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Hematologic Disorders Marissa Ferrentino, MSN RN Student Learning Outcomes Explain pathophysiological changes and clinical manifestations of various hematological disorders Contrast the effects of various hematological disorders Prioritize the treatment regime for patients with hematologic disorders...

Hematologic Disorders Marissa Ferrentino, MSN RN Student Learning Outcomes Explain pathophysiological changes and clinical manifestations of various hematological disorders Contrast the effects of various hematological disorders Prioritize the treatment regime for patients with hematologic disorders Develop a nursing care plan for patients diagnosed with hematologic disorders Determine a teaching plan for patients diagnosed with hematologic disorders Integrate the growth and development implications into the nursing care plan for the patient with hematologic disorders Some A & P for your review Cells of Hematological system are: - Erythrocytes (RBCs) - Normally round in shape, no nuclei (can squeeze through capillaries) - Leukocytes (WBCs) - Neutrophils, basophils, eosinophils, monocytes, lymphocytes - Neutrophils produced in bone marrow - Thrombocytes (platelets) - Regulate clotting Normal Ranges Blood Cell Normal Range Red Blood Cells Male: 4.51 – 6.01 million/mm Female: 4.01 – 5.51 million/mm Hemoglobin Male: 14-17.3 g/dl Female: 11.7-15.5 g/dl Hematocrit 42% - 52% in males 36% - 48% in females White blood cells 4.5-11.1 mm Platelets 150,000-450,000 mm Reticulocytes 0.5%-2.5% of total RBC count Neutrophils 59% Practice Question What is the role of erythropoietin in the regulation of red blood cells? a. To make Hgb capable to transporting oxygen b. To pick up carbon dioxide from the tissues and deliver it to the lungs c. To decrease RBC production d. To stimulate RBC production Practice Question The nurse recognizes that a deficiency in a clotting factor may cause which of the following? Select all that apply. a. Easy bruising and hematoma formation from minor injuries b. Bleeding from the gums c. Enhanced platelet aggregation d. Yellowish skin color Hematologic Disorders affecting Erythrocytes Anemias Nutritional, Blood loss, Hemolytic Retrieved from: https://physicsforme.files.wordpress.com/2011/06/red-blood-cells.jpg Anemias: Pathophysiology Decreased RBC production Deficient nutrients Decreased erythropoietin (stimulates RBC production) Decreased iron availability Increased RBC loss or destruction Acute or chronic blood loss Increased hemolysis Anemias from Decreased RBC Production Iron Deficiency Anemia - Inadequate iron in diet, blood loss - Infants, children, menstruation, pregnancy, GI diseases - Iron is needed to form hemoglobin – without iron, hemoglobin is not formed so body cannot meet o2 requirements - Pallor, glossitis, spoon-shaped fingers - CBC, iron/ferritin level to diagnose - Diet - Iron supplements – 150-200 mg daily – until levels return to normal for 2-3 months Anemias from Decreased RBC Production Thalassemia - Group of diseases involving inadequate production of normal hemoglobin - Minor thalassemia: asymptomatic/minor symptoms of anemia - Major thalassemia: growth often delayed - Pale, jaundice, splenomegaly - Managed with transfusions or exchange transfusion with chelating agent - NO iron supplements - Splenectomy may be needed Anemias from Decreased RBC Production Vitamin B12 Deficiency (pernicious anemia) - B12 is used for CNS function, formation of RBC and DNA synthesis - Body does not store B12 – must be ingested through meat, fish, eggs or dairy - Numbness/Tingling in hands and feet, weakness, reduced senses, red beefy tongue, impaired thought process - Vitamin B12 lab test - Daily supplement (vegans, vegetarians), injections Anemias from Decreased RBC Production Folic Acid Deficiency - Needed for DNA synthesis leading to RBC formation - Alcoholism, pregnancy, dialysis, GI issues - Manifestations consistent with other anemias: pallor, SOB, tachycardia, etc. - Thiamine deficiency often exists in conjunction - Daily supplement 1 mg/day Anemias from Decreased RBC Production Aplastic Anemia - Pancytopenia - Chemical agents, immunosuppressant, radiation, bacterial infections - Mild to severe symptoms, may have petechiae/purpura - If severe, may need stem cell transplant and immunosuppressive therapy Blood Loss Anemias Acute blood loss Hemorrhage Triggers compensatory mechanisms to maintain cardiac function and increase circulating volume Continual hemorrhage: hypovolemic shock and circulatory failure Chronic blood loss Menorrhagia—most common Chronic GI bleed Anemia caused by Increased RBC Destruction Sickle Cell Disease - Hemoglobin takes on a sickled (crescent-like) shape - Lessens oxygen carrying function - Replacement of normal hemoglobin with abnormal hemoglobin “S” - Autosomal recessive gene Sickle Cell Disease cont. Many patients are asymptomatic or have few generic anemia symptoms unless in a sickle cell crisis. - Crisis triggered by lack of oxygen in the blood: illness, dehydration, cold temperatures, exercise, air pressure change, etc. - Goal is to prevent crises, minimize complications (organ function) - Medication management: Hydroxyurea - Blood transfusions, HSCT - Emotional support/supportive measures - Education Sickle Cell Disease cont. During a sickle cell crisis: - Severe pain - Fever, tissue engorgement, painful swelling of hands, feet; priapism, abdominal pain - Duration: 4-6 days - History & Pain Assessments - Lab values: CBC and reticulocyte count Complications: Infection, Acute Chest Syndrome, Pneumonia, PE Sickle Cell Disease cont. Sickle Cell Crisis Nursing Interventions: Oxygen therapy Hydration: oral and IV Parenteral analgesics—Morphine, hydromorphone Patient controlled analgesia Administered around the clock Blood Transfusions - sometimes Anemias: General Clinical Manifestations Depends on severity Asymptomatic, if mild Pallor, fatigue, weakness Tachypnea, dyspnea on exertion Tachycardia, palpitations, systolic murmur, ventricular hypertrophy, angina, jaundice, vertigo, blurry vision Bone pain Retrieved from: https://www.sciencesource.com/Doc/TR1_WATERMARKED/7/b/e/5/SS21230448.jpg?d63660432604 Food Sources Anemias: Nursing Implications Screening high risk- who? Emergent care—when? Long term management---how? Patient teaching---what? why? Anemias: Nursing Assessment Health history: current complaints, hx of present complaints medical hx, medications diet ETOH and/or smoking Physical assessment: general appearance, skin color, bruising vital signs, AP, O2 sat heart and lung sounds peripheral pulses, capillary refill, dependent edema abdominal tenderness obvious bleeding Anemias: Nursing Interventions Monitor VS, O2 saturation, Cardiopulmonary status, skin, nails, edema, labs: CBC, ABGs Manage therapeutic regime: medication, IVFs, blood or blood component transfusions, oxygen therapy Assess/manage activity intolerance: ADLs, ambulation, need for assistance, O2 needs w/activity, VS changes w/activity, priorities, energy conservation, sleep Monitor/treat oral symptoms Establish/manage/teach dietary needs and modifications Assess/manage/teach pain management as needed (SCD) Teach symptoms of crisis (SCD) When to seek medical attention Practice Question Which patient is at greatest risk for developing iron deficiency anemia? a. A 6-year old with no health problems b. A 15-year old who is pregnant c. A 52- year old with hypertension d. A 72- year old with cardiac disease Practice Question Which activity should be avoided in a patient with sickle cell disease? a. Driving to the beach 3 hours away b. Going to a concert c. Swimming in cold water d. Carpentry work MG is a 10 y.o male with SCD dx’d at 4months old; this is his first day at a new school; he was worried that kids would make fun of him b/c of his small stature and limp; he avoided going to cafeteria for lunch and ate alone in the classroom; now presenting in ED, w/parent, w/ severe pain in right leg; initial assessment criteria below: Crying, holding right upper thigh BP 118/65, AP 112 bpm RR 24/min Temperature 98.4 oral All pulses palpable bilaterally What factors may have precipitated MG’s symptoms? What is the nursing priority of care and why? What teaching should be provided to reduce the risk of future recurrence of sickling? Polycythemia Polycythemia Vera Retrieved from: https://encrypted-tbn0.gstatic.com/images?q=tbn%3AANd9GcSn5r64CTKv9kFaSwGrdmzLrU8b63Sp2b9s23VYedUYJU3huldP Polycythemia Vera Increase in production of RBC (increases blood viscosity and blood volume) - High levels of hemoglobin, RBC, WBC, platelets - Caused by gene mutation - Hypoxemia develops due to impaired tissue perfusion from hyperviscosity - Goal is to decrease blood viscosity Clinical Manifestations: hypertension, pruritus, paresthesias, bleeding tendencies (nose bleeds, petechiae), gout Polycythemia: Collaborative Care Serial therapeutic phlebotomy (goal of HCT 45%) Hydration therapy Myelosuppressive agents Antiplatelet agents Anti- gout agents Monitor I & O Leg exercises, ambulation Patient teaching Coagulation Disorders Practice question The nurse is assigned to a patient with thrombocytopenia. What is the priority goal of nursing care? a. Prevention of infection b. Prevention of injury c. Prevention of dehydration d. Prevention of nutritional deficit Immune/Idiopathic Thrombocytopenia (ITP) Reduction in platelet count - Abnormal destruction of circulating platelets (platelets become coated with antibodies and get destroyed in spleen) - Can be due to other illness but usually it is unknown - Platelet count less than 150,000 Clinical Manifestations: easy bruising, petechiae, mucosal bleeding ITP Management - Corticosteroids IV Immunoglobulin (IVIG) Rituximab (monoclonal antibody) Platelet infusion (if less than 10) Splenectomy Hemophilia Group of bleeding disorders where clotting factors are inadequate - Hemophilia A – lack of factor VIII - Hereditary disorder on X chromosome - Minor injuries can cause significant blood loss - Prolonged clotting times (PT, PTT and Platelets) and reduction in factor missing - Slow, progressive bleeding, uncontrolled hemorrhage, nosebleeds, GI bleeding, hematuria, bruising, neurological signs. Hemophilia A Management *Replace Factor* - Stops bleeding - Given IV - DDAVP may stimulate an increase in factor - Anti-fribinolytic therapy - Prophylactic factor Disseminated Intravascular Coagulation (DIC) Occurs secondary to severe trauma, illness or medications - Widespread intravascular clotting & bleeding - Prolonged PT, PTT aPTT - Decreased Platelets - Increased D-Dimer DIC cont. Bleeding manifestations: pallor, petechiae, SOB, tachycardia, hypotension, abdominal distention, hematuria, dizziness, vision changes Thrombotic manifestations: cyanosis, necrosis, tachypnea, EKG changes, oliguria Resolve underlying condition! - Supportive care with blood transfusions Nursing Management for Coagulation Disorders Monitor for bleeding, labs Monitor vital signs Minimize blood loss Provide a safe environment Avoid rectal temperatures Avoid IM injections Educate families on bleeding precautions Practice Question Which nursing action is indicated for the patient with thrombocytopenia? a. Avoid intramuscular injections. b. Encourage the patient to drink plenty of fluids. c. Place the patient on isolation precautions. d. Encourage frequent rest periods. Hematologic Disorders related to Leukocytes Neutropenia Neutropenia Abnormal decrease in the number of neutrophils Often occurs alongside other conditions such as: chemotherapy agents, lupus, dialysis, leukemias, some abx and other medications, anemias Predisposed to infection from normal body flora and outside bacteria Low-grade fever is EMERGENCY Blood cultures, CBC, bone marrow aspiration Neutropenia Nursing Management - Determine cause of neutropenia Institute antibiotic therapy Identify offending organism Give hematopoietic growth factors after chemotherapy Implement protective measures - Strict hand washing, screen visitors, proper precautions, avoid crowds, dental care, avoid uncooked meats, fish, maintain daily activities, overall good hygiene Leukemia Leukemia Accumulation of dysfunctional cells because of a loss of cell regulation Type depends on stem cell involved Acute myelogenous leukemia, chronic myelgenous leukemia, acute lymphoblastic leukemia, chronic lymphoblastic leukemia CBC reveals increased WBC count, low RBC, low platelets Bone marrow aspiration Leukemia cont. Enlarged lymph nodes from bone marrow congestion Susceptible to infection Fever General signs of anemia: fatigue, pallor, weakness, SOB Bleeding tendencies: bruising, petechiae, nosebleeds Unintentional weight loss Bone pain, change in gait Leukemia Nursing Management - Chemotherapy (induction, post-induction, maintenance) - Radiation - Drug therapy - Combination therapy utilized to decrease resistance, minimize toxicity and interrupt cell growth at all points - HCST – cure, must kill off own stem cells first - Monitor signs and symptoms, labs - Institute precautions - Education: precautions, manifestations of anemia, thrombocytopenia, bleeding, adverse reactions to therapy, fertility - Drug interactions - Ambulatory Care Practice Question The nurse correlates which diagnostic result as increasing the risk of infection in the patient with leukemia? a. WBC 11,000 b. ANC 400 c. Hgb 8.6 d. Platelets 112,000 Lymphomas Lymphomas Hodgkins Lymphoma Presence of Reed Sternberg cells (b-lymphocytes) Localized Fever, night sweats and weight loss common Non-Hodgkins Lymphoma Includes various types of lymphomas (T & B cells) Disseminated Extranodal involvement (metastasis) common Diagnosis: history and physical, CBC, CXR, CT scan and PET scan, bone marrow aspiration Lymphomas cont. Often don’t seek medical treatment until systemic involvement “B” symptoms Lymphadenopathy Generalized pruritus Persistent cough – mediastinal involvement Hepatomegaly and splenomegaly in advanced disease Lymphoma Management Chemotherapy Radiation Stem cell transplant – may have own stem cells if bone marrow not affected Assessment/Education similar to leukemia (monitor labs, pain, symptom management, education on disease process, risks, complications) Complications: spinal cord compression, superior vena cava syndrome, hypercalcemia Overview of Nursing Management of Hematological Cancers Assessment of symptoms Assessment of response to Rx Supportive through Dx, Rx and disease progression Assist/comfort through diagnostic process Effects of radiation and chemotherapy Psychosocial considerations Address potential fertility issues Long term effects of therapy Overview of Nursing Management of Hematological Cancers Monitor nutritional status*: Assess/manage causative factors to inadequate intake High calorie, high protein diet, snacks, liquids Medicate for pain or nausea prior to meals , provide rest periods before meals, small frequent meals, supplements prn Assess skin and mucus membranes for signs of bleeding tendencies, swelling, or lesions*: Mouth care w/ saline* Lubricate lips, nares* Soft toothbrush or sponge* Avoid mouthwash* Culture any lesions Overview of Nursing Management of Hematological Cancers Assess coping strategies Assist in identifying sources of strength and support Discuss changing roles Evaluate cultural factors Facilitate open discussion Community resource referral Practice Question A patient with lymphoma is beginning their first course of treatment with chemotherapy. Which information is most essential for the nurse to include in the treatment plan? a. Advance directives b. Nutritional and physical activity recommendations c. Importance of frequent rest periods d. Neutropenic and bleeding precautions References Hoffman, J. & Sullivan, N. (2024). Medical-surgical nursing. 3rd ed. Philadephia, PA: F.A. Davis Company. London, M.(2014). Maternal and child nursing Care, 4th edition. NJ: Pearson Lewis, Harding, Kwong, Roberts, Hagler, Reinisch (2020). Medical surgical nursing: Assessment and Management of Clinical Problems 11th edition, St. Louis: Mosby/Elsevier. ISBN: 978-0-323-55149-6

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