Answers to the most commonly asked questions about Immune
and Idiopathic Thrombocytopenic Purpura (ITP) in children
What is ITP?
Why are platelets important?
What's a normal platelet count?
How did my child get ITP?
How can I be sure it's ITP?
Is it contagious?
Will it go away?
How is ITP treated?
Why treat at all?
What if these don't work?
What is a splenectomy?
Why the spleen?
What if it is chronic ITP?
What does that mean?
Does ITP mean I need to restrict my
child's activities?
Who else needs
to know about ITP?
Does
ITP make my child
more susceptible
to other illnesses?
Does
ITP affect my child's
life span?
OK.
So what's it like
to deal with ITP?
And
if it goes on?
How
is my child likely
to react?
What
about my other
children?
Where
can I learn more
about ITP?
The ITP Society extends its thanks to
The Bayer Corporation
Baxter Healthcare Corporation
Alpha Therapeutic Corporation
for their generous educational grants, making the printing
of this book possible.
The authors gratefully acknowledge the assistance they received
from the Massachusetts General Hospital Pediatric Hematology/Oncology
Department and the Service MGH Grant Program in preparing
and distributing the original version of this booklet.
The authors are especially grateful to Dr. Eric Grabowski
for his encouragement and input, Dr. James Bussel for his
enthusiastic support and masterful editing, and Andrea Costello
for her ability to make it all come together so beautifully.
This booklet was prepared by the parents of a child
who has ITP. Their goal was to provide answers to many
of the questions that come up after a child has been diagnosed
with ITP. Especially because medical information is ever-changing,
this booklet is not meant to replace discussions with your
child's hematologist or other health professionals-simply
to help you and your family cope with the ups and downs
of the ITP "roller coaster". We hope you find
it helpful.
ITP is a disorder of the blood that involves the immune
and blood clotting systems. ITP stands for Immune Thrombocytopenic
(a decrease in blood platelets) Purpura (purplish areas of
the skin and mucous membranes). You may also hear it referred
to as Idiopathic Thrombocytopenic Purpura. As far as experts
know, ITP occurs when a person makes antibodies, which attach
to his or her own platelets. The body's immune system eliminates
the platelets because of the antibodies on them, thinking
that they may be bacteria. This results in a decrease in
the platelet count or thrombocytopenia.
Platelets are the microscopic parts of our blood that help
stop bleeding and heal bruises.
The normal range for a child is 150,000-400,000 platelets
per microliter.
If your child is otherwise healthy, chances are he or she
has ITP as a result of a recent infection. This is the situation
in about one-half of the cases of ITP in children. Some cases
of ITP are the result of medication, other infections, or
associated with immune disorders such as systemic lupus erythematosus,
HIV infection, or Epstein-Barr virus. Infants can develop
ITP as a result of ITP in their mother during pregnancy,
premature birth, and/or a number of other syndromes, but
these cases go away rapidly.
If there is any doubt about your child's diagnosis a hematologist
(blood specialist) may ask that a bone marrow test be done.
This involves taking a sample of the liquid part of the pelvic
bone in the back. This test may also be done to rule out
leukemia.
No. And most children who may have had the same viral infection
will not develop ITP.
In most cases, yes. The vast majority of ITP cases in children
are "acute" or temporary. Statistics show that
85-90% of children with ITP recover (to normal platelet counts)
within one year-most even sooner than that. In some children,
ITP does not go away. This would be termed "chronic"
ITP. Medical professionals differ on the time frame before
ITP is considered "chronic". Some call it chronic
if it lasts more than six months, others after one year.
Treatment of ITP varies.
Medicines are stopped if they are known to be the cause.
Any infection that may result in ITP would be treated or
allowed to improve. Children with no actual bleeding and
a stable platelet account above 20,000 to 30,000 cells per
microliter probably will require no treatment. This is based
on the hope that the child's body will rid itself of the
antibody and therefore, ITP. Children in this situation can
lead an almost totally normal life.
For those children who require treatment, a hematologist
will most likely prescribe one or more drugs that change
or suppress the child's immune system. If this is successful,
fewer platelets will be eliminated and the platelet count
should rise. The thinking behind this is to "trick"
the body's spleen and liver into NOT removing or destroying
the platelets. Since fewer platelets are eliminated, the
child's platelet count should rise.
The drugs and therapies currently used to treat ITP are:
(IVIG, IgG, or GammaG)
- Corticosteroids or "steroids". High
does of these drugs over a short period of time are thought
to slow the removal of platelets by the spleen and liver
and temporarily lower the level of anti-platelet antibodies.
This class of drugs may also stabilize the blood vessels,
lowering the risk of bleeding. Steroids may be prescribed
in pill form or given intravenously (through the veins.)
- Intravenous Gamma Globulin. (IVIG, IgG, or GammaG)
This substance may be used separately or in combination
with steroids. IVIG is given intravenously over a course
of 2-6 hours for a period of 1-5 days. This product is
prepared from blood plasma and is thought to block the
antibody that results in the destruction of the platelets.
- IV-Anti-D. Recently licensed for the treatment
of ITP, this form of IVIG which is prepared from the plasma
of a smaller, more specific group of donors. IV-Anti-D
is given intravenously and functions like IVIG, acting
to provide temporary increases in the platelet count. It
is effective only in people who have a positive blood type
(i.e. A+, B+, or O+) and have not had their spleen removed.
Indications are that IV-Anti-D is particularly useful for
children who are attempting to keep their platelet count
at a safe level while waiting for their condition to improve.
None of these treatments cure ITP. The main reason to treat
a child with ITP is to keep him or her out of danger-that
is, to keep the child's platelet count in a range considered
to be safe until the body cures itself. That range is considered
by many experts to be greater than 20,000-30,000 cells per
microliter. Platelet counts below 10,000 cells per micoliter
enormously increase the risk of injury-related or spontaneous
internal bleeding and/or bleeding on the brain (intracranial
hemorrhage). Either of these conditions can be life-threatening
for a child; as a safeguard, most experts will treat children
at 20,000-30,000 cells per microliter or below.
Are there risks or side effects to these treatments?
- Steroids, as they are used in the treatment of ITP, can
cause mood changes ranging from euphoria to irritability,
weight gain, puffiness in the face and/or back of the neck,
and irritation of the stomach. Menstrual periods may also
be affected. Patients on steroids for weeks or months may
also experience an increased risk of infection, thinning
of the skin, calcium loss from bones, kidney stones, high
blood pressure, pancreatitis, cataracts, and other side
effects. Limits on dosages and duration of steroid use
can make these less likely.
- Patients who receive IVIG may experience temporary headaches,
nausea, light-headedness, or a slight fever. On very rare
occasions, a patient can be severely allergic to IVIG.
This substance is derived from human blood, but requires
a "virus inactivation" step in processing and
is therefore thought to be as free of blood born diseases
as is possible today. IVIG is significantly more expensive
than steroids.
- IV-Anti-D is a type of IVIG but comes from a much smaller
group of donors. It can cause temporary anemia, and occasionally,
fever and chills 1-2 hours after infusion. It is less expensive
than IVIG and can be given in less than an hour.
- Any of these treatments often involve needle-sticks and
therefore pain. A topical cream can be used to temporarily
numb your child's hand or arm. This often helps to minimize
the pain and fear of needle-sticks and IVs. This anesthetic
cream is available through your child's health care professional.
- For mouth or nose bleeding or in preparation for dental
work, there is a medication called AMICAR. This helps to
eliminate the bleeding without changing your platelet count.
For a few children, these treatments will not cause even
a temporary increase in platelet count. If this is the case,
your hematologist will likely test for a rarer cause of your
child's low platelet count. For others, maintaining a safe
platelet count is impossible without on-going treatments.
If this is your child's situation, your hematologist will
work with you to determine what the next step will be, based
on the risk of serious bleeding and other factors. If serious
bleeding is a risk, a splenectomy may be recommended.
It is the surgical removal of the spleen. Splenectomy is
the only treatment proven to cure ITP, and works for 70-90%
of the people who undergo it. It is major surgery, and carries
with it a life-long increased risk of serious infection.
Splenectomy is usually not used unless the ITP has persisted
for more than one year, and the child is 5 or older. It is
a serious step and needs to be discussed at length with your
child's health care professional. If splenectomy is chosen,
your child will be given medicines before, and for at least
one year afterward to strengthen him or her against infections.
In the years that follow, any fever over 102 degrees must
be treated with antibiotics on an emergency basis as a precaution.
The spleen is one of the body's organs which works like
the filter in a carburetor-removing wastes and "foreign"
bodies. Normally, platelets pass though the spleen, but with
ITP, the spleen filters out the antibody-coated platelets
"by mistake" because of the antibodies. This organ
is also thought to be where some people make the anti-platelet
antibody.
ITP is considered chronic if it lasts more than six months,
though some say after twelve months. In children, ITP may
still go away by itself after this length of time. There
is no difference in the disease itself if it is chronic.
The difference is in the management of the disease. The goal
of the treatment becomes keeping your child's life as normal
and risk-free as possible.
Assuming your child is otherwise healthy, this basically
means frequent blood tests to track his or her platelet count
and treatments when the numbers are in the range that is
risky. You can watch for visible signs of a low platelet
count, such as excessive bleeding, bruising, the small red
pin-dots (petechiae), and other typical symptoms of ITP.
The actual platelet level at which you take your child for
treatment is something to discuss with the hematologist or
other health professional. This decision should take into
consideration your child's activities and lifestyle among
other factors.
That depends on your child's platelet count and how ITP
affects his or her bleeding and bruising. Most experts advise
restricting contact and competitive team sports at platelet
counts of 30,000-50,000 cells per microliter or lower. This
is to keep the risk of internal injury and bleeding as low
as possible. However, most sports and activities are considered
okay. In general, it is important to encourage normal activity
and participation. You can ask your health care professional
for more specific guidelines. Your child should not take
aspirin or aspirin-related products such as ibuprofen.
Because of the risks associated with ITP, you should make
other medical professionals aware of your child's ITP. This
would include your child's pediatrician, dentist and any
emergency staff who may treat your child. The risk of excessive
bleeding or bruising and internal bleeding, though rare,
makes communication very important. If you do plan any dental
work beyond routine cleaning of your teeth, call your hematologist
first. He or she may ask you to get a platelet count if you
have not had one recently.
Some people wonder about this since ITP involves a child's
immune system. If your child is otherwise healthy, he or
she is no more likely to catch colds and viruses than other
children.
By itself, ITP is not known to change a person's life span.
You are likely to experience a whole range of emotions:
- Relief-that it wasn't a more serious illness like leukemia
- Anger-because this has happened to your child
- Helplessness-because you can't fix your child
- Frustration-because of the unknowns and the need to be
patient
- Worried, scared, and sad-for the pain and feelings your
child is experiencing
If your child's ITP goes away within a matter of days or
weeks and there are no other health problems, most likely
you'll feel great.
In that case, it may feel like you're on a roller coaster-going
through ups and downs with your child's platelet counts and
need for treatment. If your child responds well to treatment
and can carry on his or her normal life, you may begin to
feel that the immediate crisis has passed. The next challenge
is to "fold" ITP into your family's lifestyle as
much as possible. Blood tests can be scheduled as conveniently
as possible-closer to home perhaps, with results called in
to the hematologist's office-and around your family's activities.
That's not to say there won't be stressful times-just before
blood tests, trying to get time off for treatments, juggling
other children's schedules and child care-but it can feel
more "manageable."
A child with ITP will likely go through all the same emotional
ups and downs you experience:
- Scared-of tests, treatments, and IV needles; of dying;
of "catching" something else
- Guilt-thinking that he or she caused ITP to happen
- Embarrassed-to be "different" than other kids;
by lots of bruises
- Angry-at ITP and his or her body; at parents and health
professionals for "doing" this
- Frustrated-about restrictions on physical activities,
changes in routine; at the lack of "control"
over ITP
- Denial-of the reality of ITP and the changes to his or
her lifestyle it may require (especially true for teenagers
who may be tempted to ignore the advice of parents and
health care providers)
Talk about these feelings often. Listen carefully and let
your child know that these are normal feelings. Help your
child understand ITP by answering questions as honestly and
simply as possible. Allow your child to ask questions at,
and have some say about, the scheduling of his or her appointments.
Be sure that your child understands that he or she did NOT
cause ITP or any other illness that might develop as a result.
Offer your child a chance to express his or her feelings
through drawing, pretend play, and talks. Try to give your
child some sense of control over the management of his or
her ITP.
- Stress-from the change in routine
- Worry-for their sister or brother who has ITP; for their
own health and safety
- Mad or jealous-about all the attention their brother
or sister is getting
- Guilt-thinking he or she has caused the ITP; about being
mean or rude to the sister or brother who has ITP
Try to keep these children informed about ITP as well, and
help them find ways to express their feelings. Do what you
can to keep them feeling "special" too, so ITP
is not seen as a withdrawal of your attention and love.
As with any family issue, talking things over and finding
ways to express your emotions while on this "roller
coaster" is key. Many health professionals are available
to help you and your family sort these feelings out if you
feel the need. Many hospitals and clinics offer social support
services; this may be a good place to start.
Learning as much as possible can help too. There are a
number of sources for more information, a few of which are
detailed below:
- Your child's hematologist and other health professionals
- The ITP Society of the Children's Cancer and Blood Foundation, 111 West 57th Street, Suite 420, New York, New York 10019, 212-297-4336
- Libraries in your city, town, or in near-by hospitals
and medical universities.
- The National Heart, Lung, and Blood Institute Information Center, P.O. Box 30105, Bethesda, MD 20824-0105 301-592-8573
- The National Organization for Rare Disorders, P.O. Box 1968, Danbury, CT 06813-1968, 203-744-0100
- Health Data Brokers and Computer Bulletin Boards (These
networks and businesses provide information for a fee.
Your local library may have more specifics.)
The ITP Society of the Children's Cancer and Blood Foundation
is a non-profit organization dedicated to promoting the general
welfare of patients with ITP. The Society's goals are to
provide patient support and referral services, to support
ongoing research to advance the knowledge and treatment of
ITP, and educate the public community about this little known
disease. If you would like to join our efforts by becoming
a member, please contact us at the address below or email
us at info@childrenscbf.org
The ITP Society
c/o The Children's Cancer and Blood Foundation
111 West 57th Street, Suite 420
New York, New York 10019
A suggested membership fee of $25.00 per year can be sent
in a check or money order made out to The ITP Society. Tax
deductible donations above this amount are always welcomed.
Many thanks. If you have any questions please call us at
212-297-4336.
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