Download Nursing Management

Interferences
with
Diffusion
Anemia
Leukemia
Hemophilia
Interferences with Diffusion
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Describe clinical manifestations, causes, therapeutic interventions,
& nursing management of patients with the following Hematologic
Problems:
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Anemias –
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Decreased Erthrocyte Production
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Anemia Caused by Blood Loss
Anemia Caused by Erythrocyte Destruction
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Thrombocytopenia
Hemophilia and Von Willebrand’s Disease
Leukemias –
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Sickle Cell Disease
Acquired Hemolytic Anemia
Hemochromatosis
Polycythemia
Problems of Hemostasis:
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Iron Deficiency Anemia; Thalassemia;
Megaloblastic Anemias: Cobalamin deficiency, Folic Acid Deficiency; Aplastic Anemia
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 Diffusion
Hematologic System Review
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Complete Blood Count Studies
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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 Diffusion
Anemias Caused by Decreased Erythrocyte Production
Anemia
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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
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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 Diffusion
Anemias Caused by Decreased Erythrocyte Production
Iron-Deficiency Anemia
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Common hematologic disorder
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Etiology: Inadequate dietary intake, malabsorption, blood loss, or hemolysis
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Clinical Manifestations:
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Pallor
Glossitis – inflammation of the tongue
Cheilitis – inflammation of the lips
Headache, paresthesia, burning sensation of the tongue
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Diagnostic Studies: Lab Studies
Endoscopy to identify GI bleed
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Treatment: Drug Therapy – oral Iron replacement
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Iron absorbed best in duodenum
Ferrous sulfate – take about one hour prior to meal
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Gastric side effects: nausea / constipation
Nursing Management – Diet & Medication Instruction
Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production
Thalassemia
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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)–
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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 Anemias
Folic Acid Deficiency – Folic Acid is required for DNA
synthesis leading to RBC formation & maturation
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Causes: Poor nutrition green leafy vegetables, citrus fruits, &
beans, nuts, grains; malabsorption syndromes; drugs that
impede absorption (Dilantin); Alcohol abuse; anorexia;
hemodialysis patients
Clinical 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
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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:
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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
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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:
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Immunosuppressive therapy
Bone Marrow Transplantation
Interferences with Diffusion
Anemia Caused by Blood Loss
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Acute Blood Loss
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Chronic Blood Loss
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Body maintains its blood volume by slowly increasing plasma
volume < RBCs
Clinical Manifestations:
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Hemorrhage
 Decreased oxygen-carrying capacity
Range from fatigue with melena to orthostatic BP changes to
shock
Medical Management:
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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
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Sickle Cell Disease
Group of inherited autosomal recessive disorders
characterized by the presence of abnormal Hgb in the
erythrocyte
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Occurrence: 50,000 Americans
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Causes the erythrocyte to stiffen & elongate
Sickle shape in response to lack of oxygen
1 in 350-500 African Americans; Mediterranean, So Am; East
Indian, Arabian ancestry
Types:
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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
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Sickling Episodes:
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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
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Hemolyzed in the spleen
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Initially reversible – then becomes irreversible due to chronic
sickling
Interferences with Diffusion
Anemia Caused by Increased Erythrocyte Destruction
Sickle Cell Crisis
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Severe, painful, acute exacerbation of RBC sickling causing a
vasoocclusive crisis
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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
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Aching joints—hands & feet
Organs that have a high need for oxygen are most affected: heart, lungs,
eyes, kidneys, brain
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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
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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
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Extrinsic causes of hemolysis:
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Physical – trauma – renal dialysis; CP bypass
Autoimmune Reactions – medications; systemic
lupus erythematosus; leukemia; lymphoma
Infectious agents and toxins – parasites; antigenantibody reactions; splenomegaly
Medical Management:
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Corticosteroids; Blood product administration;
splenectomy
Interferences with Diffusion
Anemia Caused by Increased Erythrocyte Destruction
Polycythemia
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Production o& presence of increased number of RBCs
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Increased blood viscosity – hyperviscosity
Increased circulating volume – hypervolemia
Types:
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Primary – polycythemia vera / chromosomal mutation – insidious -- >50 years of age
Secondary – chronic hypoxia stimulates erythropoietin production in the kidney >
erythrocyte production
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High altitude, COPD, CV disease
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Diagnosis: Elevated RBC, WBC, Platelets; bone marrow aspiration –
hypercellularity of RBCs; splenomegaly
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Medical Management:
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Phlebotomy to maintain HCT 45-48%
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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
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Thrombocytopenia
Reduction of platelets below 150,000/ul
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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
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Decreased platelets & increased thrombosis
Interferences with Diffusion
Problems of Hemostasis
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Thrombocytopenia
Clinical Manifestations:
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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:
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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
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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
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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;
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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,
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
vWF and
Willebrand’s platelet
disease
dysfunction
Autosomal dominant; seen in
both sexes
Interferences with Diffusion
Problems of Hemostasis
Hemophilia – Disease of Childhood
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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
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Time
Early 1900’s
1970’s
1980’s
Life Expectancy
11 years
60 years
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
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XII + Surface: intrinsic path: heparin/ PT
|
XIIa
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VIIa + TF: extrinsic path: (warfarin/PT)
XI---XIa <----/
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IX-----IXa
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VIIIa |
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Thrombomodulin
X----------Xa
--------> Prot. C/S -/
| Va
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/
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Antithrombin III -----> II-------IIa ----------------------> VIIIa & Va
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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
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Hemophilia
Diagnostic Studies:
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Partial thromboplastin time: prolonged
Bleeding time:
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Prolonged in von Willebrand’s because of structural
defective platelets;
Normal in Hemophilia A & B
Factor Assays:
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Factor VIII – A
Factor IX – B
vWF – von Willebrand
Interferences with Diffusion
Problems of Hemostasis
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Hemophilia
Medical Management Goals:
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Preventive Care
Replacement Therapy during acute bleeding
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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:
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Desmospressin acetate (DDAVP) – causes a release of vWF,
which binds with factor VIII, increasing concentration
 Short-lived; IV/SQ/Intranasal
Treatment of complications
Hemophilia
Hematoma of the Ear
Interferences with Diffusion
Problems of Hemostasis
Hemophilia
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Nursing Management:
 Health Promotion:
 Acute Intervention:
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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
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Manage life-threatening complications:
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Mobilization when bleeding subsides – P & AROM exercise
Airway management – Head and neck injuries
Neuro signs – Head trauma; spine trauma
Home Care:
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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 Diffusion
Transfusion Therapy - Indications
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Packed red blood cells – anemia; Hgb < 6-9g/dL depending on
symptoms
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Washed red blood cells – hx allergic rx; bone marrow transplant
patients
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Platelets – thrombocytopenia; active bleeding with platelet count
<80,000
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Fresh frozen plasma – deficiency in plasma coagulation factors
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Cryoprecipitate – Hemophilia VII or von Willebrand’s disease
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WBC’s – Sepsis, neutropenic infection
Interferences with Diffusion
Transfusion Therapy
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Responsibilities
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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 Diffusion
Transfusion Therapy
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Transfusion reactions
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Hemolytic: blood type or Rh incompatibility – antigen-antibody reaction –
fever, chills, DIC, circulatory collapse (back pain, tachypnea, tachycardiac,
hypotension, chest pain, hemoglobinuria, apprehension
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Allergic: Urticaria, bronchospasm, anaphylaxis – hx of allergy
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Febrile: occurs in pts with anti-WBC antibodies – chills, tachycardiaa,
fevere, hypotension, tachypnea
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Bacterial: Rapid onset: occurs as a result of contamination – tachycardia;
hypotension, fever, chills, shock
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Circulatory Overload: Infused too quickly; Hx CHF – Hypertension,
bounding pulse, distended jugular veins, dyspnea, restlessness, confusion