Chapter 31: Pediatric Hematological Problems

Sickle cell anemia, an inherited hemoglobinopathy, occurs when abnormal hemoglobin S causes red blood cells to become rigid and occlude capillaries, particularly under hypoxic conditions triggered by fever, dehydration, or stress. Four distinct crisis types emerge: vaso-occlusive events causing severe pain and ischemia, splenic sequestration leading to acute anemia and hypovolemia, hyperhemolytic episodes with accelerated cell destruction, and aplastic crises from viral infection or nutritional depletion. Management centers on aggressive hydration, oxygen supplementation, scheduled analgesics while avoiding contraindicated agents, blood transfusions, and hydroxyurea therapy. Hemophilia, an X-linked recessive disorder affecting coagulation factor VIII or IX, manifests as abnormal bleeding, easy bruising, and hemarthrosis requiring factor replacement, immobilization, and activity modification. Von Willebrand disease similarly involves defective von Willebrand factor affecting platelet adhesion and factor VIII transport, presenting with mucosal bleeding and treated through factor administration. Beta-thalassemia major, an autosomal recessive globin chain deficiency prevalent in Mediterranean populations, causes severe microcytic anemia and characteristic facial features, managed through chronic transfusion therapy and iron chelation to prevent organ damage from transfusional iron overload. Iron-deficiency anemia, frequently caused by excessive cow milk consumption in children, requires oral supplementation administered carefully to avoid dental staining and gastrointestinal upset. Aplastic anemia results from bone marrow failure producing pancytopenia across all cell lines. Throughout these conditions, nursing priorities emphasize maintaining adequate perfusion, preventing complications, providing family education on genetic inheritance patterns and activity restrictions, ensuring appropriate immunizations, and supporting psychosocial adjustment to chronic disease management.