Hematological Alterations

1. Hematological Alterations NUR 264 Pediatrics Angela Jackson, RN, MSN

2. Hematological Alterations: Developmental Differences • During fetal development, blood cells are produced in the liver and the spleen • In the newborn, blood cells are produced from stem cells in the bone marrow • Full- term newborns are able to store iron in the bone marrow and liver tissue for up to 20 weeks • Premature newborns may use up their iron stores within 6-12 weeks

3. Iron Deficiency Anemia • Most common hematologic disorder of infancy and childhood • The body does not have enough iron to synthesize the hemoglobin necessary to carry oxygen to the tissues • Affects 9% of toddlers 1-2 years of age • Affects 4% of children 3-4 years of age • Incidence rates are higher in low income families

4. Iron Deficiency Anemia: Pathophysiology • Dietary iron is absorbed in the small intestine and is either passed into the bloodstream or is stored as ferritin • Iron in the blood stream binds to transferrin and is delivered to the bone marrow, where it is combined with other components to form hemoglobin • Iron may also be recycled from RBC’s that have been removed from the bloodstream and catabolized • When a child does not ingest enough iron, hemoglobin synthesis is impaired • When a child experiences blood loss, there are fewer RBC’s to be catabolized and the iron in these cells cannot be recycled

5. Iron Deficiency Anemia:Clinical Manifestations • Mild anemia: • Generally asymptomatic • May experience symptoms of moderate anemia during exertion • Moderate anemia: • Shortness of breath • Rapid, pounding heart beat • Dizziness, fainting, lethargy, irritability • Severe anemia: • Cardiac murmurs • Pale skin, mucous membranes, lips, nail beds and conjunctiva • Thinning and early graying of the hair • Abdominal pain, nausea, vomiting, anorexia, low-grade fever • Decreased physical growth, developmental delay

6. Iron Deficiency Anemia:Diagnosis • History and physical exam • Laboratory studies: • Decreased hemoglobin, hematocrit, MCV(RBC volume), MCH(weight of Hb within a RBC), MCHC(concentration of Hb in a RBC), serum iron, serum ferritin • Microcytic, hypochromic RBC’s • Increased Total iron binding capacity • Normal retic count • Normal hemoglobin electrophoresis

7. Iron Deficiency Anemia:Treatment • Infants: Breast milk or iron-fortified formula until 4-6months, then add iron-fortified cereal • School-age children and adolescents: restrict amount of milk, encourage iron-rich foods and foods high in vitamin C • Iron supplements: • Infants and preschoolers: 3mg/kg/day • School-age children: 60mg/day • Adolescents: 120mg/day

8. Iron-Deficiency Anemia:Nursing Considerations • Family Teaching • Nutrition: foods high and iron, and foods high in Vitamin C • Medication dosages and administration • Side effects of medication • Follow-up

9. Sickle Cell Anemia • Autosomal recessive disorder • Occurs in approximately 1 in every 500 African-American births and 1 in every 1000-1400 Hispanic American births • Sickle cell trait is present in 1 in 12 African-Americans

10. Sickle Cell Anemia:Pathophysiology • One amino acid replaces another, resulting in the production of sickle hemoglobin (Hb S) • This form of hemoglobin contains a semi-solid gel that caused the RBC to stretch into a sickle shape • These cells are more stiff and less able to change shape, and are unable to pass through the microcirculation • Anemia results from increased RBC destruction, worsened by the fact that sickled cells die after only 10-20 days

11. Sickle Cell Anemia: Clinical Manifestations • Vaso-occlusive crisis: aggregation of sickled cells within a vessel, causing obstruction. • Pain crisis • Hand-foot syndrome • Acute chest syndrome • Stroke • Priapism • Sequestration crisis: excessive pooling of blood in the liver and spleen. Decreased blood volume may result in shock. • Aplastic crisis: decrease in red blood cell production. Results in severe anemia • Pallor, fatigue, shortness of breath • Delayed growth • Delayed onset of puberty

12. Sickle Cell Anemia:Diagnosis • Family history and clinical manifestations • Newborn Screening • Lab tests: • Increased retic count • Decreased Hgb &Hct and TIBC • Hemoglobin electrophoresis reveals predominantly Hb S • Normocytic, normochromic, sickle shaped cells • Normal MCV, MCH, MCHC • Normal serum iron and serum ferritin

13. Sickle Cell Anemia:Treatment • Primary treatment is prevention of RBC sickling • Avoidance of fever, infection, acidosis, dehydration, constrictive clothing and exposure to cold • Immunization • Prophylactic oral penicillin until age 5 • Routine blood transfusions for children at high risk of CVA

14. Sickle Cell Anemia:Nursing Considerations • Promote comfort • Administer pain medication routinely instead of PRN • Apply heat to painful areas • Allow child to determine amount of activity tolerated • Provide passive ROM exercises • Administer IV fluids as ordered to maintain hydration • Prevent infection • Administer antibiotics as ordered • Frequent hand washing • Proper aseptic techniques • Provide education • Maintain adequate hydration • Avoid sources of infection • Promote proper nutrition • Signs and symptoms of crisis • Administration of prophylactic medications

15. Hemophilia • Group of bleeding disorders in which one factor in the first phase of coagulation is deficient • Most common type is hemophilia A (deficiency of factor VIII), occurs in approximately 1:5000 males • Hemophilia B (Christmas disease, deficiency of factor IX) second most common type, affects 10-15% of people with hemophilia • Least common type is hemophilia C (deficiency of factor XI) • Hemophilia A and B are x-linked recessive disorders, affecting mostly males. Hemophilia C is an autosomal recessive disorder that affects males and females equally

16. Hemophilia:Pathophysiology • One factor of the first phase of the intrinsic pathway is deficient • The body is unable to form clots to repair damaged blood vessels • Bleeding episodes occur

17. Hemophilia: Clinical Manifestations • Clinical manifestations vary based on severity of the disease • Mild: excessive bleeding only after severe trauma or surgery • Moderate: excessive bleeding only after trauma • Severe: excessive bleeding occurs spontaneously • Hematomas may result from injections or firm holding during the first year of life

18. Hemophilia:Clinical Manifestations • As the child learns to walk, bruising occurs easily, and hemarthrosis may develop • Persistent bleeding from minor lacerations begin to occur by 3-4 years of age • Minor occurrences of hematuria, epistaxis as well as major events such as intracranial hemorrhage and bleeding into the neck or abdomen may also occur

19. Hemophilia:Diagnosis • History and physical exam • Lab tests: • Prolonged PTT • Normal platelet count, standard bleeding time, and PT

20. Hemophilia:Treatment • Hemophilia A: • Replacement of missing coagulation factor through infusion of recombinant factor VIII concentrates • May be administered three or four times/week • Desmopressin (DDAVP) is effective for spontaneous bleeding and in preventing bleed prior to dental or surgical procedures

21. Hemophilia:Treatment • Hemophilia B: • Replacement of factor IX through plasma derived concentrates • DDAVP is not effective • Hemophilia C: Aggressive therapy is usually unnecessary because of the mild nature of the disease

22. Hemophilia:Nursing Considerations • Prevent bleeding: • Make the environment as safe as possible for infants learning to walk • Encourage noncontact sports in the school age child • Soft-bristled toothbrushes for mouth care and electric razors for shaving • Prevent IM injections whenever possible • Wear medic alert bracelet

23. Hemophilia: Nursing Considerations • Recognize and manage bleeding: • Make family aware of signs and symptoms of internal bleeding such as headache, slurred speech, loss of consciousness, black tarry stools, hematemesis, hematuria • Factor replacement should be instituted according to established medical protocol • Apply pressure to the bleeding area for at least 10-15 minutes • Immobilize and elevate the area above the level of the heart to decrease blood flow • Apply cold to promote vasoconstriction

24. Hemophilia:Nursing Considerations • Prevent crippling effects of joint degeneration resulting from hemarthrosis: • Elevate and immobilize the joint • Passive range of motion after the acute phase • Physical therapy • Nutrition counseling to maintain optimal weight • May need orthopedic intervention such as casting, traction or aspiration of blood to preserve joint function

25. Hemophilia:Nursing considerations • Family teaching: • Administration of antihemophilic factor concentrates • Methods to prevent or stop bleeding • Signs and symptoms that indicate an emergent situation and the need for immediate physician intervention • Provide support • Genetic counseling

26. Immune Thrombocytopenic Purpura (ITP) • Acquired disease characterized by thrombocytopenia and purpura • Autoimmune disorder • Peak incidence between 2 and 4 • 75% of children recover completely in 3 months • 80-90% have regained normal platelet counts within 6 months

27. ITP:Pathophysiology • Platelets adhere to injured vessel walls, release biochemical mediators, and form plugs, blocking minute ruptures occurring in the microcirculation • Inadequate numbers of platelets result in purpura under the skin and throughout the tissues • This occurs as the result of an autoimmune process that is triggered by a viral infection • Antiplatelet antibodies bind to the platelets, sequestering and destroying them in the spleen

28. ITP:Clinical Manifestations • Ecchymoses, general petechial rash occurring 1-4 weeks following a viral infection • Asymmetrical bleeding, especially on legs and trunk • Gastrointestinal or urinary tract bleeding may occur • Nose bleeding may be present and difficult to control • Intracranial hemorrhage occurs in only 1% of patients

29. ITP:Diagnosis • History and clinical presentation • Peripheral blood smear with very few, large, immature platelets • Bone marrow aspiration will be normal • Lab tests: • Decreased platelet count • Prolonged standard bleeding time • Normal PT and PTT

30. ITP:Treatment • Self-limiting condition • Prevent injury • Control bleeding • Immunosuppressive medication if sever symptoms are present • Prednisone • Gamma globulin

31. Questions?