1. Alterations in Hematologic Status
NURS 323

2. Major functions Production of cells Oxygenation
Nutrient distribution to cells
Immune protection
Waste collection from cells heat production

3. Common Diagnostic Assessments
Family History
Child’s health history
Physical exam
Medication use
Dietary pattern
Lab studies: CBC w/diff, PT, PTT, Hb, Hct, iron studies, bone marrow aspiration/bx
Prepare child & family for lab tests

4. Sickle Cell Anemia (SCA)
A global disease

5. Definition
Sickle cell anemia results from the hereditary presence of abnormal Hgb S in place of Hgb A. When Hgb S becomes deoxygenated (loses oxygen), it tends to form polymers that deform the red blood cells (RBCs) into a sickle shape. Sickled RBCs cannot pass freely through capillaries and cause blockage of small vessels. This can compromise the blood supply to various organs.

6. Incidence
Hereditary hemoglobinopathy; autosomal recessive; 1 African Americans
Hgb S trait is found in 8 to 10% of the American black population.
Sickle Cell Anemia– homozygous form (HbSS)—most prevalent in US
In Western African native blacks, up to 40% of the population carry Hgb S trait.
Sickle Hemoglobin C—a variant of sickle cell including both HbC & HbS—2nd most prevalent, not as severe.

7. Basic Defect
A defect in the structure of Hemoglobin A—substitution of 1 amino acid (valine) for another the 6th position of the beta polypeptide chain.
Hgb partially/completely replaced by Hgb S
When triggered, Hgb S sickles & obstructs cap. blood flow, s blood viscosity  tissue ischemia
Results  hemolysis (RBC destruction) & anemia;
Sickling reversible until repeated cycles leads to irreversible sickling
With STRESS (O 2, pH, Dehydration)—HgS changes to form long slender crystals which lead to tenting or crescent shaped RBC’s.
These forms are associated with  viscosity of the blood & hemolysis leading to multiple pathologies.

8. Diagnostic Tests Rarely seen before 4-6 mos.
Sickledex: sickle turbidity test; finger or heel stick; results in 3 min
Sickledex does not distinguish between sickle cell trait and sickle cell disease. Hemoglobin electrophoresis is required to distinguish between these 2 disorders.
Hgb electrophoresis to “fingerprint” protein
Hx, PE, CBC
CXR if symptoms
Sickledex Test
In the Sickledex test a deoxygenating agent is added; if 25% or more of the Hgb is Hgb S, the cells will sickle.
Normal values
A negative test result is normal for the Sickledex, though it will be abnormal also in patients with sickle trait.
Special considerations
Blood transfusions within the past 3 months or iron deficiency can cause a false negative result.

9. Diagnostic Tests (cont’d)
Peripheral Blood Smear
The routine peripheral blood smear does not contain sickled red blood cells unless hypoxemia (inadequate levels of oxygen in the blood) or a precipitating factor (such as a red blood cell poison or drug) is present.

10. Diagnostic Tests (cont’d)
Electrophoresis
Both sickle cell disease (homozygous for hemoglobin S) and sickle cell trait (heterozygous for Hgb S) can be detected by this test.
This test separates different types of hemoglobin based on electrical charge. Hgb S can be differentiated from Hgb A and other types of Hgb.

11. Diagnostic Tests (cont’d)
Normal Results
In hemoglobin electrophoresis, no Hgb S should be present. Normal hemoglobins in an adult are mostly Hgb A with small amounts of Hgb A2 and Hgb F.
What do abnormal results mean?
sickle cell trait
sickle cell anemia

12. Pathophysiology
Spleen—enlarged, if untreated, becomes fibrous & ineffective  susceptibility to infection.
Liver—failure & necrosis 2ndary to impaired circulation. Hepatomegaly and tenderness are common sx.
Kidney— perfusion 2ndary to congestion of glomerular capillaries & tubular arterioles hematuria, inability to concentrate urine, enuresis, & possible nephrotic syndrome.

13. Pathophysiology (cont’d)
Bones—
Hyperplasia of bone marrow osteoporosis weakened bones lordosis and kyphosis or other skeletal deformities
Chronic hypoxia possible osteomyelitis from salmonella
“Hand-foot Syndrome”—occurs in children 6 mos-2yrs. Caused by infarction of short-tubular bones. Sx: swelling & pain of soft tissue of hands & feet. Usually resolves in 2-3 wks.

14. Pathophysiology (cont’d)
CNS—primarily R/T ischemia
MAJOR INFARCT—Stroke or CVA paralysis
MINOR INFARCT—HA, aphasia, weakness, convulsions, visual changes 2ndary to retinopathy & retinal detachment.
Heart—Decompensation & failure 2ndary to chronic anemia  CHF, tachycardia

15. Pathophysiology (cont’d)
Blood—
Rate of destruction of RBC’s > rate of RBC production
RBC’s lifespan is  to days (compared to 120 days for nml RBC)
 Hgb, Hct & RBC count
 Reticulocyte count, bilirubin & uric acid

16. Pathphysiology (cont’d)
Other sx:
Generalized weakness
Anorexia
Joint, back, and abdominal pain
Fever
Vomiting
Possible leg ulcers
Failure to thrive,  rate of growth & development

17. Sickle Cell Crisis Triggers
Acute illness
Dehydration
Strenous exercise
Hypoxic conditions
Trauma
Respiratory illness
Results: acute pain, cell destruction, infarcts, organ damage

18. Types of Crises
Vaso-occlusive: PAIN, swollen joints, vomiting, fever, anorexia, dactylitis
Acute sequestration: liver & spleen, HR dyspnea, weakness, pallor, shock
Aplastic crisis: weakness, pallor, dyspnea, tachycardia, shock
Acute chest syndrome: chest pain, cough, fever, abdominal pain
VASO-OCCLUSIVE—most common & most painful
SEQUESTRATION—pooling of RBC’s in liver & spleen vascular collapse & circulatory shock
APLASTIC—RBC’s w/HbS + Bone marrow slows/stops production   RBC’s & profound anemia
HYPERHEMOLYTIC—most rare form. Caused by excess RBC destruction.

19. Other complications Megaloblastic anemia Hyperhemolytic crisis
Cerebrovascular injury (stroke)
Overwhelming infections

20. TREATMENT Primary Goals:
Prevent Crisis (avoid sickling phenomenon)—through adequate oxygenation & hydration in every day life activities. Hydroxyurea may be used prophylactically in adolescents and older adults. Folic Acid 1mg daily is also recommended to restore folate stores needed for erythropoiesis.
Treat crisis as an emergency when it occurs—determine etiology quickly and treat appropriately

21. Nursing Management
Adequate hydration (also any electrolyte replacement if needed)
Pain management
Promote rest
Treat any infections
Blood transfusions if needed
Monitor O2 – supplement if needed
Oral hygiene
Maintain nutrition
Family support and education

22. NURSING CARE Pain Control—strive to prevent pain.
Opioids as morphine, oxycodone, hydromorphone & methadone are preferred. Give IV or PO for severe pain round-the-clock. PCA pumps are great! Avoid Demerol (Meperidine) - ↑seizure risk
Tylenol & Ibuprofen may be used after dx is made
Use non-pharmacologic methods also
Heat (e.g. K-pad)to affected joints may be soothing by  O2 with vasodilation.

23. NURSING CARE Hydration– Prevent tissue deoxygenation
Accurate I & O essential
 IV/PO fluids up to 150ml/kg/day will promote hemodilution
Note color of urine—sometimes brown
Prevent tissue deoxygenation
Avoid strenuous activity
Avoid environments with low O2 concentration
Avoid extremes in temp- esp. cold environment
Avoid emotional stress
Avoid known sources of infection

24. Child/Family Education
How to manage & minimize crisis: avoid dehydration, cold, stress, hypoxia, acute illness, etc.
Signs of infection
Importance of immunizations
Antibiotic administration if ordered
Education & support re: long term effects
Genetic counseling

25.  Thalassemia Major
Autosomal recessive
Defective Hgb; RBCs fragile w/short life span  anemia, hemolysis
Main Tx: blood transfusions
Hemochromatosis – iron overload – occurs from hemolysis of transfused and defective RBCs
Chelation tx done to eliminate excess Fe

26. Nursing Considerations
Numerous blood transfusions
Assess s/s of hemosiderosis
Assess s/s infection
Folic acid supplementation
Iron chelation tx as ordered
Family support
Possible bone marrow transplant

27. Aplastic Anemia Anemia (↓ RBC's) Leukopenia (↓ WBC's)
Definition: Condition where all formed elements of the blood are depressed.
Pancytopenia or Triad:
Anemia (↓ RBC's)
Leukopenia (↓ WBC's)
Thrombocytopenia (↓ platelets)

28. Diagnosis -- Bone marrow aspiration or biopsy
Etiology of A.A.
Primary (congenital)
Secondary (acquired) -- often secondary to drugs e.g. chemotherapy or other meds that “shut down” bone marrow function
Diagnosis -- Bone marrow aspiration or biopsy

29. Therapeutic Management -- Goal: Restore bone marrow function
Immunosuppressive therapy
Bone marrow transplantation ** choice 85% survival rate
Nursing Considerations -- Similar to child with leukemia
Prepare family for diagnostic & therapeutic procedures
Preventing complications from severe pancytopenia
Emotionally supporting them in terms of a potentially fatal outcome

30. Hemophilia Deficiency in specific clotting factor
X-linked recessive trait; 2 most common
Hemophilia A (classic)
Hemophilia B (Christmas Disease)
Uncontrolled bleeding from wounds into joints, muscles, & other body tissues
Spontaneous & severe uncontrolled bleeding in severe form

31. Common Labs Hx & PE, symptoms PTT prolonged Factor VIII, IX levels 
Normal: PT, thrombin, bleeding time, fibrinogen, platelet count
May do liver Bx to r/o other causes

32. Signs & symptoms Excessive bleeding from circumcision
Bleeding w/teething
Hemarthosis – cardinal sign
Bruises in various stages
Hematuria
Nose bleeds
Oral bleeding

33. Nursing Management Control localized bleeding:
Direct pressure for extended period of time like 10 minutes
Ice
Elevation of affected limb
Avoid injections when possible
No rectal temps
Monitor for bleeding (joints, urine, stool, etc)
Provide pain relief
Monitor neuro status if possible head bleeds

34. Nursing Management Administration of Factor replacement as needed
Factor VIII concentrate (Antihemophilia factor {AHF} ), or DDAVP , a synthetic form of vasopressin that is the treatment of choice in mild hemophilia & von Willebrand disease. Should increase Factor VIII activity 3-4 fold.

35. Teaching Considerations
Teach family factor administration/storage
DDAVP administration
Avoid ASA type products
Avoid contact sports
Medic alert tag
Avoid constipation
Good nutrition
Good oral hygiene
Genetic counseling
Protective environment for infants/toddlers

36. Idiopathic Thrombocytopenia Purpura (ITP)
Excessive destruction of platelets Purpura (from petechiae under skin)
Normal bone marrow
Most common bleeding disorder in children
80% between 2-5 yrs old and usually last ~ 6m
Usually after viral infection
Symptoms when platelet ct < 20,000

37. Therapeutic Management
IF medical tx is indicated, then may use one of the following regimens
Prednisone
IVIG—expensive and takes long to administer and monitor pt
Anti-D antibody—controversial but may prolong survival of platelets. Can be given in 1 dose over 5-10 minutes.

38. Nursing Considerations
Stress activity restriction
Prevention of head injuries; instruct parents to get med eval if head or abd injury
Assess for bleeding/bruising
Oral care support
No rectal temps – No ASA products
Splenectomy if has for 1 yr and tx not successful
Family support & teach on how to control bleeds

39. There you have it!