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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

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

Osteosarcoma

Most common bone malignancy in children

Early symptoms Pain in extremity “growing pains”

Cause unknown Rare before adolescence

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

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

Ewing sarcoma

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

Manifestations

Pain, soft tissue swelling Anorexia, fever, malaise with mets Diagnostic workup same as for

osteosarcoma Management

Chemo Surgery (decrease tumor bulk) Radiation

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

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

Diagnostics and treatment CT, BM aspiration and biopsy Renal function and liver function

tests as well Treated with chemo, surgery and

radiation

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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