Chapter 44: Hematologic Disorders in Children Nursing Care

The crucial role of the hematopoietic system in oxygen transport, metabolism, and coagulation is explained, detailing the formation and function of erythrocytes (red blood cells), leukocytes (white blood cells), and thrombocytes (platelets) within the bone marrow. The physiological shift from fetal hemoglobin to adult hemoglobin around six months of age often reveals inherited beta-chain disorders like sickle cell anemia and thalassemia. Nursing care is guided by the Quality and Safety Education for Nurses (QSEN) competencies and Healthy People 2030 goals, emphasizing patient-centered care, preventing complications such as infection, and promoting seamless transitions across healthcare settings. Diagnostic procedures such as bone marrow aspiration (commonly performed on the iliac crests in children), along with major treatments like blood transfusions (packed red blood cells are most common) and hematopoietic stem cell transplantation (which can be allogeneic, syngeneic, or autologous), are detailed, including necessary infection control precautions and management of potential complications like graft-versus-host disease (GVHD). Key disorders of red blood cell production and function are categorized, including hypochromic microcytic anemias like iron-deficiency anemia, which is the most common anemia of childhood and is often caused by low iron intake or excessive milk consumption in toddlers. Iron-deficiency anemia is treated with oral ferrous sulfate and specific dietary counseling, including the ingestion of vitamin C to enhance absorption. Normochromic anemias, such as acute blood loss and aplastic anemia, involve impaired production of cell elements by the bone marrow and may require immunosuppression or transplantation. Hemolytic anemias, characterized by increased cell destruction, include the inherited conditions of congenital spherocytosis and G6PD deficiency, as well as autoimmune acquired hemolytic anemia. The severe hemoglobinopathy of sickle cell disease, prevalent in Black populations, involves a defect in the beta chain that causes red blood cells to sickle under low oxygen tension, resulting in painful vaso-occlusive crises. Treatment for a crisis includes pain management, intensive hydration, and oxygenation, while long-term management may involve hydroxyurea. The thalassemias, inherited disorders common in individuals of Mediterranean descent, cause severe anemia and significant iron overload (hemosiderosis) due to frequent transfusions, necessitating chelation therapy using agents like deferoxamine. Finally, coagulation disorders are addressed, including purpuras like Idiopathic Thrombocytopenic Purpura (ITP), an autoimmune condition often following a viral infection, and the X-linked recessive hemophilias (Factor VIII and IX deficiencies), which require prompt factor replacement therapy to control bleeding and prevent severe joint damage (hemarthrosis).