Chapter 32

Question 1

Nursing Care: Anemia

Answer 1

Varies based on etiology
-For mild cases: provide supportive care through diet or vitamin supplement
For moderate-severe: RBC transfusion may restore blood volume
-For decreased RBC production: administer hematopoietic growth factors

Question 2

Education/Discharge Instructions: Anemia

Answer 2

• Educate on S/S that may indicate anemia (pallor, fatigue, dizziness, lethargy)
• Teach family that daily activities may need to be altered (quiet play, rest periods, high-iron diet)
• Teach how to administer iron supplements if necessary
• Tell family that child may need regular visits for lab tests to evaluate status

Question 3

Nursing Care: Iron-Deficiency Anemia

Answer 3

• Focus on prevention, educate regarding risk factors, and early identification and recognition
• Monitor lab tests
• Communicate to parents the importance of compliance w/iron administration and of follow-up visits to monitor Hb, Hct, and reticulocyte count

Question 4

Education/Discharge Instructions: Iron-Deficiency Anemia

Answer 4

• Teach parents proper administration of oral iron
• Take between meals
• Absorption is improved in acidic environment (OJ)
• Do not take with tea or dairy
• Inform parents that liquid iron preparations may stain teeth
• Administer medication w/dropper or have child drink it through straw
• Encourage child to rinse the mouth after taking

Question 5

Nursing Care: Sickle Cell Disease

Answer 5

• Identify and treat hypoxic episodes early
• Coordinate care to allow adequate rest periods and minimize unnecessary interruptions
• Provide adequate hydration
• Assess pain and provide pain control to treat painful vaso-occlusive crisis

Question 6

Education/Discharge Instructions: Sickle Cell Disease

Answer 6

• Teach how to avoid sickle cell crisis by providing rest and adequate hydration
• Teach S/S of sickle cell crisis
• Teach steps to be taken in event of mild sickle cell crisis at home
• Educate family about goals of ongoing care, including: prevention of complications associated w/ infections, hypoxemia, and vaso-occlusive crisis
• Explain that strenuous activities may need to be avoided
• Emphasize importance of adhering to medication regimens (prophylactic penicillin to prevent overwhelming sepsis and supplemental folic acid to assist w/RBC production)
• Emphasize that preventable illnesses are potentially life-threatening for asplenic patients

Question 7

Nursing Care: Thalassemia

Answer 7

• Prevent hypoxia by providing blood transfusion therapy (q3-4 weeks for child’s lifetime)
• Be aware of risk for hemosiderosis due to chronic transfusion therapy
• Children w/Beta-thalassemia may be cured of disorder with bone marrow transplant

Question 8

Education/Discharge Instruction: Thalassemia

Answer 8

• Emphasize importance of blood transfusion therapy and chelation to ensure adherence with treatment regimen to promote quality of life
• Teach family appropriate technique for administration of chelation therapy
• Emphasize meticulous hand washing (often asplenic)
• Insist that parents must seek medical attention if child develops temp of 101.5
• Instruct family on importance of genetic counseling
• Refer to community resources for support

Question 9

Nursing Care: Hereditary Spherocytosis

Answer 9

• Child only hospitalized if severe crisis occurs, requiring transfusion
• Obtain prior consent before administering blood product
• Give parents oral/written information on risks/benefits, possible adverse rxns, and overall info about transfusion process
• Obtain CBC post-transfusion to determine treatment effectiveness
• Severe hemolysis may require folic acid supplementation
• Children w/ <80% of normal spectrin content are candidates for splenectomy

Question 10

Education/Discharge Instructions: Hereditary Spherocytosis

Answer 10

• Educate on supportive care measures
• Teach about folic acid supplementation regimen
• Educate on infection control principles for child who had splenectomy (antibiotic prophylaxis, need for appropriate immunizations, hand washing for those in contact)
• Proper method on taking temp
• Seek medical attention if child develops temp of 101.5
• Blood counts must be evaluated regularly
• Utilize community resources for psychosocial support

Question 11

Nursing Care: Hemophilia

Answer 11

• Care is collaborative and interdisciplinary
• Initiate proper tx of bleeding episodes
• Ensure deficient factors are properly identified so proper replacement factors are administered
• Focus on patient safety, complication prevention, and promoting wellness/quality of life
• Recombinant factor products are main tx

Question 12

Education/Discharge Instructions: Hemophilia

Answer 12

• Prophylactic doses of factor products may be administered at home
• Collaborate w/family on safety precautions
• Teach S/S that require prompt medical attention
• Instruct how to administer recombinant factor products by IV access
• Older, mature children may self-administer factor products at home after instruction has been completed
• Encourage medical ID bracelets to be worn

Question 13

Nursing Care: Von Willebrand’s Disease

Answer 13

• Admin. desmopressin (DDAVP), a synthetic analog of the hormone vasopressin
• Admin. Humate-P IV and/or cryoprecipitate, or fresh frozen plasma

Question 14

Education/Discharge Instructions: Von Willebrand’s Disease

Answer 14

• Teach about common bleeding sites (nose, gums, internal bleeding)
• Educate on how to control bleeding (applying pressure, ice, and seeking medical attention)
• Educate adolescent females on what constitutes excessively heavy menses
• Give tips to avoid embarrassing moment during periods of heavy menstrual flow (wearing 2 maxi pads, and not wearing light-colored paints/skirts)
• Instruct small children to avoid nose picking, vigorous nose blowing, and strenuous activity that may cause a nosebleed
• Teach children to sneeze w/mouth open and gently blow nose if needed
• Avoidance of aspirin, NSAIDs
• Children prone to epistaxis can utilize cool-mist humidifcation as a preventative measure
• Gentle flossing and use of soft-bristled toothbrush

Question 15

Nursing Care: Immune Thrombocytopenia

Answer 15

• Often spontaneous resolution with no tx
• Monitor platelet counts (counts <20,000 and asymptomatic do not require tx; toddlers/active children w/bruising and petechiae w/counts <20,000 treated aggressively to avoid intracranial bleeding)
• Perform transfusion of platelets ONLY when severe, life-threatening bleeding is present
• While patient hospitalized promote bedrest, monitor vital signs, monitor for adverse rxns during admin of IVIG, take blood counts daily
• Manage ITP medically though steroids, IV immune globulin, anti-D antibody
• Splenectomy may be needed if medical tx fails and there have been acute life-threatening bleeding episodes
• *Tx for ITO among pediatric hematologists is not consistent

Question 16

Education/Discharge Instructions: ITP

Answer 16

• Restrict activities such as contact sports and high-risk activities
• Instruct how to manage bleeding at home (apply pressure to injury site, if nosebleed have child lean down and forward and pinch bridge of nose and apply ice if possible, seek medical attention for severe bleeding not stopped by manual pressure)
• Avoid aspirin or other aspirin-containing products and injections, straight-edge razors, use of tampons, inserting thermometer or suppository into rectum
• Report S/S of bleeding immediately
• Provide safe environment to prevent trauma (padding crib, using nail file as opposed to clippers for nail care)
• Recommend child wears a medical alert bracelet

Question 17

Nursing Care: Disseminated Intravascular Coagulation

Answer 17

• Provide supportive care for symptoms
• May require management in ICU
• Admin. blood and factor products as necessary
• Monitor for hemorrhage, bleeding, petechiae, cutaneous oozing, dyspnea, lethargy, pallor, increased HR, decreased BP, headache, dizziness, muscle weakness, restlessness
• Monitor for internal bleeding by checking urine and stool for occult blood
• If child bleeding, do not disturb clots, use pressure, apply ice, and measure blood loss
• Obtain necessary lab tests

Question 18

Education/Discharge Instructions: DIC

Answer 18

• Importance of teaching infections

- Educate on triggers identified to cause this coagulation disorder

Question 19

Nursing Care: Aplastic Anemia

Answer 19

• Care varies based on severity and causative factors
• If causative factor identified, focus on resolving cause
• If cause unidentified, but there is suitable stem cell donor, pt undergoes hematopoietic stem cell transplant (HSCT)
• After transplant, provide supportive care, admin. immunosuppressive therapy, admin. hematopoietic growth factors, be aware that pt’s may not exhibit “classic signs of infection”

Question 20

Education/Discharge Instructions: Aplastic Anemia

Answer 20

• Provide reassurance and support to families
• Review prescriptions w/family and discuss possible adverse rxns
• Emphasize importance of monitoring child’s CBC
• Teach S/S of pancytopenia
• Need scheduled rest period, avoid contact with crowds, and avoid sources of infection
• Teach about injury prevention (use soft toothbrush, ensure safe play environment, adolescent girls should avoid tampon use)
• Stress importance of meticulous oral hygiene and hand washing

Question 21

Nursing Care: Neutropenia

Answer 21

• Tx may range from supportive measure to admin. of colony-stimulating factors
• If severe, bone marrow transplant often necessary
• Evaluate etiology in patients w/acquired neutropenia
• Monitor for infections by checking for fever, evaluation absolute neutrophil count, and performing physical exams
• Implement empiric therapy w/broad-spectrum antibx while culture results pending
• Admin. granulocyte colony-stimulating factor (filgrastin) to stimulate neutrophil production in bone marrow

Question 22

Education/Discharge Instructions: Neutropenia

Answer 22

• When and how of hand washing
• Properly check child’s temp
• Need for medical attention if child develops temp of 101.5
• Avoid anyone who is sick or recently received live vaccines
• Teach about meticulous oral hygiene and good skin care

Question 23

Febrile Reaction

Answer 23

• Most common blood transfusion reaction
• Develops temp >2 from baseline temp
• Occur on initiation of transfusion and have been known to occur up to 12 hrs post-transfusion
• S/S: fever and chills, may progress to tachycardia, tachypnea, hypotension
• Nursing care: pre-medicate w/acetaminophen, monitor temp, stop transfusion, monitor vital signs, notify HCP

Question 24

Allergic Reaction

Answer 24

• Occurs during transfusion in which child had previous exposure to a particular allergen in blood product
• Exposure stimulates anitbody response and an allergic transfusion reaction is evident, may occur on second or subsequent transfusions
• S/S: rash, hives, pruritus, swelling of lips, wheezing, anxiety
• Nursing care: if suspected, stop transfusion, monitor vital signs, notify HCP; admin. antihistamine (Benadryl); usually resolves allergic response; admin, histamine blocker (Zantac) to aid in symptom relief if needed; if severe rxn may require admin. of steroids (hydrocortisone and possible epinephrine); future transfusions may require prophylaxis care w/Benadryl and hydrocortisone

Question 25

Bacterial Contamination

Answer 25

• Rare non-hemolytic rxn, generally occurs during initiation of infusion
• Actual contamination of blood product
• S/S: shaking chills, fever, vomiting, diffuse erythema, onset of hypotension that may progress to shock, in severe cases may develop hemoglobinuria, acute renal failure, DIC
• Nursing Care: if identified, stop transfusion, monitor vital signs, start normal saline infusion, notify HCP, prepare for emergency care
• Obtain samples for culture and sensitivity
• Also send blood product w/tubing to blood bank to be cultured

Question 26

Circulatory Overload

Answer 26

• Rare in children, occurs when infusion is given too rapidly or an excessive quantity of blood is given
• S/S: dry cough, dyspnea, rales, distended neck veins, hypertension/hypotension, bradycardia/tachycardia, clammy skin, cyanosis of extremities
• Nursing Care: accurately verify HCPs orders, double-check volume to be infused, use IV pump, be aware of whether child is on fluid restriction, maintain accuracy of intake and output records; if symptoms identified, stop transfusion, monitor vital signs, place child upright w/feet in dependent position to increase venous resistance, notify HCP, prepare for emergency care

Question 27

Acute Hemolytic Transfusion Reaction

Answer 27

• Rare, but most severe type of reaction
• Occurs when donor RBCs and the recipient plasma are incompatible, and there is an ABO mismatch (reaction occurs after initiation, after exposure to small amount)
• S/S: fever, shaking chills, pain at IV site, tightness of chest and difficulty breathing, impending sense of doom, pallor, jaundice, N/V, red/black urine, flank pain, progressive signs of shock (tachycardia and hypotension)
• Nursing Care: if symptoms identified, stop transfusion, monitor vital signs, start normal saline infusion, verify pt ID, notify HCP, prepare for emergency care
• Obtain blood and urine samples and send to lab to analyze for Hb, indicating intravascular hemolysis
• Insert urinary catheter to monitor output more accurately

Question 28

Blood transfusions (General)

Answer 28

Education/Discharge Instructions:

• Edu tailored to type of blood product being admin.
• Ease anxiety via thorough teaching
• If available, collaborate w/child life therapist to provide developmentally appropriate medical play
• Teach S/S of adverse rxn
• Report any possible S/S of reaction to nurse immediately

Question 29

Blood Transfusion Therapy

Answer 29

Nursing Care:

• Obtain type and cross-match (good for 72 hrs) if it is almost certain that child will require blood
• Explain process and indications of blood transfusion in detail
• Obtain written consent
• Take vitals to ensure patient is clinically stable
• Take baseline vitals before initiating transfusion
• Monitor vitals throughout
• Check for pre-medications ordered before admin.
• Call for blood only when product is ready to be admin.
• Follow institution’s procedures
• Check product and verify 2 patient identifiers at bedside by 2 appropriate health-care providers
• Start transfusions slowly for first 15 mins with nurse continually present
• Do not infuse other solutions or medications w/blood through same IV line, except NS
• Always wear protective gear
• Keep all identification information attached to blood product until transfusion is completed
• Save transfusing bag for at least 1 hr after
• Blood slip must be completely filled out w/institution’s required info
• Place chart copy and blood bank copy in appropriate area to be kept on file as per policy and procedure

Question 30

Per American Association of Blood Bank Guidelines, child’s medical record must include:

Answer 30

• transfusion order
• type of blood product
• donor unit #
• date and time of transfusion
• pre/post vital signs
• volume infused
• required signatures
• any transfusion adverse events

Question 31

Nursing Care: Thrombosis

Answer 31

• Complete assessment of risk factors for developing thrombus
• If at risk: prophylactic plan of care individualized for each child’s condition, interventions reviewed daily for effectiveness
• Prophylaxis Care: may include compression stockings, intermittent pneumatic compression devices, and passive ROM (devices can be DCed when ambulatory, assess skin under stockings and devices)
• Implement early ambulation
• Admin low molecular weight Heparin
• Beware of risk of pulmonary emboli

Question 32

Education/Discharge Instructions: Thrombosis

Answer 32

• Avoid prolonged sitting and bed rest
• Smoking cessation and avoid drugs that cause blood clots (birth control pills)
• Take short walks when safely permitted if traveling for extended periods of time
• Encourage daily use of anti-embolism stockings if at high risk