Hematologic-Oncology

Hematologic-Oncology

Common Hematologic Disorders in Children
Iron-Deficiency Anemia Sickle Cell Anemia Beta-Thalasemia Major (Cooley’s anemia) Hemophilia A Von Willebrand’s Disease ITP (Immune Thrombocytopenic Pupura)

Common Heme-Oncology Diseases in Children
Acute Lymphocytic Leukemia Hodgkin’s Disease Non-Hodgkin’s Lymphoma Retinoblastoma Neuroblastoma Nephroblastoma Osteogenic Sarcoma Ewing’s Sarcoma

Complete Blood Count WBC RBC Hgb Hct Platelet

CBC with Differential WBC Neutrophils- phagocytosis
Lymphocytes – T and B cell Monoocytes – phagocytosis, antigen Eosophils- allergen Basophils-inflammatory RBC MCV- volume MCH MCHC RCW- width Hgb Hct Platelet MPV

PT, PTT The prothrombin time (PT) test measures how long it takes for a clot to form in a sample of blood. Prothrombin is one of several clotting factors that are produced by the liver. The PT test evaluates the integrated function of these factors and the body’s ability to produce a clot in a reasonable amount of time. Because the reagents used to perform the PT test vary from one laboratory to another and even within the same laboratory over time, the normal values also will fluctuate.

Other Labs Sed Rate (ESR) Iron TIBC (Transferrin) Ferritin Bilirubin

Pediatric Laboratory Normal Values: Children age 2-12 Years
RBC: HgB: Hct: % Sed: 1-8 WBC: 5,400-11,000 Platelets: 206, ,000 Fe: Ferritin: TIBC: PT: sec PTT: sec Bilirubin- less than 11.7

Anemia’s Reduction of: number of red blood cells
) Types in Children: Reduction of: number of red blood cells the quantity of hemoglobin the volume of packed red Iron-Deficiency Anemia Sickle Cell Anemia Beta-Thalasemia Major (Cooley’s anemia

Iron-Deficiency Anemia
The most common hematologic disorder of infancy and childhood 9 months- 2 years, adolescence A nutrient deficiency of inadequate dietary iron Prevention: iron fortified products

Children at Risk low birth weight infants
infants born to mothers with iron deficiency anemia infants born with GI defects chronic blood loss in older children

Pathophysiology Dietary Fe is bloodstream binds to transferrin (TIBC) and is delivered to RBC in bone marrow, combines with other cells to make Hgb Unused dietary Fe is stored in intestinal epithelial cells as ferritin

Diagnosis Low RBC’s Low HGB Low HCT Low Iron High Transferrin (TIBC)
Mild ( < 10.2), Moderate (8-9), Severe (< 7) Low HCT Low Iron High Transferrin (TIBC) Low Ferritin

Symptoms Low Hgb=low O2 tissue perfusion Hgb of 10.2 or less
May seem asymptomatic, not noticed by caregiver Pallor/Pale mucous membranes (low hgb, not enough red color to skin) Poor muscle tone, decreased activity Fatigue Increased HR, RR Hgb < 9 Above plus irritability, lack of interest in play

History Dietary history usually shows abnormally high milk intake > 32 oz day in toddler Ask parents specific questions Begin the dietary history at the time the child awoke yesterday; include all activities and exactly what the child ate

Management Iron-fortified formula
Limit cow’s milk to oz/day for children >12 months Increase age-appropriate iron-rich foods and Vit C Fe supplements- Ferrous Sulfate

Nursing Considerations
Iron-Rich Foods Vitamin C Rich Foods Meats, fish, poultry Orange juice Vegetables Citrus fruits Dried fruits Strawberries Legumes Tomatoes Enriched grain products Broccoli Whole grain cereal Leafy Green vegetables Iron-Fortified Cereal Potatoes

Manage side effects of Ferrous Sulfate Nausea, Anorexia Constipation Abdominal distress Black stools. Give on an empty stomach if possible Monitor bowel movements and suggest increased fluid and fiber.

Monitor development, sleep, and activity/fatigue patterns. Monitor hemoglobin to measure effectiveness of therapy. Instruct families to keep Ferrous Sulfate locked and out of reach of children; poisoning is a serious risk.

Sickle Cell Anemia Autosomal recessive disorder, African Americans
Characterized by abnormal hemoglobin (HbS) Clinical manifestations caused by obstructions due to the sickled RBC’s and destruction of sickled and normal RBC’s

Sickle Cell Anemia Symptoms may not appear until 6 months of age
Mortality rate children < 3 yo is 15-35% Diagnosis: Amniocentesis, CVS, Newborn Screen

Initially: fever & anemia at 6 mos
Signs & Symptoms: Initially: fever & anemia at 6 mos Pallor Fatigue SOB Irritability Jaundice

Diagnosis Moderately low Hcb and Hct Normal Iron, TIBC, Ferritin
Elevated Billirubin

3 Sequalea of SCA Vaso-Occlusive Crisis Acute Chest Syndrome
Splenic Sequestration

Vaso-occlusive crisis
Severe, sudden onset of sickling where many new sickled cells pool in a vessel and cause pain and tissue hypoxia Caused by: infection, dehydration, anxiety, cold Most common from hypoxia secondary to rapidly destroyed RBC Lasts for hours to weeks

Vaso-occlusive Crisis
Early Signs: pallor, tachycardia, fever Late Signs: acute abdominal, back, extremity pain First Crisis in infants: Dactylitis (hand & foot syndrome) swelling of hands and feet joints may be warm & swollen

Management Pain relief Adequate hydration Adequate oxygenation

Pain Assess pain every 1-2h or more frequently
Use pain scale appropriate for age Non-pharmacological pain methods AROUND THE CLOCK PAIN MEDS Tylenol for mild pain Narcotics for mod-severe pain

Prevent Occlusion Push PO fluids
IV hydration: 1.5 to 2 times normal rate Risk for fluid overload

Altered Tissue Perfusion and Prevent Further Sickling
Administer oxygen to maintain saturation of 95% or higher Pulse oximetry Semi-fowler’s position Administer PRBC’s

Acute Chest Syndrome Sickle contents break off
Bilateral pulmonary involvement Causes chest infection, embolism

Know the symptoms: Chest pain Fever Cough Wheeze Tachypnea Analgesics Oxygen Hydration Incentive spirometry, Antibiotics PRBC

Splenic Sequestration
Sickled cells block the spleen pooled blood in spleen and/or liver and enlarges Pooled blood leads to a decrease in circulating volume= hypovolemic shock CVA => coma

Know the Symptoms: Irritability Pale Tachycardia Pain to LUQ Enlarged Spleen Life Threatening- get child to ED a.s.a.p.! PRBC Remove spleen

Risk for Infection r/t Chronic Immunosuppression
Administer PCN everyday Up-to-date vaccines Educate parents: s/s infection & respiratory distress possible triggers treat pain immediately adequate fluids

Beta-Thalasemia Major (Cooley’s anemia)
Hereditary anemia due to abnormal synthesis of hemoglobin Life long disorder Mediterranean descent Life threatening symptoms

Diagnosis Low RBC’s Extremely low Hgb < 5 Increased serum iron

Symptoms Facial anomalies
Frontal bossing (prominent and protruding forehead) Maxillary prominence Wide-set eyes with a flattened nose Bronze skin color (Greenish yellow skin tone) Growth and maturation retardation

Management: RBC transfusions q2-4 weeks to get Hgb to 10-12
Iron Chelation therapy Desferal (deferoxamine) SQ Splenectomy Cure: bone marrow stem cell transplant Estimated 70% do not find a suitable donor

Observe for complications of transfusion- iron overload Supporting the child and family in dealing with a chronic life-threatening illness Monitor Growth and Development Refer the family for genetic counseling.

Compare and Contrast Iron Deficiency Sickle Cell Thalasemia Low RBC’s
Low HCT Low Hgb Low iron Low ferritin High TIBC Mod low Hgb Normal iron Normal ferritin Normal TIBC Inc Billirubin Very low Hgb Increased iron

Bleeding Disorders Hemophilia A Von Willebrand’s Disease
ITP (Immune Thrombocytopenic Pupura)

Clotting Host of factors Platelets aggregation at site of injury
Tested by coagulation time (PT/PTT)

Hemophilia A Hereditary blood coagulation deficiency (factor 8)
Ability to clot is slower X-linked recessive (white, males)

Symptoms Vary according to concentration of factor 8
Soft tissue bleeding and painful hemorrhage into joints Severe bleeding may occur in GI tract, peritoneum or CNS

Interviewing the Child with Hemophilia–Subjective Data
Recent traumas and measures used to stop bleeding Length of time pressure was applied before bleeding subsided Whether swelling increased after surface bleeding subsided Whether swelling and stiffness occurred without apparent trauma

Diagnosis Above History Suspected by Labs:
Platelet level: Normal PTT: Prolonged (elevated number) > 60 Confirmed by genetic testing for missing factor

Management of Bleeding
Acute therapy: Bleeding must be controlled by IV administration of factor 8 After trauma, surgery Pressure to laceration Prophylactic therapy: Children age 1-2 receive PO factor 8 replacement on a regular schedule if frequently symptomatic prior to surgery, dental work

Parental Education Primary Goal: Injury Prevention
Promote oral hygiene, up to date immunizations No aspirin Avoid activities that induce bleeding Provide activities for normal G&D Administration of factor replacement prn

Von Willebrand’s Disease
Most commonly inherited bleeding disorder, autosomal dominant (Males and Females) Lacks production of VWF Platelets are normal in number Inability of platelets to aggregate Varying degrees of disease VWF is deficient to defective

Diagnosis Platelets is normal PT/PTT is normal
Confirmed by genetic testing for VWF

Can be so mild that disease is undiagnosed
Signs & Symptoms Can be so mild that disease is undiagnosed Epitaxis Prolonged bleeding from cuts Excessive bleeding following surgery Bleeding from gums

Management Prophylactic therapy -Replace dysfunctional factor in blood
Treatment of Choice: DDAVP Injury Prevention

ITP (Immune Thrombocytopenic Pupura)
Autoimmune disorder (antiplatelet antibody) or cause is unknown (idiopathic) Occurs most commonly at age 2-4 years Reduction in and destruction of platelets Typically seen 2 weeks after a febrile, viral illness

Signs & Symptoms Excessive bruising and petechiae Epitaxis
Bleeding into joints Tourniquet test: shows many petechiae after inflation of BP cuff

Diagnosis Labs: Platelets < 150 (Marked thrombocytopenia)
PT and PTT: Normal

Management Prednisone IVIG (IV immunoglobulin)
PLT transfusion (only a temporary solution) Most cases are self-limiting Avoid when possible: administering intramuscular injections aspirin, aspirin-containing products, and nonsteroidal antiinflammatory medications (e.g., ibuprofen) taking temperatures rectally perform invasive procedures with extreme caution

Compare and Contrast Hemophilia A VWF ITP Normal Platelets
Elevated PT/PTT Normal PT/PTT Very Low Platelets

Oncology Cancer in adults Cancer in children
abnormal cell is transformed by genetic mutation of its DNA usually as a result from exposure to a tetragon Cancer in children usually arises from chromosomal abnormalities, genetic mutations and proliferation of embryonic cells

Oncology Treatments Chemotherapy
antineoplastic agents attempt to destroy tumor cells by interfering with cellular functions and reproduction cytotoxic drugs that are designed to cause cell death. Normal cells that have rapid growth are also affected, such as hair growth. Toxic side effects

Oncology Treatments Surgical intervention Radiation therapy
removing the entire cancerous tumor (most ideal and frequently used treatment method) Radiation therapy interrupt cellular growth by breaking the DNA stands, leading to cell death.

Types of Cancer in Children
Small percentage Carcinoma (opposed to large percentage in adults) Mostly Leukemia Followed by Lymphoma The rest is solid or soft tissue tumors

Clinical Manifestations
Differ based on type of cancer Many symptoms are similar to common childhood illnesses Symptoms may be in site other than the cancer =delay in diagnosis Often diagnosis made when cancer is advanced

Common Clinical Manifestations
Pain Anemia Anorexia, weight loss Infections Bruising Neurological symptoms Palpable mass

Psychosocial Concerns
Parents in disbelief Health child suddenly becomes ill Potentially life-threatening Treatment decisions, can last months-years Travel for treatment, heavy financial responsibilities Effects of siblings

Effects on Child Infants- unaware of diagnosis
Toddlers- aware they do not feel well Preschoolers-beginning understanding of illness, not cancer School-age-understand cancer, benefit from talking about it Adolescents-mature understanding, benefits from other adolescents with cancer

General Nursing Considerations
Provide optimal nutrition- high metabolic rate of cancer depletes stores Ensure adequate hydration-ice pops, jello Manage pain Promote growth and development Prevent Infection (next slide)

Risk for infection r/t Immunosuppressed state.
Monitor vital signs q4h Instruct parents how to measure temp at home Proper handwashing Inspect child’s skin for breakdown Inspect child’s mouth for ulcers Teach child and parents meticulous oral hygiene No live virus administration

Leukemia

Leukemia Broad term describing a group of malignant diseases
Normal Bone Marrow is replaced by abnormal immature cells Etiology: variety of agents thought to increase risk (virus, toxins, drugs) combined with genetics Two forms of leukemia ALL: Acute Lymphocytic Leukemia AML: Acute Myelogenous Leukemia

Acute Lymphocytic Leukemia
Most frequently occurring type of cancer in children < 15yo (peak 2-6) Distorted and uncontrolled proliferation of immature WBC’s (lymphoblasts) Causes decreased RBC’s, platelets, and mature WBC’s production and invasion of body organs by rapidly increasing lymphoblasts

Signs & Symptoms: Fever Bone or joint pain Bruising Decreased RBC’s
Decreased PLT’s Abnormal high WBC counts Lymphadenopathy Hepatosplenomegaly CNS invasion

Diagnosis: Based on: Signs & symptoms CBC changes
Bone marrow aspiration (> 25% of lymphoblast cells present)

Chemotherapy in 3 stages
Management Chemotherapy in 3 stages For 2-3 years Induction Sanctuary Maintenance

Induction 1st month; aim is to induce remission (blast cells to < 5%, normal Physical Findings) Approximately 95% of children achieve remission within 1 month

Sanctuary or Consolidation
Begins after remission, 4 weeks Goal: to maintain remission prevent disease from invading sanctuary sites

Maintenance goal to maintain remission
eliminate residual leukemic cells combination of drugs, outpatient basis girls treated for 2 years, boys for 3 Cure: free of disease for 4-5 years

High Doses of Chemotherapy Can Lead to:
Tumor Lysis Syndrome Metabolic emergency results from the lysis (dissolving or decomposing) of tumor cells and rapid release of their contents into the blood

Tumor lysis syndrome Rapid cell destruction releases high levels of
uric acid potassium phosphates Uric acid overloads the kidneys Leads to cardiac arrhythmias and renal failure

Children receiving chemotherapy; Monitor for: Hyperuricemia Hyperkalemia Hyperphosphatemia Hypocalcemia

Administer vigorous hydration (2–4 times rate for maintenance fluid) Administer allopurinol or urate oxidase (rasburicase) to reduce conversion of metabolic by-products to uric acid

Hodgkin's Non Hodgkin's Retinoblastoma
Soft Tissue Tumors Hodgkin's Non Hodgkin's Retinoblastoma

Lymphomas A malignancy that arises from the lymphoid system Two types:
Hodgkins Non Hodgkins

Hodgkin’s Disease Neoplasm of cervical lymphatic tissue
Starts in a single or group of lymph nodes then spreads predictably to nonnodal sites such as: spleen, liver, bone, marrow, lungs, mediastinum Affects adolescents to late 20’s Males > females Etiology unknown- infectious agent likely

Signs & Symptoms: Painless enlarged cervical node
Unexplained weight loss, unexplained fevers, night sweats

Diagnosis and Treatment
Biopsy of enlarged lymph node Staged 1-4 Treatment Chemotherapy Radiation-low doses, higher is physiologically mature Good Prognosis-single origin

Non-Hodgkin’s Lymphoma
No single origin Males > females Cause unknown Aggressive proliferation of B or T lymphocytes in lymph nodes Rapid in onset (ages 5-15) Usually found with wide-spread involvement via bloodstream (multiple enlarged nodes) Responds quickly to therapy

Signs & Symptoms: Acute abdominal and chest pain, constipation, cramping Anorexia, weight loss Painless enlarged lymph nodes found in cervical or axillary region Ascites and obstruction with vomiting a late sign Advanced disease: CNS symptoms, HA n/v, mediastinal mass, petichaie, bruising, bone pain

Diagnosis Biopsy from bone marrow or lymph node Staging 1-4 Treatment: Aggressive multi-agent chemo for 6 mos to 2 years Risk for tumor lysis syndrome Intrathecal chemo and crainal radiation

Compare and Contrast Hodgkins Non Hodgkins Males>Females
Late adolescent-20’s Single origin of cervical gland Good Prognosis Males > females ages 5-15 No single origin-wide-spread involvement Aggressive treatment

Retinoblastoma Malignant tumor of retina Inherited autosomal dominant
Immature retinal cells become malignant 6 weeks of age to preschool age Unilateral or bilateral

Signs & Symptoms Absent red reflex Whitish glow to pupil
Strabismus develops Eye pain Metastases to optic nerve, subarachnoid space, brain, 2nd eye

Retinoblastoma Treatment: If small: cryosurgery, partial vision
If mets: chemo & radiation If large: enucleation, eye prosthesis 3 weeks post-op Survival rate 90%

Neuroblastoma Nephroblastoma Osteoscaroma Ewing’s Sarcoma
Solid Tumors Neuroblastoma Nephroblastoma Osteoscaroma Ewing’s Sarcoma

Neuroblastoma Solid tumor of infants and pre-school children (peak 22mos) Cancer cells arise from sympathetic nervous system called crest cells Embryologic cells of adrenal glands Etiology: unknown

Signs & Symptoms Depend on:
extent of disease location of tumor 65% present with protuberant, firm, irregular abdominal mass that crosses midline

Neuroblastoma Other manifestations: impaired ROM & mobility
pain & limping large abdominal mass respiratory symptoms if chest tumor

Neuroblastoma Diagnosis: Chest x-ray CT scan of abdomen, pelvis, spine
Bone marrow aspiration Management: depends on the presence and extent of metastasis

Wilm’s Tumor (Nephroblastoma)
Malignant tumor of the kidneys Peak age 3-4 years Girls > boys Cause is unknown Other GU problems Occurs in asymptomatic child May have genetic predisposition Is associated with congenital anomalies

Nephroblastoma Parents usually notice a large, mobile abdominal mass while bathing or the diaper doesn’t fit anymore Grows extremely quickly, in a matter of days DO NOT PALPATE ABDOMEN can rupture the tumor and cause spreading of cancerous cells

Other Signs & Symptoms microscopic to gross hematuria hypertension
abdominal pain fatigue, anemia, fever

Diagnosis Suspected from a good history CT scan
Definitive dx made at time of surgery Staged 1-5

Staging 1 through 5 tumor confined to the kidney and completely removed surgically tumor extending beyond the kidney but completely removed surgically regional spread of disease beyond the kidney with residual abdominal disease postoperatively metastases to lung (primary site), liver, bone, distant lymph nodes bilateral disease

Treatment State 1 and 2 Stage 3-5 Nephrectomy Chemotherapy Radiation
Survival rates are good (up to 90%)

Bone Cancers

Osteogenic Sarcoma (Osteosarcoma)
Most common bone malignancy in children (teenage years) Occurs in distal long bones Attributed to extremity injury or growth spurt Originates from bone producing cells 40-50% occur at distal femur and knee

Signs & symptoms Progressive, insidious or intermittent pain at site of tumor Palpable mass & swelling Limping, progressive limited range of motion Pathological fractures

Diagnosis X-ray, CT, MRI Tumor biopsy Look for chest metastases

Cure rate: 60-65% without overt metastases
Management Remove tumor, prevent spread of disease Combination of surgery & chemo Amputation my be necessary Limb salvage operation Cure rate: 60-65% without overt metastases

Nursing care Comfort Infection Potential hemorrhage Phantom limb pain
Prosthesis Changes in body image and functioning

Ewing’s Sarcoma Highly malignant tumor in bone marrow of long bones
Can present in any bone Spreads longitudinally through bone Affects young adolescents and older children Metastases is usually present at time of dx (lungs, bone, CNS, lymph nodes)

Signs & Symptoms Intermittent pain attributed to injury
Swelling at tumor site Pain becomes constant Progresses into Weight loss Fever Increased sed rate

Diagnosis: Bone scan Bone marrow aspiration & biopsy CT of lungs Definitive dx: biopsy of tumor site

Treatment: Surgery Multi agent chemo Radiation

Compare and Contrast Osteogenic scaroma Ewing’s Sarcoma
Affects long bones Older adolescents Intermittent pain Palpable mass & swelling Limping, progressive limited range of motion Pathological fractures Metastases not as likely Surgery and chemo Affects any bone School-age and adolescents Intermittent pain becomes constant Swelling at tumor site Progresses into systemic symptoms Metastases likely Aggressive treatment

Chronically Ill Child Nursing Diagnosis
Fear Death Anxiety Anticipatory Grieving Hopelessness

Goals for Care of the Chronically Ill Child
Goals for the child Achieve and maintain normalization Obtain the highest level of health and function possible Goals for the family Remain intact Maximize function throughout the illness

Nursing Care for Children with Chronic Conditions and Their Families
Attend to the needs of the family system Revise goals frequently to meet the child’s changing developmental needs Listen carefully to the child's perception of the condition

Nursing Care for the Dying Child and the Child’s Family
Be available to assist both child and family Avoid imposing personal beliefs and expectations Provide time and attention to the dying child Recognize the need to talk about illness and death Provide adequate pain control, oral care, privacy, and information about the signs of imminent death After death, allow family members as much time as they desire with the child

Practice Questions!

A child is being admitted to the unit with thalassemia major (Cooley’s anemia). In preparing client assignments, the charge nurse wants to assign a nurse to this child who can: Teach dietary sources of iron Administer blood infusions Work with a dying child Monitor the child for bleeding tendencies

A 14-year-old boy with sickle cell anemia is admitted with severe pain in his abdomen and legs. He asks why the doctor ordered oxygen when he is not having any breathing problems. The nurse states the therapeutic action of O2 is: Prevent further sickling Prevent respiratory complications Increase O2 capacity of RBCs Decrease the potential for infection

A 10-year old in the ER has a CBC results that include a Hgb of 8, and Hct of 24. The nurse determines that based on the lab results which nursing action has a high priority? Promotion of skin integrity Promotion of hydration Promotion of nutrition Conserving energy

A 4-year-old is diagnosed with ALL
A 4-year-old is diagnosed with ALL. Following teaching about the staging and therapy, the nurse evaluates the family’s understanding of the problem. The statement by the family that indicates appropriate knowledge is “Staging will: Determine the extent of the tumor process and need for palliation Help determine if treatment is needed Determine if surgery is needed Determine the extent of malignant process and stage the leukemia

A 17-year old is being admitted for an amputation related to a bone tumor. The nurse is developing a nursing care plan and determines the most appropriate age related diagnosis is: Risk for disuse syndrome Disturbed body image Self-care deficit Activity related intolerance

The nurse is reviewing the lab work on a child admitted for fatigue
WBC 7,200 RBC3.01 Hgb 9.1 Hct 29.3 Platelets 371,000 Iron 64 Ferritin 70 Transferrin 250 Bilirubin 18.2 PTT 45 seconds

After analyzing the results, the nurse suspects the child may have:
1. Fe Deficiency Anemia 2. Cooley’s Anemia 3. Sickle Cell Anemia 4. Aplastic Anemia

The nurse is admitting a child for a swollen elbow
The nurse is admitting a child for a swollen elbow. The history indicated multiple bruising. Which of the following laboratory results heightens the nurses suspicion for Hemophilia? 1. Hbg 12,000 2. WBC 9,000 3. Platelets 356,000 4. PTT 73 seconds

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