Children's Cancer and Blood Foundation
Answers to the commonly asked questions about Immune and Idiopathic Thrombocytopenic Purpura (ITP) in Adults and Children.
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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.
Idiopathic Thrombocytopenic Purpura
What is ITP?
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.
Why are platelets important?
Platelets are the microscopic parts of our blood that help stop bleeding and heal bruises.
What’s a normal platelet count?
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.
How can I be sure it’s ITP?
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.
Is it contagious?
No. And most children who may have had the same viral infection will not develop ITP.
Will it go away?
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.
How is ITP treated?
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.
Does ITP mean I need to restrict my child’s activities?
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.
OK. So what’s it like to deal with ITP?
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, 333 East 38th Street, Suite 830, New York, New York 10016, 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, please email us at info@childrenscbf.org