My Experience with Acute Childhood I.T.P.

While many people have never heard of ITP, Immune or Idiopathic Thrombocytopenic Purpura, approximately 200,000 people in the U.S. suffer from the disease. ITP is an autoimmune disease that affects all age groups. And the cases are increasing- there are 20,000 new cases each year (“About ITP”). The scariest aspect of this odd disease is that the trigger is usually a mild illness, such as the common cold or vaccinations (“FAQ-ITP in Children”). I had never even heard of ITP until I my seven-year-old daughter exhibited the strange symptoms.

One morning my daughter said she felt a lump inside her cheek. I looked inside her mouth and saw a quarter-sized black lump. I asked her if she had been chewing on any pens. Then my husband suggested that maybe she bit her cheek during the night. We sent her to school and thought nothing of it. During the day the lump popped and her mouth was filled with blood. She rinsed her mouth out with cold water, and again went on with her day. She felt fine.

The next morning she woke up with two more lumps on the other inside cheek. I panicked, thinking, “Oh my God, they’re multiplying.” Then I noticed blood was floating in the white of her eyes. We immediately brought her to the pediatrician. When removing her pajamas to help her get ready, I noticed petechiae (pronounced pe-TEEK-ee-ay), bluish-red spots the size of freckles, covering her body. We raced to the doctor, trying to hide our anxiety from my daughter, who still was feeling great.

In the office, I let out some of my fear by joking with the doctor, “You have to help me. My daughter is exploding!”

He examined her, noticed even more bruises on her body, took blood, and said he would run some tests. For a brief moment I wondered if he thought we abused her since she looked like she had been through a traumatic accident. But when he returned, he explained that she had ITP. Her platelet count was 20,000. Normal platelet count ranges between 150,000 – 450,000 per micro liter of blood (“FAQ-ITP in Children”). He said if her white count had also been low, that would have been a sign of leukemia; however, since her white count was fine, he believed it was ITP.

I felt my breath escaping in slower and slower intervals. Since he was comparing ITP and leukemia, ITP suddenly became more serious to me. He tried to calm our nerves by explaining that ITP is not fatal, and that 80-90% of all cases are acute, meaning they last one to three months and then the patient recovers completely (“FAQ-ITP in Children”). Usually the older the patient, the more chance it will lead to chronic ITP.

The doctor then asked if my daughter had a cold recently. She had. He explained further that often colds trigger acute cases of ITP. Normally, the immune system fights off cold viruses. In ITP, the immune system becomes “confused” and believes that a person’s platelets are also viruses. Therefore, antibodies attack the platelets, and the spleen destroys them, slowly depleting the platelet count.

Since platelets are responsible for clotting the blood, the initial symptoms in my daughter were all typical signs of ITP. Her blood was not clotting properly, and since the mouth and eyes have the most sensitive blood vessels, those are the first to break. The petechiae and other bruises on her body were also typical signs; the smaller blood vessels in her skin were breaking all over her body.

Again, the doctor reassured us that while she appeared injured, all the symptoms were only superficial, neither permanent nor a serious risk to her health. However, he said that even in acute cases, the symptoms usually get worse before they get better; often the platelet count drops even lower. The lower the platelet count, the more chance of serious internal bleeding. If she was to have a serious fall or get into a car accident, she could bleed to death internally. Head injuries were particularly dangerous because she could bleed into her brain with no way to stop it. Because of this danger, he sent us to Children’s Hospital in Boston.

Lucky for us, Children’s Hospital in Boston is only an hour’s drive from our house and it has a wonderful Hematology department that is currently running a national ITP case study. They have a comprehensive program dedicated to “to treat, educate and participate in research related to the management of ITP in children”. We couldn’t have been in better hands. We arrived at 1:00 p.m. Unfortunately, we had to sit and wait for a series of tests at approximately three-hour intervals, while a team of doctors made decisions after each step.

I wish they had admitted us to the hospital. Instead, they gave my daughter a temporary bed in the emergency room wing. They withdrew blood from my daughter at least eight times that day while she screamed hysterically before they decided to insert an IV where they could more easily, and gently, take blood when necessary. To this day, my daughter still fears needles.

Her pediatrician had been right; her platelet count slowly lowered hour after hour, until it was around 1,000. She still felt fine, but when she would smile all her gums would bleed, making her teeth look brown. My heart broke. She had nosebleeds as well, and bruising where the doctors took blood. At 1:00 a.m., twelve hours later, the doctors gave us our options. We could (A.) go home. Many hospitals across the country send children home with no treatment and simply tell parents to make sure the child does not suffer any traumatic falls. Some younger children are told to wear helmets while playing. The hope is that the body will eventually realize its immune malfunction and correct itself. Doctors reexamine the platelet count in 30 days, and if there is still no change, then other options are considered.

I wanted to be more proactive. Option (B): I had the choice of three medical treatments. First, they could give her a dose of Anti-Rho D immune globulin, also known as WinRho®, a liquid concentrate of antibodies from blood donors that tricks the body into temporarily attacking red blood cells and forgetting about platelets. Unfortunately it can result in anemia and cannot be administered to people who are Rh-negative (“FAQ-ITP in Children”).

Second, Prednisone, a synthetic steroid that increases platelet count but can only be given for a few weeks because it can have serious side effects related to steroid use. It can also lower the body’s ability to fight new infections (“FAQ-ITP in Children”).

Third, Intravenous Gamma Globulin (IVIG), a liquid concentrate of antibodies from blood donors that rapidly boosts platelet count. Unfortunately, it only boosts platelet count for a short time period, so the medication must be administered for several hours a day for five days (“FAQ-ITP in Children”).

We chose WinRho. We didn’t feel extremely confident in our choice; we were trying to pick the choice with the least side effects and risks. We were afraid to give her a steroid, although it is a popular choice, and the administration of the IVIG was too cumbersome. While we were nervous about giving our daughter WinRho, a blood product that would “attack” her red blood count, we were assured that she had plenty of red blood cells and that losing some would not pose a serious risk. I also felt reassured knowing that WinRho is also the same drug they administer to women during labor when the Rh-factor differs from the baby.

Regardless of our insecurities, we were more nervous about risking our daughter to possible internal bleeding. A nurse administered the WinRho through her IV for about 30-40 minutes. Soon after, we were discharged at 3:30 a.m. My daughter did vomit on the ride home, one side effect of WinRho.

The next few mornings she woke up with nosebleeds. We kept her out of school for a week, but her pediatrician said that she could return to school as long as she didn’t participate in gym for a few weeks. We were nervous, walking around like she was made of glass. We brought her back to school and spoke to the school nurse. The nurse had never heard of ITP, so I gave her all the pamphlets from the Children’s Hospital and explained that she developed a clotting disease that would hopefully go away with time. I told her my daughter could not have any medication that thins blood, such as ibuprofen and certain cough medicines, such as Robitussin. If my daughter suffered from any falls, they would need to call me at work. If she fell and banged her head, she would need to be rushed to the hospital.

While we kept raising red flags at school and with relatives, we annoyed my daughter. She wanted to go back to normal; she still felt fine, showing no new symptoms, such as crankiness and inability to concentrate in school, that can present themselves. I work in a small high school, and two high school students approached me to say they had ITP a few years earlier. They tried to ease my nerves. I was relieved to hear that these teenagers had gone through this and were doing fine, and yet I was surprised to know two more people who had a disease that most people, including many in the medical community, know little about.

Slowly a month went by and we returned to the Boston Hematology Clinic to check our daughter’s platelet count. It was at 50,000, a disappointing number, but at least she was out of the spontaneous bleeding zone (under 50,000). However, WinRho lasts in the system for approximately 30 days, so the next monthly appointment with her pediatrician would determine whether or not her body had figured out its error without the aid of medication to artificially boost her platelets.

A month later, her pediatrician said my daughter no longer had ITP. Her platelet count was at 300,000. He said there is only a 5% chance of acute ITP returning. A year later, my daughter is doing fine with no signs of ITP. She still fears needles, and on a recent trip to the New England Aquarium, she was afraid we were bringing her back to the hospital.

While our daughter is now free from ITP, there are many children and adults still suffering from it. If ITP lasts beyond six months from the time of diagnosis, it is considered chronic ITP. Persistent bleeding in patients may be assessed for the risks and benefits of an operation to remove their spleen (splenectomy) (“FAQ-ITP in Children”). In any case, knowledge is power to ease the fear of this little known disease that is spreading.

For more information on ITP, visit:
1. pdsa.org

2. itpkids.org
3. nhlbi.nih.gov