CONTENTS:
Childhood ITP: School, Sports, Treatment
By Jennifer DiRaimo
As schools open their doors for another academic year, PDSA Medical Advisor Michele Lambert, MD, offers her guidance on childhood ITP, including strategies for managing ITP in school, sports, and treatments. Dr. Lambert is a pediatric hematologist and member of The ITP Pediatric Consortium of North America (ICON) and an Associate Professor of Clinical Pediatrics at the Children’s Hospital of Philadelphia, Medical Director of the Special Coagulation Laboratory and Director of the Pediatric Platelet Disorder Program at the Children’s Hospital of Philadelphia.
Dr. Lambert explained that due to different underlying causes of immune dysregulation involved in reducing platelet count, individuals with ITP may not respond the same to available treatments. Parents and caregivers of children with ITP often need to try more than one treatment before finding one that works well for them. Dr. Lambert described how one of the main concerns for health care providers is when to administer treatment to a child with ITP. Since treatments work differently in everyone, and most treatments cause harmful side effects, treatment is usually reserved for children that have difficult to control bleeding, bleeding in protected spaces such as the brain or other internal organs, or if they cannot participate in daily activities or their overall health related quality of life is significantly suffering. Dr. Lambert indicated that ultimately the risk for side effects must out-weight the risks associated with not treating. Most children with ITP only experience mild bleeding symptoms usually restricted to the skin, such as petechia and bruising. Therefore, treatment, frequent platelet count checking, and severe activity restrictions may not be necessary.
Dr. Lambert noted that even among hematologists, there is disagreement surrounding what activities a child with ITP should avoid, and why. Recently, she and a colleague (Dr. Char Witmer) at CHOP examined their institutional experience of patients with ITP and activity participation. The results of their study were published in September 2023. They found that in their group of 102 patients with ITP, there was minimal risk associated with sports participation for most sports in the setting of ITP. The majority of children participated in organized sports safely and most of the injuries that occurred in ITP happened during unstructured play. Only one injury in an ITP patient needed further ITP treatment (a child with platelet count <20,000 with head trauma who was treated and evaluated but did not have any further issues). Overall, this study suggested that organized sports participation, especially in sports that were not high-risk contact sports like football and ice-hockey, was likely safe for most patients with ITP. In addition, the study shows that children can take part in gym class at school and otherwise be treated normally.
Most of the existing guidance on limitations are based on those used for hemophilia patients, a very different blood disorder with very different bleeding risks. Risk categories is a reasonable way to start conversations about risk/benefit of playing particular sports with your doctor and also provides some evidence for the safety in playing types of organized sports in the setting of an ITP diagnosis.
Dr. Lambert stated that too many restrictions could negatively impact a child’s quality of life. Generally, Dr. Lambert explained that physical activities that don’t have a high risk for head trauma or a concussion are encouraged with safety measures in place as appropriate. For instance, if riding a bike wear a helmet to protect the head. She also mentioned that treatment, even if short-term, may be appropriate to increase platelet counts during a particular sport season if the risk of avoiding the sport causes more psychological harm.
At school, children with ITP are encouraged to participate in field trips, recess, and gym class. Dr. Lambert stressed the importance of not removing too much from children and encouraging them to get exercise. However, depending on their propensity to take risks and/or how they tend to play with others, restrictions on an individual basis may need to be made on the school ground. Dr. Lambert encouraged parents/caregivers to talk to their child’s school so they know what concerns to look for, and ensure there is a plan in place on how the child with ITP will be safely monitored. She also suggested that for newly diagnosed children, new and/or high-risk activities should be avoided. If children have chronic ITP, a risk-benefit framework should be applied when determining what activities can be safely encouraged in addition to having an in-depth conversation about potential risks to determine what is appropriate for your child. For instance, Dr. Lambert advises that children with ITP may need to be aware during school trips to an amusement park that certain rides may not be safe, such as roller coasters with high G-forces (induction roller coasters for example).
Dr. Lambert ended her talk by providing a summary of what physicians do know about ITP. While children who present as teenagers may be more likely to have chronic ITP, every child is unique and it is still impossible to really tell with any child on the day they are diagnosed how their ITP journey will proceed. Most children with ITP do not have severe bleeding, and the risk for life-threatening bleeding is very low. She discussed how bleeding, once it starts, becomes more difficult to control the longer it persists so it’s important for parents/caregivers to talk to their children so they know to report an injury immediately so that it can be evaluated promptly if appropriate, and to establish trust within the family so the child is comfortable saying when they have “broken the rules” about restrictions or gotten hurt.
She also urged parents to always let their child’s physician know when they see changes in their child’s behavior such as excessive sleepiness (especially if it’s coupled with a persistent or fluctuating headache, nausea, vomiting and a low grade fever, as this could be early warning signs of a brain bleed), new ITP symptoms arise, or when bleeding patterns worsen so the cause can be investigated and management can be adjusted. Dr. Lambert noted that bleeding in ITP is usually slow, even in protected areas, so there is often time to intervene if you act immediately. She urged parents to bring their child to emergency, particularly if they have had a head injury, and are showing signs of fatigue and nausea and vomiting. These signs should be taken seriously regardless of whether or not a fever or headache is present, because these could be symptoms of an intracranial bleed. If a child is bleeding, sometimes it takes a ‘kitchen sink’ of treatments to find the right one to elevate the platelet count to stop a terrible bleed.
It was suggested that secondary causes of ITP should be explored if their child is refractory or unresponsive to common ITP therapies. If there is a secondary cause, treatment can be targeted for a more successful response. Lastly, Dr. Lambert mentioned that every effort should be made to normalize a child’s life as much as possible including reasonable accommodations to mitigate risk.
For more information and resources on ITP in children and teens, click here.
Ask The Experts
This month’s e-News features patient questions about various topics important to the ITP and other platelet disorders community.
PDSA medical advisors Howard Liebman, MD; Diane Nugent, MD; John Semple, PhD; Craig Kessler, MD; David Kuter, MD, DPhil; Terry Gernsheimer, MD and Ilene Weitz, MD offer their guidance.
QUESTION:
What are some suggestions to help increase an adult’s fatigue or loss of energy when they have ITP? My doctor suggests exercising more. However, I’m so exhausted I don’t feel like I have any energy to exercise and all I want to do is sleep.
ANSWER:
There is no standard answer that works for everyone, Dr. Kessler states, it has to be individualized. In his practice, he first assesses possible explanations for the fatigue. Does it occur at a certain time of day? Is it due to insomnia from prolonged steroid use? Has the patient developed sleep apnea due to weight gain? Is it related to depression symptoms? Afterward, if the fatigue is severely affecting their quality of life, one strategy he suggests is to prescribe Modafinil, a drug that helps energize patients during their awake hours. Another strategy is to use a treatment called Ritalin at low doses, which can help with alertness.
Dr. Liebman also urges patients to remember that there are real physiological mechanisms for fatigue that are related to your immune system. He alludes to studies that have shown that fatigue is strongly associated with levels of interleukin-6, an immune hormone that is increased in inflammatory conditions. Dr. Kuter also mentions that having access to palliative care specialists to help with fatigue is helpful. To conclude, Dr. Weitz encourages that although exercise may improve fatigue, you should focus on those that are compatible with your unique lifestyle and platelet count.
QUESTION:
What is the mechanism of cyclic thrombocytopenia?
ANSWER:
Dr. Semple explains that cyclic thrombocytopenia is so named because it comes and goes over periods of time. However, the immunologic basis of these cyclical patterns has not been well researched. Dr. Gernsheimer cautions that women may experience cyclical thrombocytopenia due to hormonal changes. Sometimes, the cyclical patterns may not be physiological, Dr. Kuter explains, but rather due to the inappropriate use of thrombopoietic agents. For example, if a patient’s platelet count is responding well to Nplate®, they may be taken off therapy. However, this risks their counts dropping back down right after. Particularly for Nplate®, but also for Doptelet® and Promacta®, doctors should not take a patient off treatment immediately after they respond, but rather slowly decrease the dose. Dr. Nugent also explains that patients can develop antibodies that can destroy their anti-platelet autoantibodies (called anti-idiotype antibodies), which may provide a physiological explanation to cyclic thrombocytopenia.
If you have a question that you would like answered by one of PDSA’s medical advisors or other experts, please email your question to: pdsa@pdsa.org with “Ask the Experts” in the subject line.