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Providing Patient Centered Care for the Child with a Hematologic Disorder. Presented by Marlene Meador RN, MSN, CNE. Hematologic System. Adult. Pedi. Life cycle of RBC- 120 days Cell production- marrow and spleen RBC’s= 4.1 to 4.9 million/ml Hemoglobin= Hematocrit=. - PowerPoint PPT Presentation
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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
1243)
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 Precipitating factors (p 1248-Home Care of
the Child with Sickly Cell Disease)Signs and symptoms
Three Types 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 trait
What factor is missing or defective?
Who 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
Childhood Cancer & TreatmentChemotherapySurgeryRadiationStem Cell TransplantationSteroid TherapyBiologic AgentsComplementary and Alternative Medical (CAM)
Chemotherapy: Antinoeplastic agentsTitrated to specific formula- closely correlated with
cardiac output and blood flow to kidneys and liverHighly specialized nurses- The Association of Pediatric
Hematology/Oncology NursesAdministration routes:
OralIntravenously (large bore catheter)IntramuscularlySubcutaneouslyIntrathecally
Chemotherapy: side effects/ nursing interventions
Bone Marrow Suppression
GI and GU Integumentary Changes
Neutropenia Anemia Thrombocytopenia
Nausea/vomitingStomatitisAnorexiaRenal damage
Hair lossHyperpigmentation of skinHypersensitivity to sunlight
Chemotherapy Nursing Interventions:Protection of the patient:
Isolation- what specific type?Exposure to sun, chemicals, skin irritants
Nutritional needs:Prevent nausea & vomitingTypes of foods to meet metabolic requirementsTemperatures/textures/acid-base
Fluid balanceIntake and output
IntegumentarySelf image
Clinical Judgment:Why does the nurse increase the amount of water/liquid
intake for a patient on chemotherapy? What specific interventions does the nurse need to
follow with relation to the patient’s output? Why are stool softeners very important? What are the best foods for a patient on chemotherapy?
Why? What do parents and family need to understand about
the patient’s psycho-social needs?
Radiation TherapyPurpose
Palliative- prevent growth, reduce tumor size, pain relief Eradicate or kill a tumor
Side effects similar to chemotherapy: Sub-acute & LateSomulence syndromeFeverIrritabilityAtaxiaAnorexia Dysphasia
Surgical treatment for cancerPurpose
Confirm diagnosisDebulking or resectingRemoval of tumor as adjunct to chemotherapy/radiation
Nursing InterventionsHow does the nurse prepare the patient for surgery?What interventions apply specifically to the family?
Hematopoietic Stem Cell and Bone Marrow Transplantation :Conditioning- eradicate disease with high-dose
chemo/radiation therapyInfusion- implantation of stem cells or bone marrow
Increase in patient’s WBC, RBC, and platelets signal success!Complications
GI disturbancesGraft-Versus-Host Disease (GVHD)
Maintain patient on anti-rejection medications Prednisone Cyclosporine Tacrolimus
Graft-Versus-Host Disease (GVHD)Potentially lethal immunologic response of donor T
cells against the tissue of the recipient.Prevention: Careful tissue typing, irradiation
of blood products to inactivate mature T lymphocytes.
Signs & Symptomsrash, malaise, high fever, diarrhea, liver and spleen
enlargementTreatment
Maintain patient on anti-rejection medications Prednisone Cyclosporine Tacrolimus
Complementary /Alternative Medical Therapies (CAM)Risks-vs- benefitsQualification of practitionerDelay or interfere with conventional
treatmentCostsContraindications
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 1274-1280)
Chemotherapy: three phasesInduction phaseConsolidation
Delayed intensificationRemission and maintenance
Nursing Care for a Child Undergoing Chemotherapy: review
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?
Clinical manifestations of Neuroblastoma (p 1286)
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
Nephroblastoma- Wilm’s TumorSoft renal tumor - one or both kidneys(p 1291) 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
Retinoblastoma (p1292)
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: (p905-915)
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
Nursing Care for the NurseProviding physical and psychosocial support
for the patient and family places additional stressors on the staff and nurses
Caring for dying children and their families is emotionally demanding.
Grief counseling plays an important role for both family and staff
For questions or concerns please contact Marlene Meador RN, MSN, [email protected] References:McKinney, James, Murray, & Ashwill.
Maternal- Child Nursing forth ed.(2015). Saunders.