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Pediatric Hematologic Disorders
and Cancer
Presented by Marlene Meador RN, MSN, CNE
Hematologic SystemAdult PediLife cycle of RBC- 120
daysCell production- marrow
and spleenRBC’s= 4.1 to 4.9
million/mlHemoglobin=Hematocrit=
Life cycle of RBC- 100 days (neonate)
Cell production- red bone marrow (infant)
#RBC’s= 5million/ml at birth
Hemoglobin= 17-18 gHematocrit= 45-50%
Iron Deficiency AnemiaCauseSigns and symptoms Diagnostic testsNursing interventions
Oral supplements- What significant side effects does the nurse need to remember?
Dietary teaching- what specific foods?
Sickle Cell diseaseSickle cell trait- genetic disorders
characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbg. p. 1494-1503 Precipitating factors (p. 1496)Signs and symptoms
Types of Sickle Cell CrisisVaso-occlusive- most common effects
PainHand and foot syndrome (dactylitis)CVA- hemiplegia, aphasia, seizures, LOC
changes, vision changes, and headacheAcute chest syndrome- chest pain, fever cough
(leading cause of death in SCD) PriapismHepatomegalyHematuria
Types of Sickle Cell CrisisAplastic Crisis:
Decreased RBC production- S&S malaise, headache, pallor, lethargy, and fainting (precipitated by infection)
Splenic sequestration- life threatening S&S pallor, irritability, tachycardia, hypovolemic shock
Hyperhemolytic crisis- (not in text)- RBC’s destroyed more rapidly than usual (immature cells)
Quick Review:What is most common reason for
admission for a child with SCD?What precipitates a sickle cell
crisis?How does sickling effect the life
span of an RBC?what organs experience
complications as a result of chronic sickling crisis?
Diagnosis & Treatment
Cord blood testing if one parent is known to carry trait
Blood transfusions-
TreatmentPatient/family teaching-MedicationsImmunizations- why important?
HemophiliaX-linked trait
What factor is missing or defective? Factor VIII
Who is the carrier, and who is effected by this disorder?
Diagnosis & TreatmentWhen does diagnosis most commonly occur?
What are signs & symptoms?
Nursing Care:Factor VIII- when should the patient receive this medication?
What does the family need to know about factor VIII?
Human plasmaVasopressin (DDAVP)
Nursing Care cont…What is the primary nursing goal for a patient
with hemophilia?Prevent or stop bleeding
What are specific interventions to achieve this goal?Administer Factor VIIIApply local pressure for 10-15 minutesElevate the joint and immobilizeApply cold compresses
Complications of hemophilia
Hemarthrosis- assess child for joint pain, edema, or permanent deformity. Where most common?
Death- at risk for hemorrhage
Childhood CancerC- continual unexplained weight loss, fatigue
malaiseH- headaches with vomiting (early morning)
I- increased edema or pain in joints
L- lump or mass, persistent lymphadenopathy
D- development of whitish appearance in pupil of the eye
R- recurrent or persistent fevers, night sweats
E- excessive bruising or bleeding
N- noticeable pallor
What signs and symptoms would lead to the diagnosis of leukemia?FeverPallorOvert signs of bleedingLethargy or malaiseAnrexiaLarge joint or bone painPetechiae, frank bleedingEnlarged liver or spleen, changes in lymph
nodesNeurologic changes
Lab values for a diagnosis of leukemia: examination of CBC with at least 25% blasts confirm the diagnosis Normal Leukemia
Leukocytes < 10,000
Leukocytes> 10,000
Platelets 20-100,000
Hemoglobin 7-11
Further diagnostic findings:Bone marrow aspiration- iliac crest
(why this site?)
How does the nurse prepare the child/family for this procedure?
What are the nurse responsibilities for this procedure?
Treatment and Plan of Care:Chemotherapy: four phases
Induction phaseConsolidationDelayed intensificationRemission and maintenance
Nursing Plan of Care:Myelosupression- protect from injury
Infection/sepsis (neutropenia)- protect from infection
Renal damageGI disturbancesMetabolic emergencies
Intrathecal MedicationChemotherapy instilled into spinal canal
Assess and monitor for placement of intrathecal catheter and assess neuro checks
Cranial RadiationHead and neck tumors are more
sensitive to radiation than chemotherapy.
When would chemotherapy become an adjunct to radiation therapy?
Tumor Lysis Syndrome:What causes tumor lysis syndrome?
What are signs and symptoms of this complications
What nursing interventions apply to treatment?
Bone marrow and Stem Cell TransplantationUsed to treat leukemia, neuroblastomas
and some noncancerous conditions-aplastic anemia
Goal to administer a lethal dose to kill the cancer, and resupply the body with stem cells from the child’s own bone marrow, or a compatible donor
Develop a plan of care for the child undergoing treatment for leukemia:Risk for injury:
Soft tissue/mucous membranesGeneralized trauma
Risk for infection:Risk for alteration in bowel eliminationRisk for GI distressFluid volume delicate
Wilm’s TumorSoft tumor frequently along
sympathetic nervous system(p.1552-3) Metastasis or seeding
spread by palpation Nephrectomy treatment of Wilm’s
tumor
Nursing treatment of Wilms’ tumor:Pain management
Frequent repositionNoninvasive and pharmacologic pain interventions
Prevent circulatory overloadWeigh dailyI&O, urine for specific gravity
Prevent infectionHand washingProtective isolationHomecare needs
Clinical manifestations of neuroblastoma:P. 1549-1552Smooth, hard, non-tender along sympathetic
nervous systemFrequent location is abdomenNeck and facial edema from vena cava
syndromeIncreased ICPLimp if mets to bonePancytopenia
Nursing Management Assess by observation and inspection
(not palpation)Document bowel and bladder functionRecord height & weight, observe gaitChemotherapy, radiation, surgeryTeach parents S&S of infection. Why?
Death and Dying:Understanding of death according to
developmental age:< 3 years- no understanding/concept of death3-5 years- afraid of separation from parents 5-9 years- understand death is permanent,
irreversible and sad. Concerns for fear of pain, being left alone and leaving parents and friends.
Age 10> have adult’s concept of death
Nursing and GriefChild- encourage child to express feelings, allow choices, help maintain independence
Family- listen, answer questions, provide information, encourage expression of feelings and fears
