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Interferences with Diffusion. Anemia Leukemia Hemophilia. Interferences with Diffusion. Describe clinical manifestations, causes, therapeutic interventions, & nursing management of patients with the following Hematologic Problems: Anemias – Decreased Erthrocyte Production - PowerPoint PPT Presentation
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Interferences with
Diffusion
Anemia
Leukemia
Hemophilia
Interferences with Diffusion Describe clinical manifestations, causes, therapeutic interventions, & nursing management of patients with the following Hematologic
Problems: Anemias –
Decreased Erthrocyte Production Iron Deficiency Anemia; Thalassemia; Megaloblastic Anemias: Cobalamin deficiency, Folic Acid Deficiency; Aplastic Anemia
Anemia Caused by Blood Loss Anemia Caused by Erythrocyte Destruction
Sickle Cell Disease Acquired Hemolytic Anemia Hemochromatosis Polycythemia
Problems of Hemostasis: Thrombocytopenia Hemophilia and Von Willebrand’s Disease
Leukemias – Acute myelogenous leukemia Acute lymphocytic leukemia Chronic myelogenous leukemia Chronic lymphocytic leukemia
Lymphomas Hodgkin’s Disease / non-Hodgkin’s lymphomas Multiple Myeloma
Mature Normal Erythrocytes
Nutrients Needed for Erythrocytes
Interferences with DiffusionHematologic System Review
Complete Blood Count Studies Hgb Hct Total RBC Count Red Cell Indices
MCV – mean corpuscular volume (size of RBC) MCH – mean corpuscular hemoglobin (weight of Hb/RBC) MCHC – mean corpuscular hemoglobin concentration (saturation of
RBC with Hb) WBC
WBC Differential Platelet Count
Erythrocyte Sedimentation Rate (ESR or Sed Rate)
Development of Blood Cells
Blood Components
What are the functions of blood components?
Functions of Blood
Drugs Affecting Hematologic Function
Interferences with DiffusionAnemias Caused by Decreased Erythrocyte Production
Anemia
Deficiency in the number of erythrocytes (RBCs) The quantity of hemoglobin Volume of packed RBCs (hematocrit)
Clinical Manifestations: caused by the body’s response to hypoxia Mild (Hb 10 -14) no symptoms or minor changes Moderate – (Hg 6 – 10) CV Changes: palpitations, dyspnea,
diaphoresis Severe – (Hg<6) multiple body system CV, Cerebral, Major
Organs
Anemia Clinical Manifestations
Interferences with DiffusionAnemias Caused by Decreased Erythrocyte Production
Iron-Deficiency Anemia
Common hematologic disorder
Etiology: Inadequate dietary intake, malabsorption, blood loss, or hemolysis
Clinical Manifestations: Pallor Glossitis – inflammation of the tongue Cheilitis – inflammation of the lips Headache, paresthesia, burning sensation of the tongue
Diagnostic Studies: Lab Studies Endoscopy to identify GI bleed
Treatment: Drug Therapy – oral Iron replacement Iron absorbed best in duodenum Ferrous sulfate – take about one hour prior to meal
Gastric side effects: nausea / constipation
Nursing Management – Diet & Medication Instruction
Interferences with DiffusionAnemias Caused by Decreased Erythrocyte Production
Thalassemia
Autosomal recessive genetic disorder of inadequate production of normal hemoglobin Hemolysis occurs Abnormal Hb synthesis Ethnic groups of Mediterranean Sea & near equatorial regions of Asia
and Africa Clinical Manifestation: mild – moderate anemia with hypochromia
(pale cells) or microcytosis (small cells) Minor: one thalassemic gene – mild Major: two thalassemic genes – severe – physical & mental growth
retarded - cardiac failure is fatal Medical Management:
Medication: Chelation Therapy IV deferoxamine (Desferal) – iron binding agent to reduce iron overload
Transfusions to maintain Hg >10g/dl Nursing Management: Supportive
Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production
Megaloblastic Anemias Caused by impaired DNA synthesis & characterized
by the presence of large RBCs
Cobalamin Deficiency (Vitamin B12)– Intrinsic factor (IF) is secreted by the parietal cells of the gastric mucosa Cobalamin is not absorbed if IF is not present
Causes: Pernicious anemia, nutritional deficiency; heredity enzyme defect
Clinical Manifestations: GI—sore tongue, anorexia, N&V, abdominal pain; muscle weakness, paresthesias of feet and hands; confusion
Diagnostic Testing: Serum cobalamin levels; gastroscopy; Schilling Test – assesses parietal cell function
Medical Management: Parenteral administration of cobalamin – daily for 2 weeks, then weekly until >HCT, then monthly for life; intranasal form
Nursing Management: Health Promotion; protection from sensory injury—burns, trauma; pt compliance with replacement therapy
Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production
Megaloblastic AnemiasFolic Acid Deficiency – Folic Acid is required for DNA
synthesis leading to RBC formation & maturation Causes: Poor nutrition green leafy vegetables, citrus fruits, &
beans, nuts, grains; malabsorption syndromes; drugs that impede absorption (Dilantin); Alcohol abuse; anorexia; hemodialysis patients
Clinical ManifestationsClinical Manifestations: similar to cobalamin deficiency – dyspepsia, smooth, beefy red tongue; absence of neurologic problems
Diagnostic Testing: < Folate Level (norm: 3-25mg/ml) Medical Management: Replacement Therapy Folic Acid
1mg/day Nursing Management: Medication & dietary compliance
Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production
Anemia of Chronic Disease Associated with underproduction of RBCs and
decreased RBC survival Causes: Renal failure; advanced liver
cirrhosis; chronic inflammation; malignancy; immunosuppression
Medical Management: Correct underlying disorder Erythropoietin Therapy – Epogen, Procrit
Nursing Management: Care of the debilitated patient – dietary & medication compliance
Interferences with Diffusion Anemias Caused by Decreased Erythrocyte Production
Aplastic Anemia Pancytopenia – decrease of all blood cell types ---
RBCs, WBCs, platelets & hypocellular bone marrow Congenital Acquired – exposure to radiation & chemicals, post viral
& bacterial infections Idiopathic – 70%
Medical Management: Immunosuppressive therapy Bone Marrow Transplantation
Interferences with Diffusion Anemia Caused by Blood Loss
Acute Blood Loss Hemorrhage
Decreased oxygen-carrying capacity
Chronic Blood Loss Body maintains its blood volume by slowly increasing plasma
volume < RBCs
Clinical Manifestations: Range from fatigue with melena to orthostatic BP changes to
shock
Medical Management: Treat underlying cause – Blood replacement – packed RBCs Supplemental Iron
Lab Study Findings in Anemias
PAIR-SHARE
Name one thing you learned thus far
Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction
Sickle Cell Disease Group of inherited autosomal recessive disorders
characterized by the presence of abnormal Hgb in the erythrocyte Causes the erythrocyte to stiffen & elongate Sickle shape in response to lack of oxygen
Occurrence: 50,000 Americans 1 in 350-500 African Americans; Mediterranean, So Am; East
Indian, Arabian ancestry Types:
Sickle Cell Anemia: most severe – inherited homozygous for hemoglobin S (HbSS) from both parents
Sickle Cell Trait: mild - inherited from one parent + one normal
Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction
Sickle Cell Disease
Sickling Episodes:
Hypoxemia – triggered by stress, surgery, blood loss, viral or bacterial infection*(most common), dehydration, acidosis
Low oxygen tension in the blood Sickled cells cannot easily pass through capillaries
Hemolyzed in the spleen Initially reversible – then becomes irreversible due to chronic
sickling
Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction
Sickle Cell Crisis
Severe, painful, acute exacerbation of RBC sickling causing a vasoocclusive crisis Impaired blood flow, vasospasm, severe capillary hypoxia RBC Cell membrane permeability changes – plasma loss, & thrombi;
tissue ischemia & infarction Sudden & persists for days Clinical Manifestation: PAIN – tissue ischemia
Aching joints—hands & feet Organs that have a high need for oxygen are most affected: heart, lungs,
eyes, kidneys, brain Spleen scarring & small – auto splenectomy Bones – osteoporosis Chronic leg ulcers Prone to infection – pneumococcal pneumonia
Medical Management: Hospitalization--Oxygen, rest, fluids & electrolytes, treat infection, transfusion therapy, Chelation therapy, pain management Bone Marrow Transplant & Gene therapy technology
Sickle Cell Disease Manifestations
PAIR-SHARE
Priority Nursing Actions
for the client
in
Sickle Cell Crisis
Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction
Acquired Hemolytic Anemia
Extrinsic causes of hemolysis: Physical – trauma – renal dialysis; CP bypass Autoimmune Reactions – medications; systemic
lupus erythematosus; leukemia; lymphoma Infectious agents and toxins – parasites; antigen-
antibody reactions; splenomegaly Medical Management:
Corticosteroids; Blood product administration; splenectomy
Interferences with Diffusion Anemia Caused by Increased Erythrocyte Destruction
Polycythemia
Production o& presence of increased number of RBCs Increased blood viscosity – hyperviscosity Increased circulating volume – hypervolemia
Types: Primary – polycythemia vera / chromosomal mutation – insidious -- >50 years of age Secondary – chronic hypoxia stimulates erythropoietin production in the kidney >
erythrocyte production High altitude, COPD, CV disease
Diagnosis: Elevated RBC, WBC, Platelets; bone marrow aspiration – hypercellularity of RBCs; splenomegaly
Medical Management: Phlebotomy to maintain HCT 45-48%
300-500 ml removed every other day until <HCT Hydration Myelosuppressive therapy: busulfan (Myleran); hydroxyurea (Hydrea) = inhibits bone
marrow production Gout – Allopurinol Antiplatelet medication: Persantine, Plavix, ASA – prevent thrombotic complicatins
Primary & Secondary Polycythemia
Interferences with Diffusion Problems of Hemostasis
Thrombocytopenia Reduction of platelets below 150,000/ul
ITP: Immune Thrombocytopenic Purpura: autoimmune platelets are coated with antibodies; destroyed in spleen Women 20-40 years Normal survival 8-10 days; ITP: 1-3 days
TTP: Thrombotic Thrombocytopenic Purpura Uncommon syndrome; adults 20-50 years Characterized by hemolytic anemia, thrombocytopenia,
neurologic abnormalities, fever, renal abnormalities HITT: Heparin-Induced Thrombocytopenia & Thrombosis
Syndrome Immune-mediated response to Heparin Triggers platelet aggregation
Decreased platelets & increased thrombosis
Interferences with Diffusion Problems of Hemostasis
Thrombocytopenia Clinical Manifestations:
Bleeding: Mucosal — epistaxis, gingival, large bullous hemorrhages on
buccal mucosa Skin -- superficial ecchymoses; petechiae — flat pin-pointed
red brown microhemorrhages; purpura — numerous petechiae resulting reddish skin bruising
Prolonged bleeding – after injections & venipuncture Internal bleeding – hemorrhage – any major organ --
orthostatic hypotension -- Cerebral hemorrhage fatal Medical Management:
ITP: Corticosteroid; immunosuppressive therapy; splenectomy; platelet transfusion
TTP: Corticosteroids; plasma exchange; plasmapheresis; splenectomy
HITT: Discontinue Heparin – Protamine Sulfate
Interferences with Diffusion Anemia caused by Blood Loss or
Erythrocyte Destruction Nursing Management
Assess: Assess oral cavity, skin, nasal cavity, urine, stool – occult and overt bleeding; Lab values—CBC, platelet count; vital signs; signs & symptoms for each blood dyscrasia
Nsg Action: Ice, packing, direct pressure to control bleeding; administer medications; No ASA or platelet-acting meds; oral hygiene, skin care, IV carefully—blood product transfusion care; Avoid IM/SQ meds; pad rails & firm surfaces; pain management as needed; post splenectomy care;
Pt Education: Interpret lab values; Rationale for no sharps – electric razor; disease process; medications; avoid valsalva; blow nose gently—one side at a time; signs & symptoms of bleeding; lab values; health promotion—rest, oxygenation, nutrition; pain management
Interferences with Diffusion Problems of Hemostasis
Hemophilia
Sex-linked recessive genetic disorder caused by defective or deficient coagulation
Hemophilia
A
Factor VIII Recessive – female carrier;
Occurs primarily in male
Hemophilia
B
Factor IX Recessive – female carrier;
Occurs primarily in male
Von Willebrand’s disease
vWF and platelet dysfunction
Autosomal dominant; seen in both sexes
Interferences with Diffusion Problems of Hemostasis
Hemophilia – Disease of Childhood Clinical Manifestations:
Slow, persistent, prolonged bleeding from minor trauma and small cuts
Delayed bleeding after minor injuries Uncontrollable hemorrhage after dental extractions Epistaxis, especially after a blow to the face GI bleed – ulcers & gastritis Hematuria from GU trauma Splenic rupture from abdominal trauma Ecchymosis & subcutaneous hematomas Neurologic signs – pain, anesthesia, & paralysis – from nerve
compression caused by hematoma formation Hemarthrosis – bleeding into joints – joint deformity – may cause
crippling
SYMPTOMS OFTEN LEAD TO THE DIAGNOSIS
Interferences with Diffusion Problems of Hemostasis
Hemophilia
Time Life Expectancy Early 1900’s 11 years 1970’s 60 years 1980’s late 40’s
90% of older people are HIV+ Many are Hepatitis C+ Improved blood product testing & screening has decreased
the number of younger people with blood borne sequellae
Coagulation Mechanism
Clotting Cascade XII + Surface: intrinsic path: heparin/ PT
| XIIa | VIIa + TF: extrinsic path: (warfarin/PT) XI---XIa <----/ | IX-----IXa | VIIIa | | Thrombomodulin X----------Xa --------> Prot. C/S -- / | Va | / | | Antithrombin III -----> II-------IIa ----------------------> VIIIa & Va | | Fibrinogen----Fibrin
Specific Clotting Factors fibrinogen (factor I);
- prothrombin (factor II) - converts finbrinogen to fibrin - activates V, VIII & XIII (when bound to thrombomodulin) ; - activates protein C; - Vit K dependent;
- factor V: when activated, serves as enzyme co-factor
- factor Xa: part of Xa/Va complex which activates prothrombin;
- factor VII:
- part of factor VII/tissue factor complex-activates factor X & IX; - is activated by Xa; - Vit K dependen
- factor VIII: serves as enzyme cofactor to help activate factor X;
- factor IX: - acts w/ IXa/VIIIa/phos complex that activates factor X; - Vit K dependent;
- factor X: - acts as Xa/Va phos complex that actives prothrombin; - Vit K dependent;
- factor XII: - protein C: - when activated to Ca by thrombin bound to thrombomodulin, inhibits by proteolysis factors VIIIa and Va in
reactions requiring prot S and phospholipids as cofactors; Vit K dependent; - antithrombin III:
- is a plasma protease inhibitor that serves as a protease scavenger; - any of the blood-clotting enzymes that move away from the growing clot rapidly form a complex, and their
activities are neutralized - formation of complexes is accelerated by heparin, forms of which are located in the microvasculature on the surfaces of endothelial cells; - inhibitor of the enzymes thrombin, Xa, IXa; Is activated by heparin;
Interferences with Diffusion Problems of Hemostasis
Hemophilia Diagnostic Studies:
Partial thromboplastin time: prolonged Bleeding time:
Prolonged in von Willebrand’s because of structural defective platelets;
Normal in Hemophilia A & B Factor Assays:
Factor VIII – A Factor IX – B vWF – von Willebrand
Interferences with Diffusion Problems of Hemostasis
Hemophilia Medical Management Goals:
Preventive Care Replacement Therapy during acute bleeding
May also be given prior to surgery or dental care Factor VIII: Examples: Alpohanate, Bicolate, Koate Factor IX: Examples: Alphanine, Benefix, Konyne Mild Hemophilia A & von Willebrand’s:
Desmospressin acetate (DDAVP) – causes a release of vWF, which binds with factor VIII, increasing concentration Short-lived; IV/SQ/Intranasal
Treatment of complications
HemophiliaHematoma of the Ear
Interferences with Diffusion Problems of Hemostasis
Hemophilia Nursing Management:
Health Promotion: Acute Intervention:
Stop the topical bleeding – direct pressure, ice, Gelfoam or fibrin foam packing; topical hemostatic agents – thrombin
Administer the specific anticoagulant factor Joint bleeding: rest; pack in ice; analgesia
Mobilization when bleeding subsides – P & AROM exercise Manage life-threatening complications:
Airway management – Head and neck injuries Neuro signs – Head trauma; spine trauma
Home Care: Patient Education – Age-specific: how to live with illness: increased
oral hygiene; injury prevention; Medic Alert; routine medical follow-up; non-contact sports; self-administration of replacement factors
Local chapters of National Hemophilia Society Daily oral hygiene
Acute Hemarthrosis – Right Knee
PAIR-SHARE
What coagulation studies
are affected by hemophilia?
Interferences with DiffusionTransfusion Therapy - Indications
Packed red blood cells – anemia; Hgb < 6-9g/dL depending on symptoms
Washed red blood cells – hx allergic rx; bone marrow transplant patients
Platelets – thrombocytopenia; active bleeding with platelet count <80,000
Fresh frozen plasma – deficiency in plasma coagulation factors
Cryoprecipitate – Hemophilia VII or von Willebrand’s disease
WBC’s – Sepsis, neutropenic infection
Interferences with DiffusionTransfusion Therapy
Responsibilities Before Transfusion: assess lab values; verify order;
assess pt’s VS, UO, hx of transfusion reactions; CONSENT; patent venous access; pt ID / Blood verification RN-RN - document.
During Transfusion: Appropriate tubing & filter; normal saline infusion; prescribed rate of administration; Remain with pt for the first 20 mins of transfusion; monitor VS and assess for transfusion rx. – document
After transfusion: Assess VS; discontinue infusion & dispose of bag & tubing according to policy; document; reassess blood work
Interferences with DiffusionTransfusion Therapy
Transfusion reactions Hemolytic: blood type or Rh incompatibility – antigen-antibody reaction –
fever, chills, DIC, circulatory collapse (back pain, tachypnea, tachycardiac, hypotension, chest pain, hemoglobinuria, apprehension
Allergic: Urticaria, bronchospasm, anaphylaxis – hx of allergy
Febrile: occurs in pts with anti-WBC antibodies – chills, tachycardiaa, fevere, hypotension, tachypnea
Bacterial: Rapid onset: occurs as a result of contamination – tachycardia; hypotension, fever, chills, shock
Circulatory Overload: Infused too quickly; Hx CHF – Hypertension, bounding pulse, distended jugular veins, dyspnea, restlessness, confusion
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