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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