Hematologic disorders (pedia)

• Hematologic disorders


  Often called blood dyscrasias
• Hematologic disorders


  • Most occur in the bone marrow where blood cells are formed
  • Occur when components of the blood are formed incorrectly or either increased or decreased in amount beyond normal ranges
• Blood components


  • Blood plasma
  • Erythrocytes (RBCs)
  • Leukocytes (WBCs)
  • Thrombocytes (Platelets)
• Blood plasma


  Liquid portion of blood that contains proteins, hormones, enzymes and electrolytes
• Erythrocytes (RBCs)


  Transport oxygen to and carry CO2 away from body cells
• Leukocytes (WBCs)


  Defend the body against antigen
• Thrombocytes (Platelets)


  Function is capillary hemostasis and coagulation
• Bone marrow aspiration and biopsy

  1. Samples of bone marrow determine the type and quantity of cells being produced

  2. Common bone marrow aspiration site is the iliac crest

  3. Child lays flat on stomach for the procedure; conscious sedation is used

  4. Large bore needle introduced thru tissue to the bone. A syringe is used to aspirate bone marrow

  5. Pressure must be applied to puncture site immediately to prevent bleeding. A pressure dressing is then applied

  6. Monitor VS and temp - watch for bleeding and infection
• Blood transfusion


  1. Commonly used to treat blood disorders

  2. The product must match child's blood type

  3. Must obtain a consent form

  4. Monitor VS every 15 mins for 1 hour, then every 30 mins

  5. Administer with isotonic fluids such as NS

  6. PRBC most common kind of transfusion used with children

  7. Watch for blood transfusion reactions
• Blood transfusion reactions


  H/A, chills, back pain, dyspnea, wheezing, hypotension, hives, increased pulse or temp - STOP INFUSION
• Hematopoietic stem cell transplantation

  1. Stem cell transplant is the IV infusion of stem cells from bone marrow obtained by marrow aspiration or from peripheral or umbilical cord blood drawn from a compatible donor

  2. Re-establish bone marrow function
• Types of stem cell transplants

  • Allogenic - transfer from immune compatible donor
  • Syngeneric - Genetically identical - twin
  • Autologous - Child's own stem cells
• Stem cell transplants


  • Radiation and chemo given prior to transplant to prevent rejection
• Nursing responsibilities during stem cell transfusion

  1. Monitor cardiac rate and rhythm to detect circulatory overload and pulmonary embolism

  2. Monitor for fever, chills

  3. Administer Tylenol and Benadryl for these side effects
• Post stem cell transfer

  1. Take temp 1 hour then every 4 hours to detect infection

  2. Reinforce strict handwashing, limit diet to cooked foods to reduce exposure to bacteria
• Splenectomy


  • Spleen removes damaged or aged blood cells
  • With certain disorders such as sickle cell anemia, the spleen destroys the abnormal RBCs which causes children to have continuous anemia
• After splenectomy


  • More susceptible to thrombophlebitis and pneumonia
  • Will need oral penicillin prophylactically for 1-2 years
  • Administer flu, pneumonia and meningococcal vaccines to boost immunity
  • Teach family s/s of infection - cough, fever, sore throat, malaise, report symptoms ASAP
• Acute blood loss anemia

  • Blood loss sufficient enough to cause anemia can occur from trauma such as a car accident
  • Sudden blood loss = pale, tachycardia, tachypnea. Treat underlying cause
• Anemia of acute infection

  Can cause destruction or decreased production of erythrocytes (osteomyelitis, ulcerative colitis). Treat the underlying issue
• Anemia of renal disease

  Decreased erythropoietin. Epogen is given to increase RBC production, but will not fix the renal disease
• Congenital aplastic anemia


  Inherited disorder where a child presents with pancytopenia (reduction of all blood cell components)
• Acquired aplastic anemia


  • Decrease in bone marrow production
  • Child was exposed to excessive radiation/drugs or chemicals (insecticides)
• Assessment of aplastic anemia

  • Child will appear pale, easily fatigued, anorexic, bruises easily, petechiae, nosebleeds, GI bleeding
• Treatment of aplastic anemia

  Remove child from contact with radiation or chemical sources. D/C any drug causing anemia. Ultimate therapy - stem cell transplant
• Iron deficiency anemia


  Hgb production is inadequate, erythrocytes appear pale and reduced in diameter
• Assessment of iron deficiency anemia

  • Pale mucous membranes, enlarged heart, enlarged spleen, poor muscle tone, decreased activity
• Causes of iron deficiency anemia in newborns

  • Born prematurely or mom had an iron deficiency during pregnancy
  • Breastfed and/or an iron fortified formula. Introduce iron fortified cereal as a first food
• Causes of iron deficiency anemia in older children

  • GI tract lesions such as polyps or ulcerative colitis
  • Parasite infestation
  • Frequent epistaxis
  • Heavy bleeding in adolescent females
• Pica


  Ingesting ice, paint, chalk
• Lab values for iron deficiency anemia

  • Hgb less than 11 and Hct less than 33
• Management of iron deficiency anemia

  1. Ferrous sulfate

  2. Diet rich in iron and vitamin C

  3. When giving iron give on empty stomach with OJ or vitamin C

  4. Brush teeth thoroughly - iron will stain

  5. Use straws if liquid prep

  6. Iron will turn stools black

  7. At risk for constipation - eat raw fruit or increase water - high fiber
• Pernicious anemia (Vitamin B12 deficiency)


  B12 is necessary for maturation of RBCs. With pernicious anemia, there is a deficiency or inability to use the vitamin
• Intrinsic factor


  Must be present in gastric mucosa to absorb vitamin B12
• Symptoms of pernicious anemia in infants

  • Pale, anorexic, irritable, chronic diarrhea, tongue and smooth and beefy red
• Treatment of pernicious anemia

  1. If caused by diet, temporary B12 shots

  2. If there is a lack of intrinsic factor - lifelong monthly B12 injections
• Sickle cell anemia


  • Inherited disorder more common in African-Americans
  • RBCs have an elongated and crescent shape
  • Low blood pH and increased blood viscosity are present (occurs from dehydration and hypoxia)
  • Blood cannot move freely thru vessels, which can cause blockage resulting in acute pain and cell destruction
• Symptoms of sickle cell anemia

  • Not common in newborns, they start when infant is 6-12 months old
  • Fever, anemia, painful/swollen hands and feet
• Sickle cell crisis


  Sudden, severe onset of sickling. Pooling of too many new sickled cells in blood vessels causes tissue hypoxia
• Precipitating factors of sickle cell crisis

  • Fever
  • GI illness
  • Respiratory infection
  • Dehydration
  • Emotional/physical stress
  • Strenuous exercise
  • No obvious cause at all
• Treatment of sickle cell crisis

  1. H = hydration - IV fluids

  2. O = oxygen

  3. P = pain management - Tylenol. If something strong is needed, Morphine may be given