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Assessment and Management of Patients With Hematologic Disorders – Part 2 :Assessment and Management of Patients With Hematologic Disorders – Part 2 Loretta Elder RN MSN CAPA


Anemia :Anemia A condition of a lower than normal level of Hgb, which reflects fewer than normal RBC in circulation. As a result, the amount of oxygen delivered to tissues is decreased. Can result from dietary problems, genetic disorders, bone marrow disease or excessive bleeding (i.e. GI Bleed) See Table 33-2 1045 for Classifications of Anemia


Anemias: :Anemias: Hemolytic(result from increased destruction RBC Sickle Cell G6PD deficiency Immunohemolytic Thalessemia Anemia that result from deficiency of RBC Iron Deficiency Vitamin B12 Deficiency Folic Acid Deficiency Aplastic Polycythemia Myelodysplastic Syndrome


Sickle Cell Disease-1055 :Sickle Cell Disease-1055 Main problem in this disorder is the formation of abnormal hemoglobin chains The normal adult hemoglobin is called HemoglobinA(HbA) In sickle cell disease, there is an abnormality in the beta chains known as HemoglobinS(HbS) HbS is very sensitive to oxygen levels of the RBC SICKLE CELL CRISIS


Sickle Cell Disease :Sickle Cell Disease A pair of genes, one from each parent, is needed for the abnormal beta chains to form When the individual inherits one gene from a parent, that individual has Sickle Cell Trait Genetic counseling is needed because this individual can pass on the trait and if the genetic material is combined with another individual who also has the trait the likelihood of Sickle Cell Disease increases Sickle Cell Disease occurs when the individual receives two abnormal genes, one from each parent Abnormal hemoglobin is called Hemoglobin S (HgS)


Sickle Cell Disease :Sickle Cell Disease A genetic disorder Most common in African Americans (some Mediterranean, Caribbean, Middle Eastern, & Central American groups)


Assessment: :Assessment: Adult usually has long standing diagnosis of the disorder Adult with sickle cell trait may be unaware of a problem until there is an acute illness or anesthesia is administered History:


Sickle Cell Disease :Sickle Cell Disease Clinical Manifestations 1056 Diagnostics: Xray may show skull “crew cut” from bone cell surface destruction and regrowth. Joint xrays may show necrosis EKG, CT MRI – show organ and soft tissue damage from tissue hypoxia Diagnosis-based on percentage of Hbs Electrophoresis Sickle Cell Trait have less than 40%HbS Sickle Cell Disease have 80-100% Does not change during crisis Hematocrit (Hct) Sickle Cell Disease is low-Decreases more during crisis Bilirubin and WBC usually higher


Sickle Cell Disease :Sickle Cell Disease List Common Nursing Diagnoses and Collaborative Problems List Nursing Interventions for Each Diagnosis identified


Sickle Cell Disease :Sickle Cell Disease Medical Management Assessment of precipitating factors and tolerance to activity Prevent risk of crisis Prophylactic Therapy Analgesics Hydroxyurea (Droxia) Rest – Oxygen – Pain Management – Hydration


Sickle Cell Disease in Child :Sickle Cell Disease in Child Symptoms of disease not usually present until after 4-6 months due to large amounts of HbF present in the first few months of life. Delayed growth and puberty usually present. Child usually has small stature until age 20.


Glucose-6-Phosphate Dehydrogenase Deficiency Anemia-1061 :Glucose-6-Phosphate Dehydrogenase Deficiency Anemia-1061 A hemolytic anemia that develops when there is a deficiency of the enzyme G6PD Enzyme is responsible for energy production that helps maintain the structure of the cell membrane Cells with inadequate G6PD are more likely to lyse as the cells age These RBCs are also more likely to lyse with certain drugs sulfonamides, aspirin, quinine derivatives, thiazide diuretics, and vitamin K derivatives. Toxins also cause Stressors also trigger this anemia MUST REMOVE SOURCE


Glucose-6-Phosphate Dehydrogenase Deficiency Anemia :Glucose-6-Phosphate Dehydrogenase Deficiency Anemia Symptoms usually take several days to develop Usually asymptomatic until exposed Usually see anemia and jaundice Acute tubular necrosis (due to waste & Hgb in Kidney) Treatment Monitor for S/S anemia, fever, kidney malfunction Avoid Exacerbating Drugs Avoid Stressors Hydration Osmotic Diuretics – Mannitol (Osmitrol) Possible Transfusion


Immunohemolytic Anemia-1062RBC’s are viewed as non-self by the immune system and are destroyed :Immunohemolytic Anemia-1062RBC’s are viewed as non-self by the immune system and are destroyed Results from trauma to RBCs Caused by hemodialysis machines, cardiopulmonary bypass machines, prosthetic heart valves, burns, radiation, antibody reactions, drugs, toxins, and chemical agents. Clinical Manifestations Hepatosplenomegaly, Jaundice, Pathologic Fractures, and S/S Similar to Other Anemias Treatment Immunosuppressive Therapy, Blood Transfusions, Fluid and Electrolyte Replacement, Splenectomy, Folate, and Steroid Therapy


Thalassemia – Cooley’s Anemia-1061 :Thalassemia – Cooley’s Anemia-1061 Inherited disorder, similar to Sickle Cell When the disorder is mild, the person may have normal hemoglobin and few signs and symptoms When severe, the person may need transfusion to sustain life Incidence highest in individuals of Mediterranean descent Clinical Manifestations Small Stature (bone pain), Fractures (of long bones, ribs, and vertebrae), Hepatomegaly, Hemolysis, Severe Anemia and Hypoxia, Febrile, Pallor & Jaundice Iron overload can also occur from the repeated transfusions Diagnosis Hgb ↓ RBCs are Microcytic, Hypochromic Fragile RBCs, Bull’s eye appearance (target cells) Most often diagnosed during infancy due to low Hgb levels Prognosis Adult usually has milder form Persons with severe thalassemia may live into their 20’s


Thalassemia in the Child :Thalassemia in the Child See: Protruding forehead, maxillary prominence, wide set eyes with a flat nose, greenish yellow skin tone.


Nutritional deficits caused by:Diet, Poor Absorption,Body’s Increased Need :Nutritional deficits caused by:Diet, Poor Absorption,Body’s Increased Need Iron Deficiency Anemia (most common) Vitamin B12 Deficiency Anemia Megaloblastic (Macrocytic) Pernicious Anemia Folic Acid deficiency anemia


Iron Deficiency Anemia-1049 :Iron Deficiency Anemia-1049 Clinical Manifestations Similar to Anemia RBCs are microcytic Laboratory Values Serum Iron Concentration ↓ Total Iron-Binding Capacity ↑ Serum Ferritin Levels ↓ Inadequate supply of Iron causes a decrease in Hgb Common in elderly, women and people with poor diets.


Iron Deficiency Anemia :Iron Deficiency Anemia Nursing/Medical Treatment Increase Dietary Iron Oral Supplements (Ferrous Sulfate) Parenteral Iron Sources


Iron Deficiency AnemiaIn Children :Iron Deficiency AnemiaIn Children Most common cause of anemia in infancy, childhood and adolescence Rare before 4-6 months due to presence of maternal iron stores Decreased iron intake is often related to the intake of large amounts of cow’s milk instead of breast milk/iron fortified formula. Adolescents are at risk due to increased growth rate and poor eating habits. Usually treated with oral iron preparation as increased diet in iron is usually not enough.


Vitamin B12 Deficiency Anemia-1052 :Vitamin B12 Deficiency Anemia-1052 Megaloblastic Anemia Due to improper DNA synthesis and increased size of cells Pernicious Anemia Due to a deficiency of adequate intrinsic factor (IF) Essential for intestinal absorption of B12 Often the result of chronic gastritis or gastrectomy Other causes are loss of pancreas, loss of ileum, & strict vegetarians (rare) Clinical Manifestations


Vitamin B12 Deficiency Anemia :Vitamin B12 Deficiency Anemia Assessment and Diagnosis MCV ↑ MCHC Normal B12 level and Folic Acid level Schilling’s Test of Vitamin B12 absorption Treatment Increase Dietary B12 B12 Supplements Cyanocobalamin Injections (IM)


Folic Acid Deficiency Anemia-1052 :Folic Acid Deficiency Anemia-1052 Similar to B12 Deficiency except NO Neurological Problems Also produces an anemia where the cells are described as megaloblastic and fragile Causes Poor nutrition malabsorption Drugs Clinical Manifestations Same as B12 Deficiency without neurological symptoms Treatment Focus on Prevention! Increase intake of Folic acid PO Folic Acid 1mg/day


Aplastic Anemia-1051 :Aplastic Anemia-1051 Also known as Bone Marrow Depression Anemia Bone marrow fails to produce RBCs Cause is usually unknown, but usually related to toxin exposure (radiation/chemicals) or history of viral infection (HIV/Mono) Can occur alone, with leukopenia and thrombocytopenia, or all 3 (Pancytopenia) Clinical Manifestations Fatigue, Pallor, SOB, HA, Weakness, Tachycardia, CHF Treatment Blood Transfusions, Immunosuprressive Therapy/Steroids, Splenectomy, Bone Marrow Transplant


Polycythemia -1064Number of RBCs in Blood is Greater than Normal :Polycythemia -1064Number of RBCs in Blood is Greater than Normal Polycythemia Vera Disease with sustained increase in Hemoglobin levels, RBCs, and hematocrits. 3 major hallmarks: Massive RBC production Excess leukocyte production Excess number of platelets See extreme cell excess Blood is hyperviscous (thicker than normal) Skin and mucous membranes have flushed appearance (plethoric), intense itching, thick blood places increased demands on the heart = hypertension. Could see stasis and thrombosis Can also see gout and hyperkalemia from cell debris accumulation If untreated, person may die in 2 years Monitor CBC Phlebotomy


White Blood Cell Disorders&Coagulation Disorders :White Blood Cell Disorders&Coagulation Disorders Leukemia (classified by cell type) 1067 Acute Lymphocytic Leukemia (ALL) – comes from lymphoid pathways Acute Myelogenous Leukemia (AML)-come from hyeloid pathways Most common type Cancer Excessive growth of immature/abnormal WBCs Causes Anemia, Thrombocytopenia, and Leukopenia Can be acute or chronic


Leukemia :Leukemia Clinical Manifestations Similar to that of anemia, thrombocytopenia, and leukopenia Diagnosis Labs H&H, Platelets, BCS, CBC, Blood Clotting Factors Bone Marrow Aspiration/Biopsy Chromosome Analysis Radiologic Assessment Varies based on symptoms


Leukemia :Leukemia List Common Nursing Diagnoses and Collaborative Problems for Acute Leukemia


Leukemia :Leukemia Interventions for Acute Leukemia Prevent & Control Infection Prevent & Control Bleeding Chemotherapy: Induction, consolidation and maintenance Antibiotics, Antivirals, Antifungals Bone Marrow Transplants


Malignant Lymphoma-1080 :Malignant Lymphoma-1080 Cancer Involves the more mature lymphocytes found throughout the lymph system Hodgkin’s vs. Non-Hodgkin’s


Hodgkin’s Lymphoma :Hodgkin’s Lymphoma Marker: Reed-Sternberg Cell Most curable Clinical Manifestations Diagnosis Biopsy CT Scan of Chest/Abdomen Laparotomy CBC, Liver Function Bone Marrow Biopsy Treatment Depends on staging and extent of disease Chemotherapy & Radiation


Non-Hodgkin’s Lymphoma :Non-Hodgkin’s Lymphoma Any Lymphatic Cancer that are NOT Reed-Stemberg Cells 12 Subgroups Less responsive to treatments Decreased cure rate Clinical Manifestations Similar to Hodgkin’s Diagnosis Biopsy Treatment Same as Hodgkin's


Multiple Myeloma-1082 :Multiple Myeloma-1082 Cancer Involves the more mature lymphocytes leading to an overproduction of myeloma cells Causes ↓RBCs, ↓WBCs, and ↓Platelets Clinical Manifestations Bone destruction, fractures, bone pain, hypercalcemia Bleeding problems, easy bruising Kidney failure Immunosuppression, infection Diagnosis Bone marrow biopsy X-ray Electrophoresis Bence-Jones Protein in Urine Treatment Chemotherapy Autologous BMT


Coagulation Disorders-1082 :Coagulation Disorders-1082 Platelet Disorders Thrombocytopenia Decrease in number of platelets Autoimmune Thrombocytopenic Purpura (ITP ideopathic) Thromotic Thrombocytopenic Purpura


Clotting Factor Disorders-1090 :Clotting Factor Disorders-1090 Hemophilia A Deficiency of Factor VIII Mostly affects males Read Hemophilia section in Mckinney for Care of Child with Hemophilia starting on page 1315 Hemophilia B Deficiency of Factor IX


Transfusion Therapy :Transfusion Therapy Blood Transfusions -1104


Nursing Responsibilities: :Nursing Responsibilities: Pre: Order Consent Crossmatching IV: 18 guage preferred, NS fluids Transfusion: Take VS Begin infusion slowly Severe reaction in 1st 50 cc Stay with patient 1st 15-30 minutes Tell client to report chills, SOB, hives or itching. VS per hospital policy


Types of transfusions: :Types of transfusions: RBC transfusions Platelet transfusions Plasma Transfusions Cryoprecipitate transfusions White Cell Transfusions Autologous blood transfusions


Compatibililty: :Compatibililty: Determined by ABO and Rh antigen Type: A, B, AB, O Rh: Positive or Negative Universal donor: O negative Universal recipient: AB positive


Transfusion Reactions: :Transfusion Reactions: Hemolytic Caused by blood type or Rh incompatibility Fever, chills, apprehension, chest pain, low back pain, a sense of impending doom


Transfusion Reactions :Transfusion Reactions Allergic Transfusion Reactions Itching, bronchospasm, anaphylaxis Febrile Transfusion Reactions Chills, tachycardia, fever, hypotension Usually a reaction to the WBC Bacterial Transfusion Reactions Infusion of contaminated blood products Circulatory Overload Blood product given too quickly