N323 Module B part I

1. N323 Module B part I 1 Hematologic Problems

2. N323 Module B part I 2 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 **Gastrointestinal bleeding common cause of anemia in adults**

3. N323 Module B part I 3 Chronic vs. Acute Anemia Chronic anemias�develop 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)

4. N323 Module B part I 4 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

5. N323 Module B part I 5 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 Cultural considerations:

6. N323 Module B part I 6 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 Respiratory Manifestations Dyspnea on exertion Decreased oxygen saturation levels Neurologic Manifestations Increased somnolence and fatigue Headache

7. N323 Module B part I 7 Diagnostic Assessment Tests of cell number and function: Complete blood count Reticulocyte count Hemoglobin electrophoresis Serum ferritin transferrin total iron-binding capacity

8. N323 Module B part I 8 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.

9. N323 Module B part I 9 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

10. N323 Module B part I 10 International normalized ration (INR) is the recommended laboratory measurement system for monitoring the effect of oral coagulation therapy. Most patients on oral anticoagulants, the therapeutic range of the INR is 2-3 Dehydration: When dehydration is present hemoconcentration occurs resulting in : increased hemoglobin, hematocrit, serum osmolarity, glucose, protein, blood urea nitrogen, and various electrolytes.

11. N323 Module B part I 11 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).

12. N323 Module B part I 12 Assessment Hematologic Demographic data (age, gender) Family history and genetic risk Personal history (use of ASA, NSAIDS, antibiotic use-prolonged can lead to bone marrow suppression) Diet history Socioeconomic status Current health status (Gordon�s Hematologic assessment�activity-exercise pattern, and nutrition-metabolic pattern pg 879) Skin Head and neck Respiratory Cardiovascular Renal and urinary Musculoskeletal Abdominal Central nervous system Psychosocial

13. N323 Module B part I 13 Anemia Clinical Manifestations Mild = Hb 10 to 14 g/dl May exist without symptoms Moderate = Hb 6 to 10 g/dl Increased cardiopulmonary symptoms Experienced at rest or during activity Severe = Hb <6 g/dl Involve multiple body systems Integument Eyes Mouth Cardiovascular Pulmonary Neurologic Gastrointestinal (GI) Musculoskeletal

14. N323 Module B part I 14 ANEMIAS RESULTING FROM DECREASED PRODUCTION OF RBCs Iron Deficiency Anemia (RBC small, microcytic) Mild to severe cases A sign of underlying problem, important find cause Occurs all age groups Insufficient iron impedes synthesis of hemoglobin, reducing the amounts of oxygen transported in blood Results in microcytic (small cell) hypochromic (less color) erythrocytes due to a low concentration of hemoglobin in each cell.

15. N323 Module B part I 15 Iron-Deficiency Anemia One of the most common chronic hematologic disorders Iron is present in all RBCs as heme in hemoglobin and in a stored form Heme accounts for two thirds of the body�s iron

16. N323 Module B part I 16 Iron Deficiency Anemia (RBC small, microcytic) Causes� depleted iron stores (decreased ferritin), results in a decreased supply of iron for the manufacture of hemoglobin in RBCs Iron stores 2/3 hemoglobin, 1/3rd in the bone marrow, spleen, liver, and muscle Many causes: Lack of dietary intake of iron containing foods (adolescent growth spurt, pregnancy need higher amount of iron) Slow, chronic blood loss from bleeding ulcer, hemorrhoids, cancer, or excessive menstrual flow syndromes of GI malabsorption Occurs at any age, frequent women, older adults, people poor diets

17. N323 Module B part I 17 Iron Deficiency Anemia (RBC small, microcytic) Signs and Symptoms: Mild anemia frequently asymptomatic Pallor skin and mucous membranes Fatigue, lethargy Cold intolerance (caused by decreased cell metabolism) Irritability ( CNS hypoxia) Degenerative changes including brittle hair, spoon shaped (concave) ridged nails, Females: menstrual irregularities Severe anemia: tachycardia, palpitations, dyspnea, syncope, as well as delayed healing.

18. N323 Module B part I 18 Iron Deficiency Anemia (RBC small, microcytic) Diagnostic Tests: Hemoglobin, hematocrit (low) Mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH) Serum ferritin less than12 g/L Serum iron Erythrocytes appear hypochromic and microcytic on microscopic examination

19. N323 Module B part I 19 Iron Deficiency Anemia (RBC small, microcytic) Management� Find underlying cause increase oral intake of iron, iron supplements (take w/food to reduce gastric irritation and nausea) Oral treatment in 4 weeks raises Hgb about 2/g/dl Liquid form of iron stains teeth and dentures�use straw IM�use Z-track method, ventro-gluteal injection do not massage site

20. N323 Module B part I 20 Iron-Deficiency AnemiaDrug Therapy Oral iron Vitamin C aids in iron absorption Factors to consider (cont�d) Best absorbed as ferrous sulfate in an acidic environment Liquid iron should be diluted and ingested through a straw to prevent staining of teeth Side effects Heartburn, constipation, black stools, diarrhea

21. N323 Module B part I 21 Iron-Deficiency AnemiaNursing Management At-risk groups Premenopausal women Pregnant women Persons from low socioeconomic backgrounds Older adults Individuals experiencing blood loss

22. N323 Module B part I 22 Iron-Deficiency AnemiaNursing Management Diet teaching Supplemental iron Discuss diagnostic studies Emphasize compliance Iron therapy for 2 to 3 months after the hemoglobin levels return to normal

23. N323 Module B part I 23 Common Food sources of Iron, Vitamin B12, and Folic Acid Iron Vitamin B12 liver , organ meats liver, organ meats, nuts whole wheat breads/cereals dried beans, green leafy veges Leafy green leafy veges, carrots citrus fruit, brewers yeast Egg yolks, raisins Folic Acid Liver, organ meats, eggs, cabbage Brocolli, brussel sprouts

24. N323 Module B part I 24 Megaloblastic Anemias Group of D/O caused by DNA synthesis and characterized by large RBCs (abnormal = hemolysis) Common: Vitamin B12 Cobalamin deficiency anemia Pernicious anemia � lack of secretion of intrinsic factor (needs acidic environment) in GI tract which is necessary for absorption of Vitamin B12 Folic acid anemia Other causes: certain drugs

25. N323 Module B part I 25 Vitamin B12 Deficiency Anemia (Cobalamin) & Pernicious anemia) (megaloblastic, macrocytic) Characterized by very large nuclueated, immature erythrocyte Vitamin B12 is required for RBC maturation Causes: deficiency in B12 (cyanocobalamin) results in inhibiting folic acid acid transport into the cell =RBC maturity does not can occur. (all cell division requires adequate amount of folic acid to make DNA)

26. N323 Module B part I 26 Vitamin B12 (Cobalamin) Deficiency Anemia & Pernicious anemia) (megaloblastic, macrocytic) Results from poor intake of B12 related to vegetarian diets, or diets lacking in dairy products, OR poor absorption of B12 in conditions such as small bowel resection, tapeworm, or overgrowth of intestinal bacteria

27. N323 Module B part I 27 Vitamin B12 Deficiency Anemia & Pernicious anemia (megaloblastic, macrocytic) Key Features of Vitamin B12 Anemia: severe pallor slight jaundice Smooth, shiny, beefy tongue weight loss paresthesia of hands and feet (numbness and tingling), difficulty w/gait related lack of B12 needed for normal nerve function (impaired conduction of nerve impulses=demyelination) peripheral nerves and eventually spinal cord)

28. N323 Module B part I 28 Vitamin B12 Deficiency Anemia & Pernicious anemia) (megaloblastic, macrocytic) Diagnostic tests: Erythrocytes appear macrocytic or megaloblastic Serum vitamin B12 low Shilling test is used to measure absorption of vitamin B12 after oral administration. Treatment: prophylaxis oral supplements for pregnant women and vegetarians. Vitamin B12 injection daily X2 weeks initially, then weekly until hemoglobin normal, then monthly for maintenance for life

29. N323 Module B part I 29 Folic acid deficiency (also cause megaloblastic anemia) Required for DNA synthesis L/T RBC formation & maturation Manifestations: similar to Vitamin B12 anemia, except nervous system function remains normal. Disease develops slowly. Causes: common include poor nutrition,(r/t chronic alcoholism), malabsorption problems (Crohns disease), and drugs (oral contraceptives, anticonvulsants), HD Collaborative management: Identify high risk groups (elderly, debilitated, alcoholic, those susceptible to malnutrition, and those who require more (pregnancy) Scheduled folic acid replacement therapy

30. N323 Module B part I 30 Thalassemia (Cooleys anemia)Etiology An autosomal recessive genetic disorder of inadequate production of normal hemoglobin Normal hemoglobin, also called hemoglobin A, has four protein chains�two alpha globin and two beta globin. In Thalassemia some of these proteins are missing� L/T abnormal hemoglobin Common in ethnic groups near the Mediterranean Sea and equatorial regions of Asia and Africa

31. N323 Module B part I 31 ThalassemiaEtiology Hemolysis also occurs Problem with globulin protein Abnormal Hb synthesis One thalassemic gene (mild form) Thalassemia minor (thalassemic trait) Two thalassemic genes (severe form) Thalassemia major

32. N323 Module B part I 32 ThalassemiaClinical Manifestations Thalassemia minor Asymptomatic frequently Moderate anemia Splenomegaly Mild jaundice Thalassemia major Life-threatening Physical and mental growth often retarded Pale Symptoms develop in childhood Thalassemia major Splenomegaly Hepatomegaly Jaundice from hemolysis Chronic bone marrow hyperplasia Expansion of bone marrow space

33. N323 Module B part I 33 ThalassemiaCollaborative Care No specific drug or diet is effective in treating thalassemia Thalassemia minor Body adapts to decreased Hb Thalassemia major Blood transfusions with IV deferoxamine (binds with iron, rids through kidneys, TX, hematochromotosis) Prevention Genetic counseling with families with known history of Thalassemia Prenatal checkup

34. N323 Module B part I 34 Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Anemia Most common type of congenital hemolytic anemia where this enzyme is lacking in the RBC Enzyme lacking in the critical step of RBC energy production When exposed to certain drugs or toxins the aging RBC easily break Client usually asymptomatic until anemia or severe infection develops Acute phase: anemia and jaundice Hemolytic reaction is limited because only older RBCs containing less G6PD, are destroyed Sentence and phraseSentence and phrase

35. N323 Module B part I 35 Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Anemia Collaborative management: Hydration during episode of hemolysis to prevent acute tubular necrosis Identify and remove toxins or drug Screening for this deficiency necessary before donating blood Osmotic diuretic such as mannitol (Osmitrol) Blood transfusion to correct anemia

36. N323 Module B part I 36 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)

37. N323 Module B part I 37 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.

38. N323 Module B part I 38 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.

39. N323 Module B part I 39 Anemias of Chronic Disease Chronic inflammatory Autoimmune Infectious Malignant diseases Examples: Renal disease (decreased ereythropoietin) Myelosuppression Medication�chemotherapy Radiation

40. N323 Module B part I 40 Acute Blood Lossand Chronic Blood Loss

41. N323 Module B part I 41 Acute Blood Loss 20% volume loss may see signs or symptoms with increased activity, slight postural hypotension Result of sudden hemorrhage Trauma Complications of surgery Disruption vascular integrity Concerns Hypovolemic shock Reduced plasma volume Diminished O2 because fewer RBCs available

42. N323 Module B part I 42 Acute Blood LossClinical Manifestations Cause Body�s attempt to maintain an adequate blood volume and O2 Pain Internal hemorrhage Tissue distention, organ displacement, nerve compression Pain (cont'd) Retroperitoneal bleeding Numbness Pain in the lower extremities Shock is the major complication

43. N323 Module B part I 43 Acute Blood Loss Diagnostic Studies Laboratory data does not adequately assess RBC problems for 2 to 3 days Collaborative Care Replacing blood volume to prevent shock Identifying the source of the hemorrhage Stopping blood loss Correcting RBC loss

44. N323 Module B part I 44 Acute Blood LossNursing Management May be impossible to prevent if caused by trauma Postoperative patients Monitor blood loss No need for long-term treatment

45. N323 Module B part I 45 Chronic Blood Loss Reduced iron stores Bleeding ulcer Hemorrhoids Menstrual and postmenopausal blood loss Management Identify source Stop bleeding Possible use of supplemental iron

46. N323 Module B part I 46 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

47. N323 Module B part I 47 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

48. N323 Module B part I 48 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.

49. N323 Module B part I 49 Sickle Cell Disease (can cause anemia resulting from increased destruction of RBCs) 1.) Cause� genetic defect causing a formation of abnormal hemoglobin chains (HbS) resulting in chronic anemia, pain, disability, organ damage, increased risk for infection, and early death Sickle cell disease occurs in 1 in 350-500 African Americans Sickle cell disease state vs. sickle cell trait, variation of severity Sickle cell trait �less than 40% of total hemoglobin HbS Sickle cell disease � 80-100% of total hemoglobin contains may have abnormality of hemoglobin S (HbS)

50. N323 Module B part I 50 2.) Insufficient oxygen = cells to assume a sickle shape, cells become rigid and clumped together, obstructing small blood vessels or the capillary blood flow, cause venous stasis, anemia, pain, enlarged spleen other manifestations in the respiratory, genitourinary, cardiovascular, musculoskeletal and integumentary systems. Obstruction of the small blood vessels = repeated multiple infarctions or areas of tissue necrosis throughout the body Deoxygenation of hemoglobin in peripheral circulation = �sickle cell crisis�

51. N323 Module B part I 51 Clinical Manifestations Cardiovascular changes Skin changes Abdominal changes Musculoskeletal changes Central nervous system changes

52. N323 Module B part I 52 Diagnosis Hemoglobin S (HbS) electrophoresis Diagnosis confirmed by the presence of sickled cells in peripheral blood Hematocrit low Reticulocyte count high (chronic anemia) Mean corposcular hemoglobin concentration (MCHC) high bilirubin levels are high WBCs elevated (due to chronic inflammation and hypoxia)

53. N323 Module B part I 53 Interventions include: Protection of the client from infection in sickle cell crisis Drug therapy -pain meds, folic acid replacement - -Hydroxyurea (Droxia)

54. N323 Module B part I 54 Care of the Client in Sickle Cell Crisis Administer oxygen Pain Drug therapy: 48 hours of intravenous analgesics (opiods�Morphine or Dilaudid) IV or PCA Hydrate with IVF, encourage PO fluids 4liters/day Administer blood transfusion if required Remove constrictive clothing Encourage client to keep extremities extended to promote venous return Check circulation of extremities Complementary and alternative therapies

55. N323 Module B part I 55 Potential for Multiple Organ Dysfunction Interventions include: - pain management Hydration IV, PO Oxygen therapy Transfusion therapy Hydroxyurea (Droxia)-(stimulate HbF, fetal hemoglobin production, reducing HbS, LT use S/E bone marrow suppression, causes birth defects�females should use 2 methods of birth control) Complementary and Alternative Therapies (TENS, acupuncture)

56. N323 Module B part I 56 Acquired Hemolytic Anemia Three extrinsic categories 1. Physical factors Physical destruction of RBCs results from extreme force on the cells Hemodialysis, extracorporeal circulation, prosthetic heart valves, angiopathic disease (any disease of vessels) 2. Immune reactions Antigen�antibody reactions destroy RBCs Isoimmune reactions Antibodies develop against antigens; blood transfusions Autoimmune reactions Develop antibodies against their own RBCs

57. N323 Module B part I 57 Acquired Hemolytic Anemia Three extrinsic categories 3. Infectious agents and toxins Foster hemolysis in four ways Invading RBCs and destroying contents Releasing hemolysis substances Generating an antigen�antibody reaction Contributing to splenomegaly

58. N323 Module B part I 58 Hemochromatosis Primary hemochromatosis is an inherited disorder characterized by excessive iron accumulation due to increaxed intestinal iron absorption causing tissue damage. Symptoms do not develop until organ damage, often irreversible, develops.

59. N323 Module B part I 59 Symptoms include fatigue, hepatomegaly, bronze skin pigmentation, loss of libido, arthalgias, and manifestations of cirrhosis, diabetes, or cardiomyopathy. Diagnosis is based on serum iron studies and gene assay. Treatment serial phlebotomies

60. N323 Module B part I 60 AnemiaNursing Implementation Dietary and lifestyle changes Blood or blood product transfusions Drug therapy Oxygen therapy Patient teaching Nutrition intake Compliance with drug therapy Gerontologic Considerations Common in older adults Chronic disease Nutritional deficiencies Signs and symptoms may go unrecognized or mistaken for normal aging changes

61. 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 Polcycythemia Vera=(PV) is a rare disease with a sustained increase in blood cells (primarily red blood cells) produced by the bone marrow With unknown origin, chromosomal defect in pluripotent stem cells

62. N323 Module B part I 62 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

63. N323 Module B part I 63 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

64. N323 Module B part I 64 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

65. N323 Module B part I 65 Polycythemia vera (PV) 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

66. N323 Module B part I 66 Polycythemia vera (PV) 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 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

67. N323 Module B part I 67 White Blood Cell Disorder