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 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 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 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?