1
NURSING CARE OF THE CLIENT/FAMILY WITH A HEMATOLOGICAL HEALTH DEVIATION Nursing 304
I.
Blood types--presence or absence of A or B antigens on RBC membranes is the basis of grouping of blood types; everyone has antibodies termed anti-A or anti-B that react with the A or B antigens; Hemolysis occurs when donor blood does not match the recipient’s blood.
A. Types 1. Type A--has A antigens and anti-B antibodies; If receives B or AB blood, the anti-B antibodies will react with the B antigen present in the B blood and cause agglutination. Can receive type A and type O. 2. Type B--has B antigens and anti-A antibodies; Can receive type B and type O. 3. Type AB--has A and B antigens and no antibodies. Can receive A, B, AB, and O (universal recipient) 4. Type O--has neither A nor B antigens (no antigens) but anti-A and anti-B antibodies. (Universal donor but can receive only type O.)
B. Rh Factor--based on a third antigen, (D) 1. Rh positive person has the D antigen and no anti-D antibody 2. Rh negative person does not have the D antigen but if exposed to Rh positive blood will form the anti-D antibody which will act against the Rh antigens with future exposures
II.
Blood Component Therapy A. Common Blood Components Administered 1. Whole Blood 2. Packed RBCs 3. Granulocytes 4. Platelets 5. Fresh frozen plasma—coagulation factors 6. Cryoprecipitate—fibrinogen and some coagulation factors 7. Albumin
B. Procedure for administration of blood products
2
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18.
C. Types of Blood Transfusion Reactions
1. Acute Reactions a. Febrile, non-hemolytic
3
1. Most common--Not life threatening 2. Chills and fever within 2 hours of start of transfusion; Administer antipyretics as needed 3. Client's antibodies react to WBCs in the donor blood; happens most often in people who have had previous transfusions and developed antibodies 4. Leukocyte poor blood can be used if client known to have this problem previously; Also, special in-line filters can be used
b. Acute hemolytic 1. Life-threatening 2. Occurs when donor blood is incompatible with recipient (from errors in matching and patient identification before giving); Antibodies in recipient combine with antigens on donor erythrocytes causing agglutination and hemolysis. 3. Chills, fever, low back pain, nausea, dyspnea, anxiety, blood in urine; Hypotension, bronchospasm and anaphylaxis possible 4. Must be prevented. Must be recognized quickly and transfusion stopped. Take down all tubing. Hang new tubing and infuse NS slowly. Thorough assessment. Notify MD. Obtain required blood and urine specimens (hemolysis). Document!
c. Allergic 1. Usually because of sensitivity to a plasma protein in the blood 2. Itching, flushing 3. Stop transfusion, infuse NS. Notify MD. Usually antihistamines (Benadryl) resolve the problem and the blood can be resumed. 4. If severe, blood will not be restarted. Epinephrine and steroids may be needed. 5. In the future, this client should get antihistamine before transfusion.
d. Circulatory overload 1. Usually happens because client is sensitive to overload, such as in CHF, or if nurse allows blood to infuse too rapidly. 2. PRBCs better than whole blood unless volume needed. 3. Diuretic may be given after transfusion or between two units. 4. S&S: dyspnea, tachycardia, anxiety, crackles, pink, frothy sputum 5. Nurse should place client upright, feet down. Stop blood. Infuse NS at KVO rate. Notify MD. May need oxygen, diuretics.
2. Delayed Reactions to Blood Transfusion a. Delayed hemolytic 1. Usually mild and requires no intervention. 2. Usually will occur within 2 weeks of transfusion--takes this long for antibodies to get strong enough to do damage to donor blood. Client usually home by this time and doesn't recognize the problem.
4
2. Cells are hemolyzed per RES and is gradual 3. Fever, anemia, increased bilirubin, level possible jaundice. Subsequent transfusions could be acute hemolytic.
b. Infection from blood transfusions 1. 2. 3. 4.
Hepatitis B and C HIV Malaria Syphilis
c. Iron overload 1. Occurs with clients who get frequent transfusions 2. Can cause severe organ damage 3. Requires iron chelation therapy
d. Graft-versus-host disease
III.
Assessment of the hematological system A. Nursing assessment B. Diagnostic studies 1. Lab Tests a. CBC 1. CBC with diff--RBC, Platelets, and WBCs with the different types ID'd 2. Peripheral blood smear --sizes and shapes of cells identified b. ESR--erythrocyte sedimentation rate; indicates inflammation c. Clotting Studies d. Iron studies e. Blood typing and Rh factor f. Bence Jones protein--random urine specimen (negative normal, positive usually means multiple myeloma)
2. Radiologic Tests a. Lymphangiography (dye) b. Scans (Bone Scan, Liver/Spleen Scan)
3. Biopsies a. Bone Marrow aspiration and biopsy 1. Posterior and anterior iliac crest or sternum 2. Informed consent
5
3. 4. 5. 6.
Premedicate for pain—will be sore for several days Assess platelet count before Must empty bladder before and be very still Hold pressure 5 – 10 minutes or longer and stay in bed for about 2 hours
b. Lymph Node aspiration and biopsy 1. Local anesthetic 2. Small dressing afterwards
III.
RBCs : normal 4 – 6 million A. Main function is that they have hemoglobin that transports oxygen Hct is generally 3 x Hgb; RBCs live about 120 days; Normally removed from blood by reticuloendothelial cells (RES) which are those responsible for phagocytosis of damaged or old cells and are found primarily in the liver, spleen, and lymph nodes; Released iron is mostly recycled to make new hemoglobin in the bone marrow; Immature RBCs called reticulocytes. Stem cell>>>erythroblast>>>reticulocyte>>erythrocyte
B. Low RBCs: anemia (The more rapid onset, the more severe symptoms; the more chronic, the less severe) 1. Overall S&S with low RBCs (There are some specific S&S particular to different anemias) a. b. c. c. e. f. g.
Dyspnea (activity intolerance, Gas exchange impaired) Fatigue, malaise, headache (Activity Intolerance) Dizziness, fainting (High risk for injury) Pallor Tachycardia, palpitations, chest pain, MI Depression Cheilosis [ki-lo-sis] (inflamed lips), Glossitis (inflamed tongue)
2. General Management and Nursing Care for low RBCs-aimed at correcting underlying problem if possible and controlling symptoms a. Alternate rest and activity b. Diet Iron (organ meats, eggs, dark green leafy veggies), Folic acid (same as iron), B12 (red meats, liver) c. Meds—Iron,Folic Acid, B12 injections d. Oxygen e. Blood transfusions f. Assessment: alcohol, anticonvulsants, and oral contraceptives alter folic acid absorption
3. Anemias:
not a disease but a manifestation of a pathology;
6
a. Blood Loss Anemias b. Hypoproliferative Anemias--Decreased erythropoiesis (will see low reticulocyte count; RBCs live a normal life, but there are too few of them) 1. Lack of stimulation
2. Lack of factors needed to make RBCs a. Iron deficiency (microcytic) Specific symptoms of Iron deficiency anemia:
Care specific to Iron Deficiency Anemia:
b. Vitamin B 12 deficiency--also called Megaloblastic (large cell) anemia, Cobalamin deficiency (and Pernicious anemia when the only problem is lack of Intrinsic Factor)
Specific symptoms of Vitamin B12 deficiency: May take years to manifest due to fairly large stores of B12 in the body
7
Diagnostic test specific for Vitamin B12 deficiency: Schilling Test--receives oral dose of radioactive Vitamin B12. If absorbed, there will be radioactivity in the urine within the 24 hour urine test, and you know the small intestine is not the problem. If no radioactivity in urine, not sure if problem is intestine or stomach. So, the test is repeated with intrinsic factor added to the oral radioactive B12. If the urine has radioactivity, it means that true pernicious anemia is the problem.
Specific Nursing Care for Vitamin B12 deficiency:
c. Folic Acid Deficiency--also Megaloblastic
Specific symptoms of Folic Acid Deficiency:
Diagnostic test specific for Folic Acid Deficiency: Measuring folate within the RBC Specific Nursing Care for Folic Acid Deficiency: Diet, Folic acid supplements , oral care
c. Anemia from bone marrow damage or suppression-meds, chemicals, cancer 1. Aplastic anemia/Pancytopenia
8
2. Myelodysplastic Syndromes (MDS) a. Abnormal development of cells; Most common is RBCs being macrocytic (large), but the WBCs and platelets can also be affected. Many cells die before leaving bone marrow, and even those released have reduced ability to do what they are supposed to do. Most turn into acute myeloid leukemia (AML) b. Most often due to age, but can occur secondary to chemical exposure or chemotherapy. c. S&S like pancytopenia d. Dx test reveals macrocytic red cells e. Treatment of Myelodysplastic Syndromes 1. Symptomatic care 2. Allogenic bone marrow transplant 3. Transfusions of blood and platelets 4. Neupogen and Erythropoietin
d. Hemolytic Anemias--RBC destruction Erythrocytes destroyed for whatever reason; hypoxia develops and signals the kidney to release erythropoietin, so the bone marrow marrow is stimulated to make more RBCS and they get released prematurely as reticulocytes. As this continues, jaundice occurs. 1. Symptoms specific to hemolytic anemias
2. Many forms but we will focus on Sickle Cell Anemia. a. Hereditary. Inheritance of sickle hemoglobin gene (HbS) which causes hemoglobin to be defective. When O2 levels are low, the S hemoglobin loses round, flexible shape and become rigid and sickle-shaped. They get caught in small vessels and restrict or stop blood flow to tissue or an organ. With ischemia and infarction, pain, swelling and fever occurs. Exposure
9
to cold can cause vasoconstriction and lead to the same problem. With restored oxygen tension, the sickled cells "unsickle". Repeated episodes damage RBCs and they die. This client's RBCs only live about 30 to 40 days. Even person who only has the trait can have some symptoms in extreme situations of hypoxia and FVD. b. Diagnostic testing for Sickle Cell Anemia: 1. Low hematocrit and sickled cells on smear 2. Confirmed by hemoglobin electrophoresis which identifies the hemoglobin S c. Symptoms specific to Sickle Cell Anemia
d. Management of sickle cell anemia--goals are to
prevent effects of anemia and avoid crises 1. 2. 3. Hydroxyurea (chemotherapy)--decreases formation of sickled cells, but has many negatives (WBC suppression, secondary malignancy, birth defects if birth control not practiced) 4. 5. 6. 7. 8 9. 10. e. Management of sickle cell crisis
10
C. High RBCs: Polycythemia 1. Overall S&S with high RBCs--due to excessive volume & viscosity a. Ruddy [red] complexion b. Headache, dizziness c. Angina, dyspnea d. Pruritis (if polycythemia vera due to excessive basophils) d. Thrombosis is major complicatio
2. General Management and Nursing Care for High RBCs a. b. c. d.
Phlebotomy Avoid antiplatelet meds if bleeding a problem Avoid hot baths, antihistamines (if polycythemia vera) Measures to decrease thrombotic events (TEDS, SCDs, elevate legs, no leg crossing, adequate hydration, mobility/leg exercises)
3. Polycythemia Vera or Primary Polycythemia-Bone marrow & spleen have excessive hematopoiesis a. All myeloid cells increased, but mostly RBCs, thus Hgb and Hct are elevated b. Eventually bone marrow "burns out" and becomes fibrotic c. Dx by CBC, enlarged spleen, and S&S d. Can end up in Acute Myeloid Leukemia (AML) 4. Secondary Polycythemia--increased RBCs due to chronic
hypoxia
11
IV. WBCs: Main function protect against infection and tissue injury, [5,000 - 10,000] A. A&P 1. Leukocytes from Myeloid cells a. Granulocytes--60 - 70% of leukocytes are these 1. Immature granulocytes are called bands. “Shift to left” 2. Mature granulocytes are called Segs. 3. Types of granulocytes a. Neutrophils--Segmented neutrophils (Segs) Important for phagocytosis ; Arrive at site of inflammation very quickly but only live 1 - 2 days in this job b. Eosinophils--function in hypersensitivity reactions by working to neutralize histamine c. Basophils--produce and store histamine and other substances involved in hypersensitivity reactions b. Monocytes 1. Only about 5 % of total leukocytes but are the largest 2. Are 2nd to arrive at site of inflammation and continue phagocytosis as macrophages 3. Produced in bone marrow, circulate briefly and then become macrophages in various body tissues such as spleen (main site), liver, lung, and lymph nodes. Macrophages are the Reticuloendothelial System (RES)
2. Leukocytes from Lymphoid cells a. Produce substances that aid in attacking foreign material
12
b. 30 - 40% of leukocytes are lymphocytes but only about 1% circulating in blood. They migrate through lymphatics and blood vessels to lymphoid tissues all over the body. As they go, they mature into either B or T Lymphocytes.
1. B lymphocytes a. Produced in Bone marrow b. Can differentiate into plasma cells which produce immunoglobulins or antibodies which are proteins that destroy foreign material-- humoral immunity 2. T lymphocytes a. Produced in bone marrrow but migrate to and mature in the thymus. b. Responsible for cellular immunity (cell mediated immunity) 1. Kill foreign cells directly or release substances that enhance phagocytosis 2. Responsible for delayed allergic reactions, destruction of tumor cells, and transplant rejection
B. Low WBCs: 1. Terminology a. Leukopenia: low WBCs in general; b. Neutropenia: neutrophils less than 2,000; 1st responders Absolute neutrophil count (ANC) ANC = Total WBC X (% of neutrophils + % of bands) 100 c. Granulocytopenia (Agranulocytosis): low granulocytes d. Lymphopenia—lymphocytes < 1500
2. Overall S&S with low WBCs No real S&S until has major infection and then will manifest mildly. A significant infection may only have a low grade fever.
3. General Medical and Nursing Care for low WBCs NEUTROPENIC PRECAUTIONS (WBCs < 2,000) a. b. c. d. e.
Assess carefully for infection (urine, lungs, wounds, Temp q 4 hours) Monitor CBC with differential Careful handwashing by all No one with infection in room Everything cooked
13
f. No fresh flowers g. Oral care q 4 hours h. Perineal care after each BM i. IV site care—no multiple sticks j. Antibiotics given on time k. Avoid litter box, bird cage, etc. l. Avoid crowds m. Teach how to prevent and monitor for infection n. CSF—Neupogen, Neulasta
C. High WBCs: Leukocytosis; Can be from infection or inflammation; S&S and Tx depends if increase due to normal response or malfunction
1. Leukemias--proliferations of WBCs in bone marrow--classified according to whether from lymphoid or myeloid stem cells; Genetic? Viruses? Most deaths due to infection a. Myeloid
1. Acute Myeloid Leukemia-AML a. b. c. d. e.
Proliferation of all myeloid cells but immature Common in adults Usually poor prognosis S&S b/c of all cells abnormal (anemia, infection, bleeding) Tx: Supportive care (antibiotics, blood, etc) Chemo, Allogenic bone marrow transplant
2. Chronic Myeloid Leukemia-CML a. Proliferation of all myeloid cells, but there are also normal cells b. Usually older people get this, and asymptomatic for years, but then as it progresses symptoms begin (bone pain, fever, wt loss) c. Tx: Treated symptomatically at first, then as it becomes more acute, chemo is stepped up more aggressively, Supportive care, Bone marrow transplant before becomes acute
b. Lymphoid 1. Acute Lymphocytic Leukemia-ALL a. Proliferation from lymphoid stem cell b. Usually occurs in very young children c. Most survive about 5 years with treatment d. Immature lymphocytes proliferate and crowd out production of other cells, thus S&S are of decreased RBCs, platetlets, and large numbers of immature white cells; Also large numbers of cells pool in liver and spleen; bone pain common e. Tx: chemo, radiation, allogenic bone marrow transplant
2. Chronic Lymphocytic Leukemia-CLL
14
a. B Lymphocytes mature but are in excess and accumulate in bone marrow, circulation, lymph nodes, liver, spleen, and other organs causing pain b. Most common in US and Europe c. Usually over age 60 d. S&S of anemia and thrombocytopenia develop once they are crowded out; Also can get "B" symptoms: fever, night sweats, wt loss In this case, they have defective humoral and cell-mediated immunity so infections are common e. Tx: Not necessary when asymptomatic, but once with symptoms may get chemo, steroids, immunoglobulins
Nursing Care of Leukemias: a. b. c. d.
2. Malignant Lymphomas--cancer of lymphoid cells; Proliferation of lymphocytes a. Hodgkin's Lymphoma 1. Malignancy, easily cured in early stages; usually starts in lymphatic system but can spread outside 2. Dx: Excessional lymph node biopsy with presence of Reed Sternberg cell (giant tumor cell); then must be "staged" (to determine the extent of the disease) 3. S&S of Hodgkin’s Lymphoma a. b. c. d. Alcohol causes pain in affected nodes
15
4. Treatment of Hodgkin’s Lymphoma
b. Non-Hodgkin's Lymphoma 1.
2. S&S of Non-Hodgkin’s Lymphoma
Nursing Care of Both Lymphomas:
3. Multiple Myeloma a. Malignancy of the plasma cells (mature form of lymphocyte) b. Excessive, non-functioning immunoglobulins (remember plasma cells secrete immunoglobulins which are necessary for antibody production) c. Diagnosed by: 1. 2. 3. 4. d. S&S of multiple myeloma:
e. Treatment of multiple myeloma
16
V.
Platelets (thrombocytes) A. Main function is control of bleeding (hemostasis) Live about 7 - 10 days; Float around doing nothing, then collect at site of vascular injury forming a plug (fibrin clot)
B. Normal value is 150,000 - 400,000 C. Low Platelets 1. Overall S&S with low platelets a. b. c. d. e. f. g. h.
Petechiae Echymosis Easy bleeding Hematuria Heavy menses GI bleeding Intracranial bleed with neurological changes Anemia S&S
2. General Medical and Nursing Care for low platelets Treat underlying problem, give platelets, splenectomy, Neumega
3. BLEEDING PRECAUTIONS a. Assess frequently • Bleeding • Decreased BP, tachycardia, restlessness • Lab values b. No Foley, flossing, rectal measures, IM c. Soft toothbrush, electric razor d. Stool softeners e. 5 minute pressure after punctures; call MD if still requiring pressure after 10 minutes f. Administer blood, platelets, FFP g. Avoid suction if possible h. Teach
4. Thrombocytopenia-- platelets < 20,000 a. Caused by: 1. decreased production (marrow diseases, drug SE) 2. increased destruction (infection or immune disorders, hypersplenism) 3. increased consumption (DIC), or bleeding b. Petechiae begin with platelets < 20,000.
17
c. d. e. f.
Serious hemorrhage with platelets < 10,000 Problems occur when platelet count ok, if platelets are dysfunctional Dx with bone marrow aspiration and biopsy Management by treating underlying problem
5. Idiopathic Thrombocytopenic Purpura (ITP) a. Various causes: meds (those that alter platelet function--ASA, NSAIDS, sulfur), viruses, SLE, cause antiplatelet autoantibodies to bind to platelets and macrophages destroy them. Platelet production increases in marrow to compensate. b. Dx: bone marrow aspiration shows increased platelet precursors; CBC shows low platelets c. Tx: address underlying cause, steroids, splenectomy d. Nursing Care: bleeding precautions
6. Primary Thrombocythemia (platelets > 600,000 but poor function, thus bleeding occurs) a. Myeloproliferative disorder--not sure why b. Similar to polycythemia vera c. Nursing care focused on assessment and care of thrombosis and bleeding
7. Disseminated Intravascular Coagulation (DIC)
D. High platelets--many acquired and hereditary causes 1. Terminology a. Thrombotic disorders/thrombophilia--tendency to make clots --can be venous or arterial (Thrombocytosis) b. Secondary Thrombocytosis--Is a response to some other problem and usually & significant S&S, and resolves once underlying cause fixed
2. Overall S&S with high platelets--thrombotic episodes a. b. c. d.
Pain in extremities, ulcerations (decreased perfusion) Chest pain, MI CVA Death
3. General Medical and Nursing Care with High Platelets a. Medical Management: 1. Heparin therapy
18
2. Low-Molecular-Weight-Heparin Therapy (LMWH) Examples: Lovenox, Fragmin
3. Coumadin therapy
b. Nursing Care of Thrombotic Disorders 1. 2. 3. 4. 5.
Prevent venous stasis (mobility0 Hydration Meds (ASA, Persantine, Plavix) Monitor for clotting/bleeding Teach to avoid foods high in vitamin K (dark leafy veggies)
19
20
21
STEM CELLS IN BONE MARROW
MYELOID CELLS
ERYTHROCYTES RBCs
LYMPHOID CELLS
THROMBOCYTES Platelets NK cells B LYMPHOCYTES Can attack T LYMPHOCYTES Mature in bone marrow any target Mature in thymus Humoral Immunity Cell Mediated Immunity
LEUKOCYTES WBCs
Memory cells Acquired Immunity
Imm GRANULOCYTES Large #s Bands--babies
NEUTROPHILS Segs--mature Phagocytic 1st response
MONOCYTES Small #s Macrophages--RES 2nd Response
BASOPHILS Release histamine and heparin in allergic response
EOSINOPHILS Neutralize histamine; defend against parasites
PLASMA CELLS Make Antibodies IgM 1st formed IgG 2nd formed Most #s IgE Releases histamine IgA Mucosal IgD ?????
Memory cells Acquired Regulator T cells
Effector T cells
Helper T Suppressor T "ON" "OFF" Promotes Shuts off & proliferation limits T helper of B Lymphs and B cells and T cytotoxic
T cytotoxic Killer cells
22