Caring for the Child with a Hematological Condition

1
Chapter 33

• Caring for the Child with a Hematological
  Condition

2
A P Review

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

3
Common Hematological Conditions
4
Anemia

• 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)

5
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.
  1074)
• Discuss the Women Infants and Children (WIC)
  program
• Administer oral iron supplements

6
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

7
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
  requirements)
• Maintain oxygenation (respiratory assessment)
• Control pain (oral, IV, or PCA)
• Interpret laboratory or radiographic studies
• Educate family

8
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
  attention)
• Refer for genetic counseling

9
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
  illness

10
Hemophilia

• 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

11
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
  attention)
• Avoid the use of aspirin or NSAIDS

12
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
  episode
• Avoid aspirin, injections, tampons, nothing via
  the rectum
• Teach family to report signs and symptoms of
  bleeding
• Encourage child to wear a medical alert bracelet

13
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
  treatment

14
Aplastic Anemia

• Signs and symptoms
• Pancytopenia
• Anemia, pallor, dizziness, and fatigue
• Petechia, epistaxis
• Increased susceptibility to infections and oral
  ulcerations

• 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
  redness)

15
Neutropenia

• Signs and symptoms
• Lymphadenopathy, organomegaly, pallor, bruising,
  petechia
• 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
  transplant
• Monitor for infections (broad-spectrum
  antibiotic)
• 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)

16
Blood Transfusion Therapy

• Reasons for transfusion
• Hematological illness
• Oncological illness
• Chronic conditions

17
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

18
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.

19
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
  death.
• Children who have received multiple transfusions
  are at higher risk for developing a transfusion
  reaction.

20
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
  posttransfusion
• 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.

21
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.

22
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.

23
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.

24
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
  happening.
• 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.

25
Bone Marrow Transplantation

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

26
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
  cells)
• Allogeneic transplant (matched compatible donor,
  usually a sibling)
• Syngeneic (identical sibling)

27
Bone Marrow Transplantation

• Complications
• Acute and chronic graft versus host disease
  (GVHD)
• Infection
• Immunosuppression
• Organ dysfunction
• Psyosocial impact

28
Apheresis

• The process of apheresis is the selective removal
  of a specific blood component from a donor or
  child while re-transfusing the remaining
  components.
• 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
  patient.

29
Thrombosis

• Risk factors
• Prolonged immobility
• Disease states, obesity, medications, hereditary
  factors
• 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
  pulse

30
Thrombosis

• 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