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Caring for the Child with a Hematological Condition


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Title: Caring for the Child with a Hematological Condition

Chapter 33
  • Caring for the Child with a Hematological

A P Review
  • Blood
  • Plasma (albumin, electrolytes, proteins, clotting
    factors, fibrinogen, globulins, and circulating
  • Cells
  • RBCs (transport hemoglobin)
  • WBCs (bodys protective system)
  • Platelets (hemostasis and vascular repair after
    injury to a vessel wall)

Common Hematological Conditions
  • Signs and symptoms (initial signs)
  • Fatigue, SOB, headache, difficulty concentrating,
    dizziness, and pale skin
  • Nursing care
  • Identify underlying cause
  • Promote a healthy diet or a vitamin supplement
  • Administer red blood cells (moderate anemia)
  • Administer hematopoietic growth factors (specific
    types of anemia)
  • Teach family (signs and symptoms, alter daily
    activities, evaluate status of anemia)

Iron-Deficiency Anemia
  • Signs and symptoms
  • Asymptomatic (mild anemia)
  • Decreased Hgb and Hct
  • Irritability, fatigue, delayed motor development,
    SOB, decreased activity level, and pale skin
  • Remember an overweight child can be diagnosed
    with iron-deficiency anemia.
  • Nursing care
  • Identify early
  • Understand that primary goal is prevention
  • Facilitate nutritional counseling
  • Assist with obtaining recommended iron-fortified
    formula and cereal
  • See What to sayNutritional counseling (p.
  • Discuss the Women Infants and Children (WIC)
  • Administer oral iron supplements

Epistaxis (Nosebleeds)
  • Signs and symptoms
  • Bleeding from the nose
  • Nursing care
  • Focus on prevention, family education
  • Avoid the use of aspirin or NSAIDS
  • Use a cool mist vaporizer
  • Use first aid measures to stop bleeding

Sickle Cell Disease (SCD)
  • Signs and symptoms
  • Result of vaso-occlusion
  • Weakness
  • Pallor
  • Fatigue
  • Tissue hypoxia
  • Jaundice
  • Pain
  • Nursing care
  • Identify early and treat hypoxic episodes
  • Encourage rest
  • Hydrate (1.5 times above normal calculated
  • Maintain oxygenation (respiratory assessment)
  • Control pain (oral, IV, or PCA)
  • Interpret laboratory or radiographic studies
  • Educate family

Beta Thalassemia
  • Signs and symptoms
  • Enlarged liver and spleen
  • Mild jaundice
  • Growth retardation
  • Moderate to severe anemia
  • Bony deformities
  • Increased susceptibility to infection
  • Nursing care
  • Understand that the goal is to prevent hypoxia by
    providing blood transfusion therapy
  • Promote growth and development
  • Discuss bone marrow transplant
  • Educate family (blood transfusion and chelation
    treatment, hand washing, when to seek medical
  • Refer for genetic counseling

Hereditary Spherocytosis (HS)
  • Signs and symptoms
  • Hyperbilirubinemia
  • Splenomegaly
  • Negative Combs test
  • Nursing care
  • Give folic acid supplements
  • Discuss splenectomy
  • Educate family (folic acid supplementation and
    adhere to prescribed antibiotic regimen)
  • Promote good hand washing
  • Instruct parents on temperature taking and when
    to seek medical attention, evaluate blood counts
    and immunizations
  • Provide support to help them cope with lifelong

  • Signs and symptoms
  • Present with bleeding or known family history of
    bleeding disorders
  • Hemarthrosis
  • Soft tissue bleeding
  • Nursing care
  • Initiate prompt treatment of bleeding episodes
  • Ensure patient safety and prevent complications
  • For severe hemophilia, administer recombinant
    factor products
  • Educate family about proper administration of
    factor products

von Willebrands Disease
  • Signs and symptoms
  • Epistaxis
  • Bleeding from the oral cavity
  • Menorrhagia
  • Easy bruising
  • Nursing care
  • Administer Desmopressin (DDAVP)
  • Administer Humate-P and/or cryoprecipitate or
    fresh frozen plasma (FFP)
  • Recommend medical alert
  • Instruct parents about common sites of bleeding
    (nose, gums, internal, and menses)
  • Control bleeding (pressure, ice, and seek medical
  • Avoid the use of aspirin or NSAIDS

Idiopathic Thrombocytopenia Purpura (ITP)
  • Signs and symptoms
  • After a recent viral infection
  • Petechiae
  • Bruising
  • Mucocutaneous bleeding
  • Epitaxis
  • Menorrhagia (adolescent)
  • Nursing care
  • Administer steroids, Intravenous Immune Gamma
    Globulin (IVIG), or Anti-D antibody (WhinRho)
  • Restrict activities
  • Instruct parents on how to manage a bleeding
  • Avoid aspirin, injections, tampons, nothing via
    the rectum
  • Teach family to report signs and symptoms of
  • Encourage child to wear a medical alert bracelet

Disseminated Intravascular Coagulation (DIC)
  • Signs and symptoms
  • Excessive bleeding from orifices
  • Petechia, purpura, and hypotension
  • Multi-organ failure
  • Nursing care
  • Identify the underlying cause
  • Monitor for signs of hemorrhage, bleeding
    petechiae, cutaneous oozing, dyspnea, lethargy,
    pallor, increased heart rate, decreased blood
    pressure, headache, dizziness, muscle weakness,
    restlessness, and internal bleeding
  • Do not disturb clots, use pressure and ice to
    control bleeding
  • Obtain laboratory tests and administer blood and
    factor products
  • Inform family about plan of care and course of

Aplastic Anemia
  • Signs and symptoms
  • Pancytopenia
  • Anemia, pallor, dizziness, and fatigue
  • Petechia, epistaxis
  • Increased susceptibility to infections and oral
  • Nursing care
  • Remove causative agent
  • Administer antithymocyte globulin (ATG) and
    high-dose cyclosporine (Neoral, Gengraf)
  • Administer hematopoietic growth factors
  • Discuss hematopoietic stem cell transplant (HSCT)
  • Support and educate familyNote Traditional s/s
    of infection are not evident (swelling, pus, and

  • Signs and symptoms
  • Lymphadenopathy, organomegaly, pallor, bruising,
  • Absolute neutrophil count (ANC)
  • Total number of white blood cells multiplied by
    the percentage of neutrophils (segs and bands)
  • Nursing care
  • Evaluate etiology
  • Understand that treatment ranges from supportive
    measures to administration of colony-stimulating
    factors (GCSF Neupogen ) to bone marrow
  • Monitor for infections (broad-spectrum
  • Inform about bone marrow transplant procedure
  • Educate family (hand washing, taking temperature,
    oral hygiene, skin care, keep away from other
    sick children)
  • See Critical Nursing ActionThe Neutropenic Child
    (p. 1089)

Blood Transfusion Therapy
  • Reasons for transfusion
  • Hematological illness
  • Oncological illness
  • Chronic conditions

Blood Transfusion Therapy
  • Nurse responsibilities
  • Be knowledgeable about blood transfusion
  • Review the plan of care with the family
  • Explain in detail the indications and process
  • Obtain blood consent
  • Jehovah Witnesses
  • Obtain the required blood samples
  • Administer based on policy and procedures

The Administration of Blood
  • Strict observance to the institutional policy
    regarding the administration of blood products
    cannot be stressed enough.
  • The accuracy of patient verification is a
    critical nursing action that can help prevent a
    transfusion reaction.

Transfusion Reactions
  • Most transfusion reactions occur during the
    initiation of a transfusion, but a reaction can
    occur at any time during this process. These
    reactions can vary from a mild reaction, such as
    mild fever, to the most severe complication of
  • Children who have received multiple transfusions
    are at higher risk for developing a transfusion

Febrile Reaction
  • Child develops a fever greater than 1.8F from
    the baseline temperature
  • Occurs on initiation of the transfusion, but has
    been known to occur up to 12 hours
  • Signs and symptomsfever and chills, which then
    may progress to more serious complications such
    as tachycardia, tachypnea, and hypotension
  • Nursing carepremedicating with acetaminophen
    (childrens Tylenol) can sometimes prevent this
    type of reaction. Monitor the childs temperature
    to recognize febrile reactions early and prevent
    progression. Stop the transfusion, monitor vital
    signs closely, and notify the physician.

Allergic Reaction
  • Occurs during a transfusion in which the child
    has had a previous exposure to a particular
    allergen in the blood product
  • Signs and symptomsrash, hives, pruritus,
    swelling of the lips, wheezing, and anxiety
  • Nursing carestop the transfusion immediately,
    monitor vital signs closely, and notify the
    physician. The administration of an
    antihistamine such as diphenhydramine (Benadryl)
    resolves an allergic response.

Circulatory Overload
  • Occurs when the infusion is given too rapidly or
    an excessive quantity of blood is given
  • Signs and symptomsdry cough, dyspnea, rales,
    distended neck veins, hypertension/hypotension,
    bradycardia/tachycardia, clammy skin, and
    cyanosis of the extremities
  • Nursing careaccurate verification of physician
    orders, double-checking the volume to be infused,
    and the use of an intravenous pump. If any of the
    signs and symptoms are identified, the nurse must
    immediately stop the transfusion, monitor vital
    signs closely, place the child upright with feet
    in a dependent position to increase venous
    resistance, notify physician, and prepare
    emergency care.

Acute Hemolytic Transfusion Reaction
  • Occurs when the donor RBCs and the recipient
    plasma are incompatible and there is an ABO
    mismatch. Acute hemolytic transfusion reactions
    occur upon initiation after exposure to a small
    amount of blood.
  • Signs and symptomsfever, shaking chills, pain at
    the intravenous site, tightness of the chest,
    difficulty breathing, impending sense of doom,
    pallor, jaundice, nausea/vomiting, red or black
    urine, flank pain, and progressive signs of shock
    such as tachycardia and hypotension
  • Nursing carestop the transfusion, monitor vital
    signs closely, start a normal saline infusion,
    verify patient identification, notify the
    physician, and prepare emergency care. Other
    nursing responsibilities include obtaining blood
    and urine samples and sending them to the
    laboratory to analyze for the presence of
    hemoglobin, which indicates intravascular
    hemolysis. Insert a urinary catheter to monitor
    the childs output more accurately.

Bacterial Contamination
  • Occurs during the initiation of the infusion
  • Guidelines from the AABB (2004) require strict
    adherence to the completion of all transfusions
    in 4 hours or less to prevent this from
  • Signs and symptomsshaking chills, fever,
    vomiting, diffuse erythema, and the onset of
    hypotension that may progress to shock. In severe
    cases, hemoglobinuria, actual renal failure, and
    DIC may develop.
  • Nursing carestop the transfusion immediately,
    monitor vital signs closely, start a normal
    saline infusion, notify the physician, and
    prepare emergency care. Nursing responsibilities
    also include obtaining blood samples for culture
    and sensitivity and sending the blood product
    with tubing to the blood bank also to be cultured.

Bone Marrow Transplantation
  • Hematopoietic stem cell transplant (HSCT)
  • Bone marrow transplantation (BMT)

Bone Marrow Transplantation
  • Preparative regimen
  • Administration of near lethal doses of
    chemotherapy and/or radiation
  • Three types of hematopoietic stem cell
    transplants (HSCT)
  • Autologous transplant (childs own donor of stem
  • Allogeneic transplant (matched compatible donor,
    usually a sibling)
  • Syngeneic (identical sibling)

Bone Marrow Transplantation
  • Complications
  • Acute and chronic graft versus host disease
  • Infection
  • Immunosuppression
  • Organ dysfunction
  • Psyosocial impact

  • The process of apheresis is the selective removal
    of a specific blood component from a donor or
    child while re-transfusing the remaining
  • Blood is removed from the child, pumped through a
    special cell separator in the apheresis machine
    that removes the specific desire component by
    centrifugal force, and then is returned to the

  • Risk factors
  • Prolonged immobility
  • Disease states, obesity, medications, hereditary
  • Major surgery or trauma
  • Complications
  • Stroke
  • Deep vein thrombosis
  • Pulmonary emboli
  • Signs and symptoms
  • LungsSOB, lightheaded, and increased heart rate
  • Kidneyblood in the urine
  • Skinhemorrhagic spots
  • Artery or extremitycold, pale, blue, and absent

  • Nursing care
  • Thoroughly assess risk factors
  • Apply compression stockings
  • Use an intermittent pneumatic compression device
  • Perform passive range of motion
  • Administer lowmolecular-weight heparin
  • Promote early ambulation
  • Administer heparin in some cases