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Childhood Cancer Navigating a Course Toward Cure Dyane Bunnell, MSN, APRN, AOCNS, CPON Nemours/Alfred I. duPont Hospital for Children 1 Objectives Discuss the treatment developments and current outcomes in the field of pediatric oncology. Identify the future direction of pediatric oncology and the role of professional nurses in shaping the specialty’s future. 2 Childhood Cancer Overview A group of chronic diseases characterized by uncontrolled growth and spread of abnormal cells, which, if not adequately treated, results in death. 15,700 cases of childhood cancer diagnosed annually 2,000 cancer-related deaths annually 375,000 adult survivors of childhood cancer 3 History of Childhood Cancer 1940’s First chemotherapy agent (nitrogen mustard) discovered after use of chemical weapons in World War II Discovery of effect of folic acid in leukemia patients First remission ever induced in childhood leukemia 1950’s Many new chemotherapy agents discovered First randomized clinical trial 4 History of Childhood Cancer 1960’s Majority of present day chemotherapy agents are discovered 1970’s First combination chemotherapy was developed for childhood leukemia patients First use of intrathecal chemotherapy to prevent CNS disease First successful bone marrow transplants were performed Two-hit theory of DNA mutation to explain development of cancer Human Subjects Protection mandated in clinical trials 5 History of Childhood Cancer 1980’s Discovery of hematopoietic growth stimulating factors (GCSF) Cancer causing oncogenes were discovered 1990’s Molecular understandings of normal and malignant blood cells make new "targeted" anti-cancer drug therapies and immunestimulating therapies possible 6 Amazing Success 100 80 5-year 60 survival [%] 40 1960s 1990s 20 0 Leukemia Lymphoma Wilms 7 Room for Improvement 100 80 5-year survival [%] 60 1960 1990s 40 20 0 AML High Risk NB Brain Stem Glioma 8 In the Course of a Lifetime… Overall Survival Childhood Cancer 100% 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% 1960 1982 2016 9 Drug Development: Childhood Leukemia 1950’s – – – – – Prednisone Methotrexate Thioguanine Mercaptopurine Cyclophosphamide 1960’s – – – – – 1980’s 1990’s 2000’s – Clofarabine 2010’s – Dinutuximab Asparaginase Doxorubicin Daunorubicin Vincristine Cytarabine 1970’s – Etoposide 10 Success: Cooperative Research Groups Children’s Oncology Group Pediatric Oncology Group SWOG Children’s Cancer Group IRS NWTS CALGB 11 Success: Pediatric Trials and Treatment Centers Improved survival for patients treated on childhood leukemia protocols 16 to 20-year-olds on CCG Study: 7-year EFS=63% ©2008 by American Society of Hematology 16 to 20-year-olds on CALGB Study: 7-year EFS=34% Years Followed Poorer survival for patients treated on adult leukemia protocols Stock et al., 2008 Survival Rates 13 Navigating statistics 14 Success: Multimodal Treatment Strategies ♦ Chemotherapy ♦ Biotherapy ♦ Radiation ♦ Surgery ♦ HPCT 15 Success: Multidisciplinary Supportive Care ♦ Oncology ♦ Social work ♦ Radiation ♦ Psychology ♦ Surgery ♦ Dietary ♦ Nursing ♦ Clergy ♦ Rehabilitation ♦ Educators ♦ Pharmacy ♦ Child life ♦ Laboratory ♦ Radiology ♦ Respiratory ♦ Volunteers ♦ Interpreters ♦ Clinical research associates (CRAs) 16 Success: Evolution of Pediatric Oncology Nursing COG Nursing Discipline Clinical trials organization NCI grant funded COG member website – – – – Protocols Clinical resources Reference manuals Patient education materials COG Nursing Accomplishments 18 Success: Evolution of Pediatric Oncology Nursing Association of Pediatric Hematology Oncology Nursing Professional organization for pediatric hematology/oncology nurses Founded 1973 - > 3000 current members Promotes highest quality care for children with cancer and blood disorders Educational opportunities - annual conferences Local chapters - meetings, conferences Reference materials - books, slides, website Revolutionary Discoveries Rituxan (rituximab) – Monoclonal antibody for patients with NHL – 1997 FDA approved (first targeted therapy approved in the US) Gleevec (imatinib mesylate) – Targeted molecular therapy for patients with CML – 2001 55% 5 year survival rate; Gleevec FDA approved – 2006 90% 5 year survival The Leukemia & Lymphoma Society. Facts 2011-2012. Available at: http://www.lls.org/#/resourcecenter/freeeducationmaterials/generalcancer/facts 20 A Molecular Breakthrough A once-fatal disease (median survival 3-6 years), now an “indolent” leukemia with Gleevec for life “Druker achieved the perfect inversion of the goals of cancer medicine: his drug increased the prevalence of cancer in the world” (Mukherjee, 2010) 21 Innovations Human Genome Project Completed in 2003 A far less publicized (but vastly more complex) project: Cancer Genome Atlas Fully sequence the genome of human cancer cells 22 Innovations: Supportive Care Blood Products Diagnostic imaging Surgical innovations Colony Stimulating Factors 23 Innovations: Supportive Care Chemo-protective agents (Mesna, Zinecard) Antimicrobials Pediatric Intensive Care 24 Innovations: Supportive Care Hickman catheters Psychosocial services Discovery of serotonin receptor antagonists (ondansetron) for management of CINV 25 Current Clinical Trials Therapy Trials – Novel agents – Reduced treatments and toxicities Biology – Genomics Survivorship – Late effects Quality of Life Research – Symptom management – Psychosocial support 26 Challenges 27 Challenges Funding – “Cancer in kids is not profitable” – “Kids do not vote” Clinical Trials – “Kids’ cancers are different” – “There aren’t many kids” 28 The Horizon Immunotherapy Doctors Inject HIV Into Dying Girl, Cure Her Leukemia 29 The Horizon Cancer Therapeutics Identify the crucial cancer mutation THEN hunt for a targeted therapy against those genes 30 Next Navigation Priorities Acknowledge that cure rates in low- and middle- income countries are far behind the United States Change the fact that 1 in 8 children diagnosed with cancer today will not survive Reduce the burden of morbidity among those who survive Discover new treatment approaches for malignancies with poor prognosis Eliminate disparity in access to care and clinical trials 31 Questions and Discussion [email protected] 32 References/Resources American Cancer Society. Cancer Facts & Figures 2010. Atlanta: American Cancer Society; 2010. Association of Pediatric Hematology Nursing (APHON) (www.aphon.org) Baggott, C., Fochtman, D., Foley, G.V., & Kelly, K.P. (Eds.). (2011). Nursing care of children and adolescents with cancer and blood disorders (4th ed.). Glenview, IL: APHON. Boklan, J. (2006). Little patients, losing patience: Pediatric cancer drug development. Molecular Cancer Therapeutics, 5, 1905-1908. Brucker, M. (1999). National Childhood Cancer Foundation. Neoplasia. 1(3): 285-286. Centers for Disease Control, (2007). Trends in Childhood Cancer Mortality, United States, 1990-2004; MMWR 56(48):1257-1261. Children’s Oncology Group (www.childrensoncologygroup.org) Leukemia Lymphoma Society (www.lls.org) Mukherjee, S. (2010). The Emperor of all Maladies. New York: Scribner. Stock, W., La, M., Sanford, B., Bloomfield, C. D., Vardiman, J. W., Gaynon, P., Nachman, J. (2008). What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols? A comparison of Children’s Cancer Group and Cancer and Leukemia Group B studies. Blood, 112(5), 1646-1654. 33