NURS471 Hematology Lecture Guide PDF

Hematologic Alterations – Handout KEY NURS471 – Pediatrics Lipincott Chapter 46; ATI Chapter 21 Dr. Kelly Bates HEMATOLOGIC DISORDERS Hematology Basics: Red blood cells (RBCs) or ______ erythrocyte – Responsible for transp...

Hematologic Alterations – Handout KEY NURS471 – Pediatrics Lipincott Chapter 46; ATI Chapter 21 Dr. Kelly Bates HEMATOLOGIC DISORDERS Hematology Basics: Red blood cells (RBCs) or ______ erythrocyte – Responsible for transporting nutrients and oxygen to the body tissues and waste products from the tissues thrombocyte Platelets or ______ – Responsible for clotting White blood cells (WBCs) or ______ leukocyte – Responsible for fighting infection – Granulocytes (neutrophils, eosinophils, and basophils) and agranulocytes See Language of Hematology Handout on the last page ANEMIA - Anemia: Decrease in RBCs or hemoglobin causes decrease in amount of oxygen delivered to body tissues Three broad categories: 1) Loss of RBC's = Bleeding o hemoglobin/deficency building blocks in ↓ production of RBC 2) Hypoproliferative Anemia: ______ or 3) Hemolytic Anemia: ______ RB Excessive/premature destruction of Factors that determine Severity of Anemia: Speed of Development Crapidly severe symptoms ( = Duration (Acute vs. Chronic) Metabolic Requirements of the client Cit more activee more sks) Concurrent Disorders/Disabilities Clinical Manifestations:  most common Physical Exam - General: Weakness, Fatigue,* Malaise HEENT: Mucous membranes dry, pale. Tongue smooth, red, "beefy" (B12), glossitis, angular cheilosis, HA, Epistaxis, gum bleeding Cardio: Tachycardia, palpations, dyspnea, DOE, dizziness Skin: Pallor, Jaundice, Easy bruising - if chronic Nails: Brittle, ridged, concave. GI: Nausea, Vomiting, Coffee ground emesis, Melena stools, Anorexia GU: Female Menstrual Flow Neuro: Presence and extent of peripheral numbness, ataxia, poor coordination, confusion. Major Types of Anemia Lead Poisoning: A nonessential metal that is not a natural part of the human body. When present, causes hypo-proliferation of RBCs and possibly hemolysis Vitamin B12 Deficiency: B12 is an enzyme cofactor in DNA synthesis. Vitamin B12 deficiency can occur with: can cause anemia bic inadequate absorption , B12 needs to combine W/ intrinsic factor - Inadequate intake: beef is a great source - Atrophic gastritis: thinning of stomach lining  intrinsicis in stomach causing inability to absorb B12 - Pernicious anemia: lack of ______ - Surgery or disorders impacting the stomach or small intestine * Alcoholism common cause Folic Acid Deficiency: Deficient intake or absorption of folic acid. Caused by alcoholism, malabsorption, pregnancy, lactation. & Avoid triggers G6PD Deficiency: inherited ______ enzyme deficiency. Certain triggers cause RBC's to be broken down faster than they are produced. Most common trigger is ______ fava beans Other hemolytic anemias: can be caused by autoimmune disease, infections such as ______, malaria and adverse medication reactions Iron Deficiency Anemia in childern Desp. - Most common type of anemia. Iron is necessary for hemoglobin production - Different age groups have different risk factors o Infants: exclusive breastfeeding after the age of 6 months (bic breast mill isn't iron rich) o Toddlers: picky eating and excessive ______ consumption of milk cow ▪ Highest risk group. Annual Hgb screening age 1-5 years o Adolescents: higher demands, poor dietary intake, menstruation Expected Lab Findings X - Complete Blood Count o ______ RBC, Hgb and Hct - RBC Indices o ______ MCV small average size = microcytic o ______ MCH low average amount of hemoglobin per RBC = hypochromic o ______ Reticulocytes immature RBCs - Iron Studies o ______ Ferritin iron stores in the body o ______ TIBC transferrin is not fully saturated with iron Anemia Management & amount of iron  Iron Supplements: Ferrous Sulfate (Feosol), Ferrous Fumarate (Feostat), Ferrous Gluconate (Fergon) Oral: Best absorbed on an empty stomach (ex: 1 hour before meals) Vit ______.( increase absorption. Calcium (ex: milk and antacids) decrease absorption Liquid formulations can stain teeth - > drink through straw brush teeth , Gastric side effects are common: constipation, cramping, nausea, vomiting Green/black stools are expected IM: Causes local pain and stains the skin, so use the 2-track _______ method than & locks iron/med in the muscle rather subg IV: Mildly-moderately painful Potential for tissue staining common (nausea , cramping) , occur more wh oral supplements AG1 side effects : most * Green/Black stool Other anemia management: > - stopping the cause , stop bleed Vitamin B12: Converts folic acid from its inactive form to its active form. All cells rely on folic acid for DNA production. Folic Acid: Needed for production of NEW RBCs. Water soluble B complex vitamin. PO or IV. High dose turns urine dark yellow. Erythropoietin: Epoetin Alfa, (Epogen, Procrit). Increases production of RBCs. Monitor BP, Hgb & Hct, CV Events Blood Transfusion: Reserved for significant anemia. Immediate improvement in signs and symptoms. Nursing Management Manage Fatigue & Cluster Care ↳ Advocate limit visitors Improve Diet  Maintain Adequate Perfusion (Transfusions or IV Fluids) Promote Medication Compliance (Purpose, How to take, Side Effects, Etc.) Monitor Response and Manage Potential Complications SICKLE CELL ANEMIA: What is Sickle Cell Anemia? Hemoglobinopathy – Normal hemoglobin (HbA) is replaced by sickle hemoglobin (HbS) Genetic disorder -Autosomal ______ inheritance pattern recessive o If both parents have sickle cell trait, then 25% chance child has sickle cell disease, 50% child has sickle cell trait (a carrier) and 25% chance child is normal o Sickle cell disease: have abnormal hemoglobin (HbS) and little to no HbA o Sickle cell trait: have both normal and abnormal hemoglobin – HbA and HbS African Americans Most common among ______ o 1 in 365 have sickle cell disease o 1 in 13 have sickle cell trait Pathophysiology HbS appears ______ round under normal oxygen conditions, but becomes sickle shaped when exposed to low oxygen levels What’s different about sickled RBCs? o They’re rigid and sticky, so they don’t move through vessels well and can occlude vessels o days They have a lifespan of ______ 20 (short) normal lifespan - 100 days Clinical manifestations are a result of blood flow obstruction and increased RBC destruction o Signs and symptoms of anemia o Decreased or absent blood flow cause local hypoxia, ischemia and infarction which can cause severe pain and damage organs A high risk for infection bic spleen is affected (filters out old RBC) A & rish for sepsis becomes thick : sloggy blood = Diagnostic Evaluation Newborn screening in all states include SCD test  Positive results followed up with o Hgb electrophoresis to determine levels of HbS o Genetic testing Screening is essential for early treatment because symptoms don’t typically develop until fetal nemoglobin (hemoglobin #) months at least age 608 ______ due to the presence of ______ Clinical Manifestations of RBC lead (bic fast breakdown to excess bilirubin ( lifespan * SIs happen blc : once 191)) Sickle = 20 day crisis blockage sickle cell cause = cells Details on some of these clinical manifestations: Sign or symptom How it presents Retinopathy Damage to small vessels leading to blurred vision or blindness Avascular necrosis Most common in hip, shoulder and knee. Can cause skeletal deformities Gallstones Excess bilirubin can build up into gallstones  Autosplenectomy Due to repeated damage from sickle cell crises causing decrease in size and functionality. Causes increased susceptibility to disease Dactylitis Most common in infants and toddlers. Obstructed blood flow causes painful, swollen hands and feet Priapism Veinous flow away from the penis is obstructed causing prolonged erection Chronic ulcers More common in older children and adults SICKLE CELL CRISES Types of Crises prompt treatment & high mortality whout Sequestration Crisis: Results from pooling of blood in the liver & spleen (hepatosplenomegaly). This causes decrease in circulating blood volume (drop BP) and shock symptoms. Can result in death from cardiovascular collapse. Packed RBC’s given to raise Hgb to 9 or 10 of ruptor & risd -give enough to keep person alive but not ↳no palpating spleen : end - blood from spleen too much bic crisis will RBC = & effect, too much back to body=rebound risk for Aplastic Crisis: Decreased production of RBCs in the bone marrow resulting in profound clotting anemia. Commonly triggered byvira ______ or depletion of folic acid. infection 15th disease) slapped cheek disease Hyperhemolytic Crisis: Accelerated rate of RBC destruction characterized by anemia, jaundice and reticulocytosis. Gimmune system starts attaching them , occur w) multiple transfusions A Vaso-Occlusive Crisis Occurs when sickle cells block blood vessels à Severe PAIN Tissue inflammation, infarction and necrosis Signs and Symptoms vary by area where blood flow is occluded Severe pain, often in bones, joints, chest or abdomen Fatigue and weakness Jaundice Shortness of breath Visual disturbances Swelling of hands and feet – hand foot syndrome due to occlusion in small distal bones of hands and feet  Priapism: painful, prolonged erection due to blood flow blockage and pooling Hematuria if there's vaso-occlusive Kidney crisis in Frequency and severity of vaso-occlusive crises varies between individuals, but is often consistent for each individual (each person has own patteral RBC sickle = occlude blood resses = further hypoxia = more sickiling experience hypoxia = & person Acute Chest Syndrome Acute Chest Syndrome is a medical emergency! Blockage of small vessels in the lungs One of the most common reasons for hospitalization for patients with sickle cell disease. In - children, ______ infection is typical trigger; in adults, ______ is typical trigger. vaso- occlusive crisis a Mainly in 2-y Clinical Manifestations: Chest pain, back pain, abdominal pain, fever 101.3 or higher, productive cough, dyspnea, tachypnea, retractions and declining O2 sats. Infiltrates on CXR. Sound familiar? Presentation similar to ______Pneumonia Treatment includes: H______-O_______-P_______, yaration xygen air antibiotics, transfusions, VTE prophylaxis. management CVA (Stroke) Sudden and severe complication. Blood vessels of the brain are blocked leading to cerebral infarction. May cause variable degrees of neurologic impairment. could be silent symptoms Clinical Manifestations: Severe headache, vomiting, abnormal behavior, weakness or impaired mobility, slurred speech, visual changes Current treatment: chronic transfusion therapy – exchange transfusion then monthly infusions to maintain HbS less than 30% ↳ All of blood is replaced wl new blood t will transfusions receive monthly transcranial Children ages 2-16 are screened annually with ______ doppler to assess intracranial blood flow MANAGEMENT Sickle Cell Crisis Management to dilute blood IV fluid given don't clump so sticky RBC A A A HOP TO IT!!! (Hydration, Oxygenation & Pain Management) prevent further * give opiods on schedule not PRN pain 31C it dilate sickling *eat : relieve blood vessels  HYDRATION FORMULA 1st 10 Kg Kg x 100 mL (Max 1000 mL) Next 10 – up to 20 Kg 1000 + Kg x 50 mL (max 1500 mL) Over 20 Kg 1500 + Kg x 20 mL 14.9 Kg = 1235 54.5 Kg = 2190 OXYGENATION Manage hypoxemia. SpO2 (pulse ox) can give artificially low readings, so PaO2 (from ABG) is preferred PAIN MANAGEMENT Usually IV and scheduled (rather than PRN only) for severe pain. PCA if appropriate. infection antibotics (penicillin by 2 months)b/c & risk for A un going management & prophylactic > - blood products Therapeutic Management Aims of therapy include prevention of sickling and treatment of medical emergencies due to crises. Hydroxyurea – promotes production of ↑______ HBF to reduce vaso-occlusive crises and prolong life expectancy. Antibiotics – prophylactic penicillin by age 2 months Vaccines! Especially pneumococcal, flu and meningococcal vaccines Splenectomy - Spleen is removed after multiple sequestration crises Exchange transfusions – to maintain HbF below a threshold (ex 30% for CVA) Hematopoietic Stem Cell Transplant (bone marrow transplant) – must have severe disease and a matched donor Conly 25% have matched donor Gene editing – correct the gene mutation to reactivate production of HbF or to restore production of HbA A teach how to avoid triggers pt Nursing Management MINIMIZE TRIGGERS! Anything that could contribute to hypoxia o S: Significant blood loss o I: Illness o C: Climbing or flying to high altitudes o K: Keeping stress  o L: Low fluid intake (dehydration) o E: Elevated temperature or cold Promote normalcy – Kids with SCA can go to school and participate in normal activities Monitor and seek care for concerning symptoms Assess hydration and nutrition needs Assess and manage pain Psychological support IMMUNE THROMBOCYTOPENIC PURPURA (ITP) Immune system mistakenly attacks and destroys ______ plate let below lov =& rish of bleeding Normal: 150,000-400,000S wi ITP - below 100,000 if , Usually develops 1-3 weeks afterviral ______ illness Other risk factors include autoimmune disorders, certain medications like aspirin and pregnancy. Signs and Symptoms Easy bruising Petechiae or purpura Prolonged on spontaneous bleeding o Bleeding gums or epistaxis (nosebleeds) Menorrhagia Internal bleeding Management: treatment After 3-6 weeks / Mild cases: observation (resolve on their own port any. Moderate, severe or prolonged cases: killing platelet) Ist 2 o Corticosteroids to ______ suppress system (to stop immune it from o IVIG – replaces normal immunoglobulins to interfere with autoimmune process resort & last o Splenectomy – spleen is site of platelet destruction Safety teaching to avoid injury or trauma - whose sleed > - lean forward, head soft down , pinch at spot comin of no improvement after - ice helpful wh vasoconstriction , no contact sport ,padding on corners , get help HEMOPHILIA: What is Hemophilia?  Genetic bleeding disorder Deficiency or abnormality in clotting factors causes excessive bleeding factor more severe disease Varies in severity based on amount of clotting factor present Cless clotting = Types Hemophilia A: o Most common type (80%) o x-linked, so more common among males o deficiency in clotting factor VIII (8) Hemophilia B: o x-linked, so more common among males o deficiency in CF IX (a) von Willebrand Disease: deficency in willebrand factor ron o milder symptoms o Von Willebrand factor is a protein that helps platelet ______ stick together. It also stabilizes CF VIII (8) Clinical Presentation Spontaneous, excessive or prolonged bleeding Easy bruising Menorrhagia to maintain Hematochezia or hematuria wI PTlongterms work A Hemarthrosis: bleeding into joint cavity causing pain, swelling and decreased ROM complications o Severe or recurrent hemarthrosis can cause permanent deformity and disability Intracranial bleeding – life-threatening complication ↳ most common symptom > - sudden severe headache or prolonged headache Lab Findings * aPTT is ______ prolonged o Factors VIII and IX are involved in the intrinsic pathway A PT is _______ normal o Measures the extrinsic pathway Platelets are ______ normal Clotting factor assays are low for the relevant clotting factor Treatment main treatment & deficient in Clotting factor replacement (that person is done IV week Ausually 3 times a  o inhibitors ______: may develop antibodies over time that attack the clotting factor. Reduces effectiveness of clotting factor and complicates management Desmopressin (DDAVP, synthetic vasopressin) can increase production of CF VIII o Effective for mild Hemophilia A and von Willebrand disease (Not for hemo B). o Can be IV or intranasally Corticosteroids – to manage hemarthrosis May also receive medications to replace the function of clotting factors or to prevent clots from breaking down Gene editing – possible future cure Creplace mutated gene of normal gene) Nursing Interventions role Reduceprimary & risk for bleeding o In the hospital: hold pressure for 5 minutes after IV or injection. Avoid unnecessary invasive procedures. No rectal temps. (in infant o At home: avoid constipation, “Play it Safe”, soft toothbrush, ______ after injuries, avoid NSAIDs, medical alert tag- Avoid contact sports Monitor for bleeding Control bleeding The Language of Hematology and Oncology PREFIX MEANING a Without Hyper Above, excessive Hypo Below, deficient Pan All, total Poly Many, much Chrom Color Coagulo Clotting Erythro Red (ex RBC) Hemo or Hemato Relating to blood Leuko White (Ex WBC) Lympho Relating to lymph tissue or lymphocytes Myelo Relating to bone marrow Neutro Relating to neutrophils Thrombo Clot SUFFIX MEANING -blast Immature or embryonic -cyte or -cyto Cell -cytosis Increase in cells -ectomy Removal -genic Producing, originating, causing -globin Protein -lysis Destruction -megaly Enlargement -oma Tumor -osis Abnormal condition -poiesis Formation -rhage or -rhaggia Excessive flow -stasis Stopping or controlling A few examples: Leukopenia Thrombocytopenia Hemoglobin Myelosuppressive Leukocytosis Erythropoietin Pancytopenia Pediatric Hematologic Alterations - 7 of 7 -blast Immature or embryonic -cyte or -cyto Cell -cytosis Increase in cells -ectomy Removal -genic Producing, originating, causing -globin Protein -lysis Destruction -megaly Enlargement -oma Tumor -osis Abnormal condition -poiesis Formation -rhage or -rhaggia Excessive flow -stasis Stopping or controlling A few examples: Leukopenia Thrombocytopenia Hemoglobin Myelosuppressive Leukocytosis Erythropoietin Pancytopenia Pediatric Hematologic Alterations - 7 of 7