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Hemolytic anemia: Sickle Cell Hemolytic anemia: Sickle Cell anemia (SSA) anemia (SSA) Congenital hemolytic anemia resulting Congenital hemolytic anemia resulting from a defective Hb molecule (HbS) from a defective Hb molecule (HbS) that causes RBCs to become sickle- that causes RBCs to become sickle- shaped. shaped. Eight percent of African American are Eight percent of African American are heterozygous (carriers) for SSA heterozygous (carriers) for SSA thereby inheriting one affected gene thereby inheriting one affected gene or the sickle cell trait. or the sickle cell trait. One percent of Africa American are One percent of Africa American are homozygous (identical genes) for the homozygous (identical genes) for the disorder, thereby inheriting a disorder, thereby inheriting a defective gene form both parent or SSA defective gene form both parent or SSA Sickle cell trait (heterozygous) is a Sickle cell trait (heterozygous) is a generally mild condition that produce generally mild condition that produce few, if any manifestation few, if any manifestation

Hemolytic Anemia PPT

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  • Hemolytic anemia: Sickle Cell anemia (SSA) Congenital hemolytic anemia resulting from a defective Hb molecule (HbS) that causes RBCs to become sickle-shaped.Eight percent of African American are heterozygous (carriers) for SSA thereby inheriting one affected gene or the sickle cell trait.One percent of Africa American are homozygous (identical genes) for the disorder, thereby inheriting a defective gene form both parent or SSASickle cell trait (heterozygous) is a generally mild condition that produce few, if any manifestation

  • ContSSA is caused by an autosomal defect( one gene is affected) that results in the synthesis of hemoglobin S. Produced by a mutation in the beta chain of the hemoglobin molecule through a substitution of the amino acid valine for glutamine in both beta chainsDuring decreased oxygen tension in the plasma, the hemoglobin S causes the RBCs to elongate, become rigid, and assume a crescent, sickled shape causing the cells to clump together, obstruct capillary blood flow causing ischemia and possible tissue infraction With normal oxygenation , the sickled RBCs resume their normal shape; repeated episodes of sickling and unsickling weaken the cell membrane, causing them to hemolyze and be removed.

  • Sickled Cell anemia

  • Cont..Crisis is usually painful and can last from 4 to 6 days. Clients are always anemic Hb 7 to 10g/dl Crisis can occur in different forms.3. Sickle cell crisis: results from tissue hypoxia and necrosis due to inadequate blood flow to a specific region or organ

    2. Aplastic crisis: results from a virus. The HB drops so rapidly that the bone marrow can not compensate.

    3. Sequestration crisis: rare seen in infants ages 8 months to 2 years. Results when other organ pool the sickled cells. By age 10 children have had a splenic infarction and the spleen is no longer functional.

  • Conditions likely to trigger a sickle cell crisishypoxiaLow environmental and or body tempExcessive exerciseHigh altitudesInadequate oxygen during anesthesia InfectionStressDehydration

    .

  • Assessment findings: Sickle Cell AnemiaAching bonesChronic fatigueFrequent infectionJaundice or pallor joint swellingLeg ulcers (esp ankles)Severe localized and generalized painTachycardiaDyspneaPriapism (Unexplained, painful erection)

    Sickle cell crisis (general S&S)HematuriaIrritabilityLethargyPale lips, tongue, and nail bedsSever pain

  • Assessment findings contPainful crisisDark crisisLow grade feverSevere abdominal, thoracic, muscle or bone painTissue anoxia and necrosisWorsening jaundice

  • Assessment findings contAplastic anemiaDyspneaLethargy and sleepinessMarked decreased in bone marrow activityPallorPossible comaRBC hemolysisAcute sequestrationHypovolemic shockLethargyLiver congestionPallorWorsened chronic jaundice

  • Diagnostic and lab testHematologic studies: RBC, WBC plateletsHb electrophoresis:Hb levels:Lateral CXR:

  • Medical management Activity:Nutritional supplements:MonitoringLabs studies:IV therapyTreatments:Blood transfusionsAnalgesics:Analgescis and antipyretic:Antineoplastic:Oxygen therapyPosition for comfortAntibiotics:Genetic counseling

  • Nursing managementActivity:Nutritional supplements:MonitoringLabs studies:IV therapyTreatments:Blood transfusionsMeds Oxygen therapyPosition for comfortAntibiotics:education

  • Complications RetinopathyNephropathyCerebral vessel occlusionsHypovolemic shockNecrosis infection and gangreneDeath

  • Nursing diagnosis

    Acute PainIneffective tissue perfusionImpaired gas exchangeRisk for infectionDeficient knowledgepowerlessness

  • Evaluation Client verbalize pain reliefClient does not develop an infectionClient adheres t vaccination scheduleClient identifies precipitation factors leading to crisis state

  • PolycythemiaRefers to an circulating RBCs and concentration of HB in the blood It is a term used when the HCT is > 55% in males and > 50% in females.Also known as Polycythemia Vera, PV, or myeloproliferative red cell disorder; it can be primary or secondary.

  • Polycythemia: Primary Common in men of European Jewish Neoplastic stem cell disorder characterized by production of RBC, granulocytes and plateletsWith the overproduction of RBC, increased viscosity results in congestion of blood in tissues, the liver and spleenThrombi form, acidosis develops, and tissue infarction occurs as a result of the diminished circulatory flow of blood caused by the increase viscosity

  • Polycythemia: Secondary

    Most common formThe disturbance is not in the development of RBC but in the abnormal increase of erythropoietin, causing excessive erythropoiesis The increase in RBC production caused by increased erythropoietin release is a physiologic response to hypoxia; hypoxia stimulates the release of erythropoietin in the kidneyChronic hypoxia states may be produced by prolonged exposure to high altitudes, pulmonary diseases, hypoventilation and smokingThe results of an increased RBC production include increased viscosity of blood, which alters circulatory flow

  • Assessment Findings Ruddy completionDusky mucosaVertigoHeadachesDizzinessDyspnea and OrthopneaTachycardiaEcchymosisHepatomegaly and splenomegalyIncreased gastric secretionWeakness and fatiguePruritusExpistaxisGI bleedingAngina

  • Diagnostic finding hemoglobin and erythrocyte countWBC plateletsuric acidhistamine

  • Therapeutic management Management of the underlying condition causing the hypoxiaRepeated phlebotomy to decrease blood volume; the goal is to keep the HCT less that 45 to 48%Hydration to decrease blood viscosity

  • Planning and Implementation Assist in phlebotomyMeasures to relieve pruritus including cool and tepid bathAccurate monitor of Intake and outputNursing measures to prevent thrombotic events.Administration of medication to prevent complications

  • Medication therapy Mylosuppressant agent to inhibit bone marrow activity( hydroyurea, melphalan, redioactive phosphorus)Allopurinal and colchicineAntiplatelet agents AnalgesicHistamine antagonistsAntihistamine

  • Client education Good hydration about 3L/dayDisease processSigns and symptom of complication Prevention of bleeding Emphasize the importance of check upTeach client to avoid products that contain ironDiscuss ways to prevent thrombosis

  • Evaluation The HCT is within normal rangeThe client does not develop complication associated wit thrombusThe clients maintain adequate hydration

  • Neutropenia Leukopenia is condition of fewer WBCs than normal, results from neutropenia or lymphopeniaNeutropenia results from decreased production or increased destruction of neutrophils < 2,000/mmIs not a disease but a syndromeIt may occur as a primary Hematologic disorder but may also be caused by drugs, infections, an other medical condition such as sepsis and nutritional deficienciesIf the leukocyte count is decreased, or if immature WBC predominate in the circulation, the phagocytic function of these cells is impaired susceptibility of infections

  • Assessment There is no real symptoms associated with Neutropenia until the patient becomes infected. Routine CBC with diff such as those obtain after chemotherapy can be reveal before the onset of infection

  • Therapeutic managementTreatment depends on its causeIf the etiology is drug induced, D/C meds if possibleCorticosteroids are used if the etiology is immunologic If client develops a fever, identification and treatment of he infections is instituted

  • Planning and Implementation Monitor for signs of infectionObtain culturesAdminister antibioticsAdminister meds that stimulate the production of neutrophils( neupogen or Leukine)Enforce strict hand washing by all individuals in contact with the clientInstitute reverse isolationAvoid invasive proceduresNeutropenia precautions

  • Client education Teach client and family to report signs and symptomsTeach strict hand washingTeach clients methods to maintain good personal hygieneExplain condition and the rationale for therapeutic intervention

  • Evaluation Neutrophil count is normalizedThe client is free of infectionThe client and family verbalize methods of limiting exposure to pathogens

  • Lymphomas: Hodgkin's disease Lymphomas is a group if malignant neoplasms that affects the lymphatics system resulting in the proliferation of lymphocytes; lymphomas can be classified as Hodgkin's and non Hodgkin's lymphomas Neoplastic cells of lymphoid origin

  • Hodgkins disease More common in men and has two peaks;15 -35yrs of age and 55-75yrs; incidence is higher in whites than in African AmericanThe cause of the disease is unknown although several factors include infection with Epstein-Barr virus, familial pattern, and exposure to toxinsIs characterized by the presence of Reed-Sternberg cells that proliferate in a single lymph and travel continuously through the lymphatic to other lymph nodes and organsThe tumor originates in a lymph node (in majority of cases from the cervical nodes) and infiltrates the spleen, lungs and liver

  • Non-Hodgkins lymphoma Most common form of lymphoma affects usually adults from 50 to 70 years old. It is more common in men than women and in whitesThere is no know cause but the incidence of non-Hodgkins lymphomas is linked to viral infections, immune disorders, genetic abnormalities, exposure to chemicals, and infection with Helicobacter pylori Non-Hodgkin's lymphomas has a similar patho to Hodgkins disease, also Reed-Sternberg cells are absent and the method of lymph node infiltration is differentIn the majority of cases, the disease involves malignant B cells; the lymphoma usually originates outside the lymph nodes; the lymphoid tissues involved become infiltrated with malignant cells; the cells that make up the lymphoid tissue become abnormal and crowd out normal cells.

  • Clinical manifestation Hodgkins diseaseUsually begins with a firm and painless enlargement of one or more lymph nodes on one side of the neckFatigueWeaknessAnorexiaDysphagiaDyspneaPruritusCoughJaundiceAbnormalBone pain Development of severe but brief pain at the site of Hodgkin's after ingestion of alcoholEnlarged lymph nodes, liver and spleenB symptoms: fever without chills, night sweats, weight loss unintentionalNon-HodgkinsPainless lymph node enlargementB symptomsAbdominal pain, nausea, vomitingHematuriaPeripheral neuropathy, cranial nerves, HA, visual disturbances, changes in mental status and seizures.

  • Diagnostic findings Hodgkins diseaseNormocytic, normochromic anemiaPresence of Reed-Sternberg cells in bone BxMediastinal lymphadenopathy revealed by CXR, CT scan Mediastinal mass and pulmonary infiltrates on CXR

    Non-Hodgkins lymphoma Lymphocytopenia X-ray ma reveal pulmonary infiltratesLymph node Bx help to identify the cell type and pattern

  • Therapeutic management Hodgkins diseaseLymphangiographyStaging laparotomyRadiation therapyChemotherapy

    Non-hodgkins Staging of the disease from CT scan and bone marrowCombination chemotherapyRadiation therapyInterferon, interleukins and tumor necrosis factor

  • Planning and intervention Assist in balancing activity with rest periodsMaintain good nutritional stateProvide measures to decrease the discomfort associated with pruritusInstitute intervention for clients on chemotherapy or radiationProvide intervention to enable client to deal with body images changes refer to cancer society

  • Client education discuss with client and family the nature of he disease, the course of the therapyTeach about the medication prescribedTeach about signs and symptoms that require immediate medical intervention

  • Evaluation The client does not develop complication of bleeding of infectionThe client regains normal weightThe client verbalized absence of painThe client and family verbalize understanding of reasons of ongoing treatment and interventions.

  • Reading Thalassemia ThrombocytopeniaDICLeukemia