Pediatric Hematology/Oncology Course Director: Ricarchito B. Manera, M.D. Competency Based Goals and Objectives

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1 Pediatric Hematlgy/Onclgy Curse Directr: Ricarchit B. Manera, M.D. Cmpetency Based Gals and Objectives COMPETENCY 1. Patient Care. Prvide family centered patient care that is develpmentally and age apprpriate, cmpassinate, and effective fr the treatment f health prblems and the prmtin f health. 1. Gather essential and accurate infrmatin frm the clinical histry, systematic physical examinatin and apprpriate use f diagnstic studies in children presenting with cnditins related t hematlgy/nclgy. 2. Develp a strategic plan utilizing data btained frm the histry, physical examinatin and ancillary studies and determine if the fllwing nn-specific signs and symptms are caused by a hematlgic/nclgic disease prcess and decide if the patient needs treatment r referral prlnged fever fatigue / malaise bleeding / bruising headache seizure gait abnrmality behaviral changes lymphadenpathy hepatmegaly splenmegaly abdminal pain weight lss vmiting abdminal mass jaundice hematuria jint swelling nevi 3. Diagnse and manage cmmn childhd hematlgic cnditins such as: irn deficiency anemia f infectin transient erythrblastpenia f childhd (TEC) immune thrmbcytpenic purpura (ITP) benign neutrpenia f childhd minr transfusin reactins 4. Identify, explain, d the initial management and seek cnsultatin fr the fllwing hematlgic / nclgic cnditins: anemias: underprductin and hemlytic anemias bleeding disrders, cngenital and acquired majr cmplicatins f inherited cagulatin disrders like hemphilia and vn Willebrand disease hemglbinpathies like sickle cell disease and its cmplicatins like pain crises, fever, splenic sequestratin, acute chest syndrme, aplastic crisis and strke neutrpenia, cngenital and acquired, including neutrpenic fever in children with cancer

2 thrmbcytpenia abdminal mass mediastinal mass lytic bne lesins suspected brain tumr cnditins that predispse t malignancy r assciated with it including Dwn s syndrme, neurfibrmatsis, familial cancer and hemihypertrphy. 5. Develp a keen diagnstic acumen t recgnize and plan the initial management f cmmn hematlgic / nclgic emergencies. mediastinal mass causing superir vena caval syndrme hyperleukcytsis tumr lysis syndrme spinal crd cmpressin neutrpenic fever in children with cancer 6. Prvide supprtive care t children with malignancy r bld disrders. empiric antibitics fr neutrpenic fever antiemetic use in cancer chemtherapy use f grwth factrs i.e. GCSF and erythrpietin in cancer chemtherapy transfusin therapy central line care 7. Prvide preventive cunseling t families f children with bld disrders r cancer. prvisin f adequate diet and avidance f excessive milk intake t prevent irn deficiency anemia in children with sickle cell disrders, the imprtance f rutine immunizatins including pneumcccal vaccinatin, antibitic prphylaxis and emergent evaluatin fr fever and early signs f crises avidance f drugs knwn t induce hemlysis in children with G6PD deficiency 8. Prvide screening fr cmmn hematlgic cnditins screening fr hemglbinpathies in the newbrn perid screening fr irn deficiency anemia in the first tw years f life screening infants and tddlers fr lead pisning screening adlescents fr signs f irn deficiency anemia screening fr bleeding disrder in a pre-perative patient screening fr the risk f strke in children with sickle cell anemia 9. Observe and assist cmmn clinical prcedures in hematlgy / nclgy bne marrw aspiratin bne marrw bipsy lumbar puncture with administratin f intrathecal chemtherapy exchange transfusin pheresis prcedures COMPETENCY 2. Medical Knwledge. Understand the scpe f established and evlving bimedical, clinical, epidemilgical and scial-behaviral knwledge needed by a pediatrician; demnstrate the ability t acquire, critically interpret and apply this knwledge in patient care. 1. Distinguish nrmal frm pathlgic states f the hematlgic system Knw the changes in nrmal values f hemglbin, hematcrit, cncentratins f hemglbin A and F, and MCV with age Knw the nrmal range f white bld cell cunt and differential fr a newbrn infant, an infant belw the age f 1 year and children greater than 1 year f age

3 Distinguish nrmal lymph nde frm infectius lymphadenitis and malignant lymphadenpathy Distinguish nrmal-sized liver and spleen frm hepatsplenmegaly 2. Describe the etilgy, pathphysilgy, epidemilgy, clinical manifestatins and differential diagnses as well as management f cmmn childhd anemias. Irn deficiency anemia Describe the nrmal requirements, absrptin, metablism f irn frm birth t adlescence Identify the cmmn causes and clinical features f irn deficiency anemia Distinguish irn deficiency frm ther micrcytic, hypchrmic anemias like thalassemia trait, anemia f chrnic disease, lead pisning thrugh apprpriate use f labratry tests including red cell indices, reticulcyte cunt, ferritin, ttal irn binding capacity, transferrin saturatin and irn level. Describe the management f irn deficiency anemia including irn replacement therapy, diet mdificatin, parent educatin and apprpriate fllw-up t assess treatment respnse Hemlytic anemias Distinguish the different hemlytic anemias in terms f clinical manifestatins, pathphysilgy, labratry features and management autimmune hemlytic anemia hereditary sphercytsis G6PD deficiency hemlytic-uremic syndrme hemglbinpathy including sickle cell disrders and thalassemia Knw the principles behind the tests used t diagnse hemlytic disrders hemglbin electrphresis including quantificatin f hemglbin F and A2 direct antiglbulin test smtic fragility test enzyme assay fr G6PD Knw the characteristic mrphlgic appearance f the hemlytic disrders n a peripheral smear. Sickle cell disrders Knw the varius sickle cell syndrmes ( hmzygus sickle cell anemia, sickle beta-zer thalassemia, sickle-hemglbin C disease and sickle beta-plus thalassemia) including characteristic baseline hemglbin values, hemglbin electrphresis findings and clinical manifestatins and severity f disease. Discuss the cmmn cmplicatins and management f a child with sickle cell anemia : including pain crisis, dactylitis, splenic sequestratin, aplastic crisis, acute chest syndrme, strke, gallstnes, priapism, retinpathy and suspected bacterial infectin. Outline the management f cmmn sickle cell cmplicatins including fluid management, use f analgesics, apprpriate use f antibitics, indicatins fr bld transfusin r exchange transfusin and use f hydrxyurea. Develp a preventive care plan fr a child with sickle cell diseases: prvisin f immunizatins, prphylactic penicillin, use f flate and screening fr strke using transcranial Dppler. Thalassemia disrders Describe the clinical and labratry features f alpha and beta thalassemia traits and disease. Discuss the majr cmplicatins f thalassemia majr

4 Knw the ratinale fr chrnic transfusin therapy, prphylactic penicillin, irn chelatin therapy and prmpt evaluatin f fever. 3. Understand the physilgy, pathphysilgy and management f cmmn bleeding disrders Outline an apprpriate diagnstic evaluatin fr thrmbcytpenia in a newbrn infant and a child Discuss the pathgenesis, clinical features and management f cmmn causes f thrmbcytpenia like nenatal allimmune thrmbcytpenia, thrmbcytpenia in a baby with maternal ITP and childhd ITP. Knw the indicatins fr platelet transfusin as well as the management and clinical recgnitin f patients wh becme refractry t platelet transfusin. Describe the schema f bld cagulatin. Indicate the prper questins fr btaining a bleeding histry in the child and family members. Outline a differential diagnsis and a diagnstic wrk-up f a patient with abnrmal bleeding in the newbrn perid as well as in childhd. Cite the cmmn cnditins that wuld result in abnrmalities f cmmn cagulatin screening tests like the Ivy bleeding time, prthrmbin time and partial thrmbplastin time. State the clinical features, labratry findings, cmplicatins and management f hemphilia A, hemphilia B, vn Willebrand disease, disseminated intravascular cagulatin, vitamin K deficiency and Hench-Schnlein purpura. 4. Discuss the presentatin, pathphysilgy, and prgnsis f cmmn malignancies in children and adlescents. Summarize the presenting signs and symptms, cmmn age at presentatin, diagnstic prcedures, staging, principles f current therapy, prgnsis and lng-term cmplicatins fr the fllwing malignancies: Leukemia (ALL, AML) Brain tumrs Neurblastma Hdgkin s and nn-hdgkin s lymphma Wilms tumr Rhabdmysarcma and ther sft tissue sarcmas Bne tumrs like stesarcma and Ewing s sarcma Langerhans cell histicytsis Knw the mechanisms f actin, mde f administratin and imprtant side effects f cmmnly used chemtherapeutic agents: vincristine, asparaginase, prednisne, methtrexate, cyclphsphamide, cytarabine, anthracycline agents and epipdphylltxins. Knw the adjunctive medicatins that can increase a patient s tlerance t chemtherapy, e.g., leukvrin rescue fr methtrexate, cardiprtectants like dezrazxane fr anthracyclines, mesna fr cyclphsphamide as well as grwth factrs like GCSF and erythrpietin. Discuss the cmmn early and late cmplicatins f cancer treatment such as myelsuppressin, endcrine suppressin and risk fr secndary cancer. Discuss the principles, indicatins and cmplicatins f stem cell r bne marrw transplantatin.

5 COMPETENCY 3. Cmmunicatin Skills. Demnstrate interpersnal and cmmunicatin skills that result in infrmatin exchange and partnering with patients, their families and prfessinal assciates. 1. Obtain, recrd and present in a systematic and rganized fashin the histry, physical examinatin findings and pertinent data frm ancillary studies f patients presenting with hematlgic/nclgic cnditins. 2. Prvide accurate and detailed infrmatin t the patient, the parents, and ther family members pertaining t the patient s diagnsis, prgnsis, curse f management, alternative treatments if any, risks, side effects and cmplicatins f treatment. Assist the specialist in delivering bad news t the family f a child with a life-threatening cnditin like malignancy Assist the specialist in btaining an infrmed cnsent frm the family when enrlling a child in a clinical trial. Prepare a set f patient educatin materials a list f pertinent peer-reviewed web sites t assist the family. 3. Maintain cnstant cmmunicatin with the referring physician and ther health care prfessinals n matters pertaining t the patient s cnditin including prviding cntinuing feedback n the patient during the curse f treatment. 4. Mtivate patient and parents in maintaining and prmting health, infectin preventin thrugh anticipatry guidance and cunseling 5. Demnstrate sensitivity in cmmunicating with patients and families taking int cnsideratin their level f educatin, age, cultural backgrund, religin and persnal beliefs. COMPETENCY 4. Practice-based Learning and Imprvement. Demnstrate knwledge, skills and attitudes needed fr cntinuus self-assessment, using scientific methds and evidence t investigate, evaluate, and imprve ne s patient care practice. 1. Use scientific methds that are evidence-based t update and imprve the standards f patient care Be able t access infrmatin and d nline searches n particular presenting prblems and cnditins in a patient. Identify standardized guidelines fr diagnsis and treatment f hematlgic / nclgic cnditins such as clinical prtcls fr the treatment f children with cancer and practice guidelines in the management f patients with sickle cell disease. 2. Be aware f new trends and advances in the field f hematlgy / nclgy and be prepared t alter r mdify ne s practice in respnse t these changes 3. Critique new scientific infrmatin encuntered in the medical literature and determine its relevance t clinical practice. 4. Evaluate ne s perfrmance thrugh self-assessment as well as thrugh feedback frm patients, peers and superirs t imprve patient care. COMPETENCY 5. Prfessinalism. Demnstrate a cmmitment t carrying ut prfessinal respnsibilities, adherence t ethical principles, and sensitivity t diversity.

6 1. Demnstrate persnal respnsibility and accuntability fr patient care thrugh diligent fllw-up f a patient s prgress, labratry and ancillary prcedures, writing cmprehensive ntes and cmmunicating regularly with the referring physician 2. Display behavirs that fster and maintain a family s trust and cnfidence such as apprpriate attire, gd grming, hnesty, curtesy, respect fr cnfidentiality at all times. 3. Meet high standards f legal and ethical behavir in dealing with patients, families, as well as peers, superirs and nn-medical supprt staff 4. Advcate the interests f ne s patients ver self-interest 5. Demnstrate sensitivity t patients and clleagues gender, age, culture, religin, disabilities, ethnicity and sexual rientatin. COMPETENCY 6. Systems-Based Practice. Understand hw t practice quality health care and advcate fr patients within the cntext f the health care system. 1. Interact with the case manager and scial wrker t facilitate setting up hme care services t facilitate patient access t nursing care and ther services utside the hspital with awareness f the family situatin and resurces. 2. Help set-up a fllw-up plan fr a patient prir t discharge t ensure cmpliance and cntinuity f care 3. Interact with the scial wrker in setting up hspice care t children with chrnic debilitating cnditins r terminal illness. 4. Be aware f the supprt grups available t families dealing with children with special needs and chrnic cnditins like: Leukemia Sciety, American Cancer Sciety, Cley s Anemia Fundatin, Fancni s Anemia, Aplastic Anemia Organizatin and thers. 5. Knw the resurces available lcally and in the hspital t imprve quality f care and enhance a patient s well-being such as: Child Life Schl Prgram t tutr the children Make a Wish Fundatin Special Friends Prgram

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