Interventions for Clients with Hematologic Problems

1. Interventions for Clients with Hematologic Problems

3. Anemia • Reduction in either the number of red blood cells, the amount of hemoglobin, or the hematocrit • Clinical sign (not a specific disease); a manifestation of several abnormal conditions

4. ANEMIA 1.) Anemia is reduction in either RBCs, amount of hemoglobin, or hematocrit (% of packed RBC per deciliter of blood) “Anemia” is a symptom of an underlying disease. • Causes and types vary: • 1.)dietary problems—deficiency in components necessary to make RBC—iron, vitamin B12 (cyanocobalamin), folic acid, or intrinsic factor • 2.)genetic disorders • 3.)bone marrow disease • 4.)excessive bleeding • 5.)Immune reactions • 6.)Changes in blood chemistry • 7.)Toxins in the blood

6. Chronic anemiasdevelop gradually, more subtle symptoms-- lethargy, pallor, and anorexia (gastritis, hemorrhoids, menstrual flow) Acute anemias do not allow the body sufficient time to make physiologic adjustments -- patients symptomatic with shortness of breath, extreme fatigue, and cardiac discomfort (trauma, blood vessel rupture)

8. Hematologic Problems • Anemias • Results in: reduction in oxygen transport due to decrease in hemoglobin production, a decrease in erythrocytes, or a combination of these factors. • Reduced oxygen leads to less energy in all cells, reduced cell metabolism and reproduction. • Compensation mechanisms include tachycardia and peripheral vasoconstriction

9. Hematologic Problems • Anemias • General signs of anemia: fatigue, pallor, dyspnea, and tachycardia • Severe anemia may lead to angina if oxygen supply to the heart is insufficient • Chronic severe anemia may cause CHF • Other affects may include hair and skin changes

11. Key Features of Anemia Integumentary manifestations • Pallor, of ears, nail beds, palmar creases, conjunctiva, and around mouth • cool to touch • intolerance of cold temperatures • Nails become brittle, overtime become concave and fingers are club like in appearance. Cardiovascular Manifestations • Tachycardia, murmurs, gallops when anemia severe orthostatic hypotension

12. Key Features of Anemia Respiratory Manifestations • Dyspnea on exertion • Decreased oxygen saturation levels Neurologic Manifestations • Increased somnolence and fatigue • Headache

14. LABORATORY PROFILE Test Significance of abnormal finding Red blood cell count Decreased indicate possible anemia/hemorrhage Hemoglobin/Hematocrit Increased indicate possible chronic hypoxia, or polycythemia vera Mean cell hemoglobin (MCV) Increased levels indicate macrocytic cells, possible anemia. Decreased levels indicate microcytic cells, possible iron deficiency anemia Reticulocyte count helpful in determining bone marrow function (immature RBC) **Increased levels indicate chronic blood loss—desireable in anemic client or after hemorrhage.

15. Hemoglobin electrophoresis detects abnormal forms of hemoglobin, such as hemoglobin S in sickle cell disease, Prothrombin time /INR assesses extrinsic clotting cascade, reflects how much clotting factors II, V, VII, X is functioning. Increased=deficient in clotting factor cascade. Decreased=vitamin K excess. (monitors Coumadin tx) 25-38 sec PTT aPartial thromboplastin time assesses the intrinsic clotting cascade, factors VIII, IX, XI, XII. Prolonged w/hemophilia or disseminated intravascular coagulation (DIC). (monitors Heparin) Level maintained 1.5 to 2.5 times their baseline values

17. 3.) Hypoproliferative anemia. Hypoproliferative anemia can be subdivided into three classes based upon the size of the RBCs. The cells may be larger than normal (macrocytic), normal (normocytic), or smaller than normal (microcytic). Macrocytic anemia. Macrocytic anemia can be due to several causes. The first is a deficiency in vitamin B12 or folate, both important ingredients in RBC production. Microcytic anemia. Microcytic anemia is due to abnormalities in the production of the essential RBC protein, hemoglobin. This is often to due to underlying disease, such as thalassemia, iron deficiency anemia Normocytic anemia. Normocytic anemia may be due to chronic disease including malnutrition or mixed anemia (combined macrocytic and microcytic anemia).

19. Sickle Cell Disease • Genetic disorder resulting in chronic anemia, pain, disability, organ damage, increased risk for infection, and early death • Formation of abnormal hemoglobin chains • Conditions causing sickling: hypoxia, dehydration, infections, venous stasis, low environmental body temperatures, acidosis, strenuous exercise, and anesthesia. (Continued)

20. Clinical Manifestations • Cardiovascular changes • Skin changes • Abdominal changes • Musculoskeletal changes • Central nervous system changes

21. Interventions • Pain is the most common problem. • Drug therapy: 48 hours of intravenous analgesics • Oral hydration • Complementary and alternative therapies

24. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Anemia • Most common type of congenital hemolytic anemia • Hydration • Screening for this deficiency necessary before donating blood, because cells deficient in G6PD can be hazardous

26. Iron Deficiency Anemia • This common type of anemia can result from blood loss, poor intestinal absorption, or inadequate diet. • Evaluate adult clients for abnormal bleeding. • Supplemental iron is the treatment.

30. Vitamin B12 Deficiency Anemia • Anemia is caused by inhibiting folic acid transport and reducing DNA synthesis in precursor cells. • Vitamin B12 deficiency is a result of poor intake of foods containing vitamin B12. • Pernicious anemia is anemia caused by failure to absorb vitamin B12 and lack of intrinsic factor; • clients often exhibit • paresthesia.

31. Folic Acid Deficiency Anemia • Can cause megaloblastic anemia • Manifestations similar to those of vitamin B12 deficiency, but nervous system functions remain normal (Continued)

33. Folic Acid Deficiency Anemia(Continued) • Caused by: • Poor nutrition and chronic alcohol abuse • Malabsorption syndromes, such as Crohn’s disease • Drugs, including anticonvulsants and oral contraceptives, that slow or prevent absorption of folic acid

35. Polycythemia Vera • Disease with a sustained increase in blood hemoglobin • Massive production of red blood cells • Excessive leukocyte production • Excessive production of platelets • Phlebotomy • Increased hydration • Anticoagulants are part of therapy

36. Polycythemia Vera Due to hyperviscous (thicker than normal blood) the following may occur: • Key features: • Client’s facial skin and mucous membranes have a dark, flushed (plethoric) appearance • Distention of superficial veins • Weight loss • Intense itching • Hypertension • Fatigue, enlarged hemorrhoids • Swollen painful joints • Enlarged firm spleen • Infarctions of the heart (chest pain, heart failure), kidneys • Strokes • Bleeding tendency

37. Polycythemia Vera • Diagnostic Tests • Blood cell counts and hematocrit markedly elevated • Hyperuricemia due to high cell destruction • Bone marrow hypercellular Hgb levels to 18 g/dl Hct of 55% or > RBC count of 6 mil/mm3

38. Polycythemia vera Collaborative management: • Phlebotomy (treatment) blood drawing • Increase hydration • Anticoagulants are part of therapy to prevent clot formation • Chemotherapy to suppress bone marrow activity • Radiation therapy • Bone marrow transplantation • Significant number of individuals with PV go on to develop acute leukemia

39. Polycythemia vera • Client education guide • Drink at least 3 L day • Avoid tight or constrictive clothing, especially garters or girdles • Wear gloves when outdoors in temperature lower than 50 degrees • Contact physician first sign of infection • Use soft-bristled toothbrush • Do not floss teeth

40. Polycythemia vera • Take anticoagulants as prescribed • Wear support hose while awake and up • Elevate feet when you are seated • Exercise slowly and only on the advice of your physician • Stop activity at the first sign of chest pain • Use electric shaver

41. Polycythemia • Production and presence of increased RBCs • 2 types: Primary polycythemia = Polycythemia Vera Secondary Polycythemia = a.) hypoxia driven = high altitude, cardiopulmonary disease, defection O2 transport b.)Hypoxia independent= renal cysts or tumors

43. It is a cancer of the RBCs with 3 major hallmarks: 1.) Massive production of red blood cells 2.) Excessive leukocyte production 3.) Excessive production of platelets Hgb levels to 18 g/dl Hct of 55% or > RBC count of 6 mil/mm3

45. Aplastic Anemia (macrocytic) • Cause:deficiency of circulation RBC due to failure of bone marrow to produce these RBC cells, may occur alone or with • Leukopenia (decreased WBC) and • thrombocytopenia (decreased platelets). When ALL three occur together it is called “Pancytopenia” • Cause: 1.) Congenital in origin – chromosomal abnormality 2.)Acquired--Long term exposure to toxic agents, ionizing radiation or infection, (viral, bacterial), medications, antiseizure, antimicrobials) may cause Aplastic anemia. 3.) 70% of acquired is idiopathic (unknown cause)

46. Signs and Symptoms: Onset insidius: • manifestations include those of anemia • Those of leukopenia (recurrent multiple infections) • Those related to thrombocytopenia (petechia, tendency to bleed excessively, especially in the mouth.

47. Aplastic Anemia (macrocytic) • Diagnosis: definitive bone marrow aspiration red bone marrow is replaced by fatty red bone marrow • Treatment: blood transfusions, immunosuppressive therapy (antilymphocyte globulin (ALG), cyclosporine (Sandiuumne), prednisone, cyclophosphamide (Cytoxan) can bring about partial or complete remissions. • Splenectomy on clients with enlarged spleen that is either destroying normal RBCs or suppressing their development • Bone marrow transplant.

48. Hemolytic Anemia • Destruction or hemolysis of RBCs at a rate that exceeds production • Third major cause of anemia • Intrinsic hemolytic anemia • Abnormal hemoglobin (sickle cell) • Enzyme deficiencies • RBC membrane abnormalities • Extrinsic hemolytic anemia • Acquired (mechanical injury heart bypass, toxins) • Sites of hemolysis • Intravascular • Extravascular

49. Hemolytic Anemia • Jaundice • Destroyed RBCs cause increased bilirubin • Enlarged spleen and liver • Hyperactive with macrophage phagocytosis of the defective RBCs • Accumulation of hemoglobin molecules can obstruct renal tubules • Tubular necrosis

50. 2.) Hemolytic Anemias resulting from increased destruction (hemolysis) of RBCs a.) Sickle cell disease — b.)Glucose-6-Phosphate Dehydrogenase Deficiency anemia • c.)Thrombotic Thrombocytopenia Purpura (TTP) is a rare autoimmune reaction in blood vessels disorder in which platelets clump together abnormally in the capillaries and few remain in circulation.