11/13/20151 hematologic disorders and cancer acc susan beggs, rn msn
TRANSCRIPT
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Hematologic Disorders and Cancer
ACCSusan Beggs, RN MSN
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Comparison of adult to pedi hematologic system
Adult RBCs: 120 Lower H & H than
child Lower WBCs than
child
Pediatric RBCs: 100 days in
neonate Increased
erythropoiesis with age
Higher H & H in children (17-18g)
# of RBCs varies according to age
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Risk factors associated with blood disorders in pediatric clients More prone to anemia Genetic characteristics may impair
growth and development (sickle cell, hemophilia)
Immature immune systems to defend normal growth of cells
Higher risk of blood malignancies during childhood (ALL most common)
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RBC Maturation
Basophilic erythroblast* Orthochromic erythroblast* Proerythroblast Reticulocyte Erythrocyte Other cells that might be
suggestive of disorders:
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Components of the CBC
WBCs (leukocytes) Neutrophils Lymphocytes Monocytes Eosinophils Basophils
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Bands
Slighty smaller than other immature forms
Make up 0-6% of WBC count Indicative of a shift to the left
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Neutrophils
Segmented (segs) Make up 45-75% of peripheral
blood WBCs Elevated indicative of a shift to the
left or long term infection
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What is peak and what is trough? Peak refers to the effectiveness of
the medication; checks saturation and penetration 30 min AFTER end of infusion
Trough check if too little or too much 30 minutes PRIOR to next dose
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Iron deficiency anemia
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Iron deficiency anemia
Causes Diagnostic tests to confirm Treatments
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Diagnostic tests to confirm IDA hemoglobin hematocrit, MCV serum iron, RBC Presence of reticulocytes
(immature or newly released RBCs Changes in iron-binding capacity Serum ferritin < 15ng/ml
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Treatments for IDA
Two major treatments: Oral Dietary teaching
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Sickle cell disease (SCD)
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Types of sickle cell crisis Vaso-occlusive Aplastic crisis Splenic sequestration crisis
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Vaso-occlusive crises
Stasis of blood and clumping of cells in the microcirculation (capillaries)
May last from 1 day to several wks Manifestations: “What causes the pain?”
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SCD, continued
Manifestations: Chronic anemia (hgb 6-9) Fatigue Pain in areas of ischemia (joints) Jaundice Possible delayed sexual
maturation Susceptibility to sepsis Possible growth retardation
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What factors start the sickling? Being submitted to hypoxia Low blood pH (acidosis) Increased blood viscosity General stress Infection
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Aplastic crisis
Diminished RBC production Results in severe anemia Manifestations:
Headache Pallor Lethargy **may be precipitated by infection
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Splenic sequestration crisis Sickled cells trapped in spleen Blood flow is obstructed Resulting in splenomegaly May lead to :
Shock Hypovolemia tachycardia
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Diagnostic Studies for SCD Hemoglobin electrophoresis in NB Child > 6 months of age, quick
screen (Sickledex) CBC results:
Decreased H & H (6-9 hgb) Elevated reticulocytes (immature
RBCs)
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Nsg interventions for reactions to blood transfusions Stay with patients the 1st 5-10
minutes after beginning the transfusion
STOP the blood if rx occur, but NOT the IV
Monitor VS Listen for adventitious breath
sounds that indicate overload
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Insuring hydration in the child with SCD Educating parents s/s dehydration Instructions on # oz to replace
fluids lost Understand the “triggers” and
precipitating factors Monitoring I & O Perform regular growth and
nutritional assessments
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Goals for SCD
Oxygenation Adequate hydration Pain relief
Prevention of infection
Education of child/family
No cure, but can be managed
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Types and causes
Hemophilia A Most common (75%) Disorder with factor VIII Bleeding most common symptom Von Wildebrand is type of this
hemophilia Hemophilia B
Disorder with factor IX
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Diagnostics and Treatment Monitor studies
which may be abnormal: PTT, Bleeding time, platelet counts, Factor VIII levels
Prevention and treatment of bleeding:
Protective gear for play
Limited activities Replacement of
clotting factors Cold to cause
vasoconstriction
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Joint changes
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Labs tests to confirm hemophilia DNA testing for the trait PTT prolonged Bleeding time prolonged Platelets and PT are normal Low levels of factor VIII
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Administering Meds for Hemophilia Genetically engineered Factor VIII
blood products; reconstituted with sterile water and given IV
Human plasma, fresh whole blood, fresh or frozen plasma (1 bag of concentrate per 5 kg of body weight is usually sufficient)
Vasopressin (DDAVP) IV
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Nursing goals/interventions for the hemophiliac Prevent bleeding or STOP
bleeding **Major cause of death:
hemorrhage Apply pressure 10-15 min Elevate the joint above the heart Immobilize the extremity Apply cold compresses
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Cancer
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“…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)
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Causes of childhood cancers Unlike adults, children don’t have
the environmental exposures May be genetic? May be viral? Immune defects? Genome project has identified
genes for some of the cancers in children
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Warning signs
C H I L D R E N
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Interventions for malignancies Radiation Chemotherapy (IV, po, intrathecal) Surgery Bone marrow and stem cell
transplantation
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Leukemias
Malignancies of the blood Characterized by IMMATURE
WBCs/blast cells ALL most common (80%) ANLL also common(20%)
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Treatments for Leukemias Staging must be done first to
determine cell types Remission induction Possible CNS prophylactic therapy Maintenance Re-induction following relapse BM transplantation Prognosis
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Chemotherapy protocol for leukemia Induction phase (hopeful
remission) Consolidation phase Delayed intensification Maintenance phase
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Signs and symptoms that would suggest leukemia Fever Pallor Overt signs of bleeding Lethargy Malaise Anorexia Large joint or bone pain Petechiae Hepatomegaly, lymphadenopathy,
splenomegaly Neuro findings with CNS mets
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Nsg interventions for chemo side effects Myelosuppression: monitor labs,
prophylactics, injury awareness Infection/sepsis: neutropenia,
visitors, protective isolation Renal damage: I & O, hematuria GI: nutrition maintenance, high
calorie drinks, cold better than warm
Metabolic emergencies: tumor lysis
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BMT
Treat leukemias, neuroblastomas and apastic anemias
Kill the malignant cells and re-transfuse with stem cells from child’s BM or compatible donor
Goal of BMT: kill cancer and re-supply bone marrow with stem cells
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Safety teaching during chemo Care with brushing teeth Prevention of injury Prevention of infection Adequate hydration Treatment for GI distress
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Non-Hodgkins Lymphoma; Hodgkins Considered to be “soft tumors” May be early (one node) or
metastatic (diffuse spread) WBC and ESR may be elevated Staging must be done prior to
treatment
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Non-Hodgkins Lymphoma (NHL) Assessment findings:
“just not themselves” Lymph node enlargement Changes in sensorium Electrolyte imbalance
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Hodgkins lymphoma
Also a disease of the lymph system
Arises in a single node Peak occurrence in adolescent
boys. Occurs in clusters and may be
familial (genetic link?)
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Reed-Sternberg cells
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Treatment regimen for NHL, Hodgkins Staging Chemotherapy Radiation (for Hodgkins; not
effective in NHL)
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Nursing considerations for children with ALL, NHL, Hodgkins Preparation for diagnostic testing Education of parents Insertion of central lines Imbalanced nutrition Disturbed body image Risk for infection with
immunnosuppression Provide collaborative care
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Nursing diagnosis for children undergoing chemotherapy Imbalanced nutrition Risk for infection Activity intolerance Pain Disturbed sleep patterns Anxiety Family coping, grieving
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Wilms: manifestations
Swelling or mass within the abdomen
Metabolic alterations 2º compression from the tumor mass
Hematuria Anemia: from tumor network Weight loss and fever Mets result in shortness of breath,
chest pain
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Wilms tumor (bilateral)
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Wilms: staging, prognosis, management Undifferentiated blastoma, favoring
slightly the left kidney Survival rates are one of the
highest among all childhood cancers!
Combined treatment with surgery and chemo; may need radiation as well
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Nursing care for Wilms
Similar to other cancers Preoperative care Postoperative care Support for the family
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Wilms: treatment
Surgery: nephrectomy and lymph node dissection
Post-op chemo and/or radiation CT every 6 months for 2 yrs CXR every 3 months for 3 yrs
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Path of neuroblastoma
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Neuroblastoma, cont
Assess site of tumor by observation and inspection only
Palpation contraindicated Document elimination patterns May have all 3 methods of tx:
chemo, radiation and surgery
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Retinoblastoma
2/3 of retinoblastoma cases occur before the age of 2 years
95% occur before the age of 5 years
Overall survival is 93% Gene has been identified
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Features of Retinoblastoma
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Anatomy of the eye
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Clinical presentation of retinoblastoma “cat’s eye” reflex seen as a white
light in the pupil is the most common “leukocoria”
May have strabismus of involved eye
Red painful eye is late symptom Staging based on extent of disease
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Treatment of retinoblastoma Surgery: enucleation; utilized when
extensive retinal damage occurs Radiation: highly radiosensitive Chemotherapy: combinations of drugs Phototherapy: process that destroys the
blood vessels surround and supplying the tumor
Cryotherapy: destroys the tumor cells by forming ice crystals that disrupt the circulation of the tumor
Laser
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Osteosarcoma
Most common bone malignancy in children
Early symptoms Pain in extremity “growing pains”
Cause unknown Rare before adolescence
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Patho and manifestations Originates from bone-producing
cells invading the medullary canal of bone forming solid tumor
Manifestations Insidious pain at tumor site Palpable mass limping] Pathologic fx at site
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Diagnosis and treatment
CT, MRI, r/o mets (lungs) Staging and grading done Treatment:
Combination approach Surgery and chemo Radiation used only for palliative
care Amputation may be necessary
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Ewing sarcoma
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Ewing sarcoma
2nd most common bone tumor Mimics infection Cause unknown; no defining
characteristics Most often in midshaft of long
bones, esp femurs, vertebrae, ribs and pelvic bones
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Manifestations
Pain, soft tissue swelling Anorexia, fever, malaise with mets Diagnostic workup same as for
osteosarcoma Management
Chemo Surgery (decrease tumor bulk) Radiation
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Rhabdomyosarcoma
Malignancy of muscle or striated tissue
Usually in the periorbital areas (head and neck) in young
Trunk and extremities in older children
May be familial
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Patho and manifestations Most common soft tissue
malignancy Divided by young (<10 yrs) and
older (adolescents) in location 60% have + prognosis Soft to hard, nontender mass
(depends on location) In pelvic tumors, organ function
may be disrupted
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Diagnostics and treatment CT, BM aspiration and biopsy Renal function and liver function
tests as well Treated with chemo, surgery and
radiation
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Nursing Care of all children with malignant disease Initial focus on support of family
members Nurses facilitate the educational
process to allay fears of unknown Encourage family members to
verbalize fears and questions Postoperative care if indicated Community resources (through the
discharge planner, case worker)
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Children and death
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What do you say?
Avoid, “I know just how you feel” May become frustrating to see
children experience death May need to put aside your own
personal belief system as you try to help
Use the name of the deceased Recognize that special events and
days will be difficult
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Dealing with terminal illness Parents are informed Parents’ reaction to child’s death Grandparents…not only for the
child, but for the parents End-of-life nursing care
Meeting physiological needs Meeting the psychological needs of
the family
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Losing a child
Parents expect children to outlive them
Parents may experience strong guilt
Mention the child’s name
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“The death of a child….robs parents of what they love most, isolates partners from each other, and deafens them so that they cannot hear the cries of their other children” Barbara Rosof, The Worst Loss
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