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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 – – – – – • RBC – – – – • • • Neutrophils- phagocytosis Lymphocytes – T and B cell Monoocytes – phagocytosis, antigen Eosophils- allergen Basophils-inflammatory 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: 3.89-4.96 HgB: 10.2-13.4 Hct: 31.7-39.3% Sed: 1-8 WBC: 5,400-11,000 • • • • • • • Platelets: 206,000-403,000 Fe: 20-105 Ferritin: 47-110 TIBC: 240-508 PT: 10-11 sec PTT: 42-54 sec Bilirubin- less than 11.7 Anemia’s ) 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 – 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 24-32 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 Nursing Considerations • 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. Nursing Considerations • 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 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 1. Vaso-Occlusive Crisis 2. Acute Chest Syndrome 3. 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 Nursing Considerations • 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 Nursing Considerations • Know the Symptoms: – – – – – Irritability Pale Tachycardia Pain to LUQ Enlarged Spleen • Life Threateningget 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 1012 • Iron Chelation therapy – Desferal (deferoxamine) SQ • Splenectomy • Cure: bone marrow stem cell transplant – Estimated 70% do not find a suitable donor Nursing Considerations – 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 •Low RBC’s •Low HCT •Very low Hgb •Increased iron •Normal ferritin •Normal TIBC •Low RBC’s •Low HCT •Mod low Hgb •Normal iron •Normal ferritin •Normal TIBC •Inc Billirubin 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 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 Normal Platelets Very Low Platelets Elevated PT/PTT Normal PT/PTT Normal PT/PTT Oncology • Cancer in adults – 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 – 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) Management Chemotherapy in 3 stages For 2-3 years 1. Induction 2. Sanctuary 3. 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 Nursing Considerations • Children receiving chemotherapy; • Monitor for: – – – – Hyperuricemia Hyperkalemia Hyperphosphatemia Hypocalcemia Nursing Considerations • 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 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 Diagnosis • 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-widespread 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% 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 1. tumor confined to the kidney and completely removed surgically 2. tumor extending beyond the kidney but completely removed surgically 3. regional spread of disease beyond the kidney with residual abdominal disease postoperatively 4. metastases to lung (primary site), liver, bone, distant lymph nodes 5. bilateral disease Treatment • State 1 and 2 – Nephrectomy – Chemotherapy • Stage 3-5 – Nephrectomy – Radiation – Chemotherapy • 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 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 – Achieve and maintain normalization – 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: 1. 2. 3. 4. 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: 1. 2. 3. 4. 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? 1. 2. 3. 4. Promotion of skin integrity Promotion of hydration Promotion of nutrition Conserving energy 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: 1. Determine the extent of the tumor process and need for palliation 2. Help determine if treatment is needed 3. Determine if surgery is needed 4. 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: 1.Risk for disuse syndrome 2.Disturbed body image 3.Self-care deficit 4.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 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