Hematologic problems lecture

8/8/2019 Hematologic problems lecture

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HEMATOLOGIC SYSTEM

DISTURBANCES


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-Myeloproliferative disorder in whichthere is increased production of blood

cells (RBCs, granulocytes, and platelets).


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ProliferatingCellular

Elements

Increase RBCcount, viscosity

and volume

Congestion inthe liver and

spleen

Stasis andthrombosis

Bone Marrow-aplastic, fibrotic

and leukemic


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GOALS OF CARE

Reduction of blood volume and

viscosity

Reduction of bone marrow activity

Venesection (phlebotomy regimen)


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Myelosuppressive agents

Activity

Fluid Balance


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DIAGNOSTIC PROCEDURES

BONE MARROW EXAMINATIONBONE MARROW EXAMINATION-- percutaneous removal of bone marrow and an examination

of erythrocytes, leukocytes, thrombocytes, and precursor cells.

*Before: IC

Determine the patients ability to lie still during

aspiration

Tell the patient that he may experience a

burning sensation as the bone marrow aspirated.

*After: Maintain a pressure dressing at the aspiration site.

Check the aspiration site for bleeding and

infection.

Maintain bed rest, as ordered.


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2. ERYTHROCYTE LIFE SPAN2. ERYTHROCYTE LIFE SPAN--

determination involves a reinjection of the patientsblood that has been tagged with chromium 51.Purpose: to measure the life span of circulatingRBCs.

*Before: Inform the patient that frequent bloodsamples will be drawn over a 2-week pd

*After: Check the venipuncture site for bleeding

Apply a pressure dressing to thevenipuncture site


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- Refers to a condition in which there is a

decrease in hemoglobin concentration, the

number of circulating RBCs, or the volume

of packed cells (hematocrit) compared with

normal values.


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3 Broad Etiologic Categories of Anemia:

Loss of RBCsLoss of RBCs

Decreased production of RBCsDecreased production of RBCs

Increased destruction of RBCsIncreased destruction of RBCs


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TWO KINDS OF DIVISIONTWO KINDS OF DIVISION

1. Those caused by impaired RBC formation

2. Those caused by excessive loss or destructionof RBCs.

*Reticulocyte Count- is of primary importance indiagnosis.

*Morphologic Characteristic- used in classifyingAnemias.


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TERMS USEDTERMS USED

1. Normolytic/ Normochromic- normal sizeand color of RBCs imparted from hemoglobinconcentration

2. Microcytic/ Hypochromic- decreased sizeand color of RBCs caused by inadequatehemoglobin concentration

3. Macrocytic- large size of RBC.

4. Anisocytosis- variation in size of RBCs.

5. Poikilocytosis- variation in RBCs shape.


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CLASSIFICATIONS OF ANEMIAS

TYPE MORPHOLOGIC

CHARACTERISTICS

CHARACTERISTICS

1. APLASTIC Normocytic, normochromicRBCs, depletion of leukocytes

and platelets.

Drug toxicity, genetic failure, radiation,chemicals, infections.

2. HEMOLYTIC

(SICKLE CELL

ANEMIA)

Normocytic, normochromic,

increased number of

reticulocytes

Mechanical injury, RBC antigen-

antibody reaction, complement

binding, chemical reactions, hereditary

membrane defect.

3. MACROCYTIC /

MEGALOBLASTIC:

(PERNICIOUS or

FOLIC ACID)

Macrocytic with variation in

size (anisocytosis), shape

(piokilocytosis) of RBCs.

Inadequate diet, lack of intrinsic factor-

Pernicious Anemia, Impaired

absorption.

4. MICROCYTIC:Iron defiency,

Chronic Blood Loss

Microcytic, hypochromic Inadequate diet, blood loss and chronicincreased need.

5. Post-

Hemorrhagic; Acute

Hemorrhagic

Normocytic, normochromic,

increased number of

reticulocytes within 48-72

hours.

Loss of blood leading to hemodilution

from interstitial fluid within 48-72

hours. Internal and external

hemorrhage


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APLASTIC ANEMIA

REMEMBER: Bone Marrow


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Idiopathic

Genetic failure of bone marrow

development and/or injury to stem cells may

explains cellular differentation. Physical agent (whole body irradiation)

Chemical agent (cytotoxic drug used to treat

malignant disease)

Antimicrobial agent

Anticoagulant

Anti-inflammatory

Etiology/Risk Factors


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Causative factors: Congenital or acquired, Idiopathic

Decreased or damaged marrow stem cells

Drop in all levels of blood elements

Blood forming cells are not formed and dont mature

Decrease circulating blood cells in the circulation

Hypoxia or decrease in oxygen

transport to the tissues(fatigue, Infections, Hemorrhage or bleeding)

Anemia (pallor and dyspnea)


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Assessment

Weakness Dyspnea

Headaches

Syncope

Recurrent infections

Bleeding tendencies

Cervical lymphadenopathy (if patient had arepeated throat infections)

Retinal hemorrhages


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Diagnostic Findings

Bone marrow aspirate shows an extremely

hypoplastic or even aplastic (very few to no

cells) marrow replaced with fat

Uptake of iron by the marrow is decreasedand serum iron is increased

Smears most frequently show normocytic,

normochromic RBCs that are profoundlydecreased in number.


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Medical Management

BONE MARROW TRANSPLANTATION orPERIPHERAL STEM CELLTRANSPLANTATION.

Immunosuppressive therapy Supportive therapy: transfusion of RBCs

and platelets as necessary


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Intervention

Assess carefully for signs of infection andbleeding

Counsel the patient to conserve energy during

treatment

Assist with bone marrow biopsies when

necessary to r/o potential problem with

hematopoiesis

Administer transfusion when ordered


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SICKLE CELL ANEMIA

Sickle hemoglobin gene The sickle hemoglobin (HbS) acquires a

crystal like formation when exposed to lowoxygen tension

The oxygen level in venous blood can be lowenough to cause this change; the RBCcontaining HbS loses its round, very pliable,biconcave disk shape and becomes

deformed, rigid, and sickled-shaped


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If two peoplewith sickle

cell trait havechildren, thechildren mayinherit twoabnormalgenes. Thesechildren willproduce onlyHbS and

therefore willhave cellanemia.


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Pathophysiology

Oxygen level in venous blood is low (deoxygenated)

RBC containing HbS loses its round, biconcave disk shape,

becomes deformed, rigid and sickled shaped

This long rigid RBC can adhere to endothelium

of small vessels

Stiff, viscous sickled cell pile up

against each other


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Blood flow to an organ maybesignificantly reduced

Capillary Venule

Occlusion

Shortened Red

Cell survivalHemolytic

Anemia

MicroinfarctionIschemic tissue painIschemic organ malfunctionAutoinfarction of spleen

JaundiceGallstoneLeg Ulcers


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Symptoms are due to three underlyingSymptoms are due to three underlyingfactors:factors:

1.Hemolytic anemia from the destruction ofsickle cells

2.Thrombosis and Infarction from the occludedmicrocirculation

3.An elevated bilirubin from the releasedhemoglobin that may result in gallstoneformation (cholelithiasis)


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Sickle cell crisis

VASOOCCLUSIVE CRISIS stasis, ischemia andinfarction. Signs include fever, pain, and tissueengorgement.

SPLENIC SEQUESTRATIONpooling of blood in thespleen.

APLASTIC CRISIS APLASTIC CRISIS diminishedproduction and increased destruction of redblood cells,


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Intervention

S

T

P

O

SUPPLEMENTAL OXYGEN

TRANSFUSION (BLOOD)

ORAL FLUIDS

PAIN RELIEVER


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MEGALOBLASTIC ANEMIA

Deficiencies of vitamin B12 and folic acid

megaloblast (large primitive

erythrocytes in the blood and bonemarrow.

Both vitamins are essential for normal

DNA synthesis causing abnormal

maturation of erythrocytes, leukocytes

and platelets


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Folic Acid Deficiency:

Found in people who rarely eatuncooked vegetables

Alcohol increases folic acid

requirements


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Pathophysiology

Inadequate intake of folic acid1. Malabsorptive diseases2. Hemolytic anemia3. Alcoholism

4. Pregnant women

Depleted stores of folates in the body

DNA synthesis is abnormal


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Hyperplasia of the bone marrow

1. RBCs (Macrocytic)2. Other cells are also abnormal (WBC, platelets)

Abnormal RBCs and Myeloid cells are destroyed withinthe marrow

Mature cells that do leave the marrow are fewer innumber

Pancytopenia ( a decrease in all myeloid derived cells)

Anemia


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Intervention

Oral doses of folic acid (0.1 to 5 mg) daily

until the blood pictures improve or until

malabsorption is corrected

People with malabsorption syndromes

may need parenteral folic acid initially,

followed by maintenance therapy with

oral doses.


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PERNICIOUS ANEMIA

Vitamin B12 Deficiency


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Absorption of Vit. B12:

Vitamin B12

To be absorbed in the GIT, must bindto The INTRINSIC FACTOR

Intrinsic Factor is secreted byparietal cells of the gastric mucosa

Intrinsic Factors then binds available vitamin B12 in the GIT

B12 is then absorbed and then stored in the liver, which isavailable for the production of new erythrocytes


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1. Abnormally large erythrocytes (macrocytic

anemia)

2. Hypochlorhydria (deficiency of gastric

hydrochloric acid)

3. Neurologic and gastrointestinal symptoms

4. A fatal outcome unless the person receives

lifelong injections of vitamin B12


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Etiology & Risk Factors

Lack of intrinsic factor

Heredity

Prolonged iron deficiency

May also be an autoimmune disorder

Following gastric surgery


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SCHILLING

TEST


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--aspiration of stomach contents through NGT.

*Before: NPO x 12 hrs

(-) smoking, (-) chewing x 8 hrs

(-) meds affecting gastric secretions

*After: Assess the reactions to gastric acid

stimulant

GASTRIC ANALYSIS


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Interventions

Lifelong therapy

Acute phase: the person may be treated with

vitamin B12 injections

Oral iron supplementations

Blood transfusion

Intensive physical therapy and rehabilitation

({+} neurologic sx )


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IRON DEFICIENCY ANEMIA

Fe intake < hgb synthesis Most common


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Bleeding (from ULCERS, GASTRITIS, INFLAMMATORY

BOWEL DISEASE, or GASTROINTESTINAL TUMORS)

Pre-menopausal : Menorrhagia (excessive menstrualbleeding) and pregnancy with inadequate iron

supplementation

Chronic Alcoholism

Iron Malabsorption ( Gastrectomy and Celiac Disease)

Etiology/Risk Factors


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Inadequateiron in thebloodduetoseveral factorsand

causes: Bleeding, Menorrhagia, Chronicalcoholism, Iron

malabsorption

Lessiron willbeabsorbed

in theduodenum

Decreasedionization andabsorption ofiron in the

blood

Hemoglobin synthesisisaltered

Decreased Hgb and oxygenbinding capacity due to decreasein iron in the heme component of

hemoglobin

Hypoxiain allthetissuesofthebody

Iron DeficiencyAnemia


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THALASSEMIA

A group of hereditary disorders associated with

defective hemoglobin-chain synthesis.

Hypochromia

Extreme Microcystosis

Hemolysis

Variable degrees of anemia


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2 MajorGroups

Alpha thalassemias-RBCs are extremely microcytic, but the anemia, ifpresent, is mild.

Beta thalassemias-Classic thalassemia


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Interventions

Transfusion therapy

Individual with Thalassemia receive packed red cells,

which maybe given:

In a monthly or bi monthly basis (regular transfusion regimen)

Whenever the hemoglobin falls below 3 to 4 gm/100 ml

(nonsystematic Transfusion)

Every 15 days in order to maintain the hemoglobin at 12 to 15

gm/100 ml (hypertransfusion regimen)

Because of transfusion therapy, patient can develop an ironoverload which may cause cardiac arrhythmias.

Excessive iron can be remove from the blood to some

extent by Chelating agents such as desferrioxamine


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1. VASOCONSTRICTION

2. FORMATION OF A HEMOSTATICPLATELET PLUG

3. BLOOD COAGULATION

4. CLOT FORMATION


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FACTORFACTORCOMMONCOMMON

SYNONYMSSYNONYMSREMARKSREMARKS

II FibrinogenFibrinogen Soluble macromolecule, synthesized in theSoluble macromolecule, synthesized in theliver, fibrin precursor.liver, fibrin precursor.

IIII ProthrombinProthrombin Synthesized in the liver, vit K required forSynthesized in the liver, vit K required for

formation.formation.

IIIIII TissueTissuethromboplastin;thromboplastin;thrombokinasethrombokinase

Phospholipid; involved in activation ofPhospholipid; involved in activation ofextrinsic pathwayextrinsic pathway

IVIV CalciumCalcium Involved in several complexes ofInvolved in several complexes of

coagulation process.coagulation process.

VV Proaccelerin;Proaccelerin;labile factor;labile factor;

AcAc--globulin;globulin;

AcAc--GG

Synthesized in the liver; modifier protein,Synthesized in the liver; modifier protein,not enzyme; required innot enzyme; required in prothrombinprothrombin

activator complexactivator complex


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VIVI Obsolete termObsolete term Same as factor V.Same as factor V.

VIIVII Proconvertin; stableProconvertin; stable

factor; serumfactor; serum

prothrombinprothrombin

conversionconversion

acceleratoraccelerator

Part of enzyme complex in extrinsic pathway;Part of enzyme complex in extrinsic pathway;

synthesized in the liver;synthesized in the liver; vitvit K required forK required for

fromationfromation..

VIIIVIII Anti hemophilicAnti hemophilic

globulin (AHG);globulin (AHG);

AntihemophilicAntihemophilic factorfactor

(AHF);(AHF); antihemophilicantihemophilic

factor Afactor A

Required for intrinsic pathway function,Required for intrinsic pathway function,

possibly synthesized in the liver, spleen, RES,possibly synthesized in the liver, spleen, RES,

or kidneys.or kidneys.


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FACTORFACTORCOMMONCOMMON

SYNONYMSSYNONYMSREMARKSREMARKS

IXIX Plasma thromboplastinPlasma thromboplastin

component (PTC),component (PTC),

Christmas Factor,Christmas Factor,

antihemophilic Factor Bantihemophilic Factor B

Synthesized in the liver, requires Vit K neededSynthesized in the liver, requires Vit K needed

for intrinsic pathway function.for intrinsic pathway function.

XX StuartStuart--Prower factor;Prower factor;

Stuat FactorStuat Factor

Synthesized in the liver, requires vit K, neededSynthesized in the liver, requires vit K, needed

for both intrinsic and extrinsic pathwayfor both intrinsic and extrinsic pathway

functionsfunctions

XIXI Plasma tromboplastinPlasma tromboplastin

antecedent (PTA);antecedent (PTA);

antihemophilic factor Cantihemophilic factor C

Substrate in intrinsic activator of intrinsic factor,Substrate in intrinsic activator of intrinsic factor,

area of intrinsic factor is unknownarea of intrinsic factor is unknown

XIIXII Hageman Factor,Hageman Factor,

contact factor,contact factor,antihemophilic factor Dantihemophilic factor D

Involve in the first step of activation of intrinsicInvolve in the first step of activation of intrinsic

system; area of sythesis is unknown.system; area of sythesis is unknown.

XIIIXIII Fibrin Stabilizing factorFibrin Stabilizing factor

(FSF), fibrinase(FSF), fibrinase

Causes amide cross linkage fibrin, stabilizes clotCauses amide cross linkage fibrin, stabilizes clot

formation, synthesized by platelets and possiblyformation, synthesized by platelets and possibly

other proteins, may be activated by liver.other proteins, may be activated by liver.


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ALTERATIONS IN BLOOD COAGULATION

HEMOPHILIAHEMOPHILIA-Loosely defines several different hereditary deficiencies of

coagulation factors of intrinsic pathway.

3 TYPES OF HEMOPHILIA3 TYPES OF HEMOPHILIA

1. CLASSIC HEMOPHILIA OR HEMOPHILIA A/ VON WILLEBRAND

DISEASE

2. FACTOR IX DEFICIENCY/ HEMOPHILIA B/ CHRISTMAS DISEASE

3. FACTOR XI DEFICIENCY/ HEMOPHILI C/ ROSENTHALS DISEASE


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VITAMIN K DEFICIENCY

-Vit K is required for the synthesis of factors II, VII,

IX, and X.

LIVER DISEASE

-liver is essential for the synthesis of the

coagulation proteins and for the removal of

activated coagulation products from the

circulation


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DIC/ CONSUMPTIVE COAGULOPATHY

Activation of the sequence causing coagulation

Hypercoagulable state

Thrombosis esp. in small vessels

Consumption of clotting factors esp platelets and fibrin

Secondary activation of Fibrinolytic System

Diffuse Fibrinolysis


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PLATELET DISORDERS

Etiology:

1.Defective platelet production

2.Increase platelet destruction

3.Sequestration of platelets

4.Loss of platelets from the system

THROMBOCYTOPENIA


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2 MAJOR TYPES OF THROMBOCYTOPENIA

1. Idiopathic thrombocytopenia purpura

2. Secondary thrombocytopenia


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IDIOPATHIC THROMBOCYTOPENIC PURPURA

-An autoimmune condition that causes an increasedrate of destruction of platelets.

Acute - viral infection Chronic- autoimmunehemolytic anemia


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Assessment

Diffuse petechiae

Ecchymosis

Epistaxis

Hemorrhages into the soft tissues Melena

Hematuria


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SECONDARY THROMBOCYTOPENIA

Drug sensitivity-cholothiazide derivatives, gold thiomalate,diphenylhydantoin, acetaminphen, quinidine, heparin, sulfonamides,

chloramphenicol, antimetabolites and antihistamine.

Platelet destruction

Decrease in platelet count

Bleeding disorder


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PLATELET FUNCTION DISORDER

- NORMAL platelet COUNT but w/ ALTERATIONSin function due to defective membrane.

Thrombocytosis- An increase in number of platelet in peripheral blood.

Etiology

1. Infection2. Trauma

3. Post splenectomy-when platelets are circulated I the blood

they can no longer pool in the spleen.


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Potential for fluidvolumedeficitrelatedtobleedingGoal: Hemodynamicstatusmaintainedmanifestedby urine

output >30 ml/hr.

Avoid procedures/activities that can increase intracranial

pressure (coughing, straining to have a bowel movement)Rationale: Prevents intracranial bleeding

Monitor vital signs closely, including neurologic checks:Rationale: Identifies sign of hemorrhage/shock quickly

Avoid medications that interefere with platelet function ifpossible (ASA, NSAIDS, beta lactam antibiotics): Rationale:Decreases problems with platelet aggregation andadhesion


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Avoid IM injections

Monitor amount of external bleeding carefully Monitor number of dressings, % of dressing saturated;

time to saturate a dressing is more objective than

dressing saturated a moderate amount

Monitor suction output (all excreta).

Females may receive progesterone to prevent menses.

Use low pressure with any suctioning

neededAdminister oral hygiene carefully Avoid lemon glycerine swabs, hydrogen peroxide,

commercial mouthwashes

Use sponge-tipped swabs, salt/baking soda mouthrinses

Avoid dislodging any clots, including those around

IV sites and injection sites


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Potential forimpairedskin integritysecondarytoischemia

orbleeding

Goal:skin integrityremainsintact;oralmucosaremainsintact

Assess skin, with particular attention to bony

prominences, skin folds

Reposition carefully; use pressure-reducing mattress

Perform careful skin care every 2 hour emphasizing

dependent areas, all bony prominence, perineum

Use lambs wool between digits, around ears, as needed

Use prolonged pressure after injection or procedurewhen such measures must be performed (at lat 5 min)

Administer oral hygiene carefully


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Potential for fluidvolumeexcess

Goals:A

bsen

ceo

fedema;

abse

nce

o

frales;

intake notgreaterthan output.

Auscultate breath sounds every 2 4 hour:

crackles can develop quickly. Monitor extent of edema .

Monitor volume of IVs, blood products;

decrease volume of IV medications ifpossible.

Administer diuretics as prescribed.

Hematologic problems lecture

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