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

Other Labs PT/PTT 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 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

Iron-Deficiency Anemia A nutrient deficiency of inadequate dietary iron The most common hematologic disorder of infancy and childhood Peaks at 9 months- 2 years, adolescence Prevented by use of iron fortified productsn

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

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

Diagnosis Low RBC 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 symptoms plus irritability, lack of interest in play

Nursing Considerations Consume 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 May be ordered to take 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

Sickle Cell Anemia Autosomal recessive disorder Seen in 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 Can be diagnosed in-utero Symptoms may not appear until 6 months of age Mortality rate children < 3 years old is 35%

Signs and Symptoms Pallor Fatigue SOB Irritability Jaundice

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

3 Complications of Sickle Cell Anemia 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 fever tachycardia pallor Late Signs abdominal pain back pain extremity pain First Crisis in infants Dactylitis (hand & foot syndrome) swelling of hands and feet joints may be warm & swollen

Prevent/Treat occulsions Adequate oxygenation Management Pain relief Prevent/Treat occulsions 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

Hydration Prevents and treats occlusion Push PO fluids IV hydration 1.5 to 2 times normal rate Risk for fluid overload Listen for crackles

Treat Sickling-Oxygenation Administer oxygen Maintain saturation of 95% or higher Semi-fowler’s position

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

Nursing Considerations Symptoms Chest pain Fever Cough Wheeze Tachypnea Analgesics Oxygen Hydration Incentive spirometry Antibiotics PRBC

Splenic Sequestration Sickled cells block the spleen Blood pools in spleen and/or liver and enlarges Pooled blood leads to a decrease in circulating volume Can lead to hypovolemic shock

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

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

Beta-Thalasemia Major (Cooley’s Anemia)

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 q 2-4 weeks Iron Chelation therapy Desferal (deferoxamine) SQ Splenectomy Cure isbone 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 Mod low Hgb Normal iron Normal ferritin Normal TIBC Inc Bilirubin Very low Hgb Increased iron

Bleeding Disorders

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

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

Hemophilia A

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

If Suspected ask about 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

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

Management of Bleeding Acute therapy Acute bleeding stopped by IV administration of factor 8 Pressure to laceration Prophylactic therapy PO factor 8 replacement on a regular schedule if frequently symptomatic (prior to surgery, dental work)

Parental Education Primary Goal is 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

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 Bleeding from gums Epitaxis Prolonged bleeding from cuts Excessive bleeding following surgery

Management Primary Goal: Injury Prevention Prophylactic therapy Replace dysfunctional factor in blood when bleeding with DDAVP

ITP (Immune Thrombocytopenic Pupura)

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

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

Management PLT transfusion (only a temporary solution) Injury Prevention Avoid when possible: administering intramuscular injections aspirin, aspirin-containing products, and nonsteroidal antiinflammatory medications (e.g., ibuprofen) taking temperatures rectally

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

Oncology

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 Treatment Surgical intervention Removing the entire cancerous tumor Most ideal and frequently used treatment method

Oncology Treatment destroy tumor cells by cause cell death Chemotherapy destroy tumor cells by cause cell death normal cells that have rapid growth are also affected, such as hair growth toxic side effects

Oncology Treatment Radiation therapy Least preferred treatment in children 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 Develops from a variety of agents thought to increase risk (virus, toxins, drugs) combined with genetics

Acute Lymphocytic Leukemia Occurs in children< 15 years old (peak 2-6 years old) Distorted and uncontrolled proliferation of immature WBC’s (lymphoblasts) Causes decreased RBC’s, platelets, and mature WBC’s production Invasion of body organs by rapidly increasing lymphoblasts

Signs and Symptoms Fever Bone or joint pain Bruising Lymphadenopathy CBC Changes Physical Exam Fever Bone or joint pain Bruising Lymphadenopathy Hepatosplenomegaly Decreased RBCs Decreased PLTs Very high WBC

Diagnosis Signs & symptoms CBC changes Confirmed with bone marrow aspiration (> 25% of lymphoblast cells present)

Management High dose chemotherapy administered for 2-3 years Returns blast cells in bone marrow to less than 5% Physical assessment findings are normal

Tumor Lysis Syndrome Metabolic emergency Lysis (dissolving or decomposing) of tumor cells 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) 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

Hodgkins Lymphoma

Hodgkins Lymphoma Neoplasm of the lymph tissue Affects adolescents to late 20’s Males > females Etiology unknown- infectious agent likely

Signs and Symptoms Begins as a single painless enlarged cervical node Spreads predictably to nonnodal sites spleen, liver, bone, marrow, lungs, mediastinum

Signs and Symptoms As cancer progresses Unexplained weight loss Unexplained fevers Night sweats

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

Non-Hodgkins Lymphoma

Non-Hodgkins Lymphoma Aggressive neoplasm of many lymph nodes No single origin Rapid in onset Affects younger children ages 5-15 Males > females Cause unknown-infectious agent likely

Signs and Symptoms Multiple enlarged painless lymph nodes Acute abdominal and chest pain, constipation, cramping Anorexia, weight loss

Signs and Symptoms As cancer progresses CNS symptoms, Headache Nausea Vomiting Mediastinal mass Petichaie Bruising Bone pain

Diagnosis and Treament Biopsy from bone marrow or lymph node Treatment Aggressive multi-agent chemo for 2 years Risk for tumor lysis syndrome May need 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- may have poorer progrosis

Retinoblastoma

Retinoblastoma Malignant tumor of retina Immature retinal cells become malignant Affects children 6 weeks of age to preschool age May be unilateral or bilateral

Signs and Symptoms Absent red reflex Whitish glow to pupil Strabismus Eye pain

Treatment Small retinoblastoma Cryosurgery Partial vision Large retinoblastoma and/or metastases brain/ 2nd eye Chemo Radiation Enucleation Eye prosthesis

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

Neuroblastoma

Neuroblastoma Solid tumor usually in abdomen Affects infants to pre-school age children Cancer cells arise from sympathetic nervous system called crest cells Embryologic cells of adrenal glands Etiology: unknown

Signs and Symptoms Depend on 65% of neuroblastomas extent of disease location of tumor 65% of neuroblastomas protuberant, firm, irregular abdominal mass that crosses midline

Signs and Symptoms impaired ROM and mobility pain & limping respiratory symptoms Management depends on the presence and extent of metastasis

Nephroblastoma

Nephroblastoma Malignant tumor of the kidneys Peak age 3-4 years Girls > boys Cause is unknown

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

Hematuria Hypertension Abdominal pain Fatigue Anemia Fever Other Signs & Symptoms Hematuria Hypertension Abdominal pain Fatigue Anemia Fever

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 Nephrectomy Chemotherapy Stage 3-5 Radiation DO NOT PALPATE ABDOMEN can rupture the tumor and cause spreading of cancerous cells State 1 and 2 Nephrectomy Chemotherapy Stage 3-5 Radiation

Bone Cancers

Osteosarcoma

Osteosarcoma Bone cancer from osteoblasts Affects adolescents Attributed to extremity injury or growth spurt 40-50% occur at distal femur and knee

Signs and symptoms Progressive pain at site of tumor Palpable mass & swelling Limping Limited range of motion Pathological fractures

Management Remove tumor, prevent spread of disease Combination of surgery & chemo Amputation my be necessary Limb salvage operation

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

Ewing’s Sarcoma

Ewing’s Sarcoma Highly malignant tumor in bone marrow Can present in any bone Spreads longitudinally through bone Affects older children and young adolescents

Signs and Symptoms Intermittent pain attributed to injury Swelling at tumor site Pain becomes constant Progresses into Weight loss Fever Increased sed rate Metastases is usually present at time of dx (lungs, bone, CNS, lymph nodes)

Treatment Surgery Multi agent chemo Radiation Risk for tumor lysis syndrome 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

Care of the Chronically Ill Child

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 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