Sport and Children with Immune Thrombocytopenia: Never Give Up

Allowing children to participate in extracurricular activities, such as sports, helps them explore and develop skills which are considered beneficial throughout their lives. By participating in sports, children are exposed to various mental, social, emotional, physical, and educational benefits. The physical benefits of sports participation are obvious: it reduces the risk of obesity, improves cardiovascular performance, strengthens the osteoarticular system, and stabilizes the sleep-wake biorhythm. The psychological benefits are no less important: it develops self-esteem, facilitates social skills, and teaches the value of defeat (1). These benefits, both physical and psychological, also can intervene in the process of caring for a sick child by promoting healing and/or delaying chronic complications. Various experiences of physical exercise prescription in children with chronic diseases are known. For example, in children affected by cystic fibrosis, aerobic and anaerobic training programs can reduce the annual rate of decline in lung function. In juvenile idiopathic arthritis, resistance training can improve muscle strength and in children with an attention-deficit hyperactivity disorder, it can help with attention span and motor coordination (2). It is not always easy to start sick children in sport. Some pathologies are still considered in contrast with sporting activity. In particular, sport is often denied in children with diseases at risk of bleeding. We underlined, instead, the importance of physical activities in hemophilic children, and we tried to hypothesize a path that would allow children to practice sports safely (3). Primary immune thrombocytopenia (ITP) is an autoimmune hematological disorder characterized by a platelet count <100,000/μL. The goal in the management of children with ITP is to reduce the burden of therapies and hospitalization. Limited or very mild cutaneous hemorrhages must be treated with a “wait and see” approach. Pediatric ITP is usually of short duration, with at least two thirds of the patients recovering spontaneously within 12 months (4). However, 20% to 30% of children with ITP have chronic thrombocytopenia (more than 12 months) (5). The platelet count in ITP patients does not necessarily correlate with the bleeding risk (6), and signs and symptoms vary widely (7). One third of the children with ITP are asymptomatic, whereas the remainder may suffer from mild or severe bleeding manifestations (8,9). Treatment in asymptomatic or mildly symptomatic patients consists of observation, whereas patients with severe disease usually receive intravenous human immunoglobulin and/or steroids as first-line treatment and immunosuppressive agents (rituximab, mycophenolate mofetil, sirolimus) as second-line treatment (10,11). Splenectomy, while effective, is a third-line therapeutic option due to the postoperative risk of infection (12). Three obstacles limit sports activity in children with thrombocytopenia. The first issue is a lack of consistent scientific production to support the low risk of bleeding during sports activities. The International Consensus Report on the Investigation and Management of Primary Immune Thrombocytopenia, considered the leading document on this topic, does not cover the subject at all (10). The Italian recommendations for the management of children with chronic ITP do not give up on the subject. In the document, contact sports (combat sports, rugby, diving) are denied. Instead, it advises, after a case-by-case evaluation based on clinical history and psycho-physical condition, to practice noncontact sports with low traumatic risk (swimming, jogging, etc.) (13). The second point is the poor value attributed by doctors to the therapeutic role of sports activity in improving the quality of life and psychological health. People with chronic diseases suffer from a clinical condition known as “fatigue.” The “fatigue” symptoms are often reported by caregivers of thrombocytopenic children (14). Therefore, sports programs are fundamental in the process of the care for these children. Finally, some therapies could cause problems during sports activities. Indeed long-term corticosteroids therapies may increase the risk of traumatic fractures or can open problematic scenarios with antidoping rules. Otherwise the use of immunosuppressive agents contraindicates the frequency of crowded (locker rooms, sports halls) or damp (swimming pools) places. In recent years, a new class of drugs has risen to prominence in the ITP therapy: thrombopoietin-receptor agonists (TPORA). Two TPORA agents, romiplostim and eltrombopag, are FDA- and EMA-approved for the treatment of ITP. The main therapeutic approach of TPORA is to stimulate platelet production rather than modulating the immune system. Romiplostim is administered by subcutaneous injection and Eltrombopag is an oral drug (15). The increasingly constant use of TPORA in the clinical routine could facilitate access to children's sports in ITP. The use of TPORA allows us to answer each of the three issues described above. First, TPORA are usually used in a prophylactic way to prevent hemorrhage. The goal of TPORA is a platelet count around 50,000/μL. A platelet count around 50,000/μL is considered safe for surgery and guarantees a low risk during sport (16). Second, subcutaneous or oral administration reduces the impact of pathology and improves the quality of life. Children are free of hospitalization and can live an active social life. Third, TPORA are not steroid or immunosuppressive agents. TPORA's side effects do not include an increased risk of infection, and therefore, there is no limitation to frequenting schools or other crowded areas (17). Moreover, TPORA are not listed in the World Anti-Doping Agency list of prohibited substances and methods updated in 2019 (18). In conclusion, sports participation in the children at risk for bleeding has clear health and psychosocial benefits but can be associated with harm. When counseling patients at risk for bleeding, decision making should be patient-centered and the relevant variables including disease, sport, and patient-related factors considered. For patients with ITP, management of the psychosocial stressors caused by removal of high-risk sports participation is imperative. To maximize injury prevention, it is important to choose a sport wisely, manage the coagulopathy during the highest risk times, apply appropriate athletic techniques, and always wear appropriate safety gear (19).