BLOOD PHYSIOLOGY/DISORDERS N-103 MMCCOLLEGE OF NURSING 2011-2012 FIRST SEM MAJOR FUNCTIONS 1. TRANSPORT 2.

DEFENSE PLASMA Clear, straw-colored 91-92% water PLASMA CONTENTS WATER PROTEIN.. Albumin, globulin, fibrinogen, prothrombin, nutrients, met wastes, electrolytes ERYTHROCYTES 4.5 6.2 million/ cu mm Red bone marrow Erythropoiesis 120 days/4 mo hematocrit Fraction of the blood occupied by RBC ***NORMAL : 3X the hb value BLOOD CLASSIFICATION MAJOR BLOOD GROUPS Rh FACTOR Present in RBC POSITIVE or NEGATIVE **O NEGATIVE no antigen, no Rh factor AB positive no antibodies in serum, Rh factor present AGE-RELATED ASSESSMENT FINDINGS FOR HEMO DISORDERS AREAS : NAIL BEDS (CAPILLARY REFILL) HAIR DISTRIBUTION SKIN MOISTURE AND COLOR 1. Nail beds Pallor, cyanosis, and decreased capillary refill **ELDERLY - ASSESS LIPS *nails are typically thickened and discolored 2. Hair distribution Thin or absent hair on trunk and extremities **ELDERLY lack of hair or lower extremities and toes *loss of body hair in an even pattern over time 3. Skin moisture and color Skin dryness, pallor, jaundice

 ELDERLY DRY SKIN IS normal Pigment loss and skin changes like jaundice may occur Pallor ANEMIAS Excessive blood loss, inadequate production, excessive rbc destruction ORIGINS Defect in bone marrow production Loss of RBC e.g. hemorrhage, chronic bleeding, hemolytic processes Hereditary disorders Inadequate nutritional intake of iron, B12, and folic acid IRON DEFICIENCY ANEMIA inadequate intake/ excessive loss Common in adolescents, infant on milk as primary diet, pregnancy; chronic blood loss ( gi bleed, menses, hookworm infestation); Common in the Elderly**** poor dietary intake and decreased absorption in the small intestines

DIAGNOSTICS HEMOGLOBIN; HEMATOCRIT Serum iron and ferritin(M-18=270ng/mL, F-18-160ng/mL) <11 g/100..headache, dizziness, fatigue, tinninus, palpitations, DOE, pallor, cheilosis, smooth sore tongue

 Koilonychia Pica Dx sources of chronic blood loss TREATMENT Supplemental iron for 6 months Empty stomach; with meals if GI upset occurs Liquid/enteric-coated EGGS, MILK, CHEESE, ANTACIDS INHIBIT ORAL IRON Increased dietary intake Supplemental folic acid NURSING DIAGNOSES Imbalanced nutrition, less than body requirements r/2 inadequate intake Ineffective tissue perfusion r/2 decreased oxygen carrying capacity of the blood PERNICIOUS ANEMIA loss of intrinsic factor NOT ASSOCIATED WITH INADEQUATE DIETARY INTAKE **NEEDED FOR NORMAL DNA SYNTHESIS IN MATURING rbc DIAGNOSTIC SCHILLING TEST RBC MORPHOLOGY GASTRIC ANALYSIS for free HCl SCHILLING'S TEST NPO PO RADIOACTIVE CYANOCOBALAMINE IM RADIOACTIVE B12 URINE COLLECTION (24H) NORMAL: at least 5%(+)RADIOACTIVE b12 IN URINE <5%....? NURSING DIAGNOSES DISTURBED THOUGHT PROCESSES IMPAIRED SENSORY PERCEPTION (KINESTHETIC) FOLIC ACID DEFICIENCY ANEMIA ETIOLOGY dietary deficiency, malabsorption syndromes, infancy, adolescence, pregnancy; anticonvulsants and oral contraception s/s slow, insiduous onset, weight loss, emaciated, malnourished DIAGNOSTICS DIFFErenTIATE BETWEEN B12 and FOLIC ACID deficiency Serum folate acid = < 4 ng/ml treatment IV AND ORAL REPLACEMENT Avocado, black beans, asparagus

APLASTIC ANEMIA BONE MARROW DEPRESSION ETIOLOGY: MEDICATIONS e.g. chemotx, benzene, chloramphenicol, anticonvulsants Radiation Idiopathic Congenital (Fanconis anemia) DIAGNOSTIC BONE MARROW BIOPSY Site : posterior iliac crest Prep local anesthetic, analgesic, **feeling of pressure & pain Post bleeding at site; apply pressure to site; bed rest for 30 min analgesic as indicated CLINICAL MANIFESTATIONS Anemia s/s Fever, infections Bleeding s/s NURSING DIAGNOSES RISK FOR INFECTION RISK FOR INJURY TREATMENT BONE MARROW TRANSPLANTATION ANDROGENIC HORMONE THERAPY to stimulate bone marrow regeneration CORTICOSTEROIDS / immunosuppressive treatments BONE MARROW TRANSPLANTATION GOAL : TO RESTOR HEMATOLOGICAL AND IMMUNOLOGICAL FUNCTION BONE MARROW T - PROCEDURE ADULT CLIENT ASP OF 400 800 ML OF BONE MARROW FROM DONORS ILIAC CREST; PROCESsED AND TRANSFUSED TO RECIPIENT PROCEDURE TYPING ALLOGENIC HISTOCOMPATIBLE DONOR E.G. RELATIVE AUTOLOGOUS FROM CLIENTS OWN BONE NARROW WITHOUT DISEASE AND IS FROZEN SYNGENEIC FROM IDENTICAL TWIN WITH PERFECT TISSUE MATCH PREPARATION IMMUNOABLATIVE PREP chemo and radiotx to produce immunologically suppressed state before marrow transfusion; takes 5 7 days pre COMPLICATIONS - BMT

 GVHD

INFECTION due to immunosuppression Bleeding from severe thrombocytopenia GRAFT-VERSUS-HOST DISEASE / REJECTION (GVHD)

ACUTE REJECTION 7-30 days post CHRONIC REJ occurs in 100 days S/S erythematous rash on palms and feet, spreading to trunk; altered liver enzymes, liver tenderness and jaundice; GI s/s Gvhd nsg implications Prep for immunosuppression with chemo and radiotx Confirmed by SKIN or ORAL MUCOSAL BIOPSY SUCCESSFUL ENGRAFTMENT hematopoiesis after 2 5 weeks post Care of an immunosuppressed client POLYCYTHEMIA VERA (PRIMARY) Proliferation/HYPERPLASIA of all red marrow cells Occurs during middle age s/s ruddy complexion, headache, hepatosplenomegaly, pruritus, s/s poor perfusion e.g. angina, claudication, thrombophlebitis, hypertension NURSING DIAGNOSIS INEFFECTIVE TISSUE PERFUSION (multiple organs) DIAGNOSTICS CBC BONE MARROW ASPIRATE AND BIOPSY URIC ACID TREATMENT PHLEBOTOMY 2-3 TIMES PER WEEK ANTICOAGULANTS immunosuppressants LEUKEMIAS Uncontrolled proliferation of abnormal WBC and eventual cellular destruction occurs as a result of the infiltration of the leukemic cells into body tissue Primarily affected are high vascular organs of RES..spleen, liver, lymph nodes Infiltration, enlargement, fibrosis LEUKEMIAS PRIMARY CONSEQUENCES: ANEMIA INFECTION BLEEDING TENDENCY LEUKEMIA : types ACUTE LYMPHOCYTIC (BLAST OR STEM CELL

 ACUTE MYELOGENOUS (AML) CHRONIC MYELOGENOUS (CML) CHRONIC LYMPHOCYTIC TYPE: ACUTE LYMPHOCYTIC PEAK = 4 YRS OLD; 65 YRS OLD Favorable prognosis with chemotherapy Can infiltrate meninges causing increased ICP TYPE: AML ADULTS**disease of older people with median age of 67 ONSET age 15 35 yrs TYPE : cml Common after age of 20 Slow onset s/s less severe TYPE : CML COMMON OLDER ADULTS FREQ ASYMPTOMATIC DIAGNOSTICS BONE MARROW ASPIRATION ** increased number of blast cells Lumbar puncture ** presence of leukemic cells CHRONIC MYELOCYTIC LEUKEMIA TREATMENT PHASES: INDUCTION - 2 destroy leukemic cells INTENSIFICATION starts stat after induction targeting undetected leukemic cells lasting several months CONSOLIDATION after remission to eliminate any remaining cells MAINTENANCE lower doses to keep d body free of leukemic cells CHRONIC LYMPHOCYTIC LEUKEMIA (CLL) Proliferation of morphologically normal but functional inert lymphocytes Lymphocytes are immunoincompetent and responds poorly to antigenic stimulation Indolent for years with gradual transformation to more malignant disease NURSING DIAGNOSES ACUTE PAIN ACTIVITY INTOLERANCE HEMOSTASIS MECHANISMS Extrinsic initiated by tissue damage and blood loss Intrinsic mechanism within the blood vessel where blood loss and tissue trauma are not present THREE PHASES

 FIRST : tissue injury..release of platelet factor..calcium and accessory factors leads to thromboplastin formation SECOND : conversion of PROTHROMBIN to THROMBIN THIRD : conversion of FIBRINOGEN to FIBRIN **RBCs are trapped in the fibrin and gives color to the clot

FIBRINOLYSIS Clots are dissolved by FIBRINOLYSIN BLEEDING DISORDERS THROMBOCYTOPENIA ATP/ITP DIC

CLASS. and ETIOLOGY Decreased platelet production Increased platelet destruction Abnormal distribution/sequestration in spleen Dilutional c/m No s/s Platelets <20,000/cu mm ..spontaneous petechiae, ecchymosis (venipuncture, pressure); mucosal bleeding, menorrhagia Excessive bleeding post-procedures DIAGNOSTICS CBC, PLATELET COUNT hemostasis test TREATMENT/MGT TREAT underlying cause Platelet transfusions(300 ml; 15-30 min) Steroids/immunotx NURSING ASSESSMENT Hx prior illness, bleeding episodes, surgeries, exposure to toxins/irradiation, family hx Current/recent meds/ OTC, herbal/dietary supplements PE bleeding s/s NURSING DIAGNOSIS RISK for injury : bleeding NURSING INTERVENTIONS MINIMIZE BLEDING Institute bleeding p/c Restrict activity/exercise..when? NSG INTERVENTIONS Monitor pad count, amount of saturation during menses Adm blood productsmonitor Evaluate urine, stool, emesis for gross/occult blood ATP/ITP ACUTE/CHRONIC Results from immune destruction of platelets by antiplatelet antibodies characteristics Autoantibodies(IgG and IgM) directed against a platelet associated antigen- destruction of platelets in liver, spleen Acute childhood ff viral illness; good prognosis Chronic - ages 20-40, F> M; asstd with pregnancy, SLE, thyroid disease MGT Supportive care bleeding control, platelet transfusion High- dose immunotherapy

Splenectomy DIC/disseminated intravascular coagulopathy Acquired thrombotic and hemorrhagic syndrome Abn activation of the clotting cascade and accelerated hemolysis Widespread clotting in small BV; consumption of clotting factors and platelets Simultaneous bleeding and thrombosis

Etiology/patho Event: overwhelming infection(bacterial sepsis), OB c/p (abr placentae, eclampsia, amniotic fluid embolism, retention of dead fetus); massive tissue injury ; vascular and circ collapse Hemolytic BT rx, snakebite CA c/m Abn clotting extremity coolness & mottling; acrocyanosis; dyspnea, abn breath sounds; altered mental status, acute renal failure, pain (ischemia, infarct) Abn bleeding Dx/mgt Treat underlying disorder Replacement tx fresh frozen plasma, platelet Supportive fluid rep, O2, maintain BP, renal perfusion COMPLICATIONS THROMBOEMBOLIC HEMORRHAGIC (CEREBRAL)** the most common cause of death NURSING DIAGNOSES RISK FOR INJURY INEFFECTIVE TISSUE PERFUSION

NURSING INTERVENTIONS MINIMIZE BLEEDING PROMOTE TISSUE PERFUSION OBJ: minimize bleeding **avoid dislodging clots Apply pressure to site of bleeding 20 min Cautious use of tape Bedrest Internal bleeding = BS, abd girth Evaluate fluid status and bleeding freq VS check, CVP, I and O OBJ: PROMOTE TISSUE PERFUSION Keep client warm Avoid vasoconstrictive agents Change position frequently, perform ROM Monitor ECG and lab tests arrhythmia, ABG, BUN, creatinine MONITOR s/s vascular occlusion, REPORT STAT Brain, eyes, bone, pulmonary, extremities, coronary, bowel SICKLE CELL ANEMIA Severe, chronic, hemolytic anemia Genetically determined, inherited disease autosomal recessive(next slide) CLINICAL COURSE: PAIN -> occlusion of capillaries by sickled RBC INCIDENCE: AFRICAN-AMERICAN; ARABIC/MEDITERRANEAN ANCESTRY PARENTS HETEROZYGOUS for HbS AUTOSOMAL RECESSIVE A genetic condition that appears only in individuals who have received two copies of an autosomal(nonsex chromosome) gene, one copy from each parent The parents are carriers who have only one copy of the gene and DO NOT EXHIBIT THE TRAIT because the gene is recessive to its normal counterpart gene. If both parents are carriers, there is a 25% chance of a child inheriting both abnormal genes and, consequently, developing the disease. There is a 50% chance of a child inheriting only one abnormal gene and of being a carrier, like the parents, and there is a 25% chance of the child inheriting both normal genes.

CAUSE inheritance of a gene from the structurally abnormal portion of the hemoglobin chain characteristics Hemoglobin A(HbA) is partly/completely replaced by abnormal sickle hemoglobin S(HbS) **sensitive to changes in the 02 content of the RBC deficient O2 causes cells to assume the SICKLE shape, become rigid and clumps-> obstructing capillaries PRECIPITATING FACTORS: sickling Any condition that increases need for O2 and alters O2 transport Dehydration Infection Trauma Strenuous physical exertion Cold exposure Hypoxia Acidosis Surgery pregnancy Sickle cell crisis 1. vaso-occlusive crisis 2. splenic sequestration 3. aplastic crisis Crisis: vaso-occlusive Blood stasis and clumping in capillaries -> ischemia, infarction s/s fever; painful swelling of hands & feet(dactylitis), joints; abdominal pain; hepatomegaly; icteric sclera; vomiting; acute back pain; priapism

**MOST COMMON EVENT in INFANTS and TODDLERS Crisis: splenic sequestration Pooling of and clumping of blood (hypersplenism) Rapid onset s/s profound anemia; hypovolemia, shock ***fragile rbc..rapid destruction(6-20 days) ***frequent cause of death in INFANTS APLASTIC CRISIS BONE MARROW ceases RBC PRODUCTION INCREASED DESTRUCTION OF RBC LOW RETICULOCYTE COUNT MANAGEMENT:prevention of sickling Promote adequate oxygenation and hemodilution Increased fluid intake AVOID HIGH ALTITUDES and low-O2 environment AVOID physical exertion AVOID extreme heat or cold Blood transfusion Interventions IVF normal saline, oral fluids O2 adm, BT as prescribed Adm analgesics (RTC) ***Meperidine is AVOIDED (risk of meperidine-induced seizures POSITION: elevate head (< 30deg) extremities extended (to promote venous return); AVOID strain to joints Adm antibiotics as ordered to prevent infection VACCINES H influenzae type B, menincococcus THALLASEMIA A group of genetic (inherited) blood disorders COMMON FEATURE: defective production of hemoglobin COMMON: Greek and Italian descent BETA THALLASEMIA decreased production of normal adult hemoglobin (HbA), the predominant type of hemoglobin from soon after birth until death HEMOGLOBIN: heme, globin globin has 4 protein sections called polypeptide chains Two are identical (the alpha chains) The other two chains are also identical to one another (beta chains) In persons with beta thalassemia, there is reduced or NO production of beta globin chains. MINOR THALASSEMIA only one copy of the beta thalassemia gene (together with one perfectly normal beta-chain gene).

 The person is said to be HETEROZYGOUS for beta thalassemia MAJOR THALASSEMIA/COOLEYS ANEMIA two genes for beta thalassemia and no normal beta-chain gene The child is homozygous for beta thalassemia. A striking deficiency in beta chain production and in the production of Hb a serious disease CHARACTERISTICS Anemia begins: within the first months after birth; becomes progressively more and more severe S/S failure to thrive Feeding problems(easy fatigue from lack of oxygen, with the profound anemia) bouts of fever ( predisposed to infection) diarrhea and other intestinal problems. pale skin, irritability, growth retardation Abdominal swelling (hepatosplenomegaly) jaundice (hemolytic anemia) TREATMENT BLOOD TRANSFUSION FOLIC ACID GENE THERAPY HEMOPHILIA INHERITED, congenital bleeding disorder SEX-LINKED, RECESSIVE TRAIT caused by gene carried on their X chromosome Affect males; CARRIERS: FEMALES Most common transmission: union of an unaffected male with a traitcarrier female

 PATHOPHYSIOLOGY and ETIOLOGY LACK OF FACTORS VIII (hemophilia A/ classic hemophilia) and IX(hemophilia B/Christmas hemophilia)..defect in intrinsic phase of coagulation process Unstable blood clot Platelet number and functions are normal CLINICAL MANIFESTATIONS Diagnosed after the child becomes active Severity varies***depends on plasma level of the coagulation factor involved Hx of prolonged bleeding after circumcision, easy bruising, spontaneous hematomas, hemarthrosis, hematuria, GI bleed DIAGNOSTIC EVALUATION Protime and BT normal PTT prolonged Prothrombin consumption decreased Thromboplastin increased Gene analysis to detect carrier state, for prenatal diagnosis MANAGEMENT PROMPT, appropriate treatment**KEY REPLACEMENT OF type-specific coagulation concentrates during bleeding episodes Supportive therapy : NSAIDS, PT COMPLICATIONS AIRWAY obstruction Repeated hemorrhage ->degenerative joint changes Intestinal obstructions Nerve compression - > paralysis

 Intracranial hemorrhage NURSING PLANS AND INTERVENTIONS Prevent hypovolemia through bleeding control Provide protection against bleeding Preserve mobility Relieving pain Enhancing family coping Bleeding control Apply cold and pressure for 10-15 min to site of injection/venipuncture NO SUTURING AND CAUTERIZATION Immobilize and elevate above heart level Adm coagulation concentrates as ordered AVOID RAPID adm to reduce transfusion reaction; 2-3ml/min STOP transfusion if with hives, tingling, chills, fever, flushing Bleeding protection AVOID rectal temp AVOID injections if possible NO ASA and ASA products Safety p/c pad cribs/siderails; inspect toys; protection devices; remove environmental hazards NO hard candy, suckers, candy canes, straw, sharp eatingutensils Preserve mobility Treat bleeds stat Supportive care to bleeding joints: Immobilize(slight flexion); elevate; ice packs followed by heat Gentle passive exercise 48 h after acute hemarthrosis Physical therapy Pain relief increasing pain means that bleeding continues NSAIDs during acute phase Opioids sparingly