Hematologic Disorders
Epistaxis: Short, isolated episodes are normal and nonemergent although they cause stress
• Risk Factors
o Trauma (nose picking)
o Low humidity, allergies
o Can be the result of underlying diseases like von Willebrand, hemophilia
• Nursing Care
o Maintain calmness with child and family
o Place sitting up with head tilted forwards slightly to prevent aspiration of blood
o Apply pressure for at least 10 mins
o Can use ice if preferred
Iron Deficiency Anemia
• Risk Factors
o Premature birth: decreased iron stores
o Adolescents d/t poor diet, rapid growth, obesity
o Excessive intake of cow's milk in toddlers
o Infants: GERD, pyloric stenosis
o Older child: GI polyps, colitis
o Adolescents: Menorrhagia
• Assessment
o Tachycardia
o Pallor
o Brittle, spoon-shaped fingernails
o Systolic heart murmur
o Cravings for non-nutritive things (ice, dirt, paper)
• Lab Tests
o CBC: Decreased RBC, Hgb, Hct
o Stool sample: Guaiac test
o Treatment can take up to 3 months
o Will need to monitor the child’s blood count routinely
• Medications
o Iron supplements: 1 hr before and 2 hrs after meals, milk, and antacids
o Watch for GI upset
o If giving liquid iron, use the dropper, place it directly in the back of the mouth, can mix it with formula
(not milk), juice, or cereal
o Give vitamin C to help with the absorption
o Watch for tarry stools and constipation
o IV ferrous sulfate: in severe anemia
o Very painful
• Nursing Care
o Iron supplements for preterm and low-birth-weight infants by 2 mos old
o Iron supplements to full-term infants at 4-6 mos
o Iron-fortified formula
o Include high-iron foods (iron-fortified cereals, dried beans, red meat, potatoes, peas, chicken) and
vitaminC
Sickle Cell Anemia
• Risk Factors
o An autosomal recessive genetic disorder
, o African American people
o Children who have the trait without manifestations of the disease can pass it to their children
• Assessment
o Family history
o Pain when in crisis
o Shortness of breath, fatigue
o Pallor
o Jaundice
o Vaso-occlusive crisis (painful)
▪ Acute: r/t dehydration and decreased oxygen. Pain, swollen joints, hematuria, jaundice
▪ Chronic: Increased risk of resp inf and osteomyelitis, retinal detachment and blindness, murmurs,
seizures
• Assessment cont.
o Sequestration
o Excessive pooling of blood primarily in the spleen (splenomegaly) and sometimes in the liver
(hepatomegaly)
o Shock d/t decreased blood volume
o Aplastic Crisis
▪ Extreme anemia d/t temporary decreased RBC production
▪ Triggered by a viral infection
o Hyper hemolytic Crisis
▪ Decreased rate of RBC destruction leading to anemia, jaundice, and/or reticulocytotic
• Laboratory Tests: Screening for newborns is mandatory nationwide
o CBC: detect anemia
o Hgb electrophoresis
o Sickle Cell Crisis:
▪ Hbg decreased, WBC, bili & reticulocyte elevated, Peripheral blood smear reveals sickled cells
• Diagnostic Procedures
o Transcranial Doppler Test (TCD)
▪ Assesses intracranial vascular flow and detects the risk for CVA
▪ Done annually for kids 2-16 yrs with sickle cell disease
• Nursing Care
o Decrease oxygen consumption
o Administer blood products
o Give oral prophylactic penicillin
• Nursing Care continued Pain Mgmt
o Treat mild pain with acetaminophen or ibuprofen. Manage severe pain with opioids.
o Comfort measures
• Medications
o Opioids: Administer on a schedule to prevent pain
o Antineoplastics: can cause anorexia
• Therapeutic Procedures
o Exchange Transfusion
o Hematopoietic Stem Cell Transplant
• Client Education
o Emotional support
o Promote rest and adequate hydration
o Wear a medical ID bracelet
o Avoid contact sports
• Complications
, o Stroke: d/t blood vessel occlusion
o Blood transfusions are usually done monthly to prevent a repeat stroke.
• Acute chest syndrome:
o Can be life-threatening
o Common in adolescents
o Increased risk of developing pneumonia d/t decreased oxygen to lung tissue
o Assess: chest, back, or abd pain, fever of 101.3 or higher, couch, tachypnea, decreased oxygen saturation
o Increased risk of infection
• Kidney Scarring
• Priapism (persistent and painful erection)
Hemophilia
• A group of bleeding disorders characterized by difficulty in controlling bleeding. Deficiencies in the clotting
factors
• Sometimes recognized during infancy following circumcision or later in childhood after injuries occur
• Varies in severity based on the percentage of clotting factor a child’s body contains
• For example, a child with mild hemophilia can have up to 40% of normal factor VIII but a child with
severehemophilia has very little factor VIII.
• Hemophilia A & B are X-linked recessive disorders.
• Types of Hemophilia
o Hemophilia A: Deficiency of factor VIII, “classic” hemophilia, 80% of cases of hemophilia
o Hemophilia B: Deficiency of factor IX, “Christmas disease”
o Von Willebrand: Inherited lack of the von Willebrand protein, inability of the platelets to aggregate
• Assessment
o Bleeding, joint pain and stiffness, impaired mobility, activity intolerance
• Lab Tests
o Prolonged partial thromboplastin time (aPTT)
o Facto-specific assays (tests)
o Platelets and prothrombin time WNL
o Whole blood clotting time can be WNL or prolonged
• Diagnostic Procedures
o DNA testing
• Nursing Care
o Management of bleeding,
o Avoid IM, sub-cut ok, prefer venipuncture over a finger or heel sticks for blood sampling,
o rest,
o elevation of the affected part,
o compression at the site.
o Do not administer aspirin.
• Medications
o DDAVP: increases plasma factor VIII
o Factor VIII: to treat and prevent hemorrhage
o Corticosteroids: treat hematuria and hemarthrosis
o E-aminocaproic acid: inhibit clot destruction
o NSAIDs: use cautiously d/t inhibition of platelets function
• Client Education
o Prevent bleeding at home
o Safe environment for play
o Up-to-date immunizations
o Wear a medical ID bracelet
Epistaxis: Short, isolated episodes are normal and nonemergent although they cause stress
• Risk Factors
o Trauma (nose picking)
o Low humidity, allergies
o Can be the result of underlying diseases like von Willebrand, hemophilia
• Nursing Care
o Maintain calmness with child and family
o Place sitting up with head tilted forwards slightly to prevent aspiration of blood
o Apply pressure for at least 10 mins
o Can use ice if preferred
Iron Deficiency Anemia
• Risk Factors
o Premature birth: decreased iron stores
o Adolescents d/t poor diet, rapid growth, obesity
o Excessive intake of cow's milk in toddlers
o Infants: GERD, pyloric stenosis
o Older child: GI polyps, colitis
o Adolescents: Menorrhagia
• Assessment
o Tachycardia
o Pallor
o Brittle, spoon-shaped fingernails
o Systolic heart murmur
o Cravings for non-nutritive things (ice, dirt, paper)
• Lab Tests
o CBC: Decreased RBC, Hgb, Hct
o Stool sample: Guaiac test
o Treatment can take up to 3 months
o Will need to monitor the child’s blood count routinely
• Medications
o Iron supplements: 1 hr before and 2 hrs after meals, milk, and antacids
o Watch for GI upset
o If giving liquid iron, use the dropper, place it directly in the back of the mouth, can mix it with formula
(not milk), juice, or cereal
o Give vitamin C to help with the absorption
o Watch for tarry stools and constipation
o IV ferrous sulfate: in severe anemia
o Very painful
• Nursing Care
o Iron supplements for preterm and low-birth-weight infants by 2 mos old
o Iron supplements to full-term infants at 4-6 mos
o Iron-fortified formula
o Include high-iron foods (iron-fortified cereals, dried beans, red meat, potatoes, peas, chicken) and
vitaminC
Sickle Cell Anemia
• Risk Factors
o An autosomal recessive genetic disorder
, o African American people
o Children who have the trait without manifestations of the disease can pass it to their children
• Assessment
o Family history
o Pain when in crisis
o Shortness of breath, fatigue
o Pallor
o Jaundice
o Vaso-occlusive crisis (painful)
▪ Acute: r/t dehydration and decreased oxygen. Pain, swollen joints, hematuria, jaundice
▪ Chronic: Increased risk of resp inf and osteomyelitis, retinal detachment and blindness, murmurs,
seizures
• Assessment cont.
o Sequestration
o Excessive pooling of blood primarily in the spleen (splenomegaly) and sometimes in the liver
(hepatomegaly)
o Shock d/t decreased blood volume
o Aplastic Crisis
▪ Extreme anemia d/t temporary decreased RBC production
▪ Triggered by a viral infection
o Hyper hemolytic Crisis
▪ Decreased rate of RBC destruction leading to anemia, jaundice, and/or reticulocytotic
• Laboratory Tests: Screening for newborns is mandatory nationwide
o CBC: detect anemia
o Hgb electrophoresis
o Sickle Cell Crisis:
▪ Hbg decreased, WBC, bili & reticulocyte elevated, Peripheral blood smear reveals sickled cells
• Diagnostic Procedures
o Transcranial Doppler Test (TCD)
▪ Assesses intracranial vascular flow and detects the risk for CVA
▪ Done annually for kids 2-16 yrs with sickle cell disease
• Nursing Care
o Decrease oxygen consumption
o Administer blood products
o Give oral prophylactic penicillin
• Nursing Care continued Pain Mgmt
o Treat mild pain with acetaminophen or ibuprofen. Manage severe pain with opioids.
o Comfort measures
• Medications
o Opioids: Administer on a schedule to prevent pain
o Antineoplastics: can cause anorexia
• Therapeutic Procedures
o Exchange Transfusion
o Hematopoietic Stem Cell Transplant
• Client Education
o Emotional support
o Promote rest and adequate hydration
o Wear a medical ID bracelet
o Avoid contact sports
• Complications
, o Stroke: d/t blood vessel occlusion
o Blood transfusions are usually done monthly to prevent a repeat stroke.
• Acute chest syndrome:
o Can be life-threatening
o Common in adolescents
o Increased risk of developing pneumonia d/t decreased oxygen to lung tissue
o Assess: chest, back, or abd pain, fever of 101.3 or higher, couch, tachypnea, decreased oxygen saturation
o Increased risk of infection
• Kidney Scarring
• Priapism (persistent and painful erection)
Hemophilia
• A group of bleeding disorders characterized by difficulty in controlling bleeding. Deficiencies in the clotting
factors
• Sometimes recognized during infancy following circumcision or later in childhood after injuries occur
• Varies in severity based on the percentage of clotting factor a child’s body contains
• For example, a child with mild hemophilia can have up to 40% of normal factor VIII but a child with
severehemophilia has very little factor VIII.
• Hemophilia A & B are X-linked recessive disorders.
• Types of Hemophilia
o Hemophilia A: Deficiency of factor VIII, “classic” hemophilia, 80% of cases of hemophilia
o Hemophilia B: Deficiency of factor IX, “Christmas disease”
o Von Willebrand: Inherited lack of the von Willebrand protein, inability of the platelets to aggregate
• Assessment
o Bleeding, joint pain and stiffness, impaired mobility, activity intolerance
• Lab Tests
o Prolonged partial thromboplastin time (aPTT)
o Facto-specific assays (tests)
o Platelets and prothrombin time WNL
o Whole blood clotting time can be WNL or prolonged
• Diagnostic Procedures
o DNA testing
• Nursing Care
o Management of bleeding,
o Avoid IM, sub-cut ok, prefer venipuncture over a finger or heel sticks for blood sampling,
o rest,
o elevation of the affected part,
o compression at the site.
o Do not administer aspirin.
• Medications
o DDAVP: increases plasma factor VIII
o Factor VIII: to treat and prevent hemorrhage
o Corticosteroids: treat hematuria and hemarthrosis
o E-aminocaproic acid: inhibit clot destruction
o NSAIDs: use cautiously d/t inhibition of platelets function
• Client Education
o Prevent bleeding at home
o Safe environment for play
o Up-to-date immunizations
o Wear a medical ID bracelet
