ITP is a very rare disease that only affects 200,000 people each year, half of them are children. ITP stands for Idiopathic Thrombocytopenia Purpura. Idiopathic indicates that the cause of the illness is unknown;
thrombocytopenia means that there is a decrease in the number of platelets, and purpura refers to the purple discoloration of the skin. ITP is a non-contagious auto-immune disease that causes a person to bruise and bleed easily. In ITP the platelets are the target of the immune system. This compromises the both the production of platelets and the number of platelets circulating the blood. This disease primarily affects children although it can strike at any age.
The exact causes of ITP are unknown; however some doctors believe that it may occur after a viral or bacterial infection, a vaccination, or an exposure to a cytotoxic agent. The immune system marks the platelets a “foreign invader” and the platelets are destroyed or “eaten” by special white blood cells called macrophages and T-cells. These cells destroy the platelets in the blood and they get eliminated from the body by the spleen. There are two types of ITP, acute and chronic. Acute ITP is often linked to an infection, is self limiting, and is the most common type among children. Chronic ITP is always of an unknown origin and usually affects adults. It is important that the parents understand that there is nothing they did or could have done differently for their child to not have this disease.
Before a parent can understand the full spectrum of their child’s illness it is important that they understand normal. A normal platelet count for anybody, even children is 150,000 to 400,000 platelets per milliliter of blood. A child that has ITP can have platelets counts of 2,000 – 3,000 platelets per milliliter. Although a platelet count of 30,000 is enough to prevent severe bleeding in children. The goal of all treatment is to protect the child from severe bleeding.
Parents tend to ignore most of the symptoms of ITP until they are more severe, because most of the symptoms are very vague, and most children get bumps and bruises from normal play. This leads to the child being at a much higher risk of severe bleeding at the time of diagnosis. All of the symptoms associated with are ITP are related to the low platelets and bleeding. Parents will see easy bruising, small red pinpoint spots (called petechiae), or purpura. Purpura look like small to medium blood blisters that form under the skin. The child might have cuts that take much too long to stop bleeding, unexplained bleeding from the nose or mouth/gums. The parent might also notice blood in the urine, stool or vomit. If a child has any of these signs they should be seen by a doctor right away.
A Hematology doctor or blood doctor is usually needed to diagnose ITP. Because of its rarity most doctors have never seen a case of it. The only way to have definitive diagnosis is to do a bone marrow aspiration. This will rule out all other possibilities such as leukemia and cancers. The doctors will also run several blood tests and a urine analysis. the blood studies will show a count of the cells in the blood and how long it takes for the blood to clot. With the bone marrow aspiration the doctors are looking to see if platelets are being produced, because one of the problems with ITP is that the bone marrow stops producing the platelets.
The goal of all treatment is raise the platelets to safe level and restore a normal or near normal lifestyle. The treatments for ITP vary depending on the severity and how the body reacts to the treatment. The primary course of treatment is high dose corticosteroids, also known as steroids, primarily they use prednisone. They will also try Gammagloblins such as IVIg or IgG. Immunosuppressive drugs are also frequently use in conjunction with steroids or IgG. Immunosuppressive drugs are often called Chemotherapy drugs. There is also a new medication that is being tested by the FDA called Eltrombopag. It is still in the final stage of the clinical trials, but shows a great outcome. The last and final treatment that is done when nothing else has worked is to do total Splenectomy or removal of the spleen. The goal of all treatment is raise the platelets to safe level and restore a normal or near normal lifestyle. The goal of all of these treatments is to suppress the immune system to a point where it can “reboot” and not have a tendency to attack platelets.
How a child’s life will be affected by the treatment is highly dependent on the treatment. A child with ITP can live a normal life (i.e. attend school, play non-contact sports) unless platelets drop below 20,000. some of the side effects that might be seen with the prednisone treatment are moodiness and irritability, they may develop a puffy face, have frequent urination increased appetite, and weight gain. When taking prednisone long term they will have growth impairment, they may not reach a full expected height. When taking the immunosuppressive medications the child will have side effects that similar to chemotherapy treatments. This is because a lot of the same medications are used to treat ITP as many cancers. Some of those side effects include weight loss, anorexia, hair loss, and over all ill feeling. When a splenectomy is done the child will have to be cautious of becoming sick. With a splenectomy the child is extremely immune-compromised and cannot fight even a simple cold.
ITP will affect a child in many aspects of their life, but it also depends on the age of the child when they get it. As a preschooler they may never remember having it, but a school aged child or adolescent will remember what it was like. It is a scary process to go through. I hope that one day they will find a way to treat this without all the side effects that the disease and its treatments bring.
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