Ch 42: Hematological Interventions (per Amendolair)

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    Interventionsfor Clientswith

    HematologicProblems

    Chapter 42

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    Red Blood Disorders

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    Anemia

    Reduction in either the number of red blood cells,the amount of hemoglobin, or the hematocrit

    Clinical sign (not a specific disease); amanifestation of several abnormal conditions

    See Table 43-1 Common Causes ofAnemia

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    Anemias: RBC

    Anemias Caused by Increase Destruction of RBC

    Sickle Cell

    Glucose-6-Phosphate Dehydrognenase Deficiency Anemia

    Anemias resulting from Decrease Production of

    RBC Iron Deficiency Anemia

    Vitamin B12 Deficiency Anemia

    Aplastic Anemia

    Polycythemia Vera Myelodysplastic syndromes

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    Anemias: WBC Disorders

    Leukemia

    Malignant Lymphomas Hodgkins Lymphoma

    Non-Hodgkins Lymphoma

    Multiple Myeloma

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    Anemia: Coagulation Disorders

    Platelet Disorders

    Autoimune Thrombocytopenic Purpura

    Thrombotic Thrombocytopenic Purpura

    Clotting Factor Disoders

    Hemophilia

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    Sickle Cell Disease

    Genetic disorder resulting in chronic anemia, pain,disability, organ damage, increased risk for infection,and early death

    Formation of abnormal hemoglobin chains(Continued)

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    Sickle Cell Disease (Continued)

    Conditions causing sickling: hypoxia, dehydration,infections, venous stasis, low environmental bodytemperatures, acidosis, strenuous exercise, andanesthesia.

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    Figure 43-01. Red blood cell actions under conditions of low tissue oxygenation

    . HbA,H

    emoglobin A; HbS, hemoglobin S.

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    To have an autosomal recessive disorder, you inherit twomutated genes, one from each parent. These

    disorders are usually passed on by two carriers. Their health is rarely affected, but they have one mutated

    gene (recessive gene) and one normal gene (dominant gene) for the condition. Two carriers have a 25 percent

    chance of having an unaffected child with two normal genes (left), a 50 percent chance of having an unaffected

    child who also is a carrier (middle), and a 25 percent chance of having an affected child with two recessive

    genes (right).

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    Clinical Manifestations

    Cardiovascular changes

    Skin changes

    Abdominal changes

    Musculoskeletal changes

    Central nervous system changes

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    Interventions

    Pain is the most common problem.

    Drug therapy: 48 hours of intravenous analgesics

    Oral hydration

    Complementary and alternative therapies

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    Potential for Sepsis

    Interventions include:

    Protection of the client from infection in sickle cell crisis

    Drug therapy

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    Potential for Multiple Organ Dysfunction

    Interventions include:

    Hydration

    Oxygen therapy

    Transfusion therapy

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    Glucose-6-Phosphate Dehydrogenase (G6PD)Deficiency Anemia

    Most common type of congenital hemolytic anemia

    Hydration

    Screening for this deficiency necessary beforedonating blood, because cells deficient in G6PD

    can be hazardous

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    Iron Deficiency Anemia

    This common type of anemia can result fromblood loss, poor intestinal absorption, orinadequate diet.

    Evaluate adult clients for abnormal bleeding.

    Supplemental iron is the treatment.

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    Vitamin B12 Deficiency Anemia

    Anemia is caused by inhibiting folic acid transportand reducing DNA synthesis in precursor cells.

    Vitamin B12 deficiency is a result of poor intake offoods containing vitamin B12.

    (Continued)

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    Vitamin B12 Deficiency Anemia (Continued)

    Pernicious anemia is anemia caused by failure toabsorb vitamin B12 and lack of intrinsic factor;clients often exhibit paresthesia.

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    Folic Acid Deficiency Anemia

    Can cause megaloblastic anemia

    Manifestations similar to those of vitamin B12deficiency, but nervous system functions remainnormal

    (Continued)

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    Folic Acid Deficiency Anemia (Continued)

    Caused by:

    Poor nutrition and chronic alcohol abuse

    Malabsorption syndromes, such as Crohns disease

    Drugs, including anticonvulsants and oral contraceptives,

    that slow or prevent absorption of folic acid

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    Aplastic Anemia

    Deficiency of circulating RBC due to bone marrowfailure to produce RBC

    Cause: Injury to immature RBC

    Long term exposure to toxic agents and drugs Ionizing radiation exposure

    Infection

    Unknown

    Fanconis anemia hereditary aplastic anemia

    Associated with leukopenia and thrombocytopenia

    Assess

    Management

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    Polycythemia Vera

    Disease with a sustained increase in bloodhemoglobin

    Massive production of red blood cells

    Excessive leukocyte production

    Excessive production of platelets

    Assessment

    Bone Marrow aspiration

    Phlebotomy

    Increased hydration

    Anticoagulants are part of therapy

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    In a bone marrow aspiration and biopsy, a doctoror nurse uses a thin needle to

    remove a small amount of liquid bone marrow, usually from a spot in the back of

    your hipbone called the posterior iliac crest. A bone marrow biopsy isoften taken at

    the same time. Thissecond procedure removes a small piece of bone tissue and the

    enclosed marrow.

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    Leukemia

    Type of cancer with uncontrolled production of immaturewhite blood cells in the bone marrow

    Acute or chronic

    Classified by cell type

    Risk factors: ionizing radiation, exposure to certainchemicals and drugs, bone marrow hypoplasia, geneticfactors, immunologic factors, environmental factors

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    Your body's lymphatic system is part of your immune system, which protects you

    against infection and disease. It includes yourspleen, thymus, lymph nodes and

    bonemarrow. In people with leukemia, the bone marrow produces a large number

    of abnormal blood cells. These blood cells don't function properly and eventually

    overwhelm production of healthy cells.

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    Clinical Manifestations

    Cardiovascular: heart rate is increased; bloodpressure is decreased.

    Respiratory rate increases.

    Skin grows pale and cool to the touch.

    Intestinal manifestations include weight loss,nausea, and anorexia.

    Central nervous system disturbances includeheadache.

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    Laboratory Assessment

    Decreased hemoglobin and hematocrit levels

    Low platelet count

    Abnormal white blood cell count, may be low, normal orelevated, but is usually quite high

    Poorer prognosis: client with high white blood cell countat diagnosis

    (Continued)

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    Laboratory Assessment(Continued)

    Definitive test: examination of cells obtained frombone marrow aspiration and biopsy

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    Risk for Infections

    Infection is a major cause of death in the clientwith leukemia, and sepsis is a commoncomplication.

    Autocontamination

    Cross-contamination

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    Drug Therapy for Acute Leukemia

    Induction therapy

    Consolidation therapy

    Maintenance therapy

    New drug therapies

    Drug therapy for infection

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    Infection Protection

    Frequent handwashing

    Private room

    HEPA filtration or laminar airflow system

    Mask for visitor with upper respiratory infection(Continued)

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    Infection Protection (Continued)

    Minimal bacteria diet without uncooked foods

    Monitoring of daily laboratory results

    Assessment of vital signs

    Skin care, respiratory care

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    Bone Marrow Transplantation

    Standard treatment for leukemia

    Purges present marrow of the leukemic cells

    After conditioning, new, healthy marrow given tothe client toward a cure

    Sources of stem cells

    Conditioning regimen

    Transplantation

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    Risk for Injury

    Nadir: period of greatest bone marrow suppression Bleeding precautions Fatigue Interventions:

    Diet therapy Blood replacement therapy Drug therapy Energy conservation

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    Hodgkins Lymphoma

    Cancer that starts in a single lymph node or asingle chain of nodes

    Marker: Reed-Sternberg cell

    Large, painless lymph node usually in the neck;

    fever, malaise, night sweats One of the most curable cancers

    Treatment: external radiation alone or withcombination chemotherapy

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    Lymph nodes, such as these normal lymph nodes in the neck (left), are located

    throughout your body. Swollen lymph nodes in the neck (right) sometimes indicate the

    presence of Hodgkin's disease.

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    Non-Hodgkins Lymphoma

    All lymphoid cancers that do not have the Reed-Sternberg cell

    More than 12 types of non-Hodgkins lymphoma

    Low-grade lymphomas less responsive to

    treatment; cures are rare Treatment: radiation therapy and multiagent

    chemotherapy, or single-agent therapy withfludarabine

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    Multiple Myeloma

    White blood cell cancer that involves a more maturelymphocyte than either leukemia or lymphoma

    Uncommon cancer

    Manifestations: fatigue, easy bruising, bone pain,fractures, hypertension, increased infection,hypercalcemia, and fluid imbalance

    Treatment: chemotherapy

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    Autoimmune Thrombocytopenic Purpura

    Large ecchymosis or petechial rash on arms, legs,upper chest, and neck

    Diagnosed by decreased platelet count and largenumbers of megakaryocytes in the bone marrow

    (Continued)

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    Petechiae, a common sign of

    idio

    pathic throm

    bo

    cyto

    penicpurpura, occur when small blood

    vessels (capillaries) in the skin

    begin to bleed. They appear as

    tiny dots that range in color from

    red to bluish purple.

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    Autoimmune Thrombocytopenic Purpura (Continued)

    Interventions include: Therapy to prevent bleeding

    Drug therapy to suppress immune function

    Blood replacement therapy

    Splenectomy

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    Thrombotic Thrombocytopenic Purpura

    Rare disorder; platelets clump together abnormallyin the capillaries and too few platelets remain incirculation

    Inappropriate clotting, yet blood fails to clot

    properly when trauma occurs Plasma pheresis, infusion of FFP

    Aspirin, alprostadil, plicamycin

    Immunosuppressive therapy

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    Hemophilia

    Hemophilia A is deficiency of factor VIII andaccounts for 80% of cases.

    Hemophilia B (Christmas disease) is deficiency offactor IX and accounts for 20% of cases.

    For hemophilia Awith blood transfusion and factorVIII therapy, survival time has increased greatly.

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    Transfusion Therapy

    Pretransfusion responsibilities to prevent adversetransfusion reactions:

    Verify prescription.

    Test donors and recipients blood for compatibility.

    Examine blood bag for identification.

    Check expiration date.

    Inspect blood for discoloration, gas bubbles, or cloudiness.

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    Transfusion Responsibilities

    Provide client education.

    Assess vital signs.

    Begin transfusion slowly and stay with client first15 to 30 minutes.

    Ask client to report unusual sensations such aschills, shortness of breath, hives, or itching.

    Administer blood product per protocol.

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    Types of Transfusions

    Red blood cell

    Platelet transfusions

    Plasma transfusions: fresh frozen plasma

    Cryoprecipitate

    Granulocyte (white cell) transfusions

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    Transfusion Reactions

    Clients can develop any of the followingtransfusion reactions:

    Hemolytic

    Allergic

    Febrile Bacterial

    Circulatory overload

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    Autologous Blood Transfusion

    Collection and infusion of clients own blood

    Eliminates compatibility problems; reduces risk fortransmission of bloodborne disease

    Preoperative autologous blood donation

    (Continued)

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    Autologous Blood Transfusion (Continued)

    Acute normovolemic hemodilution

    Intraoperative autologous transfusion

    Postoperative blood salvage