activity.
Although there is no diet that will cure JDMS, it
is important for your child to eat a regular, balanced diet. Protein in your
child’s diet is important for normal growth and to repair damaged muscles.
Calcium is important for strong bones. If your child does not feel well and
does not want to eat, try to keep her interested in foods by letting her help
prepare meals, and by preparing many different foods. A nutritionist can help
plan a good diet for your child.
Your doctor, nurse and other health care workers
can help teach you about JDMS. By teaming about the illness and how to treat
it, both you and your child will be able to take better care of the disease.
Your
child’s regular doctor is the best person to see when your child first becomes
ill.
Because JDMS may be hard to diagnose or treat, the
doctor may send your child to a specialist such as a pediatric rheumatologist,
a doctor who specializes in childhood arthritis. If there is no pediatric
rheumatologist in your area, your doctor may refer you to another specialist
who is knowledgeable about JDMS.
Several other health professionals may be involved
in your child’s treatment program:
·
Nurse - teaches your child about JDMS and how to take
care of it. This includes teaching pain control and play therapy to help the
child deal with the illness.
·
Physical therapist - tests muscle
strength and designs exercises which strengthen large muscles I such as the
arms and legs) and keep joints flexible.
A physical therapist can also help your child regain lost coordination
and movement.
·
Occupational therapist - teaches new ways to do daily activities such as eating,
combing hair, and dressing, which may be more difficult for a child with JDMS.
These activities strengthen smaller muscles (such as those in the hand) and
help the child feel more independent.
·
Social worker – helps with the personal,
emotional, family, or financial problems that may have occur with JDMS.
Other persons such as nutritionist, pharmacist,
psychiatrist or psychologist can help you handle special problems.
Try not to overprotect your
child, even though this is a natural desire. Your child might become too
dependent if you do everything for her or if you do tasks, which she is capable
of doing.
Don’t be “blackmailed” into
allowing activities that shouldn’t be done, but com-promise when you can. Being
as consistent as possible will help your child team what is expected.
Plan special time to spend
alone with your spouse, or with the entire family. When your child first becomes ill, you may set aside
relationships with other family members. It is important, however, to continue to
talk to and spend time with all family members.
Overall, it is important for
the whole family to fit JDMS into its regular life pat-tern and to adapt to the
illness rather than fight it. This means fitting your child’s treatment program
into the family’s regular daily schedule. It means asking for special help from
people when you need it. Adapting also means talking (and crying) about JDMS
now and then. Of course, JDMS will make life more difficult than it used to be
- but accept this as a challenge rather than a burden.
WHERE CAN YOU GO FOR MORE
HELP?
Sometimes, the stress of having JDMS may cause
your child to develop emotional or behavioral problems that you cannot deal
with alone. Other family members may also feel overwhelmed. Whatever the case
may be, counseling or support groups can help work things out. Sometimes all
that is needed is a talk with a sympathetic person, another family who has a
child with a rheumatic disease, or a professional who is trained to help in
these situations. The doctor, nurse, or medical social worker may be able to
help you or refer you to other sources of help.
Generally, children with JDMS should attend a
regular school and should not be isolated from other children their same
age. But because of JDMS your
child may need special services, such as special transportation or supplies, to
help her get along in a regular school.
How can you help your child avoid problems at
school?
· Be informed
·
Inform others
There are two federal laws, which affect your
child’s rights in school:
Section 504 of The Rehabilitation Act of 1973
This law forbids all federal programs from
discriminating against disabled persons. It means your child should be able to
receive services from all programs and agencies (including schools) that
receive money from the federal government.
Public Law 94-142 (The
Education for All Handicapped Children Act)
This law says that every child has a right to a
free public education, whether or not the child has a disability. It means you
can work with your child’s school to make sure she receives the services she
needs in order to attend public school.
For more details about these laws see When Yom
Student Has Arthritis, available from your local Arthritis Foundation chapter.
If you have problems protecting your child’s
rights, you can contact the following organizations:
The American
Juvenile Arthritis Organization
Arthritis Foundation
1314 Spring Street, N.
Atlanta, Georgia 30309
The Arthritis Foundation’s national membership
organization that focuses on the problems related to arthritis and rheumatic
diseases in children.
The Federation for
Children With Special Needs
312 Stuart Street
Boston, Massachusetts 021 16
A national organization that helps children with
special needs obtain an equal opportunity for education.
Muscular Dystrophy
Association
(MDA)
810 Seventh Avenue
New York, NY 10019
A national organization that provides support
services for persons with neuromuscular disorders.
National Information
Center for Children and Youth with
Handicaps (NICHCY)
Box 1492
Washington, DC20013
A national resource center for parents of disabled
children.
Talk to people at the school about your child’s
illness. Tell your child’s teachers, the school nurse, and the principal about:
·
how JDMS affects your child, and problems that may
occur in school
·
your child’s treatment program, including
medications taken during school hours
·
how to reach you or your child’s doctor, if
necessary
·
this booklet and other Arthritis Foundation
booklets such as, When Your Student Has Arthritis
Most children have active
JDMS for about two years. Since JDMS affects every child differently, some
children may have active disease longer than two years, and some may have more
severe symptoms than others.
JDMS is a treatable disease.
Although most children recover completely from the disease, some children will
have permanent muscle weakness from it. Despite the challenges children with
JDMS and their families face, the majority of these children grow up to lead a
fulfilling, happy life.
By reading this booklet, you
have begun to learn about your child’s condition and how to treat it. Most
importantly, you have learned some of the ways to approach the daily challenges
of the illness. Continue to read and learn about JDMS. You will be helping
yourself, your child, and your family adjusts to the challenges of the illness:
you will be helping them to an active and happy future.
Antinuclear antibody (ANA) -
a type of protein found in the blood of some children with different forms of
autoimmune disease.
Arthritis - general term meaning
inflammation of a joint.
Connective tissue - tissue that
supports, binds, and/or separates other tissues and organs. Bones, cartilage,
and tendons are types of connective tissue.
Chronic disease - an illness which
lasts for a long time, (months to years) or for the life of the affected
individual.
Juvenile rheumatoid arthritis -
a form of arthritis that occurs in children. Its most characteristic feature is
joint swelling and pain.
Polymyositis - (PAH-lee-my-o-SI-tis) a
rheumatic illness that affects muscles. It occurs most frequently in adults.
Polymyo-sitis is different in many ways from JDMS and from dermatomyositis in
adults.
Rheumatic disease - (roo-MA-tick) general term for
diseases that involve inflammation of connective tissue. Juvenile
dermatomyositis, juvenile rheumatoid arthritis, scleroderma, and systemic lupus
erythematosus, are types of rheumatic diseases that affect children.
Systemic lupus erythematosus-
(ee-RIH-them-a-TOE-sus) a chronic inflammatory disease which can affect skin,
muscles, joints, and internal organs.
The mission of the Arthritis
Foundation is to support research to find the cure for and prevention of
arthritis and to improve the quality of life for those affected by arthritis.
Formed in 1948, the Arthritis
Foundation is the only national, voluntary health organization that works on
behalf of all people with any of the more than 100 forms of arthritis or
related diseases. Volunteers in chapters nationwide help to support research,
professional and community education programs, services for people with
arthritis, government advocacy and fund-raising activities.
In 1981, the Arthritis
Foundation established the American Juvenile Arthritis Organization (AJAO) - a
national member-ship organization, which works as a part of the Foundation to
focus its efforts on the specific problems, related to arthritis in children.
This group was formed for
children with arthritis and rheumatic diseases, for their parents and friends,
and for health professionals who treat these children. The AJAO publishes a
newsletter and provides information about arthritis in children to parents and
the general public.
Some chapters of the
Arthritis Foundation have helped parents organize AJAO groups. Many of these
groups meet regularly and provide informative programs on the medical,
financial, and emotional issues surrounding arthritis in children. The groups also provide an opportunity
to talk with other parents of children with arthritis. Contact your local
chapter of the Arthritis Foundation for more information about AJAO and local parent
groups.