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 birthHemoglobin= 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?

What Parents Want to Know: (p

1281)

Specific foods: (based on age of child)Cream of wheat or iron fortified cerealApricots, prunes, raisins and other dried fruitsEgg yolks Dark green leafy vegetables

Administration of Iron Supplements:Give with vitamin C –rich fluidsPrevent staining from liquid iron supplementsChanges in stool patternsAvoid mixing supplement with food/drink containing calcium

Sickle Cell diseaseSickle cell trait- genetic disorders

characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbg p 1282-1283

Precipitating factors (p 1284)Signs and symptoms

Three Forms of Sickle Cell CrisisVaso-occlusiveAcute sequestrationAplastic

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

to the ED 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 & TreatmentCord blood testing if one parent

is known to carry traitBlood transfusions

ComplicationsNursing interventions before/during/after

TreatmentPatient/family teachingMedicationsImmunizations- why important?

Clinical Judgment:Why are blood transfusions ordered for

the patient in sickle cell crisis?Can a neonate have a diagnosis of sickle

cell disease? What ethical issues relate to this

diagnosis?

Hemophilia (p 1291)X-linked traitWhat factor is missing or defective?

Factor VIIIWho is the carrier, and who is effected

by this disorder?

Diagnosis & TreatmentWhen does diagnosis most

commonly occur? What specific laboratory tests

and values?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 hemophiliaHemarthrosis- assess child for joint pain,

edema, or permanent deformity. Where most common?

At risk for hemorrhageDeath

Childhood Cancers“…communication promotes understanding and clarity; with understanding, fear diminishes; in the absence of fear, hope emerges; and in the presence of hope, anything is possible” (Stovall, 1995)

Childhood CancerC- continual unexplained weight loss, fatigue

malaiseH- headaches with vomiting (early morning)I- increased edema or pain in jointsL- lump or mass, persistent lymphadenopathy D- development of whitish appearance in pupil of the eyeR- recurrent or persistent fevers, night sweatsE- excessive bruising or bleedingN- 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 LeukemiaLeukocytes < 10,000 Leukocytes> 10,000

Platelets 20-100,000Hemoglobin 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: (p 1313/1321)

Chemotherapy: three phasesInduction phaseConsolidation

Delayed intensificationRemission and maintenance

Nursing Plan of Care for a Child Undergoing Chemotherapy:Myelosupression- protect from injuryInfection/sepsis (neutropenia)- protect

from infectionRenal damageGI disturbancesMetabolic emergencies

Intrathecal MedicationChemotherapy instilled

into spinal canalAssess 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 complicationsWhat 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

Nephroblastoma- Wilm’s TumorSoft renal tumor - one or both kidneys(p 1332) 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 1327)

Smooth, hard, non-tender along sympathetic nervous system

Frequent location is abdomenNeck and facial edema from vena cava

syndromeIncreased ICPLimp if metastasis 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?

Osteosarcoma- most common primary bone malignancy in children Goal of treatment- remove tumor and prevent spread of

diseaseBiopsy Chemo Surgery Chemo

(radiation=palliative pain control)Promote self esteem

Side effects of chemotherapyAmputation of extremity Separation from friends and family

Ewing Sarcoma- second most common bone tumor associated with childrenPain, soft tissue swellingAnorexia, fever, malaise with metastasis Diagnosis same as osteosarcomaManagement

ChemoSurgery (decrease tumor bulk)Radiation

Rhabdomyosarcoma

Pathophysiology and ManifestationsMost common soft tissue malignancyDivided by young (<10 yrs) and older

(adolescents) in location60% have positive prognosisSoft to hard, nontender mass (depends

on location)In pelvic tumors, may disrupt organ

function

Diagnosis and TreatmentCT, BM aspiration and biopsyRenal function and liver function testsTreated with chemo, surgery and

radiation

Retinoblastoma (p1333)

Retinoblastoma- rare malignant tumor of the neural retina“cat’s eye” reflex seen as a white light in the

pupil is the most common “leukocoria”May have strabismus of involved eyeRed painful eye is late symptomStaging based on extent of disease

Nursing care of the child/family with a malignant disease:Initial focus on support of family membersNurses facilitate the educational process to allay

fears of unknownEncourage family members to verbalize fears and

questionsPostoperative care if indicatedCommunity resources (through the discharge

planner, case worker)

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 Care and GriefChild- encourage child to express

feelings, allow choices, help maintain independence

Family- listen, answer questions, provide information, encourage expression of feelings and fears

For questions or concerns please contact Marlene Meador RN, MSN, CNE Email mmeador@austincc.edu References:McKinney, James, Murray, & Ashwill.

Maternal- Child Nursing Third ed (2009). Saunders.