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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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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) Together with the lymphocytes,

make up 75-90% of peripheral blood

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:

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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 Plt 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

Central lines (implanted ports) Intrathecal Steroids

Surgery Bone marrow and stem cell

transplantation

Intrathecal chemotherapy

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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 Induction Consolidation Delayed intensification Remission Maintenance

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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: tymor 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

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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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Lymphoma

Considered to be “soft tumor” 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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Lymphoma, cont.

Assessment findings: “just not themselves” Lymph node enlargement Changes in sensorium Electrolyte imbalance

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Treatment regimen for lymphoma Staging Chemotherapy Radiation NOT effective for

lymphoma

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Nursing considerations for children with leukemia & lymphoma Preparation for diagnostic testing Education of parents Insertion of central lines Imbalanced nutrition Disturbed body image Risk for infection with

immunosuppression Provide collaborative care

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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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Types and treatment

Embryonal (75%) Alveolar (25%) Treatment:

Surgery Radiation chemotherapy

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Neuroblastoma, cont.

Manifestations: May have altered bowel and

bladder function Weight loss, abdominal distention Dyspnea when tumor is

mediastinal Edema of face and neck with vena

cava syndrome Pancytopenia

Treatment of neuroblastoma Depends on the site Biopsy used for diagnosis and

staging NSE (enzyme in neural tissue)

may be elevated in blood Most responsive to treatment

under one year of age

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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 “leukoria”

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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Link to see!

http://www.youtube.com/watch?=UeVOKFgXv08

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Children and death

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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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