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Alterations in Hematologic Status

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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!

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