Chapter 37: Disorders of Hematopoietic System

1. Chapter 37: Disorders of Hematopoietic System Medical Surgical 1 Fall Semester

2. Blood Dyscrasias pg 564 Abnormalities in the numbers and types of blood cells and bleeding disorders develop from both treatable and chronic pathologic processes. Some are life threatening Many have similar symptoms and require diagnostic test

4. Anemia Decrease in number of erythrocytes (Red blood cells) and a lower than normal Hgb level. Causes a decrease in amount of oxygen carried to tissues Caused by blood loss, inadequate nutrition, and genetic or acquired disorders

5. Hypovolemic Anemia Loss of blood volume results in a decrease of blood cells. Because erythrocytes are the most abundant type of blood cell, the most critical consequence is hypovolemic anemia.

6. Pathophysiology pg 564 Caused by sudden loss of a large amount of blood or a gradual loss of small amounts over a prolonged period

7. Signs & Symptoms Acute S/S of acute hypovolemic anemia from sudden severe blood loss--shock: extreme pallor, tachycardia, hypotension, reduction of urinary output and altered consciousness

8. Signs & Symptoms Chronic Symptoms from chronic blood loss: pallor, fatigue, feeling chilled, postural hypotension, and rapid heart and respiratory rate CBC, Hgb and Hct levels used to diagnose

9. Hemoglobin Levels Normal results vary, but in general are: Male: 13.8 to 17.2 gm/dL Female: 12.1 to 15.1 gm/dL

10. Medical Management Treatment of sudden blood loss --transfusions Treatment of chronic (uterine tumors, hemorrhoids) underlying condition is treated May need transfusion or IV or IM iron to help compensate loss of hgb. Oxygen helps if severe

11. Nursing Process pg. 565 (Hypovolemia) Monitor I & O--report urine below 30 to 50 ml/hour Assess vitals q 2 to 4 hours. Report systolic BP less than 90 or heart rate above 100 Monitor oxygen saturation ..report levels less than 90% Apply direct pressure to stop bleeding Use modified trendelenburg

12. Trendelenburg Position Facilitates Blood Flow To Brain

13. Nursing Care (Hypoxemia) Monitor oxygen saturation continually with a pulse oximeter. (it measures the percentage of oxygen bound to hemoglobin) Report a sustained oxygen saturation value below 90%. (normal is 95 � 100%) Supplement oxygen as ordered by physician.

14. Nursing Care (Activity Intolerance & Body Temp) Space activity..let him rest Provide extra blanket, if chilled warm blanket Close door, raise temp of room

15. Iron Deficiency Anemia pg 567 Develops when there is an insufficient amount of iron to produce hemoglobin. Occurs when: Heme cannot be recycled d/t blood loss Dietary intake of iron is insufficient Absorption of iron from food is inadequate The need for iron exceeds the reserves

16. Pathophysiology Even when a healthy diet is consumed, less than 10% of the iron is absorbed.

17. Pathophysiology Iron is needed to produce Hgb. Need for iron increases if rapid growth, pregnancy, reproductive years and during menses.

18. Signs & Symptoms Causes reduced energy, feel cold all the time, and experience fatigue and dyspnea with minor physical exertion. Heart rate is fast even at rest

19. Nursing Management 567 Improve the nutritional intake of iron. Use Z tract if iron given IM. Insert the needle at a 90 degree angle. Give test dose Review clinical procedure 37-1 pg. 568 Z-Track Technique on own.

20. Stop Think Respond Why does the nurse use the Z-track method to administer parenteral iron???

21. Z-track method ensures that the medication does not seep into SQ tissue. Iron can irritate and stain tissue

22. Teaching Dilute liquid preparations of iron with another liquid such as juice & drink with a straw to avoid staining the teeth. Take with food or immediately after a meal to avoid gastric distress Take with vitamin C to promote its absorption Expect that iron colors stool dark green or black

23. Sickle Cell Anemia Erythrocytes become sickle or crescent shaped when there is low oxygen in blood Caused by abnormal form of Hgb...Hgb S Hereditary, found primarily in African Americans but also occurs in people from Mediterranean and Middle Eastern countries Must inherit gene from each parent to have the disease but can have the trait if only one parent has the gene..

24. Anemia: Sickle Cell Anemia

26. Sickle Cell Anemia Sickle cell disease--100% Hgb affected Sickle cell trait--40% Hgb affected, so less chance of developing symptoms Abnormality in Hgb shortens life span of erythrocytes and they become hemolyzed (destroyed) Spleen becomes obstructed by excess volume of destroyed RBCs so risk of infection high

27. Sickle Cell Bone marrow enlarges to compensate for the continuous need to produce more RBCs Persistent anemia causes tachycardia, dyspnea, cardiomegaly and arrhythmias Vascular occlusion causes severe pain Sickle cells can lodge in small blood vessels and block blood flow Stroke is common even in small children

28. Signs and Symptoms Severe joint pain is a frequent symptom seen Will have jaundice Chronic leg ulcers as blockage to legs Priapism (prolonged erection as delayed emptying of thick blood Other signs of other types of anemia also present

29. Sickle Cell Crisis Blockage of small vessels reduces blood flow and causes localized ischemia and severe pain Fever, pain, and swelling of one or more joints are common Can lead to CVA, pulmonary infarction, shock and renal failure

30. Sickle Cell Anemia To diagnose sickle cell a sickle cell screening is done. Sickle cell anemia is present when the sickle cell screening test is positive for hemoglobin S.

31. Medical Management pg 569 Treatment is supportive as can�t be cured Blood transfusion may be given (however, transfused blood increases blood viscosity (thickness), which potentially does more harm than good. Every infection, no matter how minor, treated with antibiotics

32. Management A few people have been cured of sickle cell disease, using bone marrow transplantation. The possibility of curing sickle cell disease with gene replacement therapy is undergoing research, but practical application of the technique is remote at this time.

33. Medical management Narcotics given for pain, oxygen given Complete bedrest, hydrated with IV fluids Iron chelating agent (Desferal) given to remove excess iron from transfusions and erythrocyte destruction

34. Stop think, and Respond What is the correlation between sickle cell anemia and stroke (cerebrovascular accident)?????

35. Sickling causes the abnormally shaped RBCs to occlude small blood vessels. Blockage of small blood vessels in the brain results in cerebral injury from a loss of oxygen to the tissue and ultimately cellular death.

36. Teaching Box 37-1 pg 571 Drink extra fluids Dress warmly in cold temperatures Avoid vigorous physical exercise Avoid leg positions or clothing that cause vasoconstriction Stop smoking or other use of nicotine Avoid high altitudes, get flu and pneumonia immunizations and see Dr stat if infection

37. Hemolytic Anemia pg 571 Chronic premature destruction of erythrocytes (RBCs) Causes --cardiopulmonary bypass during surgery, arsenic or lead poisoning, malaria, infectious agents or toxins and exposure to hazardous chemicals and blood transfusion reactions

38. Signs & Symptoms & Treatment Similar to other anemias. In severe form, they may have jaundice and spleen enlargement Steriods given, blood transfusions and if doesn�t respond, spleenectomy done

39. Thalassemia & Cooley�s Anemia pg 571 Group of genetic inherited syndromes of abnormal Hgb synthesis Results in impaired synthesis of either alpha or beta chain of adult Hgb.

40. Thalassemias Pg 571 Hereditary hemolytic anemias Cooley�s anemia, a severe form of beta-thalassemia, has symptoms of severe anemia and a bronzing of the skin caused by hemolysis of RBCs Iron chelation required because of iron deposits in skin Must be on bedrest and protected from infection

41. Thalassemias

42. Pernicious Anemia Pg 571 Develops when there is a lack of intrinsic factor in gastric secretions causes it. The intrinsic factor is necessary for absorption of vitamin B12 B12 and the intrinsic factor is necessary for the maturation of erythrocytes

43. Pathophysology Aging and gastric mucosa atrophy reduces intrinsic factor. Also secondary to the surgical removal of the stomach or small bowel resection in which the ileum, the site for vitamin B12, erythrocytes remain in an immature form.

44. Pernicious Anemia If not recognized, degeneration of nervous system develops and can be permanent if not treated stat.

45. Signs & Symptoms pg 572 Stomatitis, glossitis (red, beefy looking tongue), digestive disturbances and diarrhea. If severe, dyspnea, jaundice, irritability, confusion and depression occur

46. glossitis (red, beefy looking tongue)

47. Signs & Symptoms Numbness and tingling in arms and legs and ataxia (incoordination) are common signs of neurological damage. Vibratory and position sense are sometimes lost.

48. Management pg 572 B12 is given IM for life

49. Folic Acid Anemia Pg 572 Characterized by immature erythrocytes. Commonly caused by insufficient dietary intake of folic acid (Vit B9) Elderly, alcoholics, ones with intestinal disorder that affect food absorption, malignancy and chronically ill may have folic acid deficiency because of poor nutrition Pregnancy and hemolytic anemia can cause

50. Signs & Symptoms Severe fatigue, sore tongue that is beefy red, dyspnea, nausea, anorexia, headache, weakness and light-headedness occur Will have low Hgb and Hct and serum folate is decreased A schilling test differentiates pernicious anemia and one caused by folic acid Only one that has neuro symptoms is pernicious anemia

51. FYI: Schilling Test (Not in book) Schilling test: Give B12 IM to load up patient and then give radioactive B12 P.O. to see if it is discarded in urine. It should be discarded as body has all the B12 it needs. If not discarded then it is positive for pernicious anemia

52. Medical Management Parenteral folic acid given if absorption disorder otherwise a high folic acid diet given As in any other type of anemia, should space activity and plan frequent rest periods Vitamin C enhances absorption of folic acid and iron

53. Erythrocytosis pg 573 An increase in circulating erythrocytes. One of these conditions is called polycythemia vera Polycythemia vera has a greater than normal RBC, WBC and platelets (also called thrombocytes) If living at higher altitudes it is normal to have erythrocytosis

54. Polycythemia Vera Despite the abundance of erythrocytes, their life span is shorter. The dead RBC release potassium and uric acid (causes gout) Oxygen combining capacity is lower and since blood is thicker, clots form Complications include hypertension, CHF, stroke, areas of infarction and hemorrhage

55. Signs and Symptoms Face and lips are reddish purple. Fatigue, weakness, headache, pruritis, exertional dyspnea and dizziness are common Excessive bleeding after minor injury as engorged capillaries and veins. Spleenomegaly and swollen and painful joints because of uric acid levels.

56. Medical Management pg 573 A phlebotomy (opening a vein to withdraw blood) is done several times a week; 500 ml is removed at a time. Anticoagulants are given and radiation can be given to slow production of RBC in the bone marrow. Cancer drugs (Mustargen) given to curb excessive bone marrow activity

57. Nursing Management Drink 3 quarts of fluid daily Avoid crossing legs and wearing tight clothing that impairs circulation Change position frequently and elevate lower extremities Do isometric exercises --contracting and relaxing quadriceps and gluteal--wiggle toes Wear TED hose or support hose, rest stat if chest pain

58. Leukemia Pg 573 Malignant blood disorder--too many leukocytes (WBC) formed but they are immature. Besides making too many WBCs, there is a decrease in RBC formation. WBC is too immature to fight infection Cause unknown but exposure to toxic chemicals and radiation, viruses, and certain drugs precipitate

59. Pathophysiology Severe anemia eventually occurs, and the reduction of platelets leads to bleeding. The excessive numbers of leukocytes infiltrate the spleen, liver, lymph nodes, and brain if unchecked

60. Types of Leukemia (Table 37-3 pg 574) Review on own

61. Signs & Symptoms Infections, fatigue from anemia, and easy bruising are hallmarks of leukemia. Fever is present, spleen and lymph nodes enlarge and internal and external bleeding occurs. Nosebleeds, mouth and GI tract are common sites of bleeding

62. Medical Management Drug therapy is the primary weapon for arresting leukemia The type of drug or combination of drugs depends on the form of leukemia. Erythrocyte & platelet transfusions are necessary to treat the anemia and decrease in platelets.

63. Treatment Antibiotics to prevent infection, and bone marrow transplant and stem cell transplantation have all increased the survival rate. Can be from oneself (autologous) or from another (allogenic)

64. Nursing management Drugs used to fight leukemia are highly toxic and can impair formation of all blood cells Antineoplastic drugs cause alopecia, nausea, vomiting, diarrhea, excessive bleeding, anorexia, stomatitis and oral ulcerations Give small frequent feedings of bland foods and frequent sips of cool water to maintain fluid and nutrition

65. Nursing Management Initial assessment includes a history of current symptoms and past symptoms Joint pain and other symptoms associated with leukocyte infiltration of the central nervous system ( headache, confusion) can occur. Observe for symptoms of kidney, ureteral, or bladder stones as high uric acid form chemo--increase fluids

66. Nursing for Leukemia (Care plan pg 575) Assess for signs of infection (swelling and tenderness) Protective isolation Neutropenic precautions--box 37-2 page 576--no raw fruits or vegetables or flowers and etc. Stay away from people who are ill, including nurses

67. Nursing for Leukemia Monitor platelet count--inspect skin for bruises and petechiae Handle gently to avoid bruises Report melena (black tarry stools), hematuria, epistaxis Apply prolonged pressure after injections or IV sites Spongy toothettes for oral hygiene, use electric razor

68. Teaching for Leukemia Box 37-2 pg 577 See Dr stat is excessive bleeding or bruising or symptoms of illness or infection If sores in mouth so not self treat see Dr. stat. Call Dr. stat if severe nausea with prolonged vomiting, severe diarrhea, fever and chills, excessive bruises or bleeding, cough, chest pain, cloudy urine, rash, blood in stool or urine, severe headache, extreme fatigue, increased resp rate or dyspnea

69. Multiple Myeloma pg 576 Malignancy involving plasma cells, lymphocyte-like cells in the bone marrow. Prognosis is poor, with estimated survival of 1 to 5 years Associated with aging and rarely occurs before 40. Immature plasma cells proliferate (multiply rapidly) in the bone marrow forming single or multiple osteolytic (bone destroying) tumors

70. Multiple Myeloma Malignant plasma cells release an abnormal protein called M-type globulin. Excess production of plasma cells reduces production of erythrocytes, leukocytes and platelets. Later the liver, spleen, soft tissues and kidneys are affected

71. Multiple Myeloma First symptom is usually vague pain in pelvis, spine or ribs. As it progresses the pain is more severe and localized When bone marrow is replaced by tumors, pathologic fractures occur. Resistance to infection is decreased as lymphocytes make antibodies Bone marrow causes anemia and renal calculi and renal failure occur

72. Multiple Myeloma Skeletal X-ray shows punched out bone lesions in bone marrow. Serum calcium levels are elevated due to bone destruction and uric acid levels are high

73. Nursing for Multiple Myeloma Assess for signs of infection, excessive fatigue and pain in new areas Assist with ambulation because immobility can worsen loss of calcium from bone Push fluids up to 4000 cc daily to prevent renal damage Safety 1st as any injury, no matter how slight can cause fractures. Give analgesics before care & delay bath until med helps

74. Agranulocytosis pg 579 Reduction in production of granulocytes (neutrophils, basophils and eosinophils. Causes higher risk for infection. Number one defense against bacterial infection are granulocytes. Most common cause is toxicity from drugs

75. Signs & Symptoms Fatigue, fever, chills, headache, and opportunistic infections in mouth, throat, nose, rectum or vagina If cause found and treatment started early usually recover

76. Nursing management Determine names of all drugs taken in last 6 months to a year. Protective isolation Protective isolation is necessary if the leukocyte count is extremely low. Visitors or staff with any type of an infection are restricted from close client contact until the infection has cleared.

77. Pancytopenia pg 579 Refers to conditions such as aplastic anemia in which numbers of all marrow-produced blood cells are reduced. Aplastic anemia is more than just a deficiency of erythrocytes---�failure to develop�

78. Aplastic Anemia pg 579 Consequence of inadequate stem production in bone marrow �so insufficient numbers of erythrocytes, leukocytes, and platelets, collectively described as pancytopenia. Exposure to toxic chemicals, radiation and drug therapy with anticancer drugs and some antibiotics. Death rate is high

79. Signs & Symptoms Typical symptoms of anemia, frequent opportunistic infections, plus coagulation abnormalities, unusual bleeding, small skin hemorrhages (petechiae and ecchymosis. spleen is enlarged Bone marrow confirms diagnosis In some cases the bone marrow will regenerate and have normal function if cause found and corrected

80. Medical Management Transfusions given Antibiotics given to prevent infection Steroids given in cases of an autoimmune connection Bone marrow transplantation if donor found Stem cell transplantation is an alternative

81. Nursing Management Assess s/s severe anemia, infection, and bleeding Soft foods and oral hygiene techniques to avoid bleeding gums. Avoid chewing as much as possible Give good oral hygiene Protective isolation, restrict visitors if WBCs very low Apply pressure if injections must be given

82. Coagulopathies pg. 580 The term coagulopathy refers to conditions in which a component that is necessary to control bleeding is missing or inadequate. Two common examples are thrombocytopenia and hemophilia.

83. Thrombocytopenia pg 580 Lower than normal number of platelets or thrombocytes Accompanies leukemia and other malignant blood disease and caused by severe infections, and certain drugs Idiopathic thrombocytopenia purpura is thrombocytopenia without a known cause

84. Thrombocytopenia Purpura, small hemorrhages in the skin, mucous membranes, or subQ tissue. Bleeding from other parts of body also occur (nose, oral mucous membranes, GI. Internal hemorrhage can be fatal Spleenectomy may be needed to raise platelet count and relieve symptoms

85. Hemophilia pg 581 Disorder involving clotting factors. Three types but most common types A, B, & C. Type A is most common & results from a deficiency of factor IIIV Genetic-Inherited from mom to son as a sex-linked recessive characteristic. Daughters inherit the trait and can pass on disease to their sons but seldom develop the disease. Bleeding noted in infancy and childhood. Milder may go unrecognized for years

86. Hemophelia Persistent oozing and sometimes severe bleeding that occurs spontaneously after injury. Bleeding in joints will damage the joints and lead to deformity and limitation of movement. Deficiency of factor 8 or 9 (Christmas disease) Treatment is transfusions , direct pressure, cold compresses and measures to control bleeding

87. Nursing management Assess joints and mobility Inspect skin for purpura or hemorrhagic areas Ask if BP cuff causes bleeding Temp taken using tympanic to avoid oral or rectal injury Prevent trauma, reduce pain, conserve energy

88. CONSIDERATIONS Review Nutritional, Pharmacologic, Gerontologic Considerations !!!!! There are a lot of similarities so focus on what makes it different��