Hematologic Disorders and Cancer

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Hematologic Disorders and Cancer

• ACC
• Susan 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 lt 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 gt 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 dont 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

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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 childs 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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Wilm's Tumor
(nephroblastoma)
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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
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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

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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
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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

• cats 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 childs death
• Grandparentsnot 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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The End