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Stony Brook Pediatrics Hematology/Oncology Outpatient Elective Rotation Competency-based Goals and Objectives Primary Goals for this Rotation Competencies GOAL I: Prevention, Counseling and Screening (Hematology/Oncology). Understand the role of the pediatrician in preventing hematologic or oncologic conditions, and in counseling and screening individuals at risk for these diseases. 1. Provide routine preventive counseling about hematology to all patients and families, addressing: a) Adequate diet and iron intake to prevent iron deficiency b) Signs, symptoms and risk of sickle cell crises c) Signs, symptoms, and risks for thrombotic events in a child identified of being at risk K, PC, IPC, P 2. Provide preventive counseling to parents and patients with specific hematology/oncology conditions, addressing: a) In a child with a sickle hemoglobinopathy, the importance of antibiotic prophylaxis, pneumococcal and routine immunizations, folic acid supplementation, and urgent need for evaluation for fever b) Risk of infections related to transfusion of blood or blood products, and K, PC, alternatives to routine transfusion (i.e., direct donation, irradiation, freezing, filtration) SBP, P c) Expected course of common childhood malignancies, with good and bad prognosticators d) Support groups and information available for children with cancer e) Thrombosis risks in patients with known inherited risk factors for thrombosis 3. Describe regular hematology/oncology screening for patients: a) Screen for hemoglobinopathies in the newborn period. b) Screen for iron deficiency anemia during the first two years of life, with particular emphasis on premature infants. c) Screen adolescent females for signs of iron deficiency and anemia yearly after menses has begun. d) Screen for -thalassemia trait GOAL II: Normal Vs. Abnormal (Hematology/Oncology). Distinguish normal from pathologic states of the hematologic and lymphatic systems. K, PC IPC, 1. Explain the findings on clinical history and examination that suggest a hematologic or oncologic disease that requires further evaluation and K, treatment. 2. Interpret clinical and laboratory tests to identify hematologic or oncologic disease (CBC, including indices and blood smear review, reticulocyte count, PT, PTT, hemoglobin electrophoresis, iron, iron K, binding capacity, ferritin, transferrin, uric acid, LDH, electrolytes, renal function, urinalysis). 3. Describe the changes that occur over time in the hematologic indices of K the normal infant and child (e.g. Hemoglobin, hematocrit, MCV) 4. Discuss the interpretation of a bone marrow aspirate. K PC PC GOAL III: Undifferentiated Signs and Symptoms (Hematology/Oncology). Evaluate, treat, and/or refer patients with presenting signs and symptoms that may indicate a hematologic or oncologic disease process. Evaluates, treats and/or refers patients with presenting signs and symptoms of commonly or less commonly or rarely seen conditions Develops a strategy to determine if the following presenting signs and symptoms are caused by a hematology/oncology disease process and determine if the patient needs treatment or referral: a) b) c) d) e) f) g) h) i) j) k) l) Fatigue/malaise Fever Bruising/bleeding Headache Limb pain/limp Lymphadenopathy Hepatomegaly and/or splenomegaly Weight loss Abdominal pain Vomiting Dizziness and gait disturbances Pallor Mass K, PC GOAL IV: Common Conditions Diagnose and manage patients with common hematological disorders 1. Diagnose, explain, and manage the following hematologic or oncologic conditions: a) b) c) d) e) f) Iron deficiency Idiopathic thrombocytopenic purpura Major and Minor reactions to blood or blood product transfusions Hemoglobinopathies Transient erythroblastopenia of childhood Bone lesions (differentiation between benign and malignant lesions)Congenital and acquired bleeding disorders GOAL V: Conditions Generally Referred (Hematology/Oncology). Diagnose and initiate management of patients with hematological or oncological disorders that generally need referrals. K, PC 1. Identify and explain conditions a – m (below): Initially manage, and seek consultation or refer the following hematology/oncology conditions: a) Anemia (exclusive of common iron deficiency or transient erythropenia b) Abnormal bruising or bleeding (inherited and acquired) c) Hemoglobinopathies (sickle cell and other sickling disorders), including severe pain crisis, fever, stroke, sequestration and aplastic crises d) Urgent conditions in children under treatment for cancer, including fever and neutropenia, chicken pox exposure or illness, bleeding e) Neutropenia f) Thrombocytopenia including ITP g) Abdominal masses h) Major complications of inherited bleeding disorders i) Mediastinal masses j) Suspected or confirmed CNS tumor k) Conditions that might predispose to malignancy (e.g.. , Bloom syndrome (retinoblastoma), and familial cancer), hemihypertrophy l) Coagulation disorders K, PC, IPC 2. In cases of serious or life-threatening disease, counsel the patient's families with sensitivity to their desire and need to know about: a) b) c) d) Prognosis and possible impact of the disease Likely steps in immediate and future treatment Decisions about treatment options which they may face Support services that they may seek in the hospital and community 3. Identify the role and general scope of practice of hematology/oncology; recognize situations where children benefit from the skills of specialists trained in the care of children Identify the role and general scope of practice of hematology/oncology; recognize situations where children benefit from the skills of specialists trained in the care of children; and work effectively with these professionals in the care of children with hematologic or oncologic diseases. K, PC, IPC, P, SBP K, PC, IPC, SBP GOAL VI: Common Malignancies. Discuss the presentation, pathophysiology, and prognosis of important malignancies in children and adolescents. 1. Summarize the common ages, presenting signs and symptoms, diagnostic procedures for the following malignancies and conditions: a) b) c) d) e) f) g) h) Leukemia (ALL, AML) Brain tumor Hodgkin's and non-Hodgkin's lymphoma Neuroblastoma Wilms' tumor Soft tissue sarcomas (rhabdomyosarcoma) Bone tumors (osteosarcoma and Ewing's sarcoma) Retinoblastoma K i) Langerhans cell histiocytosis 2. Compare and contrast the common acute side effects of commonly used chemotherapeutic agents. 3. Discuss the common late complications of childhood cancer treatment that may present in childhood or adolescence. These include: learning disabilities, endocrine suppression and second malignant neoplasms. 4. Be familiar with adjunctive medications that increase patients' tolerance of chemotherapy, e.g. folinic acid, recombinant human erythropoietin, GCSF. K, PC K, PC K, PC GOAL VII: Iron Disorders. Discuss the appropriate methods of diagnosis and management of a patient with iron disorders. 1. Describe the normal requirements, absorption, and metabolism of iron from birth through adolescence. 2. Differentiate iron deficiency anemia from other causes of anemia common to children. 3. Describe the diagnosis and treatment of iron deficiency, and discuss the follow-up necessary to assure success in treatment. 4. Develop a treatment and education plan for managing iron deficiency. This should include: dietary management, replacement therapy, parent education, and follow-up. K K K, PC K, PC, IPC GOAL VIII: Blood Products. Understand indications for and complications related to the use of blood products. 1. Explain the appropriate indications for and potential risks of various blood products (e.g., red blood cell products, platelet concentrates, coagulation factors). 2. Describe the indications for leukofiltration, irradiation of blood products, and use of CMV negative blood products. 3. Summarize the signs and symptoms of a transfusion reaction. Develop an effective treatment plan to manage a transfusion reaction. 4. Describe alternatives to blood transfusions. These should include: erythropoietin K K K, PC K GOAL IX: Sickle Cell Disease. Understand the general pediatrician's role in the diagnosis and management of patients with sickle cell disease. 1. Explain the findings on clinical history, examination, and laboratory tests (including newborn screening) that suggest a diagnosis of sickle cell disease. 2. Compare and contrast the different sickle cell syndromes, including presentation, treatment and complications. These syndromes include sickle cell anemia, hemoglobin SC, and hemoglobin S -thalassemia. 3. Discuss the common complications seen in a child with sickle cell disease. These include: hemolysis, hand-foot syndrome, anemia, aplastic crises, bone infarction, stroke, skin ulcers, pain episodes, priapism, sepsis and infections, cholelithiasis, chest syndrome, retinopathy, renal failure, and sequestration crises. Pulmonary hypertension 4. Outline the management of a patient who presents with a sickle crisis. These should include discussion and proper use of IV fluids, analgesics, antibiotics, blood transfusions, and indications for hydroxyurea and stem cell transplant. K K K K, PC 5. Develop a preventive care plan for a patient with a sickle disease. These should include use of folate, prophylactic antibiotics, immunizations, prompt evaluation of febrile episodes and stroke screening. K, PC Procedures GOAL X: Technical and therapeutic procedures. PL-1 & PL-3: Describe the following procedures, including how they work and when they should be used; competently perform those commonly used by the pediatrician in practice. Bone marrow: aspiration/biopsy (not expected to perform this) Central line: use/care Lumbar puncture (not expected to perform this) GOAL XI: Diagnostic and screening procedures. PL-1 & PL-3: Describe the following tests or procedures, including how they work and when they should be used; competently interpret reports of procedures and correlate clinically those commonly used by the pediatrician in practice. Radiologic interpretation: abdominal ultrasound Radiologic interpretation: Abdominal CT (with and without contrast) Radiologic interpretation: CT of head Radiologic interpretation: extremity X-ray Radiologic interpretation: MRI of head Radiologic interpretation: renal ultrasound Radiologic interpretation: PET scan Radiologic interpretation: MIBG Core Competencies: K Medical Knowledge PC - Patient Care IPC - Interpersonal and Communication Skills PProfessionalism PBLI - Practice-Based Learning and Improvement SBP - Systems-Based Practice K, PC K, PC K, PC K, PC K, PC K, PC K, PC K, PC K, PC Performance Expectations by Level of Training Beginning Developing Accomplished Competent Description of identifiable performance characteristics reflecting a beginning level of performance. Description of identifiable performance characteristics reflecting development and movement toward mastery of performance. Description of identifiable performance characteristics reflecting near mastery of performance. Description of identifiable performance characteristics reflecting the highest level of performance. Medical Knowledge Patient Care PL1 PL1, PL2 PL2, PL3 PL3 PL1 PL1, PL2 PL2, PL3 PL3 Interpersonal and Communication Skills Professionalism PL1 PL1, PL2 PL2, PL3 PL3 PL1 PL2, PL3 PL3 Practice-Based Learning and Improvement Systems-Based Practice PL1 PL1, PL2 PL2, PL3 PL3 PL1 PL1, PL2 PL2, PL3 PL3