Juvenile Dermatomyositis
(JDMS) What Is It?
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.
We don't know what causes JDMS and why some children get it and others do not. Once the immune system is turned on it will damage it's intended enemy, in this situation, the body itself. 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 once the virus is gone, the immune system does not realize that it is time to stop the fight and attacks the body's own tissue(s). We now also know that the tendency for the immune system to react in this way is, at least, partially inherited. This is not a simple genetic inheritance, there is usually only one affected person in the entire extended family, but we do think that there are genes or groups of genes which either allow for the autoimmune illness to occur or fail to protect the person from developing it.
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.
Remember: JDMS can be mild in some children and severe in others. Your child
may not have all of these symptoms, and may have only minor problems with some
of them. For example, some children with JDMS may only complain of inability to
play on the monkey bars while others may become weak so quickly as to be unable
to rise from bed or stand up from floor.
Skin Rash
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 usually appears
before muscle weakness becomes apparent but it may occur after or at the same
time as the muscle weakness. The rash may be made worse by sunlight.
Weak Muscles
The body's central muscles, those that are 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. This happens after the central muscles are already affected and could be
dangerous and be the reason the child with JDMS is admitted to the
hospital.
At the start of JDMS, some children have a fever along with the other symptoms described
above.
OTHER POSSIBLE SYMPTOMS
The following symptoms may also occur in JDMS. Some are less common, but since
they can occur you should know about them.
Stiff and Swollen Joints
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
joint damage.
Vasculitic Ulcers
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 gastrointestinal tract through which food travels from the
esophagus to the rectum).
· severe pain in the abdomen
· coal-black stools
· change in pattern of bowel movement
·
passing blood in stools.
Calcinosis
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. The lumps are not caused by too much
calcium in your child's diet - they are a late result of the illness and occur
in more than half of all children with JDMS. Calcium lumps are usually not
present at the beginning of the illness. It is thought that calcium is more
likely in children who have untreated muscle inflammation either because they were
not diagnosed in time or were not treated aggressively enough to control
inflammation promptly. It is possible however that no matter
what is done or not done, some children with JDMS are at risk for calcinosis as part of their particular illness.
In most children, after a while, the calcium lumps are usually absorbed back
into the body. Rarely, the calcinosis becomes
extensive and significantly affects movement.
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:
Slowed Growth
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.
How is JDMS Diagnosed?
Child's Health History
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?
· How are your child's activities affected by the illness
· Has she had any choking or regurgitation
·
Can
she carry her back pack and books
Physical Examination
The doctor will examine your child, paying special attention to skin and
muscles. In particular, the doctor will look for these signs:
· a rash on the face, knuckles, elbows, knees, and/or cuticles
·
swelling around the eyes
· nasal speech pattern
·
sore and/or weak muscles
·
sore
and/or swollen joints
Laboratory Tests
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:
Muscle Enzymes
When the
muscles are inflamed or damaged, certain proteins from inside the muscle cells
(called enzymes) leak into the blood stream and their levels can be measured.
The doctor will look for these enzymes such as CPK and aldolase.
Your doctor may also tell you about other enzymes, called AST, ALT and LDH.
These are less specific than CPK and aldolase but are
often used to assess how medications are working. 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
antibodies against the body's cells. This is not a specific test for DMS, it is
often positive in other rheumatic diseases.
Electromyogram (EMG)
This test measures electrical activity in the muscles. Children with JDMS have
a specific electrical pattern that shows the muscle is inflamed.
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. Unfortunately, it is painful and therefore it is not done frequently.
Muscle
Biopsy
This test provides the most specific information about your child's inflamed
muscles. It 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.
Most doctors no longer perform EMG or muscle biopsy if the child has a typical
rash, demonstrable muscle weakness during the physical examination, and
elevated muscle enzymes in the blood test. However, if there is no rash, or
muscle enzymes are normal despite a typical rash, EMG and/or muscle biopsy is
needed.
MRI
(magnetic resonance imaging)
MRI, a very sophisticated Xray, is
sometimes done to find inflamed muscles in children who remain strong and have
normal or just slightly elevated levels of muscle enzymes; the MRI helps
identify muscles most suitable for biopsy.
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.
What Is the Treatment for JDMS?
· 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.
· medication
· exercise
· a balanced diet
·
education
Medication
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 (also known as: steroid, corticosteroid, cortisone, solumedrol,
prednisolone)
Prednisone is by far the most common
and most important of the medications used in autoimmune diseases. It works
quickly to slow down the immune system and help control inflammation in the
muscles and joints. This drug is similar to cortisone, a natural body hormone.
When children take this medicine, their body stops producing the natural
hormone and your child becomes dependent on the medication and cannot simply
stop it. In order to allow the body to resume production of the natural
hormone, your doctor will slowly lower the medication so the body can gradually
make it again.
Steroids can be given as pills, or as a large intravenous infusion weekly, or
both. Some pediatric rheumatologists prefer to give a large dose intravenously
initially and repeat this dose several times while giving a lower daily dose.
Other doctors give only pills, while another one may advise both. No matter how
it is administered, steroid is very successful in controlling your child's
inflammation.
Prednisone is used to:
· relieve muscle and joint pain
· improve muscle strength
· control fever
·
control
skin rash
· increased appetite
·
weight gain
· stretch marks on the skin
·
round, full cheeks
· high blood pressure
·
mood changes
· weak or damaged bones
· small cataracts
· slowed growth rate
·
greater chance of developing
diabetes, if it runs in the family.
If prednisone alone can't control
your child's illness, a stronger drug such as Methotrexate is often used.
This drug also slows down the body's immune system but it works completely
differently from prednisone and has a different set of potential side effects.
· increased risk for serious infections
· nausea and vomiting
· diarrhea
· mouth sores
· skin rash
· damage to some internal organs, such as the liver
·
decreased
number of blood cells
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).
· upset stomach
·
eye
problems
If methotrexate and prednisone don't fully control
your child's DMS, your doctor will probably advise cyclosporine (CSA). This is
another immunosuppressive drug developed for organ transplantation. It helps
control DMS especially in combination with Methotrexate. These two agents
together often allow the prednisone to be tapered away. CSA may cause:
· extra hair growth on face and arms
· thickening and swelling of gums
·
kidney
problems including high blood pressure
Exercise
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 muscles as strong and flexible as possible
·
keep
joints flexible
A Balanced Diet
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
because muscle is made of protein. Calcium is important for strong bones.
However, because prednisone is used to treat your child, the diet must be low
in salt and in sugar. Once, your child regains her strength, her diet should be
low in fat and overall calories but it must stay balanced. 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. Meals
prepared at home are much healthier than anything you buy ready made.
Specifically, fast foods should be discouraged and meals out
need to become special treats. A nutritionist can help plan a good diet
for your child.
Education
Your doctor, nurse and other health care workers can
help teach you about JDMS. By learning about the illness and how to treat it,
both you and your child will be able to take better care of the disease, answer
people's questions and feel in more control over your lives.
The Health Care Team
Several other health professionals may be involved in your child's treatment
program:
Remember: your child may not need to see all of these persons, and may need
to see others only a few times.
· 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 (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 occur with JDMS.
Finding Medical Care
To find out about these health professionals in your
area, contact any of these organizations:
· your local Arthritis Foundation chapter
· a local children's hospital or medical center
·
your
county medical society
What You May Be Feeling
Parents When you are
first told your child has JDMS, you might feel shocked, numbed, or
disbelieving. You might also feel guilty, and ask yourself if something you did
or didn't do caused your child's JDMS. You are experiencing the grieving
process because you have in fact "lost" your healthy child. It takes
time to go through this process, weeks to months, and it is a necessary part of
accepting your child's chronic illness and coping with it most effectively and
productively.
The child with JDMS The child
with JDMS may feel many different emotions. Children can feel "hurt"
by an illness that isn't their fault, blame parents for the illness, adopt a
"why me?" attitude, and indulge in self-pity or anger because of
restrictions on activities. They may also resent other children who are well,
including brothers and sisters. Talk to your health care team about how to best
help your child.
Brothers and Sisters Other
children in the family may feel left out because of the amount of time and
attention the child with JDMS requires. Or they may feel guilty, as if their
normal "bad thoughts" towards their brother or sister had somehow
caused the illness. They may be afraid they will also get JDMS or that their
ill brother or sister may die from the disease.
What Can You Do To Help Them? They key to dealing with these emotions is
to talk about them with one another.
For Your Child with JDMS Talk to
your child about how she feels about the illness. Allow her to express her
anger about JDMS from time to time.
Encourage your child to develop her special talents. Also,
expect her to behave as well as other children: for example, make sure that she
is given household chores which she can complete.
For Other Family Members Talk to
your child's brothers and sisters about JDMS - let them express their feelings
about the disease.
For You and Your Spouse 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 it you do tasks which she
is capable of doing.
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.
What About
School?
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.
· Be informed
·
Inform
others
Federal Laws
There are two federal laws which affect your child's rights in school:
·
Section
504 of The Rehabilitation Act of 1973
·
Public
Law 94-142 (The Education for All Handicapped Children Act)
For More Help
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.W.
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
A national organization that helps children with special needs obtain an equal
opportunity for education.
·
Muscular
Dystrophy Association (MDA)
A national organization that provides support services for persons with
neuromuscular disorders.
·
Inform Others
Talk to people at the school about your child's illness. Tell your child's
teachers, the school nurse, and the principal about:
· the disease, JDMS
· 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
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. Younger children may have an easier course than
adolescents. Some children regain their strength quickly and medications can be
eliminated during the first or second year but others may have recurrences or
may have the type of disease that does not easily respond to medications and
needs to be treated for many years. It is impossible to predict how your child
will respond, only time will tell. The most important
thing is to have confidence in your child's doctor and to insist that your
child receive care from a Board Certified Pediatric Rheumatologist.