Carol S. Viele RN MS OCNClinical Nurse Specialist

Hematology-Oncology-Bone Marrow TransplantUCSF

Associate Clinical; ProfessorDepartment of Physiological Nursing

UCSF School of Nursing

OverviewHeld in Boston, MassachusettsApril 29-May 1, 201155 sessions over 3.5 days of the meetingSession topics included:

Infection, Sepsis UpdateClinical Trials/ Protocol IssuesInternational OncologyEthicsPreparing for the future of Oncology NursingBMT ToxicitiesSafe Handling IssuesGenotype directed therapyGenetics

Highlights in Crash Course in BMTPresenters from Johns Hopkins, Seattle Cancer Care Alliance and

Stanford UniversityTopics: Pulmonary Issues, Hepatic Toxicity and Hepatic GVHD and

Skin ToxicityPulmonary Issues

Incidence 30-60% Cause of death 60% Diagnosis

Bronchoscopy Lung biopsy

Risk Factors/Etiology Aspergillus CMV Pneumocystis jiroveci Bronchiolitis Obliterans BOOP DAH

Highlights in Crash Course in BMTInterventions

AntibioticsAntifungalsAntiviralsSteroidsAnxiolytics

BenzodiazepinesDypsnea management

Highlights in Crash Course in BMTHepatic Complications

Sinusoidal obstruction syndromeGraft versus host diseaseDrug induced lung injuryInfections

Bacterial Fungal Viral

Cholecystitis

Highlights in Crash Course in BMTDiagnostic tests

Laboratory dataImagingLiver biopsy

PreventionUrsodiolAntifungalsAntiviralsConditioning regimens

Decreased intensity regimens No cytoxan

Highlights in Crash Course in BMTTreatments

Low dose tissue plasminogen activator20% response

Antithrombin IIIDefibrotide

> 36 % response rate

Highlights in Crash Course BMTHepatic Graft versus Host disease

Onset 2-4 weeks post BMTJaundice and increased LFT’sStaging directly related to level of bilirubinPrevention and Treatment

Calcineurin inhibitors Mycophenolate mofetil Methotrexate Ursodiol Steroids ATG Sirolimus Rapamycin Monoclonal antibodies

Highlights Genotype Directed Therapy Lung CancerBy Lecia Sequist MD, MPHNSCL Cancer therapy

Chemotherapy- modestly successfulMolecular targetingKey pieces to understand the cell biology of each

individual’s tumorTreatment effective against the particular biology of

tumor EGFR dysregulation

Tyrosine kinase inhibitors in lung Gefitinib- Iressa Erlotinib- Tarceva

Highlights Gene Directed Therapy Lung CancerTreatment:

Find EGFR mutations in patients10% of lung cancer patient have EGFR mutationsResponse rates as high as 70% in this group of patients 1

Based on the Mok trial US is looking at need for molecular testing of tumors

More common in: Women Never smokers Little smoking history

Mok 2009 NEJM

Highlights Gene Directed Therapy Lung CancerTargeted therapy eventually develops resistanceMass General is doing repeat biopsies to track

resistance development in tumorsInitial response is usually 12 monthsLooking at another pathway the MET inhibitorAdding a MET inhibitor with ErlotinibAnother pathway is ALK translocation

First described in 2007Can be responsible for lung cancer progression

Highlights Gene Directed Therapy Lung CancerCrizotinib – a new agent being trialed and the target

is ALKPhase I study150 PatientsDramatic responses? FDA approval in 2011

Highlights Gene Directed Therapy Lung CancerFuture genotype directed therapy in lung cancer

KRASALKBRAFMETPDGFREGFR

Highlights Biology of Pediatric and Adult CancersJohn Maris MD Children’s Hospital PhiladelphiaBelinda Mandrell PhD RN PNPThe future in cancer treatment is a “ personal

approach”Need to understand hereditary cancersGenomic profilingPractical and ethical implications

Highlights Biology of Pediatric and Adult CancersChildhood cancers

Continue to cause significant morbidity and mortalityCure rates are stagnantLate effects are significantChildhood cancers represent a microcosm of cancers

in generalCancer is the leading cause of death in children

except for accidents2/3 of children who survive have life long disabilities1/4 of the children who survive have significant life

long disabilities such as CHF and hearing loss

Highlights Biology of Pediatric and Adult CancersMolecularly targeted agents

Increase the cure ratesDecrease the toxicity rates

Highlights Biology of Pediatric and Adult CancersNeuroblastoma

Median age at diagnosis 17 months15% of childhood mortalityInduces significant morbidity30% of cases spontaneously resolve50% of cases are high risk diseaseNeed to define the molecular targets

Genetic basis of diseaseDefine the oncogenic drivers of this disease

Highlights Biology of Pediatric and adult CancersGenomic profiling

ALK (Anaplastic lymphoma kinase) gene is the major familial neuroblastoma gene and is located on chromosome 2 Occurs in 80% of familial disease

PHOX 2B occurs in 10% of Familial neuroblastoma

Highlights Biology of Pediatric and adult CancersGenomic Profiling includes:

DNA copy numbers- Single Nucleotide Polymorphism arrays

RNA copy numbers – Expression arraysMutations- Sequencing analysisVision for all patients they will all have DNA

sequencing done at diagnosis. As we treat patients mutations will occur and moving forward we can profile the DNA and RNA alterations

Highlights Biology of Pediatric and Adult CancersGenetic Profiling Considerations

Family history may suggest a genetic cancer syndrome

Tests need to be adequately interpretedConsent for testing must occurPre and Post counseling needs to occurPatients need to know the results may affect

their ability to obtain life insurance not health insurance

Highlights Biology of Pediatric and Adult CancersInformed Consent Issues:

Clinical ImplicationsImportance for childrenAccuracy of testingFeesPsychological issuesConfidentiality issuesInsurance issues