ITP in a 4 Year-Old Boy Who Recovered Without Specific Treatments
Seth was taken to the Emergency room when he was 4 years old because of bruises that covered his body. The bruises had been notable for about two weeks and started only where he usually had them, such as on his shins. His parents grew concerned however when they continued and appeared on his arms and back. When he developed “little red spots” all over his body the family took him to the local emergency room. It was late in the day when they took him to the crowded emergency room and had to wait for test results. When the results came back, a complete blood count (CBC) showed that Seth had very few platelets. Seth’s parents were told that based on his labs and physical exam this was “possibly leukemia” and to take him immediately to the emergency room at the Children’s Hospital.
When Seth arrived at the Children’s Hospital it was now midnight and he still had to undergo more exams and laboratory tests by the new doctors. His parents waited without a clear diagnosis and feared the worst. “We feared that this was leukemia and that Seth would die,” his Dad recalls. Fortunately, when the laboratory tests came back the only thing wrong with Seth was an extremely low platelet count (3,000, normal is over 150,000) and nothing else. Seth was diagnosed with ITP and told to follow up in the hematology clinic in the morning. The doctors spoke with the hematologist on call and decided it was not necessary to give Seth any medicine since he had no major bleeding.
In the morning Seth came to the hematology clinic. He had no new bleeding although he was still covered in bruises. His platelet count was 6,000 and his blood looked normal under the microscope except for the low number of platelets. He had a normal red blood cell count and a normal white blood cell count. On exam Seth had no enlargement of his liver, spleen, or lymph nodes. Seth did indeed have ITP. There was no evidence for leukemia either on the examination or from the laboratory data. The doctors spent a lot of time during that visit reviewing the diagnosis of ITP with Seth’s family and talking about the treatment options available for a boy like him. Different medications were discussed that might increase Seth’s platelet count right away; however the doctors explained that there was an excellent chance that Seth would get completely better without any medicine, probably within 2 months. In addition, the family understood that many of the medications were associated with side effects such as; severe headache with IVIG, the breakdown of red blood cells leading to anemia with anti-D, and the increased moodiness and nausea that can accompany steroid use. Given all of this information and the fact that IVIG and anti-D are very expensive, the family decided that they would rather watch Seth and not give him medicine at that time. “I was glad to hear that just closely watching him would be his treatment. I think that medications are many times given unnecessarily,” his Dad said, thinking back on the decision. His parents stated, “We felt reassured that this was something that could be outgrown and that there were medications available if necessary to boost his counts.”
The parents received an informational booklet about ITP that described the diagnosis of ITP. It also had important information on things to avoid when the platelet count is low, like aspirin and ibuprofen that can decrease the functional ability of the few circulating platelets, rough housing, and contact sports. Reasons to call the doctor were outlined along with the appropriate phone numbers of the staff. All this information was useful in reassuring the family and providing education, however the family still feels that the best thing was the time the doctors spent with them reviewing all the possible treatment options. They knew that they would have to keep him from rough housing at home and give him options for more “quiet activities”, but thought this was the best thing for him. Seth continued to go to his regular nursery school and the parents spent time going over the diagnosis and restrictions with the teachers. His Dad stated, “Boys will be boys. Care and explaining to everyone (family members, friends, and school staff) about his condition was important. I was very, very protective at first. Seth acted perfectly normal when he was diagnosed with ITP so it was hard for him to understand why we would restrict him from certain activities. He said, ‘I feel fine, Dad, I’m OK!’.” Despite how good he felt he was restricted from bike riding, playground activities, rough housing with his brother, and recess during preschool.
Seth’s platelet count was measured again in one week and then every couple of weeks and continued to be low. Despite this he had only mild bruising and no serious bleeding, such as nosebleeds or bleeding from his gums. The family stood by the decision to avoid medication and watch him. He did so well that even though we had established a return appointment in one month, he was lost to follow-up.
Three months after diagnosis Seth returned to clinic because he needed to have some dental work performed, and the dentist wanted to make sure the platelet count was normal. At that visit his platelet was 62,000. While this was much better, and at a level that is safe and can prevent any serious bleeding, it was still below normal. Seth had now had ITP for 3 months, but had recently not even noticed that he was any different than other children. He was not bruising at all, at least not more than normal. Seth had his dental work done without any medications or bleeding problems.
Seth came back 1 month later for a check-up and much to everyone’s delight had a normal platelet count of 243,000. So Seth has had a full recovery from his ITP with watchful waiting as his treatment. Seth is now a healthy six-year-old boy active in baseball and tennis. He is doing well in school. Because of his diagnosis he learned about hospitals and what they do. Looking back on the whole experience his Dad said, “He had no side effects and did well. This is why I’m glad ITP can be treated with close monitoring rather than medication.” The family says that they would have done everything the same way again.
Seth Update: February 2011
Seth continues to do well and has not required any further visits to our clinic or treatment for his ITP. The family knows that they can always call us if they notice that he starts bruising or bleeding again, but fortunately this has not happened. Seth is now a healthy and active boy who only sees his doctors for regular check ups.
Comments by Dr. Cindy Neunert
Seth’s story illustrates the natural course of most cases of ITP in children. ITP is one of the most common disorders bringing children to the attention of a pediatric hematologist. The majority of children with ITP will have bruising and petechiae like Seth, but more significant bleeding is uncommon. The disorder can be alarming when children are first seen because of the underlying fear that the child has leukemia or is at risk for serious bleeding. In Seth’s case this is why he was transferred to the Children’s Hospital for evaluation in the middle of the night. His parents recall very clearly the fear that their whole life was about to change.
Seth’s case also illustrates several points about the management of children with ITP. First, the diagnosis of ITP can be made by physical exam and evaluation of the blood under the microscope. But this requires a physician who is experienced in the diagnosis of ITP. In a typical emergency room, the most serious potential problems are considered first, just as leukemia was first considered when Seth first went to the emergency room. In most cases a bone marrow examination to rule out leukemia or cancer is not needed if everything else is normal. Seth’s parents felt comfortable because the doctors spent a lot of time with them explaining the diagnosis and management options. While there are medications available to help with low platelet counts, often what parents need is just reassurance. In this case, education helped the parents feel comfortable taking Seth home without medication. Treatment with drugs should be based on a discussion with each family and evaluation of the amount of bleeding the child is having. While Seth clearly illustrates the case of a child who did not have severe bleeding, some children with ITP will have more significant bleeding when they are seen by the doctors. If that is the case or the family is less comfortable with the risk of bleeding, then I am more likely to recommend therapy. In addition, for families that live far away, don’t have adequate access to transportation or medical care, or those who I am not certain will call with a significant change, medications can offer an immediate increase in platelet count and decrease in anxiety over the possibility of significant bleeding.
Seth is a delightful little boy who despite having ITP was completely unaware that he was “sick”. Like most children with ITP, what Seth really needed was time. Most children will recover and never have any further problems after several months. While it took Seth longer than most to recover, his ITP “disappeared” in the end. In my experience, ITP will completely resolve in most children Seth’s age within approximately 6 to 8 weeks. He is now a youngster who is doing great and enjoying all of his normal activities. He is full of energy and keeping his parents busy. Seth has a very small chance (1-2%) of having another episode of ITP and is not at risk for other blood disorders or cancers because of his ITP. Unlike adults, most children Seth’s age with ITP usually resume their normal childhood activities and have only memories of visiting the hospital.
While the most common age group of children with ITP is between 2 and 8 years of age, ITP can occur in older children and adolescents. These patients can present a unique treatment challenge in that they are “little adults”. Older children can often times have a clinical course very similar to that of adults, with slower onset and a more “chronic” course. It is important to remember that each child is different and continued communication with your doctor is the most important part of your child’s treatment.
Seth’s ongoing story brings to light a very important fact about ITP in children. Full recovery from ITP occurs in most children and recurrence is rare. Full recovery requires documentation that the platelet has risen to the normal range without recent medication. Even in children with ITP who seem to recover quickly, we request another platelet count six months following diagnosis to be certain that all is well. At that time, once we have documented two normal platelet counts, we feel comfortable that the children should not have more trouble with ITP.
Despite being told that the platelet is now normal most parents wonder what having ITP in the past means for their child’s future. For the most part we think that ITP does not lead to any problems in the future. ITP does not turn into a more serious blood disorder, like leukemia or aplastic anemia. It is usually not a sign that their child will later develop other autoimmune conditions, such as systemic lupus erythematosus (SLE or “lupus”). While we believe that ITP is truly gone forever and will not recur in the majority of children, about 1 to 3% of patients many months to many years later exhibit petechiae, bruising, or other bleeding, and again have marked thrombocytopenia. Most of the time the cause of the new episode, just like the cause of the initial event, is unknown. Some children have been reported with as many as four or five separate episodes of “acute ITP”. It is likely that these are not separate events, but rather that these patients had borderline thrombocytopenia some or much of the time without any bleeding that brings them back to the doctor. We are currently trying to study this at our center by following children with resolved ITP for a long time to see if the platelet counts go below normal even after the first episode of ITP is considered resolved. There are even cases where a person with apparent acute ITP as a child develops such a recurrence 20 or 30 years later or rare cases where pregnant women who had ITP during childhood give birth to babies with temporary thrombocytopenia.
Because ITP can sometimes recur we always tell parents to contact us if they notice that the child begins to bruise again or has any other bleeding. Usually this is easy as children are active and therefore any increase in bruising would be easily noticed by the parents. Because the risk of ITP returning is so low, children with resolved ITP do not need to continue to be seen by a hematologist and should resume care with their regular pediatrician for routine well child visits.