Showing posts with label hematologic disorder. Show all posts
Showing posts with label hematologic disorder. Show all posts

DISORDERS OF THE HEMATOLOGIC SYSTEM

Thursday, May 22, 2008 | Labels: | 0 Comments  

Idiopathic Thrombocytopenic Purpura



  1. General information
    1. Increased destruction of platelets with resultant platelet count of less than 100,000/mm3 characterized by petechiae and ecchymoses of the skin
    2. Exact cause unknown; may be an autoimmune mechanism; onset sudden, often preceded by a viral illness
    3. The spleen is the site for destruction of platelets; spleen is not enlarged
  2. Medical management
    1. Drug therapy: steroids and immunosuppressive agents
    2. Platelet transfusion
    3. Surgery: splenectomy
  3. Assessment findings
    1. Petechiae: spider-web appearance of bleeding under skin due to small size of platelets
    2. Ecchymosis
    3. Blood in any body secretions, bleeding from mucous membranes, nosebleeds
    4. Diagnostic tests: platelet count decreases, anemia
  4. Nursing interventions
    1. Control bleeding
      1. Administer platelet transfusions as ordered.
      2. Apply pressure to bleeding sites as needed.
      3. Position bleeding part above heart level if possible.
    2. Prevent bruising.
    3. Provide support to client and be sensitive to change in body image.
    4. Protect from infection.
    5. Measure normal circumference of extremities for baseline.
    6. Administer medications orally, rectally, or IV, rather than IM; if administering immunizations, give subcutaneously (SC) and hold pressure on site for 5 minutes.
    7. Administer analgesics (acetaminophen) as ordered; avoid aspirin.
    8. Provide care for the client with a splenectomy (see Splenectomy, in Unit 4).
    9. Provide client teaching and discharge planning concerning
      1. Pad crib and playpen, use rugs wherever possible.
      2. Provide soft toys.
      3. Sew pads in knees and elbows of clothing.
      4. Provide protective headgear during toddlerhood.
      5. Use soft Toothettes instead of bristle toothbrushes.
      6. Keep weight to low normal to decrease extra stress on joints.
      7. Use stool softeners to prevent straining.
      8. Avoid contact sports; suggest swimming, biking, golf, pool.

Tuesday, May 20, 2008 | Labels: | 0 Comments  

Hemophilia



  1. General information
    1. A group of bleeding disorders where there is a deficit of one of several factors in clotting mechanism
    2. Sex-linked, inherited disorder; classic form affects males only
    3. Types
      1. Hemophilia A: factor VIII deficiency (75% of all hemophilia)
      2. Hemophilia B (Christmas disease): factor IX deficiency (10%-12% of all hemophilia)
      3. Hemophilia C: factor XI deficiency (autosomal recessive, affects both sexes)
    4. Only the intrinsic system is involved; platelets are not affected, but fibrin clot does not always form; bleeding from minor cuts may be stopped by platelets.
    5. If individual has less than 20%-30% of factor VIII or IX, there is an impairment of clotting and clot is jelly-like.
    6. Bleeding in neck, mouth, and thorax requires immediate professional care.
  2. Assessment findings
    1. Prolonged bleeding after minor injury
      1. At birth after cutting of cord
      2. Following circumcision
      3. Following IM immunizations
      4. Following loss of baby teeth
      5. Increased bruising as child learns to crawl and walk
    2. Bruising and hematomas but no petechiae
    3. Peripheral neuropathies (due to bleeding near peripheral nerves): pain, paresthesias, muscle atrophy
    4. Hemarthrosis
      1. Repeated bleeding into a joint results in a swollen and painful joint with limited mobility
      2. May result in contractures and possible degeneration of joint
      3. Knees, ankles, elbows, wrists most often affected
    5. Diagnostic tests
      1. Platelet count normal
      2. Prolonged coagulation time: PTT increased
      3. Anemia
  3. Nursing interventions
    1. Control acute bleeding episode.
      1. Apply ice compress for vasoconstriction.
      2. Immobilize area to prevent clots from being dislodged.
      3. Elevate affected extremity above heart level.
      4. Provide manual pressure or pressure dressing for 15 minutes; do not keep lifting dressing to check for bleeding status.
      5. Maintain calm environment to decrease pulse.
      6. Avoid sutures, cauterization, aspirin: all exacerbate bleeding.
      7. Administer hemostatic agents as ordered.
        1. fibrin foam
        2. topical application of adrenalin/epinephrine to promote vasoconstriction
    2. Provide care for hemarthrosis.
      1. Immobilize joint and control acute bleeding.
      2. Elevate joint in a slightly flexed position.
      3. Avoid excessive handling of joint.
      4. Administer analgesics as ordered; pain relief will minimize increases in pulse rate and blood loss.
      5. Aspirin should not be given because it inhibits platelet function.
      6. Instruct to avoid weight bearing for 48 hours after bleeding episode if bleeding is in lower extremities.
      7. Provide active or passive ROM exercises after bleeding has been controlled (48 hours), as long as exercises do not cause pain or irritate trauma site.
    3. Administer cryoprecipitate (frozen factor VIII) as ordered.
      1. Thaw slowly.
      2. Gently rotate bottle; shaking deteriorates antihemophilic factor.
      3. Infuse immediately when thawed; factor VIII deteriorates at room temperature.
    4. Provide client teaching and discharge planning concerning
      1. Prevention of trauma (see Idiopathic Thrombocytopenic Purpura)
      2. Genetic counseling
        1. when mother is carrier: 50% chance with each pregnancy for sons to have hemophilia, 50% chance with each pregnancy for daughters to be carriers
        2. when father has hemophilia, mother is normal: no chance for children to have disease, but all daughters will be carriers
      3. Availability of support/counseling agencies

Tuesday, May 20, 2008 | Labels: | 0 Comments  

Disseminated Intravascular Coagulation (DIC)

  1. General information
    1. Diffuse fibrin deposition within arterioles and capillaries with widespread coagulation all over the body and subsequent depletion of clotting factors.
    2. Hemorrhage from kidneys, brain, adrenals, heart, and other organs.
    3. Cause unknown
    4. Clients are usually critically ill with an obstetric, surgical, hemolytic, or neoplastic disease.
    5. May be linked with entry of thromboplastic substances into the blood.
    6. Pathophysiology
      1. Underlying disease (e.g., toxemia of pregnancy, cancer) causes release of thromboplastic substances that promote the deposition of fibrin throughout the microcirculation.
      2. Microthrombi form in many organs, causing microinfarcts and tissue necrosis.
      3. RBCs are trapped in fibrin strands and are hemolysed.
      4. Platelets, prothrombin, and other clotting factors are destroyed, leading to bleeding.
      5. Excessive clotting activates the fibrinolytic system, which inhibits platelet function, causing further bleeding.
    7. Mortality rate is high, usually because underlying disease cannot be corrected.
  2. Medical management
    1. Identification and control of underlying disease is key
    2. Blood transfusions: include whole blood, packed RBCs, platelets, plasma, cryoprecipitates, and volume expanders
    3. Heparin administration
      1. Somewhat controversial
      2. Inhibits thrombin thus preventing further clot formation, allowing coagulation factors to accumulate
  3. Assessment findings
    1. Petechiae and ecchymoses on the skin, mucous membranes, heart, lungs, and other organs
    2. Prolonged bleeding from breaks in the skin (e.g., IV or venipuncture sites)
    3. Severe and uncontrollable hemorrhage during childbirth or surgical procedures
    4. Oliguria and acute renal failure
    5. Convulsions, coma, death
    6. Laboratory findings
      1. PT prolonged
      2. PTT usually prolonged
      3. Thrombin time usually prolonged
      4. Fibrinogen level usually depressed
      5. Platelet count usually depressed
      6. Fibrin split products elevated
      7. Protamine sulfate test strongly positive
      8. Factor assays (II, V, VII) depressed
  4. Nursing interventions
    1. Monitor blood loss and attempt to quantify.
    2. Observe for signs of additional bleeding or thrombus formation.
    3. Monitor appropriate laboratory data.
    4. Prevent further injury.
      1. Avoid IM injections.
      2. Apply pressure to bleeding sites.
      3. Turn and position client frequently and gently.
      4. Provide frequent nontraumatic mouth care (e.g., soft toothbrush or gauze sponge).
    5. Provide emotional support to client and significant others.
    6. Administer blood transfusions and medications as ordered.
    7. Teach client the importance of avoiding aspirin or aspirin-containing compounds.

Tuesday, May 20, 2008 | Labels: | 0 Comments  

Sickle-Cell Anemia


  1. General information (see Figure 5.2 below)
    1. Most common inherited disorder in US African American population; sickle cell trait found in 10% of African Americans
    2. Autosomal recessive inheritance pattern
    3. Individuals who are homozygous for the sickle cell gene have the disease (more than 80% of their hemoglobin is abnormal [HgbS]).
    4. Those who are heterozygous for the gene have sickle cell trait (normal hemoglobin predominates, may have 25%-50% HgbS). Although sickle cell trait is not a disease, carriers may exhibit symptoms under periods of severe anoxia or dehydration.
    5. In this disease, the structure of hemoglobin is changed; the sixth rung of the beta chain changes glutamine for valine.
    6. HgbS (abnormal Hgb), which has reduced oxygen-carrying capacity, replaces all or part of the hemoglobin in the RBCs.
    7. When oxygen is released, the shape of the RBCs changes from round and pliable to crescent shaped, rigid, and inflexible.
    8. Local hypoxia and continued sickling lead to plugging of vessels.
    9. Sickled RBCs live for 6-20 days instead of 120, causing hemolytic anemia.
    10. Usually no symptoms prior to age 6 months; presence of increased level of fetal hemoglobin tends to inhibit sickling.
    11. Death often occurs in early adulthood due to occlusion or infection.
    12. Sickle cell crisis
      1. Vaso-occlusive (thrombocytic) crisis: most common type
        1. crescent-shaped RBCs clump together; agglutination causes blockage of small blood vessels.
        2. blockage causes the blood viscosity to increase, producing sludging and resulting in further hypoxia and increased sickling.
      2. Splenic sequestration: often seen in toddler/preschooler
        1. sickled cells block outflow tract resulting in sudden and massive collection of sickled cells in spleen.
        2. blockage leads to hypovolemia and severe decrease in hemoglobin and blood pressure, leading to shock.
  2. Medical management: sickle cell crisis
    1. Drug therapy
      1. Urea: interferes with hydrophobic bonds of the HgbS molecules
      2. Analgesics/narcotics to control pain
      3. Antibiotics to control infection
    2. Exchange transfusions
    3. Hydration: oral and IV
    4. Bed rest
    5. Surgery: splenectomy
  3. Assessment findings
    1. First sign in infancy may be "colic" due to abdominal pain (abdominal infarct)
    2. Infants may have dactylitis (hand-foot syndrome): symmetrical painful soft tissue swelling of hands and feet in absence of trauma (aseptic, self-limiting)
    3. Splenomegaly: initially due to hemolysis and phagocytosis; later due to fibrosis from repeated infarct to spleen
    4. Weak bones or spinal defects due to hyperplasia of marrow and osteoporosis
    5. Frequent infections, especially with H. influenzae and D. pneumoniae
    6. Leg ulcers, especially in adolescents, due to blockage of blood supply to skin of legs
    7. Delayed growth and development, especially delay in sexual development
    8. CVA/infarct in the CNS
    9. Renal failure: difficulty concentrating urine due to infarcts; enuresis
    10. Heart failure due to hemosiderosis
    11. Priapism: may result in impotence
    12. Pain wherever vaso-occlusive crisis occurs
    13. Development of collateral circulation
    14. Diagnostic tests
      1. Hgb indicates anemia, usually 6-9 g/dl
      2. Sickling tests
        1. sickle cell test: deoxygenation of a drop of blood on a slide with a cover slip; takes several hours for results to be read; false negatives for the trait possible.
        2. Sickledex: a drop of blood from a finger stick is mixed with a solution; mixture turns cloudy in presence of HgbS; results available within a few minutes; false negatives in anemia clients or young infants possible.
      3. Hgb electrophoresis: diagnostic for the disease and the trait; provides accurate, fast results.
  4. Nursing interventions: sickle cell crisis
    1. Keep child well hydrated and oxygenated.
    2. Avoid tight clothing that could impair circulation.
    3. Keep wounds clean and dry.
    4. Provide bed rest to decrease energy expenditure and oxygen use.
    5. Correct metabolic acidosis.
    6. Administer medications as ordered.
      1. Analgesics: acetaminophen, meperidine, morphine (avoid aspirin as it enhances acidosis, which promotes sickling)
      2. Avoid anticoagulants (sludging is not due to clotting)
      3. Antibiotics
    7. Administer blood transfusions as ordered.
    8. Keep arms and legs from becoming cold.
    9. Decrease emotional stress.
    10. Provide good skin care, especially to legs.
    11. Test siblings for presence of sickle cell trait/disease.
    12. Provide client teaching and discharge planning concerning
      1. Pre-op teaching for splenectomy if needed
      2. Genetic counseling
      3. Need to avoid activities that interfere with oxygenation, such as mountain climbing, flying in unpressurized planes

Tuesday, May 20, 2008 | Labels: | 0 Comments  

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