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Chapter 23

Chapter 23. The Child With a Hematolgic Alteration. Review of the Hematologic System. Bone marrow is the site of red blood cell production Red blood cells are responsible for oxygen and carbon dioxide transport

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Chapter 23

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  1. Chapter 23 The Child With a Hematolgic Alteration

  2. Review of the Hematologic System • Bone marrow is the site of red blood cell production • Red blood cells are responsible for oxygen and carbon dioxide transport • Platelets are cell fragments that work to stop blood flow from an injury by adhering to the walls of blood vessels and forming platelet plugs

  3. Types of Leukocytes: Granulocytes • Neutrophil function: phagocytosis; seen in an acute inflammatory response and infectious disease • Eosinophils mediates allergic response, also have parasiticidal properties • Basophils seen in the healing process of inflammation and during conditions of chronic inflammation

  4. Types of Leukocytes: Agranulocytes • Lymphocytes function: Humoral immunity (B cell), cellular immunity (T cell) • Monocytes (macrophages) function: phagocytosis and antigen processing; is also ass w/chronic infections

  5. Anatomical and Physiological Differences in the Infant and Child • Early production of blood cells in the fetus occurs in the liver and spleen • After birth, the liver is the primary site for production of most of the blood clotting factors and prothrombin • In infants and young children, hematopoiesis occurs in almost every bone until age five (5) because all of the bone contains red marrow.

  6. Anatomical and Physiological Differences in the Infant and Child • After the age of five, hematopoiesis in the shafts of the long bones is reduced • As bone growth ceases near the end of adolescence (between 18-20), only the marrow of the ribs, sternum, vertebrae, skull, clavicles, scapulas and pelvis continue to produce blood cells

  7. Anatomical and Physiological Differences in the Infant and Child • RBC’s are formed through the process of erythropoiesis • The newborn has a higher level of erythropoietin which stimulates RBC production • Fetal hemoglobin (HgbF) is present up to the first 5 mos with adult Hgh steadily increasing through the first half of infancy

  8. Anatomical and Physiological Differences in the Infant and Child • The RBC count, Hgb and Hct levels are high in infants, but will stabilize in childhood • Platelet levels in newborns are lower than in older children and adults

  9. History • Frequent or easy bruising? • Frequent or heavy nose bleeds? • Recurrent or chronic infections? • Medications (prescription or OTC) that might impair hematolgic function • Past medical hx r/t hematologic dysfunction: illnesses, surgeries or injuries • Excessively heavy menstrual Flow?

  10. Physical Assessment • Inspection • Palpation • Auscultation

  11. Common Diagnostic Studies • CBC with differential • Direct and indirect Coombs’ test • Prothrombin time (PT) and Partial thromboplastin time • Bleeding time • Platelet agglutination/aggregation to evaluate platelet clumping ability • Hemoglobin electrophoresis • Serum Bone Marrow aspiration and biopsy

  12. Administration of Blood and Blood Products • Identify the child and verify blood with another nurse • Take v/s including blood pressure before administering blood, then q 15 minutes for the first 2 hrs, then q 30 minutes thereafter until infusion is complete

  13. Administration of Blood and Blood Products • Administer blood with normal saline on a piggyback setup, through an appropriate filter • Use blood within 30 minutes of its arrival form the blood bank • Check blood products-products that appear purplish or are bubbling should not be used because of risk of bacterial contamination • The rate of infusion of packed RBC’s is approximately 5 ml/kg/hr over not ore than 4 hours

  14. Administration of Blood and Blood Products • Run transfusion slowly x first 15 minutes; monitor closely for transfusion reaction during this time period • During administration of blood or blood products, the child and parents should be instructed to notify the nurse immediately if the child feels “bad”, or has fever or chills, headache, nausea, pain at he needle site, or difficulty breathing

  15. Administration of Blood and Blood Products • In neonates and small infants, auscultate the lungs before and frequently during a transfusion to detect signs of respiratory distress form fluid overload • If reaction is suspected, stop the transfusion immediately and infuse normal saline through new tubing, notify MD and continue to monitor v/s and hourly urine output • Send samples of child’s blood to lab

  16. Effects of Anemia • Cognitive development • Attention span • Concentration • Memory Learning ability • Behavior • Social interaction • Physical growth • Immunity to illness • Appetite • Muscle function • Manual dexterity

  17. Iron Deficiency Anemia Occurs as a result of • Blood loss • Poor nutritional intake • Rapid growth w/increased internal demands for blood production • Chronic blood loss • Chronic diarrhea • Parasitic GI problems • Females who have heavy menstrual periods

  18. Iron Deficiency Anemia Initial Clinical Manifestations • Pallor • Irritability • Fatigue Clinical Manifestations seen in prolonged anemia • Nail bed deformities • Tachycardia • Growth Retardation • Systolic heat murmur • Developmental delay

  19. Iron Deficiency Anemia Diagnostic Evaluation includes: • CBC • Reticulocyte Count • Serum Ferritin level; serum Iron Therapeutic Management • Oral Fe supplements may be ordered: ferrous sulfate is the form preferred as it is absorbed best

  20. Iron Deficiency Anemia Nursing Considerations: • Correct bleeding if it is the cause of anemia • Implement dietary modifications to provide a high Fe diet • Promote rest, protect from infection; monitor cardiac functioning • Transfusion if ordered • Protein and Vitamin C in order to produce new cells; folic acid to help convert the Fe from ferritin to hemoglobin • If Fe deficiency due to ingestion of lg quantities of milk at the expense of solid food intake, limit to 1 qt of milk/day

  21. Lead Poisoning in Children Symptoms of lead poisoning may include: • Anemia • Hyperirritability • Aggressive behavior • Decreased appetite and energy • Poor sleeping • Headaches • Constipation • Loss of recently acquired developmental skills (in young children) • Abdominal cramping

  22. Lead Poisoning in Children Sources of Lead • Lead based house paint • Soil • Water • Food • Parents work or hobbies Lead Exposure • Eating/Drinking • Breathing

  23. Lead Poisoning in Children Therapeutic Management for long-term exposure: • Isolation from or disposal of the lead source • Chelation therapy to remove lead from the body In cases where someone has potentially eaten toxic doses of lead in a short period of time, the following treatments may be administered: • Gastric lavage • Bowel irrigation with polyethylene glycol solution

  24. Lead Poisoning in Children Prevention of Lead Exposure • Find Sources of Lead • Remove Source of Lead • Good Handwashing

  25. Sickle Cell Anemia • Autosomal recessive condition • Normal hemoglobin partially or completely replaced by the sickle shaped, hemoglobin S (Hgb S) • Symptoms do not manifest until 4-6 months of age • Many children must undergo a splenectomy in early childhood, leading to severely compromised immunity

  26. Sickle Cell Anemia

  27. Sickle Cell Anemia Precipitating factors for sickle cell crisis include: • Increased Blood viscosity • Hypoxia • High altitudes • Hypoventilation • Vasoconstriction (when cold) • Emotional stress

  28. Sickle Cell Anemia • Vaso-occlusive crisis • Painful Episode • Acute Chest syndrome • Dactylitis • Priapism • CVA • Acute Sequestration crisis (Splenic Sequestration) • Aplastic Anemia

  29. Sickle Cell Anemia Therapeutic Management: • Opioids (Morphine; No Demerol) • NSAIDs • IV hydration • Oxygen (if patient is hypoxic) • Exchange Transfusions (erythrocytapheresis) • Hydroxyuria • Chelation therapy (for long term RBC transfusion therapy)

  30. Sickle Cell Anemia Nursing Considerations: • Hydration through IVF’s as ordered • Hydration through oral intake • Administration of O2 and blood products as ordered • Administer Antibiotics as ordered • Promote/Encourage rest • Assist child and family to avoid emotional stress as much as possible • Administer analgesics around the clock as ordered when child is in crisis; position for comfort and to decrease stress on painful joints (pain control) • Assess for signs and symptoms of worsening anemia, shock and altered neuro function • Provide emotional support during crisis

  31. Beta Thalassemia (Cooley Anemia) • Thalassemias are a group of hereditary blood disorders of hemoglobin synthesis characterized by mild to severe anemia • Autosomal recessive disorder • Causes the synthesis of defective hemoglobin; leads to anemia and hypoxia

  32. Beta Thalassemia (Cooley Anemia) Clinical Manifestations: • Anemia • Frequent epistaxis • Skeletal Changes • Facial deformities • Heart: CHF, myocardial fibrosis, murmurs • Liver/Gallbladder: hepatomegaly, hepatic insufficiency

  33. Beta Thalassemia (Cooley Anemia) Clinical Manifestations Cont: • Endocrine • Splenomegaly • Darkening of skin Therapeutic Management: • Erythrocyte Transfusion • Chelation therapy • Splenectomy

  34. Beta Thalassemia (Cooley Anemia) Nursing Considerations: • Adm. Blood product as ordered, observing for complications of multiple transfusions • Assess for signs of Fe overload and hepatitis • Observe for signs of infection • Adm. folic acid as ordered • Fx prevention by encouraging physical activities that do not increase risk • Iron chelation therapy as ordered

  35. Hemophilia • X linked recessive trait expressed almost exclusively as carrier females and affected males • Two (2) types: • Hemophilia A (classic hemophilia): factor VIII deficiency; most common type accounts for 80% of hemophilia cases • Hemophilia B (Christmas Disease): factor IX deficiency; accounts for 15% of all hemophilia cases

  36. Hemarthrosis and Joint Destruction in Hemophilia

  37. Hemophilia Clinical Manifestations: Range from mild to moderate or severe • Hemarthrosis - one of the most commonly seen symptoms • Significant deep tissue and intramuscular hemorrhages may occur • Extensive bleeding may be seen after circumcision, tooth extractions, lesser injuries, and minor surgical procedures • Hematuria • Ecchymosis • Epistaxis

  38. Hemophilia Diagnostic Evaluation: • Hx • Presenting symptoms • Lab work (PT, PTT, fibrinogen level, platelet count, quantitative immunoelectrophoretic assay, Factor VIII & IX assays) Therapeutic Management To prevent excessive bleeding & tissue damage through the administration of the missing or ineffective factors

  39. Hemophilia Nursing Considerations: • Control localized bleeding through topical coagulants, pressure, elevation and ice • Control bleeding of significant bleeds through administration of the necessary factor; monitor factor levels as ordered • Transfuse as ordered to help minimize disease complications • Provide opportunities for normal growth and devel activities; as indicated, provide injury protection for activities • PT to prevent flexion contractures and to strengthen muscles and joints when bleeding into joints has been controlled • DDAVP IV as ordered for mild hemophilia • No rectal temps • Analgesics as ordered for pain • RICE (Rest, Ice, Compression, Elevation) if joint involved in a bleeding episode to reduce damage

  40. Immune Thrombocytopenic Purpura (ITP) • Reduction in & destruction of platelets, despite normal platelet production in the bone marrow • Usually develops 1-3 weeks after a viral infection such as chicken pox measles or rubella Clinical manifestations: • Multiple ecchymosis • Petechiae

  41. Petechiae with Thrombocytopenic Purpura

  42. Immune Thrombocytopenic Purpura (ITP) Diagnostic Evaluation: • Thorough Hx • CBC Therapeutic Management: • Administration of IV or Oral corticosteroids and intravenous immunoglobulins • Splenectomy may be indicated if treatment ineffective

  43. Aplastic Anemia • Is a deficiency of the blood cells that results from failure of the bone marrow to produce adequate numbers of circulating blood cells • Can be congenital or acquired Clinical Manifestations: • Petechiae • Ecchymosis • Bleeding • Pallor • Epistaxis • Weakness • Tachycardia • Fatigue • Anorexia

  44. Aplastic Anemia Diagnostic Evaluation: • Hx of bruising immediately after an event that would not normally cause a bruise • CBC • Bone Marrow aspiration and biopsy confirms the diagnosis Therapeutic Management: • If acquired: exposure to the causative agent is discontinued immediately; then tx based on symptoms

  45. Aplastic Anemia Nursing Considerations: • Bleeding prevention • Administration and monitoring of blood transfusions as ordered • Infection prevention • Encouraging mobility as tolerated

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