Hematological/ Oncological
Erythrocytes (Red Blood Cells)
- Carries oxygen - Typically live & circulate for 100-120 days - Hemoglobin (Hgb) is the oxygen-carrying protein molecule of the RBC .....Requires circulating iron
Hemophilia symptoms
- Spontaneous hemorrhage can happen internally or externally - Male newborn hemorrhage after circumcision or separation of umbilical cord!!! - Infant mouth or frenulum bleeding, gingival oozing - IM vaccine injections resulting in large hematomas - Minimal lacerations causing prolonged bleeding - Excessive bruising!!! - Joint hematomas (hemarthroses) ....Appear when children become mobile .....Pain & swelling of elbows first, then shoulders, wrists, hips, knees, & ankles Hematuria!!! Epistaxis GI bleed Intracranial hemorrhage - Cerebral, subdural, subarachnoid, epidural - Leads to mental retardation, seizures, motor impairment
Hemostasis
- The clotting of blood. - Prevents the spread of infection by trapping microorganisms & foreign bodies. - Stops bleeding. Provide a framework for future repair & healing. - Interaction between platelets, fibrin, and coagulant factors.
Tumor Lysis Syndrome (TLS)
-oncologic emergency with rapid lysis of malignant cells -usually the result of chemotherapy or sometimes radiation -may occur 24 hours-7 days after antineoplastic therapy is initiated -develops when chemotherapy or irradiations causes the destruction of a large number of rapidly dividing malignant cells -intracellular contents are rapidly released into the bloodstream
A child is diagnosed with iron deficiency anemia. What will the nurse see as common clinical presentation findings? Irritability, tachycardia, brittle and concave nails Increased serum concentration of the proteins albumin, gamma globulin, and transferrin Anorexia, bradycardia, and systolic murmur Normal growth, spastic muscle tone, and delayed learning
1
A child with a vegan diet is diagnosed with iron-deficiency anemia. What iron-rich foods will the nurse suggest the parents add to child's diet? Dark leafy vegetables, fish, lean beef Poultry, fortified cereals, fish Organ meat, shellfish, legumes Legumes, fortified cereals, molasses
1
Long term side effects of chemo and radiation
Anthracyclines - Cardiotoxicity Alkalinizers - Secondary malignancies Radiation - Significant impairment of intellectual & physical development ....Risk of secondary malignancies
Average platelet count
150,000-400,000
A 10-year-old child is admitted to the hospital with a suspected brain tumor and is scheduled for a magnetic resonance imaging (MRI) in the morning. What should the nurse include in the child's teaching? "The MRI will explain the type of abnormal growth." "The MRI will locate the exact place that is causing you to be sick." "The MRI room is hot and noisy during the study." "The next test will differentiate the cells and help with future treatments."
2
An adolescent asks the nurse what a biopsy will do for a new growth in the abdomen. What is the nurse's best response? "The biopsy will help confirm the cancer type of cells and treatment needed." "The biopsy will determine the type of benign or malignant tumor." "The biopsy will identify if a central intravenous line is needed." "The biopsy will determine if antibiotics are needed."
2
ALL relapse
20% of children with ALL will relapse - Relapse sites include bone marrow, CNS, testes - Early relapse < 18 months since therapy initiated has grim prognosis - 1/3 of those who relapse will go on to have long-term remission Relapse Therapies Blinatumomab (monoclonal antibody) Inotuzumab (monoclonal antibody) Rituximab (monoclonal antibody) HSCT (hematopoietic stem cell transplant)!!!! CAR-T: engineering autologous T-cells to express chimeric antigen receptors targeting leukemia cells - Exciting, potentially curative therapy in refractory of multiple relapsed pre-B-cell ALL - FDA Approved: tisagenlecleucel
A 12-year-old male child is diagnosed with acquired thrombocytopenia (ATP) after an infection with Rocky Mountain spotted fever. The nurse understands that which diagnostic exam will identify ATP? Partial thromboplastin time: 28 seconds Total iron-binding capacity values: 280 mg/dL Platelet count: 80 x 103/mcL Hemoglobin: 13.5 g/dL
3
A patient is diagnosed with a terminal brain tumor. What teaching should the nurse include with the recommended palliative treatments? The chemotherapy will shrink the brain tumor. The child will not have pain as therapy continues. Radiotherapy will shrink the brain tumor. There are no side effects with radiation therapy.
3
The nurse is admitting a child after a trauma with a history of acquired thrombocytopenia (ATP). What nursing care is important to prevent further bleeding? Encourage mouth care with a firm toothbrush to prevent plaque buildup. Perform suctioning of the oral airway. Administer prescribed steroids. Maintain bed rest.
3
The nurse is discharging a child home after a sickle cell crisis. What statement indicates the parents need further teaching? "We will talk to the dentist about any planned dental care visits. " "We will contact the healthcare provider if the child has a fever." "We will report any signs of a respiratory infection immediately to prevent recurrent crises." "If my child has a headache, I will let him rest."
4
Non-Hodgkin lymphoma (NHL)
500 children & 400 adolescents diagnosed each year - Affects more males - Occurs in second decade of life - Associated with immune system dysfunction, EBV & CMV 25% present with mediastinal mass - Respiratory symptoms from obstruction (cough, dyspnea, orthopnea), SVC syndrome Most common site of BL origin: GI tract - Acute abdomen: abdominal pain, distention, fullness, nausea, vomiting constipation Nontender lymph node enlargement Evaluation - Biopsy - CBC, CMP, LDH, Uric Acid, TLS labs - Bone marrow aspiration, LP - CXR, CT of head, neck, abdomen, pelvis, MRI, PET Treated with chemotherapy Treated with radiation if CNS involvement - Prognosis: 85% 5 year survival
The nurse on the pediatric floor is reviewing her 4-year-old female patient's chart. Laboratory results show decreased leukocytes, platelets, and erythrocytes. The history states the patient was admitted for a complaint of "chicken pox that will not go away." What diagnosis are these findings consistent with?
Aplastic anemia
hydrocephalus and CNS tumors
Half of infratentorial tumors have associated hydrocephalus Causes increased intracranial pressure (ICP) - Lethargy - Vomiting - Irritability Less prominent in infants as cranial sutures remain open ....Skull can accommodate for increased ventricular size bc the fontanelles aren't closed yet
Infection and SCA
High risk for sepsis - S. pneumoniae - H. influenza - N. meningitidis - Parvovirus B19 PCN ppx starts at 2 months of age Immunized with HiB & PCV13, PPsV23 after 24 months. Annual Flu, now COVID. If febrile (temp > 38 C).. 100.4 F, must have undergo septic w/u CBC Blood Cx Urine Cx LP if sx meningitis Hospitalization for infants & ill appearing children CXR to evaluate for Acute Chest Syndrome
A nurse is teaching the parent of a child who has a neuroblastoma. Which of the following statementsshould the nurse include in the teaching? (Select all that apply.) A. "Chemotherapy and radiotherapy may be necessary for treatment." B. "Your child will need a bone marrow biopsy." C. "Your child will be paralyzed because of this tumor." D. "Most children are diagnosed around age 12." E. "Your child will need surgery for resection of the tumor."
A, B, E
A nurse is caring for a child who is experiencing neuropathy due to chemotherapy. Which of the following are manifestations of neuropathy? (Select all that apply.) A. Constipation B. Skin breakdown C. Foot drop D. Jaw pain E. Hemorrhage cystitis
A. CORRECT: Constipation is a manifestation of neuropathy. C. CORRECT: Foot drop is a manifestation of neuropathy. D. CORRECT: Jaw pain is a manifestation of neuropathy.
A nurse is providing teaching about epistaxis to the parent of a school‑age child. Which of the following should the nurse include as an appropriate action to take when managing an episode of epistaxis? (Select all that apply.) A. Press the nares together for at least 10 min. B. Breathe through the nose until bleeding stops. C. Pack cotton or tissue into the naris that is bleeding. D. Apply a warm cloth across the bridge of the nose. E. Insert petroleum into the naris after the bleeding stops.
A. CORRECT: Pressing the nares together for at least 10 min is an appropriate action to take when managing an episode of epistaxis. C. CORRECT: Packing cotton or tissue into the naris that is bleeding is an appropriate action when managing an episode of epistaxis.
A nurse is caring for a child who has thrombocytopenia. Which of the following actions should the nurse take? (Select all that apply.) A. Monitor for signs of bleeding. B. Administer routine immunizations. C. Obtain rectal temperatures. D. Avoid peripheral venipunctures. E. Limit visitors.
A. CORRECT: The child who has thrombocytopenia is at risk for hemorrhage. Monitoring for findings of bleeding is an appropriate action for the nurse to take. D. CORRECT: The child who has thrombocytopenia is at risk for bleeding. Avoiding venipunctures is an appropriate action for the nurse to take.
A nurse is providing teaching about the management of epistaxis to a child and his family. Which of the following positions should the nurse instruct the child to take when experiencing a nosebleed? A. Sit up and lean forward. B. Sit up and tilt the head up. C. Lie in a supine position. D. Lie in a prone position.
A. CORRECT: The nurse should instruct the child to sit up and lean to prevent aspiration when experiencing a nosebleed.
A nurse is assessing a child who has rhabdomyosarcoma of an extremity. Which of the following areclinical manifestations of this condition? (Select all that apply.) A. Pain B. Discoloration of the skin C. Lymph node enlargement D. Moveable mass E. Palpable mass
A. Pain is a clinical manifestation of rhabdomyosarcoma of an extremity. C. Lymph node enlargement is a clinical manifestation of rhabdomyosarcoma of an extremity. E. Palpable mass is a clinical manifestation of rhabdomyosarcoma of an extremity.
A nurse is assessing a child who has rhabdomyosarcoma of the nasopharynx. Which of the following areclinical manifestations of this disorder? (Select all that apply.) A. Enlarged neck lymph nodes B. Pain C. Vomiting D. Epistaxis E. Diplopia
A. Palpable neck lymph nodes is a clinical manifestation of rhabdomyosarcoma of thenasopharynx. B. Pain is a clinical manifestation of rhabdomyosarcoma of the nasopharynx. D. Epistaxis is a clinical manifestation of rhabdomyosarcoma of the nasopharynx.
Acute lymphoid leukemia vs Acute myeloid leukemia
ALL - More common, accounts for 75% of cases - Proliferation of lymphoblasts - Younger age of presentation, typically diagnosed between 1-4 year of age - Presents with mild fever, mild bleeding, bone pain & pathologic fractures - Often has Tumor Lysis Syndrome (TLS) with presentation or initiation of treatment - Typically treated with chemotherapy & radiation - Good prognosis, 80% survival - Relapse rate is 20%, only 1/3 of those will achieve long-term remission - Hematopoietic stem cell transplantation (HSCT) for relapsed ALL Acute non-lymphoid leukemia/acute myeloid leukemia (ANLL/AML) - Rarer than ALL, accounts for 20% of cases - Proliferation of myeloblasts - Age of dx ranges from 1-16 years - Presents with fever, bone pain, life-threatening leukocytosis & pancytopenia - Life threatening bleeding, unlikely to have TLS - Very high risk of infection: sepsis, fungal infections - Treated with the most toxic therapies including multiagent chemotherapy & HSCT - Often need prolonged hospitalizations with isolation precautions, TPN for nutrition, frequent blood product transfusions - Poorer prognosis, 50-60% survival
leukocytosis
Abnormally high WBC count... this happens in acute leukemia because of from proliferation of lymphoblasts or myeloblasts
Neutropenia
Absolute Neutrophil Count - Tells body how well body fights infection - Normal > 1,000 - Abnormal < 1,000 - Neutropenic < 500!!! Maintain integrity of mucous membranes & skin (HRF mouth ulcers) Ensure adequate nutrition & fluids Avoid sick contacts Maintain personal hygiene Get adequate rest Patients must report signs/symptoms of infection ASAP If febrile, admitted to the hospital for F + N
Acute Myelogenous Leukemia (AML)
Accounts for 20% of leukemia diagnosed in children - Secondary AML due to prior anthracycline chemotherapy exposure & radiation therapy Poorer prognosis compared to ALL Females do better than males Older children do better than infants Caucasians do better than non-white patients Improved outcome with initial WBC < 20 109/L Worse outcome with initial WBC > 100 109/L Long term survival 50-60% Treatment Multiagent chemotherapy HSCT Most toxic therapies in childhood cancer
Iron deficiency anemia cont.
Accounts of half of anemia cases in early childhood Most commonly due to: Cow's milk protein intolerance Poor diet Menstruation Chronic blood loss Evaluation CBC w/ Differential ....Low Hgb < 12 g/dL, Low Hct, Microcytic (low MCV), Hypochromic (low MCH) Elevated RDW (early marker of Fe def) Low Serum Ferritin: Reflects current iron stores, acute phase reactant, increased with inflammatory conditions. Elevated Total iron-binding capacity (TIBC)
Jenai was recently bit by a tick while on a hike. She is now exhibiting signs and symptoms of petechiae, blood in her stools, and frequent and spontaneous nosebleeds. Her blood work reveals a decrease in platelet counts. What disorder does Jenai have?
Acquired thrombocytopenia
Acute vs chronic anemia
Acute Acute Blood Loss - Trauma - GI bleed - Coagulopathy Hemolytic Anemia - Mechanical destruction of RBCs, rapid fall in Hgb with increased retic count Symptomatic - Tachycardia, pallor, irritability/poor PO intake, lethargy Symptoms of bleeding - Hematemesis, hematochezia, epistaxis, heavy menses - Underlying coagulopathy Symptoms of hemolysis - Jaundice, hepatosplenomegaly, scleral icterus Chronic May be asymptomatic May have subtle symptoms Related to body's compensatory abilities No tachycardia May be related to Iron Deficiency Anemia May be related to chronic hemolysis (stable Hgb, high retic count) May be related to anemia of chronic disease - Infection - Inflammation - Malignancy - Mild anemia
Aplastic crisis
Acute drop in Hgb level caused by transient arrest of erythropoiesis & increased destruction of RBCs - Profound anemia, baseline Hgb decreased by more than 2 g/dL Leads to markedly reduced reticulocyte count .....Reticulocytopenia can last 2-14 days Symptoms include: fatigue, pallor, SOB, dizziness Typically caused by viral infection, most commonly parvovirus B19 Treatment: - Requires hospitalization - PRBC transfusion
A 4 year old presents for her WCC. Family states they noticed she has lost weight over the past few months. She is usually very active and has lots of bumps and bruises. However, she seems to be more tired than usual. She recently started complaining about leg pain. Her PCP obtains a CBC, notable for anemia, leukocytosis, neutropenia, and thrombocytopenia. What oncologic condition does she likely have?
Acute leukemia
Treatment of tumor lysis syndrome
Aggressive hydration - Hyperhydration with 2x MIVF Urine alkalinization with sodium bicarbonate Diuretics to promote increased UOP Hyperuricemia Tx: Allopurinol Rasburicase Hyperkalemia Tx: Remove K from IVF IV loop diuretics Insulin/Dextrose Kayexalate Hyperphosphatemia Tx: Phosphate binders Hypocalcemia Tx: Calcium gluconate in symptomatic patients (seizure, tetany)
HSCT and AML
Allogenic HSCT recommended for high-risk AML & AML without remission following induction chemotherapy Standard risk AML receives HSCT if AML relapse & 2nd remission achieved - Prognosis for relapsed AML patients is poor - Chemotherapy alone results in < 10% survival at 4 years - Improved survival with HSCT
Types of lymphoma
Hodgkin's Lymphoma Non-Hodgkin's Lymphoma - Diverse group of solid tumors of the lymphatic tissues that form from malignant proliferation of T cells, B cells, or intermediate lymphocyte cells Burkitt lymphoma Diffuse large B-cell lymphoma T and B Lymphoblastic lymphoma Anaplastic large-cell lymphoma
Aplastic vs hemolytic anemia
Aplastic anemia.... Inherited or acquired Pancytopenia (no wbc, rbc or platelets being created... very bad) Hypocellular bone marrow Risk of life-threatening infections & bleeding May need chronic PRBC transfusions Hematopoietic cell transplantation is the only curative treatment for bone marrow failure Hemolytic anemia.... Inherited or acquired Premature destruction of RBCs Increased marrow production of reticulocytes No obvious bleeding PRBC transfusions for severe anemia Folic Acid supplementation Splenectomy for severe cases
Long term effects of FE deficiency anemia
Associated with cognitive deficits Comorbidity with lead poisoning Lead poisoning screening at 9-12 months and 24 months Blood lead levels > 5 mcg/dL are a public health concern
Acute Chest Syndrome (ACS)
Associated with vaso-occlusion of the lungs Caused by community-acquired pathogens (bacterial, viral) Presents with fever and/or respiratory symptoms!!! Symptoms include shortness of breath, tachypnea, chest pain, cough, wheezing, crackles!!! - Assess SpO2 - Obtain CXR to evaluate for pulmonary infiltrate (pneumonia) Requires hospitalization - can progress quickly to respiratory distress and respiratory failure - Administer O2, bronchodilators, incentive spirometry - May need intubation & mechanical ventilation Treatment: IV hydration IV analgesics IV antibiotics Consider PRBC transfusion or exchange transfusion to correct anemia, decrease Hgb S, increase oxygen binding capacity
Biomedical ethics
Autonomy Free choice Child cannot make choice Parent/guardian Proxy Beneficience Beneficial to patient Cure attained Nonmaleficence Not harming patient Chemo side effects Psychosocial effects Justice Distributing benefits & burdens proportionally For many cancers there are effective therapies
A nurse is assessing a child who has neuroblastoma of the adrenal gland. Which of the following areclinical manifestations of metastasis from the primary site? (Select all that apply.) A. Weight gain B. Bone pain C. Periorbital ecchymoses D. Proptosis E. Weight loss
B. A child who has metastatic neuroblastoma will report bone pain. C. A child who has metastatic neuroblastoma will have periorbital ecchymoses. D. A child who has metastatic neuroblastoma will have proptosis. E. A child who has metastatic neuroblastoma will have an ill appearance.
A nurse is assessing a child who has leukemia. Which of thefollowing are early manifestations of leukemia? (Select all that apply.) A. Hematuria B. Anorexia C. Petechiae D. Ulcerations in the mouth E. Unsteady gait
B. CORRECT: Anorexia is an early manifestation of leukemia. C. CORRECT: Petechiae is an early manifestation of leukemia. E. CORRECT: Unsteady gait is an early manifestation of leukemia.
A nurse is caring for an infant whose screening test reveals that he might have sickle cell disease. Which of the following tests should be performed to distinguish if the infant has the trait or the disease? A. Sickle solubility test B. Hemoglobin electrophoresis C. Complete blood count D. Transcranial Doppler
B. CORRECT: The hemoglobin electrophoresis test should be performed to distinguish if the infant has the trait or the disease
A nurse is preparing to administer iron dextran IM to a school‑age child who has iron deficiency anemia.Which of the following actions by the nurse is appropriate? A. Administer the dose in the deltoid muscle. B. Use the Z‑track method when administering the dose. C. Avoid injecting more than 2 mL with each dose. D. Massage the injection site for 1 min after administering the dose.
B. CORRECT: The nurse should use the Z‑trackmethod when administering the dose.
A nurse is caring for a toddler who has a Wilms' tumor. Which of the following should be included inthe plan of care? A. Abdominal palpation to identify the size of the tumor B. Preparation for surgery C. Teaching about dialysis D. Obtaining 24-hr urine specimen
B. Removal of the tumor occurs within 24 to 48 hr of admission. Therefore, preparationfor surgery should be included in the plan of care.
Sickle cell trait
Benign clinical course Hgb A + Hgb S (30-40% RBCs) Rare sickling except with hypoxia, shock, high altitudes Risk of sudden death in adolescents and young adult athletes Extreme exercise, exhaustion, dehydration, & hypoxia are leading causes Need genetic counseling ....Educating adolescents with trait about their status & risk of disease transmission
History of anemia
Birth history - early - neonatal complications GI disorder Growth Nutritional history - excessive milk intake - lack of green leafy veggies or red meat Physical changes - pallor - petechiae/bruising - extremity pain/swelling Behavioral changes - irrtable - fatigue - school issues Bone fractures Recent illness Bleeding - nosebleeds - gum - bloody urine - bloody stool Drug or toxin exposure Pica/ eating ice or dirt Family hx Traveling
Sickle cell demographics
One of the most common genetic disorders in children Detected on newborn screen!!! Autosomal recessive inheritance pattern Most common in African descent, as well as Mediterranean, Caribbean, Central & South America, & India - 1 in 600 African American infants has sickle cell anemia - Sickle cell trait occurs in 8% of African Americans Sickle cell is related to the evolution protecting individuals from malaria
Procedures
PIV insertion Bone marrow aspiration lumbar punctures Imageings Radiation Surgery
Chronic hematological disorders and growth/development
Chronic illness stunts growth - SCA height & weight velocity stalls in school aged children - Delayed puberty Children spending more time hospitalized that in school learning Risk of strokes can affect cognition & learning
Physical exam of anemia
Pale/pallor - Conjunctivae, buccal mucosa, palm, nailbeds Fatigue/lethargy Irritability, poor PO intake (infants) Headache Jaundice, scleral icterus Petechiae, bleeding - Retinal hemorrhages, glossitis, bleeding from mucous membranes Heart murmurs, tachycardia, shock, heart failure Lymphadenopathy, hepatosplenomegaly Frontal bossing, prominent maxilla Join or extremity pain, spoon nails
A nurse is caring for a child who is postoperative following surgical removal of a Wilms' tumor. Whichof the following assessments is an indication to continue NPO status? A. Abdominal girth 1 cm larger than yesterday B. Report of pain at the operative site C. Absent bowel sounds D. Passing of flatus every 30 min
C. Absent bowel sounds is an indication that gastrointestinal motility is absent and areason to continue with NPO status.
A nurse is caring for a child who has oral mucositis. Which of the following actions should the nurse take? (Select all that apply.) A. Swab the mucosa with lemon glycerin swabs. B. Apply viscous lidocaine. C. Offer soft foods. D. Use a soft, disposable toothbrush for oral care. E. Encourage gargling with a warm saline mouthwash.
C. CORRECT: Offering soft foods decreases the amount of chewing needed and possible irritation. D. CORRECT: A soft toothbrush allows for adequate cleaning of the mouth and decreases irritation. E. CORRECT: A warm saline mouthwash iseffective in soothing mucositis.
A nurse is planning care for an infant who is scheduled to have a lumbar puncture. Which of the following actions should the nurse include in the plan of care? A. Cleanse the thoracic area of the infant's back withan antiseptic solution. B. Apply a eutectic mixture of local anesthetic cream justbefore the procedure begins. C. Restrain the infant during the procedure to prevent movement. D. Position the infant with his head extended and chin raised.
C. CORRECT: Restraining the infant during the procedure to prevent movement will decrease the potential for injury. It is an appropriate action for the nurse to take.
A nurse is teaching the parent of a child who has a Wilms' tumor. Which of the following statementsshould the nurse include in the teaching? (Select all that apply.) A. "Your child will need to have chemotherapy for 12 months." B. "Wilms' tumors are typically genetic in nature." C. "Surgery is done usually within 48 hours of diagnosis." D. "Palpating the tumor could cause spread of the cancer." E. "Further treatments will start immediately after surgery
C. Prompt removal of the tumor is best practice for treatment of Wilms' tumor. Therefore,this should be included in the teaching. D. Palpating the tumor could cause rupture of the encapsulated tumor. Therefore, thisshould be included in the teaching. E. Chemotherapy and/or radiation are started immediately after surgery. Therefore, thisshould be included in the teaching.
A nurse is caring for a child following an above-the-knee left-leg amputation. Which of the following isan appropriate action for the nurse to take? A. Avoid discussing the amputation. B. Administer aspirin for phantom pain. C. Prepare the child for a prosthesis fitting. D. Maintain the affected limb in the dependent position.
C. Temporary prostheses are fitted soon after surgery. Therefore, preparing the child for aprosthesis is an appropriate action for the nurse to take.
A nurse is caring for an adolescent who has been diagnosed with osteosarcoma. Which of the followingactions should the nurse take? A. Ensure that the adolescent has a referral for a psychiatrist visit. B. Prepare a teaching plan to educate the adolescent in detail about what he should know regardinghis diagnosis and treatment. C. Spend time with the adolescent to answer any questions he may have. D. Perform a mental status examination to assess the adolescent's thought patterns.
C. The nurse should be available to answer the client's questions and to listen as he talksabout his feelings.
A 3 year old has a one month history of headache and morning vomiting. No fevers or exposure to GI illness. She has been increasingly irritable. Parents deny fever. What oncologic disorder may she have?
CNS tumor
Brain tumor diagnostics
CT scan Widespread availability Quick scan Can rule out intracranial hemorrhage or pressure Significant radiation exposure MRI scan Greater sensitivity of brain & spine tumors Not widely available Long study, may need sedation PET scan LP Bone scan (evaluate medulloblastoma mets)
Complications of ALL therapy
Causes myelosuppression - Neutropenia (admission for fever/presumed infection) - Need for antibiotics, antivirals, antifungals - Need for transfusions (RBCs, PLT) Anthracyclines & cardiotoxicity (baseline EKG & ECHO) Vincristine & peripheral neurotoxicity IT MTX & neurotoxicity Steroids & avascular necrosis Radx & risk of secondary cancers
Family impact of cancer
Child feels isolated and low self esteem Cannot go to school, see friends, participate in sports Physical changes: hair loss, weight gain, scarring Bullying Anxiety, depression Parents feel sense of isolation after diagnosis Prolonged hospitalization far from home, family, friends Half of parents report drifting away from friendships Parental financial burden Medical expenses Loss of income/job Parents feel grief, stress, anxiety and depression High incidence in divorce following diagnosis of childhood cancer Affects siblings Causes friction, negative emotions (anger, resentful, fearful, withdrawn), poor quality of life School performance may decline PTSD
SCA chronic pain management
Complementary Therapy Acupuncture Massage therapy Cognitive behavioral therapy Biofeedback Relaxation therapy Distraction therapies Warm packs Whirlpool therapy
Platelets (thrombocytes)
Controls bleeding Normal: 150-450,000 Abnormal: < 20,000 Easily bruises Gum bleeding Nose bleeds
Complications of AML treatment
Cytopenias - Frequent blood transfusions Severe prolonged myelosuppression Infection - With fever, immediately start empiric coverage for Strep. Viridans (vancomycin) Minimize risk of CVL infections Risk of sepsis Risk of gram negative organisms Antibx ppx (fluroquinolone) PJP ppx (Bactrim, pentamidine) - Risk for invasive fungal disease Antifungal ppx [fluconazole, voriconazole, Posaconazole] Mucositis - Need for TPN Liver toxicity Prolonged hospitalization
Clinical presentation of acute leukemia
Pancytopenia - Anemia (fatigue, pallor, tachycardia, irritability) - Leukocytosis (increased WBC) ....Neutropenia (low neutrophil count) - Thrombocytopenia: (petechiae, bruising, gingival oozing, epistaxis, menorrhagia) Fever/Infection Pain Bone pain (long bones), joint pain, painful lymph nodes - Pathologic fractures (ALL) - Refusal to walk or irritability
Pain management of SCA
Prevention: Hydroxyurea!!!! Increase hydration (PO or IV) Analgesics Acetaminophen NSAIDs (PO or IV) Ibuprofen Ketorolac Opioids Fentanyl Morphine Hydromorphone Adjuvant Medications Ketamine
Bleeding disorders
Fibrinogen Deficiency Prothrombin Deficiency Factor V Deficiency Factor VIII Deficiency Factor IX Deficiency Factor X Deficiency Factor XI Deficiency Factor XIII Deficiency von Willebrand Disease
White blood cells (leukocytes)
Fight infection Normal WBC count: 5-10 x109/L - Leukocytosis (elevated WBC) Symptoms Fever > 101.5 F Chills, diaphoresis Diarrhea Burning with urination Erythema, edema, drainage of wounds Leukopenia (decreased WBC) ANC: 2,500-6,000 avg Absolute Neutrophil Count (ANC) Telling of how well the body is fighting infections .....< 1,000: Abnormally Low ....< 500: Neutropenic
A nurse is providing teaching to the parent of a child who has a new prescription for liquid oral iron supplements. Which of the following statements by the parent indicates an understanding of the teaching? A. "I should take my child to the emergency department if his stools become dark." B. "My child should avoid eating citrus fruits while taking the supplements." C. "I should give the iron with milk to help prevent an upset stomach." D. "My child should take the supplement through a straw."
D. CORRECT: The child should take the supplement through a straw to prevent or minimize staining of the teeth.
Types of anemia
Depends on RBC size/mean corpuscular volume (MCV).... Normocytic Macrocytic (increased MCV) Microcytic (decreased MCV) Depends on appearance/ mean corpuscular hemoglobin... Normochromic Hypochromic (decreased MCH) Depends on reticulocyte count.... Helps to distinguish disorders resulting from: Hemolysis (rapid destruction), reflective in increased retic count Bleeding (loss of RBCs) Inability to produce RBCs (bone marrow depression), reflective in decreased retic count
Management of hemophilia
Depends on severity of disease Depends on the site of bleeding Prophylactic therapy (prior to dental procedures, surgery) Give SQ immunizations with 26G needle & IM immunizations with 23G needle, apply firm pressure & ice for several minutes Physical therapy - Apply cold & pressure to affected joints - Avoids NSAIDs, ASA - Avoid contact sports - Wear protective gear (helmets) Dental care Treatment options: Plasma-derived preparations Recombinant FVIII Recombinant FIX Emicizumab (monoclonal antibody)
Diagnosis and treatment of neuroblastoma
Diagnostics - CBC, CMP - Ultrasound, CT, MRI of chest & abdomen, skeletal survey or bone scan - Urine catecholamines Treatment - Surgical resection Chemotherapy High dose chemo with autologous SCT Immunotherapy Radiation ....Prognosis depends on age & stage of tumor
lumbar puncture
Evaluates CNS involvement May need PLT transfusion prior to procedure - Initial LP: PLT > 100 - Subsequent LP: PLT > 20-50
Diagnosis and treatment of Wilms tumor
Evaluation - Obtain chest, abdomen, pelvis imaging (CXR, KUB, CT, MRI, U/S) - Abdominal Ultrasound - U/A: microscopic hematuria - BP: HTN Treatment: - Nephrectomy, possible resection of ureter & adrenal gland - Chemotherapy - Radiation - 90% of children are cured Risk of HTN if renal ischemia
Types of inherited aplastic anemia
Fanconi anemia Shwachman-Diamond syndrome Congenital amegakaryocytic thrombocytopenia Dyskeratosis congenita
Symptoms of SCA
Fatigue After 6 months of age, painful vaso-occlusive crises occurs Pain crises - Swelling & inflammation of hands & feed (arthritis and or dactylitis) - Bone necrosis Abdominal pain - Splenic sequestration (sudden pooling of blood in the spleen) Liver congestion Bacterial infections - Slowed weight and height gains after age 7 - Delayed puberty by 3-4 years
Normal H/H values of newborns, infants and children
Fetal hemoglobin increases with gestational age Full term infants have high Hgb levels > 14 g/dL Hgb levels drops off shortly after birth - Caused by shortened survival of fetal RBCs & rapid expansion of blood volume - Known as physiologic anemia - Reach their NADIR at 6-9 weeks (11 g/dL) Normal Childhood Range of Hemoglobin Level 11-15 g/dL - Anemia is Hgb < 10 -11 g/dL - Severe anemia < 5 g/dL, leads to serious tissue hypoxia & cyanosis - Normal Hematocrit 30-45%
Chronic illness and hematological conditions
Frequent lab draws, clinic visits, frequent hospitalizations and transfusions (iron and blood), medications, surgeries, HSCT
Chronic hematologic disorders and family dynamics
Genetic counseling Educating adolescents with trait about their status & risk of disease transmission Family medical education to assess child for symptoms of illness/crisis Support groups - Sickle Cell Disease Association of America - National Hemophilia Foundation
Parental refusal of cancer treatment
Goal is for shared decision making among family & oncology team - Committing to therapy does require several years of chemotherapy, clinic visits, hospitalizations, and close monitoring Parents may opt to pursue alternative holistic approaches including nutritional supplements, vitamins, or herbal remedies. May prefer to be followed by holistic provider. However if there is a high likelihood of long-term survival & reasonable quality of life with treatment, provider cannot withhold therapy If parents refuse treatment that places child at substantial risk, providers are obliged to seek legal recourse according to the AAP & Society of Pediatric Oncology - DCYF should be contacted for medical neglect - Inability to provide adequate care for child is a criminal offense Ultimately depends on court rulings to favor the family or medical community - Consider prognosis, risks of treatment, parental believes, alternative therapy - Muddy given a parent may feel their decision is in the best interest of their child Holistic physicians can be reported to medical board for failing to conform to minimal standards of care However, in patients with poor prognosis limiting medical therapy may outweigh the benefits, keeping in mind the child's quality of life
AML treatment
Goal is to induce clinical & biological remission by rapidly reducing the number of malignant cells Treatment failure due to relapse or treatment-related mortality Chemotherapy Cytarabine Anthracycline (danorubicin) Etoposide 6-thioguanine L-asparaginase Intrathecal chemo IT cytarabine Radx if refractory CNS involvement Monoclonal antibody therapy - Gemtuzumab
ALL treatment
Goal is to induce permanent biological & clinical remission - 85% of children with ALL will be cured of their disease Induction: First 28-35 days Consolidation: Further systemic chemo + CNS prophylaxis Interim Maintenance: Intensified maintenance regimen Delayed Intensification: Re-induction & reconsolidation Maintenance: Continuation of therapy for 2-3 years CNS directed therapy: Intrathecal chemo
Long term complications of surviving childhood cancer
Growth deficiencies - short stature - delayed puberty or obesity and fast puberty cognitive dysfuntion - impaired IQ - learning disabilities Chronic health conditions - growth pain - peripheral neuropathy CHF Psychosocial Secondary malignancies
Clinical presentation of brain tumors
Headaches Nausea/vomiting Neurologic deficits: ataxia, focal weakness, cranial nerve palsies Vision changes Seizures (uncommon) Back pain (spinal cord tumor) Failure to thrive Developmental delays, regression Behavior/personality changes Changes in academic performance
Types of hemolytic anemia
Hemolytic disease of the newborn Hereditary Spherocytosis G6PD deficiency Autoimmune hemolytic anemia Microangiopathic hemolytic anemia Sickle cell anemia
A newborn boy was just circumcised in the nursery. He is hemorrhaging from the circumcision site despite gentle pressure. What hematological condition should be considered?
Hemophilia
Doug is 12 years old and all his friends play contact sports. Doug's parents encourage him to join the swim team instead due to his condition. Doug often suffers from nosebleeds, bruising, and blood in the urine. What disorder does Doug have?
Hemophilia
Types of hemophilia
Hemophilia A /Factor VIII Deficiency - 1 in 5,000 males (X-linked disorder), females are only carriers - Treat with recombinant FVIII Hemophilia B / Factor IX Deficiency - 1 in 25,000 males (X-linked disorder), females are only carriers - Treat with recombinant FIX
Peripheral smear SCA
Hgb 6-10 g/dL Elevated Retic: 5-15% Morphology of sickled cells, with predominance of Hgb S, not Hgb A Signs of functional asplenia - Nucleated RBC - RBC with Howell-Jolly body - Target cells
ALL consolidation chemotherapy
High Risk Cyclophosphamide Cytarabine PEG-asparaginase 6-mercaptopurine (6-MP) Intrathecal MTX +/- Radiation Standard Risk - IT Chemo
Supporting children and families with chronic illness
Honest communication with age-appropriate language Give clear & honest answers to child's questions Explain & prepare for procedures/treatments - Avoid saying "that won't hurt" if a procedure will be painful - Instead say the child may feel pressure, discomfort, or stinging Allow child to express feelings, concerns, fears Remind them their illness is not their fault! If they ask "Why me?" its OK to respond "I don't know" If they ask "Am I going to die" consider their age, maturity, prognosis Consider talking about religious, spiritual or cultural beliefs Allow for non-verbal communication Music, writing, or drawing to express emotions Maintain routine Set boundaries (bad behavior, overindulgence)
Long term management of SCA
Hydration (8-10 cups of fluid/day; 1.5 x MIVF) Infection Prevention Folic Acid Hydroxyurea beginning at age 2 !!!!! - Increases Hgb F, decreases Hgb S - Decreases hemolysis - Decrease number of painful crises - Decreases ACS - Decreases risk of stroke - Decreases need for RBC transfusion - Preserves organ function Glutamine beginning at age 5 - Reduces the frequency of vaso-occlusive events & hospitalization
clinical presentation of AML
Indistinguishable from ALL Life-threatening manifestations - Extreme leukocytosis (WBC > 200 x109/L) - Increase blood viscosity with myeloblasts & decreased flow through vessels Risk of hypoxia in lungs & brain (HRF stroke!) Life-threatening bleeding Fever very common - No apparent infectious etiology Leukemia cutis in infants (blueberry muffin rash) Myeloid sarcomas
Sickle Cell Anemia (SCA)
Individuals do not form the normal Hgb A molecule, instead Hgb S!!!! As fetal Hgb (Hgb F) declines after 6 months of age, Hgb S increases Hgb S collapses the RBC giving it a rigid "sickled" shape - Causes severe chronic hemolytic anemia - Increased blood viscosity & obstruction of small blood vessels - Leads to thrombosis, tissue ischemia,& necrosis, leads to excruciating pain - Unpredictable acute complications that may become life-threatening - Possible chronic organ damage
Symptoms of FE deficiency anemia
Infants & Toddlers - Irritability or restlessness if Hgb < 8 g/dL Pica - Appetite for non-food items such as paper, dirt, clay - Pagophagia (eating ice) Developmental delays - Cognitive & motor delays Social emotional behavioral disturbances
Hemophilia
Inherited bleeding disorder caused by low levels or absence of blood protein that is essential for clotting Mild hemophilia - Bleeding only with significant trauma or surgery Severe hemophilia - Bleeding with minimal trauma - Apparent when infant learns to crawl & walk
Hemolytic anemia
Inherited or acquired - Occurs in infancy or early childhood Premature destruction of RBCs!!!! - Reduced red cell survival - Intravascular destructions happens within blood circulation - Extravascular destruction due to phagocytic cells of the bone marrow, liver, or spleen Increased marrow production of reticulocytes!!! - Retic count is elevated in proportion to the extent of hemolysis No obvious bleeding!!!
Types of anemia
Iron Deficiency Anemia Aplastic Anemia Hemolytic Anemia Sickle Cell Anemia
12-month-old Brian is seen at the local pediatrician's office. The nurse documents the following findings during her assessment: poor muscle tone, irritability, concave nailbeds, and edematous. These findings are consistent with what disorder?
Iron deficiency anemia
A 24 month old boy presents to his PCP for his 2 year old WCC. Family reports he loves drinking milk. He drinks 5 cups a day. He is not a fan of vegetables, especially green ones. On physical exam he appears pale. His routine Hgb is 10 g/dL. What hematologic condition may he have ???
Iron deficiency anemia
ALL maintenance therapy
Lasts 2-3 years - Pulse dose steroids Prednisone or dexamethasone Vincristine PO MTX & 6-MP CNS PPX - IT MTX - Cranial RDX
During a well-child examination of a 3-year-old girl with known hearing loss and developmental delays, the mother reports that the family lives in a house built 50 years ago that has copper pipes and chipped paint. The nurse then asks the mother if the child ever has abdominal pain, vomiting, constipation, headache, or fever. What condition does the nurse suspect the child to have?
Lead poisoning
During patient rounds on the pediatric hematology/oncology unit, the nurse is told to prepare her patient for a bone marrow test. Laboratory values show a low reticulocyte count and thrombocytopenia. These findings are consistent with what diagnosis?
Leukemia
Acute leukemia lab derangements
Leukemia WBC > 100,000-400,000 (avg wbc is 4,000-11,000) Pancytopenia (anemia, neutropenia, thrombocytopenia) Transaminitis (elevated LFTs) Elevated uric acid Elevated lactate dehydrogenase (LDH)
Pathophysiology of whole blood
Made of cellular components ...Plasma - Fluid components, serves as transport medium - Contains proteins (albumin, globulin, fibrinogen) - Maintains intravascular volume, coagulation, & acid-base balance ....Leukocytes/ White Blood Cells (WBCs) - Fight infections .....Erythrocytes/ Red Blood Cells (RBCs) - Carries oxygen Thrombocytes/ Platelets (PLTs) - Controls bleeding
Hodgkin lymphoma
Malignancy of the reticuloendothelial & lymphatic systems, involves B cells - Originates in cervical lymph node & spreads to other lymph nodes Diagnosed in adolescents aged 15-19 years old - Accounts for 6% of childhood cancers Painless enlargement of cervical lymph nodes (firm, rubbery, non-tender) Chronic cough if tracheal compression from large mediastinal mass Fever, decreased appetite, weight loss, drenching night sweats Evaluate with CBC, CMP, LDH, ESR, CRP, CXR, BM aspiration, biopsy Treated with chemotherapy & radiation Risk of infertility Risk of secondary malignancy
A nurse is providing home care instructions to a parent of a child who is receiving chemotherapy. Which of the following instructions should the nurse include in the teaching?
Manifestations of infection, bleeding precautions, hand hygiene
Hemolytic anemia
Marked RBC fragmentation Mircospherocytes (large arrow)
Hematological system
Massive fluid organ Permeates the entire body Delivers nutrients & vital elements throughout the body Transfer of respiratory gases Hemostasis Phagocytosis Provision of cellular & humoral agents to fight infection
Types of CNS tumors
Medulloblastoma - Most common CNS tumor, 20% of cases - SR in children > 3 years: 80% chance survival Gliomas - Includes astrocytoma, ependymoma Atypical teratoid/rhabdoid tumor (ATRT) - Most common CNS tumor in children < 1 year CNS tumors: Highest rate of long term complications than any other type of cancer
Types of pediatric cancer
Most common Leukemia Lymphoma Burkitt's Lymphoma Hodgkin's Lymphoma Brain tumors Infants- Toddlers (0-4) Neuroblastoma Wilms Tumor Acute Lymphoblastic Leukemia (1-4 years old) Children 10+ Lymphoma and Sarcoma
CNS tumors
Most common cancer diagnosis after leukemia Accounts for 20% of pediatric malignancies Link to certain genetic disorders Neurofibromatosis, tuberous sclerosis, von Hippel-Lindau syndrome, Li-Fraumeni syndrome Caused by radiation therapy (secondary malignancy) Locations - Infratentorial (cerebrum) in children up to 14 years - Supratentorial (cerebellum) in adolescents - Spinal cord tumors in adolescents
Acute leukemia
Most common cause of cancer in children - Acute leukemia accounts for 25% of newly diagnosed cancers in children < 15 years Malignant hematologic disease Normal bone marrow elements are replaced by abnormal, poorly differentiated lymphocytes of myelocytes known as blast cells Leads to unregulated proliferation of malignant blast cells Leads to cellular destruction - Normal cell production is decreased - Competition for metabolic elements - Infiltration of blasts induces pain
Iron deficiency anemia
Most common hematological condition in the world Most common nutritional disorder in the world Risk Factors Prematurity, Low Birth Weight!!! Exclusive breastfeeding without Fe supplementation or infants fed non-Fe fortified formula!!! Early weaning to cow's milk (before 12 months)!!!! Excessive intake of cow's milk, goat's milk, or soy milk in children 1-5 years old (> 25 oz.) !!!! Children with feeding problems or malabsorption IBD, Celiac disease, Crohn's disease, bowel resection, giardia Low socioeconomic status, food insecurity Lead exposure (high comorbidity, lead molecules block iron from biding) Children with restricted or alternative diets (Eating disorders, Obesity, Vegans) Adolescent females given combination of pubertal growth spurt, heavy menses, nutritionally inadequate diets!! Chronic use of ASA or NSAIDs
Wilms tumor/nephroblastoma
Most common malignancy of the GU tract - 6% of childhood cancers Occurs in children aged 1-5 years old Associated congenital anomalies: renal abnormalities, duplicated collecting system, cryptorchidism, hypospadias, ambiguous genitalia, cardiac abnormalities Associated with Beckwith-Widemann, Denys-Drash, Perlman syndromes Typically found as increasing abdominal size or firm, smooth mass in the abdomen or flank found by parent or PCP - Fever, dyspnea, diarrhea, vomiting, abdominal pain, weight loss, malaise
Retinoblastoma
Most common malignant ocular tumor in childhood - Affects 200-300 children each year Diagnosed before age 5 Symptoms Leukocoria "white eye" Often noticed by family or photo with white reflex Strabismus Vision loss Inflammation of eye Treated by oncologists & ophthalmologists Enucleation & prosthetic placement Laser therapy Chemotherapy Radiation Risk of secondary malignancy
Management of CNS tumors
Neurosurgery - Goal is to achieve maximal tumor removal while minimizing morbidity & mortality - Children with increased ICP c/b obstructive hydrocephalus will need CSF diversion Radiation therapy - Destroys remaining tumor in cases of incomplete resection - Treats microscopic residual disease & metastatic disease Chemotherapy - Vincristine, MTX, temozolomide, procarbazine, lomustine (CCNU), cisplatin, carboplatin, cyclophosphamide, etoposide
Pediatric oncology nursing ethics
Nurses must assist uncomfortable medical procedures that cause pain to the child Witness conflicting medical decisions between parents or healthcare professional to determine best interest of child Moral distress can lead to anger, sadness, frustration, burn out, withdrawal from patient, diminished quality of care, leaving clinical position Disagreement on end-of-life care, not all grief & patient loss leads to moral distress Moral distress from short staffing, causing busy work shifts & limited time to support families Nurturing good collegial relationships & developing manageable workloads can reduce moral distress among nurses
CSF diversion
Obstructive hydrocephalus r/t CNS tumor Extraventricular drain (EVD) placed at time of tumor resection - Left in place post-op, will be clamped to determine if normal CSF flow has been restored post-resection Developing sx increased ICP with EVD clamped indicative of need for permanent CSF diversion - Placement of ventriculoperitoneal shunt (VPS)
SCA and stroke
Occurs due to cerebral vascular stenosis & occlusion of the major arteries of the brain (including carotids) leading to cerebral hypoxia & infarction - Most SCA patient have annual transcranial doppler Ischemic Symptoms: seizures, hemiparesis, visual field deficits, aphasia, cranial nerve palsies, coma Intracranial Hemorrhage Symptoms: severe headache and vomiting Obtain head CT & brain MRI/MRA Treatment: - Administer exchange transfusion
Pediatric oncology manifestations
Often presents with symptoms of benign illness - Weight loss - Fatigue - Swelling or pain in bones, joints, back, or legs - Lumps or masses Lymphadenopathy Enlarged lymph nodes > 10 mm Enlarged supraclavicular, axillary, or epitrochlear nodes Nodes that har hard, non-mobile, non-tender Excessive bruising or bleeding Rash Recurrent infections Recurrent fevers Headaches Nausea or vomiting
Aplastic anemia
Pancytopenia - Anemia: fatigue & pallor - Neutropenia: fever, mucosal alterations, & bacterial infection - Thrombocytopenia: hemorrhagic manifestations Hypocellular bone marrow/bone marrow failure without malignancy Inherited or acquired - Acquired is typically due to immunosuppressive therapy Risk of life-threatening infections & bleeding!!! May need chronic PRBC transfusions Hematopoietic cell transplantation is the only curative treatment for bone marrow failure!!!!
Pediatric cancer management
Pediatric Cancer Centers - More than 200 across the U.S. Children's Oncology Group (COG) Collaborative group of multidisciplinary professionals involved in the treatment of children with cancer Cares for 90% of children diagnosed with cancer COG Clinical Trials Therapy can last months to years Rate of survival now up to 80-85%, improved from the 1970s
Parental coping
Plan day by day instead of year by year encourage rest and hobbies Leaning in on inner circle Nurture personal relationships Support groups Social worker Financial advisement
Acute lymphoblastic leukemia (ALL)
Pre-B cell AlL - majority if cases T-cell ALL 15% of cases More common in males WBC > 100 Bulky lymphadenopathy Presents with mediastinal mass Airway or cardiovascular compromise Increased incidence of CNS disease
Iron supplementation
Premature infants < 37 weeks - Require iron supplementation from 2 weeks of age to 12 months of age Exclusively breastfed infants - Should begin Fe supplementation at 4 months of age unless - Fe containing foods are introduced Fe Fortified Formula fed infants do not need additional supplementation - At 6 months of age, iron fortified cereal & pureed meats should be introduced - Avoid whole cow's milk until 12 month of age (low Fe content), then limit cow's milk to 24 ounces/day Iron Supp - < 3 years: liquid supp (use dropper!) - > 3 years: chewable multivitamin - Tastes bad - Give with OJ to promote absorption, do not give with milk!!!!!! - May stain teeth but can be removed with dental cleaning - Constipation, iron may turn stools black
Peripheral smear of thrombocytes
Purple dots are PLT Increased PLT number with clumps of large abnormal PLT in thrombocythemia
Preventative care and hematological disease
RN, therapist, immunizations, prophylactic ABX, nutritional assessment, stroke preventions, lung function w PFT screening, bone health, pain management, monitor vision, cardiac function
Etiology of acute leukemia
Radiation exposure Benzene exposure Genetics (Down syndrome, Fanconi anemia, neurofibromatosis, Diamond-Blackfan anemia) When child has has AL, siblings are 4x more likely to develop AL
Splenic sequestration
Rapid onset of Intravascular pooling of large amounts of blood & platelets in the spleen - CBC notable for significant drop from baseline Hgb - Leads to life-threatening anemia, thrombocytopenia, hypovolemic shock, cardiovascular collapse, & sudden death within hours of onset Begins in infants aged 6 months when Hgb F disappears and Hgb S circulates Symptoms include sudden weakness, pallor of lips & mucous membranes, breathlessness, rapid pulse, faintness, abdominal fullness, splenomegaly.!!! Treatment: PRBC transfusion to restore circulating blood volume With recurrent splenic sequestration, spleen becomes fibrotic due to multiple infarctions - Splenectomy should be considered in children who have splenic sequestration crisis.... you can live without a spleen bc the liver will take over its actions
Pediatric cancer
Rare -m15,000 children and adolescents are diagnosed with cancer each year in the U.S. Leading cause of death after accidents Survival rates have increased over the years - More than 80% of children diagnosed are expected to be cured of their disease! Some cancers are related to genetic disease - Down syndrome, Beckwith-Wiedemann syndrome, neurofibromatosis Some cancers are related to prior malignancy Only a small percentage are preventable, unlike adults - Radiation exposure (ex. CT or history of radiation treatment)
Hyperhemolytic crisis
Rare, sudden exacerbation of hemolysis with worsening anemia despite ongoing reticulocyte production - Likely reflects occult splenic sequestration or aplastic crisis detected during a period of resolving reticulocytosis - May be a delayed hemolytic transfusion reaction - Accelerated hemolysis with acute vaso-occlusion event, acute pain, or ACS Infection or drug exposure Symptoms may include fatigue, pallor, dyspnea Treatment: - Potentially fatal if cause of hemolysis is not addressed - Rapid PRBC transfusion... bc RBC are being destroyed so quick and not being produced quick enough
Red flag symptoms of headaches
Recurrent morning headache Headache that wakes a child from sleep Intense, incapacitating headache Headache with vomiting
Anemia
Reduction in circulating RBCs Decrease in hemoglobin (Hgb) - Decreased RBC production - Blood Loss - RBC destruction - Abnormalities of RBCs Affects oxygen transport to tissues
Sarcomas
Rhabdosarcoma Most common soft tissue malignancy 3rd most common solid tumor in children, 250-400 new cases annually Painless growing mass found in head & neck, GU, extremities May cause compression of airway, cranial nerves, or urinary obstruction Treated with chemotherapy, surgery, radiation Survival > 70% Osteosarcoma Most common bone malignancy Aggressive cancerous tumor of the bone Typically occurs in the metaphysis of most rapidly growing bones, the long bones (femur, tibia, humerus) Symptoms include bone pain, palpable mass Typically attributed to minor trauma May present with pathologic fracture of weakened bone Treatment includes chemotherapy & radical surgery (limb-sparing if possible), possible radiation Survival > 70% Poor prognosis with mets (10-30%) Ewing's Sarcoma 2nd most common bone malignancy Typically occurs flat bones or in the midshaft of long bones (pelvis, chest wall, long bones of lower extremities) Symptoms include bone pain, swelling Treated with chemotherapy, surgery (resection with limb-salvage procedures), and radiation Survival 60-70%
Side effects of Chemotherapy
Risk for Infection (neutropenia) Handwashing Monitoring closely for fever, signs of infection Continue routine immunizations, except for live vaccines Risk for injury (thrombocytopenia) - fall risk - skin puncture/ trauma - no rectal temps - no ASA/NSAIDs Risk for constipation - hydration - mobility - stool softeners Pain - meds - distract - relaxation Poor nutrition - n/v Alopecia Anxiety
Bone marrow aspiration
Sample obtained from iliac crest!!!! Morphology Blast Count - Normal Bone Marrow: < 5% blasts - Acute Leukemia Suspected: > 5% blasts ALL: Diagnosed if > 25% lymphoblasts ...Typically 80-100% lymphoblasts at time of diagnosis of ALL! AML: Diagnosed if > 20% myeloblasts Immunophenotyping by flow cytometry Karyotype Molecular studies
AL diagnostic studies
Serum Labs.... CBC CMP (lytes, BUN, creat) Coags (PT, INR, PTT, fibrinogen, D-Dimer) LDH Uric Acid Blood culture CXR Xray of long bone if bone pain Bone Marrow Aspiration Lumbar Puncture EKG ECHO
An adolescent African American female presents to the ED with shortness of breath, chest pain, and wheezing. Her RR is 40 and her SpO2 is 90%. Her Hgb is 7 g/dL. CXR reveals pulmonary infiltrate. What hematological disorder might she have?
Sickle cell anemia
Tonya is a 16-year-old African American girl in the hospital with complaints of pain in both hands, chest pain, fever, and moderate respiratory distress. The nurse is providing IV therapy for hydration and encouraging oral fluid intake. Medications include hydroxyurea and pain medication. What disorder does Tonya have?
Sickle cell disease
Vaso-occlusive episodes and SCA
Sickled RBCs cause vascular obstruction & vaso-occlusion!!! May be precipitated by fever, viral illness, or dehydration Pain is typically the consequence, most often musculoskeletal pain Can last for several days Major site of pain include bones & joints - Back pain - Dactylitis in children < 5 years (painful swelling of hands & feet) Support with analgesics & hydration Abdominal pain - Risk for cholecystitis, pancreatitis, ileus, UTI, PID, PNA, ACS Priapism.. persistent and painful errection
Neuroblastoma
Solid tumor of sympathetic nervous system, arises from neural crest cells - Most common solid tumor of early childhood, peak incidence 2 years (600 cases diagnosed each year) Typically develops in the abdomen & along the sympathetic neural pathway, with primary tumor in the adrenal gland - Tumor can extend to neck, thorax, & pelvis Symptoms Unexplained fever, pain, weight loss, irritability Ascites, palpable hard, fixed irregular mass in abdomen HTN r/t compression of renal vasculature, catecholamine secretion Spinal cord compression Horner's syndrome, heterochromia, racoon's eyes Splenomegaly, lymphadenopathy Anemia, coagulopathy
SCA multiorgan involvement
Spleen - Splenomegaly disappears after age 5 given autoinfarction of the organ Bones - osteonecrosis of humerus and femur heads, may need hip replacement Skin - leg ulcers from decreased perfussion Kidneys - nephropathy, asymptomatic proteinuria, renal failure Eyes - retinopathy, blindness Lungs - restrictive lung disease, pulmonary hypertension Heart - cardiomegaly, CHF Brain - cerebrovascular disease leads to hemiparesis, coma, death
Stem cell research
Stem cell transplantation may be considered in children with severe sickle cell anemia, may be curative in some patients Matched sibling donor Unrelated bone marrow Umbilical cord blood transplantation Stem cells can be taken from an embryo but this is an ethical problem bc of religion and politics
Conventional ALL induction therapy
Steroids - Prednisone or dexamethasone Chemotherapy - PEG-asparaginase - Vincristine - Danorubicin (HR ALL) Intrathecal Chemotherapy Cytarabine Methotrexate
Geography of pediatric cancer
The Northeast has the highest rate of child/adolescent cancer in the U.S. - The highest incidence of lymphoma & brain tumors is in the Northeast The West has the highest incidence of leukemia The South has the lowest rate of child/adolescent cancer
Management of mild FE deficiency anemia
Treat - ferrous sulfate (iron) for 4-6 weeks Dose - 3-6mg/kg/day of elemental iron in 2-3 divided doses daily max: 200mg Repeat - repeat retic count in one week - reliculocyte count should increase >3% within 48-96 hours for starting FE sup Repeat - repeat hub in 1 month - hub should increase by 1-2 g/dl..... continue until normal (4-6 weeks)....continue for 2-3 months to replenish iron stores Recheck - recheck after 6 months
Screening for FE deficiency anemia
Universal Hgb on all infants age 12 months!!!! Perform if higher risk screening - 12 - 36 months in children with excessive cow's milk intake - Onset of menarche in adolescent females Venous sampling - Most reliable - With capillary sample, excessive squeezing of finger may produce inaccurate results Diagnostic Criteria Age of the child History of low iron intake Hgb: 11 g/dL Microcytic Anemia!!!!!!
Peripheral smear of leukocytes
Variety of Leukocytes Neutrophil: Nucleus is segmented into 4 lobes connected by thin chromatin strands. Cytoplasm is pink with fine blue granules. Band: Immature neutrophils Lymphocyte: Small cell, heavily stained condensed nucleus with small rim of blue cytoplasm Monocyte: Irregular nucleus with grayish/blue cytoplasm with few granules Metamyelocyte: Kidney bean shaped nucleus with granulated cytoplasm
complete blood count (CBC)
WBC count Differential Neutrophils Absolute Neutrophil Count (ANC)!!! Lymphocytes Monocytes Eosinophils Basophils RBC Hematocrit Hemoglobin Platelet count
Tumor Lysis Syndrome (TLS)
When a pt is being treated for cancer and the chemo breaks up the cancer so quick and it goes into the bloodstream faster than the chemo is killing it. In patients with large tumor burden & rapid cell turnover (ex. ALL, non-Hodgkin lymphomas) High risk in patients with WBC >100 or bulky disease (organomegaly, adenopathy, mediastinal mass) Oncologic emergency given severe metabolic derangements Anticipate with initiation of treatment Hyperuricemia Hyperkalemia Hyperphosphatemia Hypocalcemia Acute kidney injury
All risk stratification
Younger children, females, & pre-B cell ALL have better prognosis overall. Standard Risk - Children 1-10 years - WBC < 50 - 4 Year Survival: > 90% ......Low risk patients receive less intensive regimen to decrease recurrence & minimize exposure to unnecessary agents & decreases toxicity High Risk - Children < 1 year (worse prognosis) - Children > 10 years - WBC > 50 - 4 Year Survival: 75% ....High risk patients receive more intensive and potentially more toxic therapies
Five-year-old Roxy is in the hospital with failure to thrive. She was adopted as a baby from a mother who had a positive drug history. Current assessment reveals lymphadenopathy, oral candidiasis, and swelling between the ear and jaw. Her foster mother reports diarrhea for the past 7 days. What diagnosis are these findings consistent with?
acquired immunodeficiency syndrome
cancer
any malignant growth or tumor caused by abnormal and uncontrolled cell division
hematopoietic stem cells
cell in the bone marrow that gives rise to all types of blood cells
Reticulocyte
immature red blood cell
Normal peripheral blood smear
◼ RBCs with uniform size & shape ◼ Normal central clearing/pallor should be 1/3 of diameter of cell ◼ Large purple stain is lymphocyte ◼ Normal size of RBCs = nucleus of lymphocyte ◼ Small purple circles are PLT
Types of acute leukemia
◼Classified according to cell type involvement ◼Acute Lymphoblastic Leukemia (ALL) -Accounts for 75% of AL cases ◼Acute Myeloid Leukemia (AML) - Accounts for 20% of AL cases ◼The remainder are undifferentiated or mixed phenotypic ALs (MPALs)
Peripheral smear of FE deficiency anemia
◼Normal size of RBCs = nucleus of lymphocyte Small microcytic RBCs Thin rim of pink (hypochromia) Occasional pencil shaped cells