“A bridge without gaps”
Goals of Care in AYA and other disparate populations
Dr Rachel Hughes Lead Clinician, Western Australian Youth Cancer Service
Palliative Care Outreach Physician, Pilbara, Western Australia
Acknowledgements
WA YOUTH CANCER SERVICE
Session Overview: What lessons are learnt from a “disparate cohort”?
AYA oncology – do we
need ANOTHER
specialty?:
•Who are the AYAs?
•When cancer and youth
health collide
Health During Cancer:
• AYA are socially and
biologically unique
• Survivorship
• Incurable disease
A model of care for
AYA and what have we
learnt?
• Youth Cancer
Services
• Care Coordination
• Sustainability
Edward Morris : History of the London Hospital (3rd Ed) 1926
10
years
20 year222s
30
years
Industrial
revolution
Agicultural
settlement
Mid 20th
Century
Menarche
Psychosocial
maturation
Hunter
gatherers
Menarche & social transitions in high income world
AAGluckman, P. D., and M. A. Hanson. "Changing times: the evolution of puberty." Molecular and cellular endocrinology 254 (2006): 26-
31.
Present
Day
Adolescence
10 years
20 years
30 years
The road is long:
Hunter
gatherersHunter
gatherers
Hunter
gatherers
Agricultural
settlement
Industrial
Revolution
Mid 20th
centuryPresent day
Social determinants?
• (Seeman et al., Synapse, 1987) (see also Huttenlocher & Dabholkar, J Comp Neurol,1997;
• Petanjek et al., PNAS, 2011)WA YOUTH CANCER SERVICE
The adolescent brain:
• (Seeman et al., Synapse, 1987) (see also Huttenlocher & Dabholkar, J Comp Neurol,1997;
• Petanjek et al., PNAS, 2011)
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Decisions, decisions….
Health of Adolescents and Young Adults
Adolescents : Health Risks and Solutions – WHO (2014)
•Sexual Health
•Mental Health
•Violence
•Alcohol and Drugs
•Injuries
•Nutrition – malnutrition and obesity
•Tobacco
•Exercise
•Adolescent Rights
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birth 15 30 40 years
ChildrenCancers of early growth
Adolescents and Young Adults
AdultsCancers of aging
ALLNHLPNETWilmsNeuroblastomaRetinoblastomaCraniopharyngiomaRhabdomyosarcoma
OsteosarcomaEwing sarcomaGCT boneRhabdomyosarcomaSoft tissue sarcomasDesmoplastic small round cellOligodendrogliomaCNS germ cell tumoursMelanomaHodgkinPh+ ALLAPMLAMLTesticular CaOvarian CaColon CaHepatic CaNasopharyngeal CaBronchoalveolar Ca
CarcinomasAerodigestiveGenitourinary
BreastSkin
etc
Diagnostic Delay
Unfavourablecytogenetics and
histology
Host biology and pharmacokinetics
Referral pathways and treatment setting
A correlation between survival and accrual to clinical trials
Bleyer et al 2003
30%
20%
10%
0%
2%
1%
0%
-1%
-2%
-3%0-4 5-9 10-14 15-19 20-24 25-29 30-34 35-39 40-44 45+
Ave
rage
An
nu
alC
han
ge 5
Yr
Surv
ival
10% 20% 30%
P=0.003
2.4%
1.2%
0%
-1.2%
-2.4%
-3.6%0%
Accrual %
Age
Ave
rage
An
nu
al C
han
ge 5
Yr
Surv
ival A
ccrual o
nto
Clin
ical Trials (%)
Soft-TissueSarcomas
Increased toxicity for AYA receiving paediatric protocols?
Age is recognised to influence toxicity profiles.
Observations from the literature:
• Treatment related adverse effects and mortality (St Jude)
• Osteonecrosis (AIEOP-ALL 95, ALL-BFM95)
• Thromboembolic events (DFCI ALL protocols)
• Tumour lysis
• Hyperglycemia, Diabetic ketoacidosis
• Higher infection rates (CCG 2961, CCG2891, AML-PFM 93, AML-PFM 98)
• Obesity
Wood, W. A., & Lee, S. J. (2011). Malignant hematologic diseases in adolescents and young adults. Blood, 117(22), 5803-
5815.
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AYA prevalent toxicities:
Vincristine Neuropathy
Myelosuppression
Osteonecrosis
Bone marrow transplantation complications
Late effects of treatment
Bukowinski : Toxicity of cancer therapy in adolescents and young adults.
Seminars in oncology nursing Aug 2015
Fertility Preservation - female
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Stern, C., Conyers, R., Orme, L. M., Barak, S., Agresta, F., & Seymour, J. (2013). Reproductive concerns of children and adolescents with cancer: challenges and potential solutions. Clinical Oncology in Adolescents & Young Adults, 3.
Patient age General medical conditioncytopenias coagulopathy
Treatment Type
Chemotherapy:Alkylating agentsCumulative Effect
Radiotherapy:CranialWhole BodyAbdo / pelvic
Pre-transplant
Risks of processHormonal
manipulationAnaesthetic
Diagnosis – treatment urgency
Disease contamination of reproductive tissues
Fertility preservation - male
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Symptoms in AYA cancer patients
Most common symptoms
during treatment
• Fatigue
• Sleep-wake disturbances
• Nausea
• Anorexia / eating problems
• Pain
• Mood disturbance
• Appearance Changes
Erickson, J. M., MacPherson, C. F., Ameringer, S., Baggott, C., Linder, L., & Stegenga, K. (2013).
Symptoms and symptom clusters in adolescents receiving cancer treatment: A review of the
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• Is the profile unique?
• Is the burden different?
• Should management differ?
AYA have many significant years ahead of them.
They enter these years with a unique and heavy burden
• Higher rates obesity and physical morbidity
• Lower educational achievement
• Greater levels of social isolation
• Higher rates of anxiety and depression
• High prevalence post traumatic stress disorder
• Significant fear of cancer recurrence
OnTrac at Peter Mac : Survivorship Connections
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After treatment:Young cancer survivors are a high risk medical cohort
Higher risk:
•Cardiovascular toxicity
•Neurocognitive effects
•Chronic Diseases – obesity, diabetes, hypertension
•High risk pregnancy
•Poor general health
•Recurrent malignancy
•Second malignancy
•Premature ovarian failure
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After treatment:Young cancer survivors are a high risk medical cohort
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Hodgkins Lymphoma cure rate 95-97% in AYA
Thyroid problems 30%
Second Malignancy: Breast, lung, thyroid, stomach, bone, soft tissueHaematological malignancyBreast cancer secondary to radiotherapy
15-25x populationCumulative risk 12-17% at 30 years
(Horwich, Swerdlow. British journal of cancer (2004)
Cardiomyopathies: Radiotherapy, anthracyclinesHigh risk pregnancy
Others:
Continuing the transition
• “AYAs occupy the world between child and adulthood.”
• “The speed of this process depends on the individual and their circumstance.”
• “It is important to balance unachievable goals with goals that can be realised, however short a life may be”.
Rob George and Finella Craig
Palliative Care for Young People with Cancer (2009)
Picture: www.stefaniaboleso.com/is-google-plus-growing-up/
What do young people want from their health care provider?
Balancing hope and realism
“They know what my future will be like. They
know how desperate and sad and painful and
short my situation is. But without ever being
unrealistic or instilling false hope, they somehow
contribute to making sure I’m not a desperate or
sad human being. They tell it how it is, no spin.
Then they proceed to assure me that I can still
do excellent things and be an excellent person.
They encourage me to make my life, shall I say,
more potent. And to enjoy everything I have the
capacity to enjoy.”
Jack 21yo
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Tricky Disease – Creative Solutions
Palliative chemotherapy
Surgery – metastectomy, pneumonectomy
Stenting
Bronchoscopy
Embolisation
Radiotherapy
Pain procedures eg nerve blocks
Clinical trials
Control of bleeding
“He had a very difficult hour and a half; the cancer had filled his lungs and his lungs had filled with fluid so he essentially suffocated, and that is not an easy way to die, but that was the way he wanted to die. He wanted to be conscious and he was. As hard as those memories are to be there while he suffered so much, we’re so blessed because we were there — we were all there”
Did we do a good enough job? – Uncontrolled symptoms
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A role for consultative youth cancer services:
Health Before:
• Adolescent Health
• Advocacy
• Diagnostic delay
• Referral pathways
• Sarcoma MDT
Health During:
• Fertility Preservation
• Psychosocial support
• Symptomatology
• Care coordination
• Clinical trial access
• Incurable Illness
• GP communication
Health Beyond:
• End of treatment
• Late Effects
• Surveillance
• Chronic disease
• Transition
• Palliative Care
Sustainable services
Educated champions are the best advocates
Sustainable services
Consultation with your cohort
Sustainable services
Don’t be modest – demonstrate your work
Acknowledgments
YOUTH CANCER SERVICE
• 1.Palmer, S., & Thomas, D. (2008). A practice framework for working with 15–25 year-old cancer patients treated within the adult health sector. Melbourne: onTrac@PeterMac: Victorian Adolescent & Young Adult Cancer Service.
• 2.Jones, B. L. (2008). Promoting healthy development among survivors of adolescent cancer. Fam Community Health, 31 Suppl 1, S61-70.
• 3.Langeveld, N. E., Stam, H., Grootenhuis, M. A., & Last, B. F. (2002). Quality of life in young adult survivors of childhood cancer. Support Care Cancer, 10, 22.
• 4.Shama, W., & Lucchetta, S. (2007). Psychosocial issues of the adolescent cancer patient and the development of the teenage outreach programme (TOP). Journal of Psychosocial Oncology, 25(3), 99-112.
• 5.Zebrack, B. (2009). Information and service needs for young adult cancer survivors. Support Care Cancer, 17(18543006), 349-357
• 6.Kwak, M., et al. (2013). "Prevalence and predictors of post-traumatic stress symptoms in adolescent and young adult cancer survivors: a 1-year follow-up study." Psycho-oncology 22(8): 1798-1806.
• 7.Varela, V. S., et al. (2013). "Posttraumatic stress disorder (PTSD) in survivors of Hodgkin's lymphoma: prevalence of PTSD and partial PTSD compared with sibling controls." Psycho-oncology 22(2): 434-440.
• 8.Palmer, S., Mitchell, A., Thompson, K., & Sexton, M., (2007). Unmet needs among adolescent cancer patients: A pilot study. Palliative and Supportive Care, 5, 127-134.
• 9.Ganz, P. A., Earle, C. C., & Goodwin, P. J. (2012). Journal of Clinical Oncology Update on Progress in Cancer Survivorship Care and Research. Journal of Clinical Oncology, 30(30), 3655-3656.
• 10.Thompson, K., Palmer, S., & Dyson, G. (2009). Adolescents and young adults: Issues in transition from active therapy into follow-up care. European Journal of Oncology Nursing, 1-6.
• 11.Von Essen, L., Enskär, K., Kreuger, A., Larsson, B., & Sjödén, P.O. (2000). Self-esteem, depression and anxiety among Swedishchildren and adolescents on and off cancer treatment. Acta Pædiatr, 89, 229-236.
• 12.Whyte, F., & Smith, L., (1997). A literature review of adolescence and cancer. European Journal of Cancer Care, 6, 137-146.
• 13.Zebrack, B. J. (2000). Cancer Survivor Identity and Quality of Life. Cancer Practove, 8(5), 4.
• 14.Decker, C. L. (2006). Coping in adolescents with cancer: a review of the literature. J Psychosoc Oncol, 24(4), 123-140.
• 15.Towle, A., Godolphin, W., & Van Staalduinen, S. (2006). Enhancing the relationship and improving communication between adolescents and their health care providers: A sc
• 16.Wood, W. A., & Lee, S. J. (2011). Malignant hematologic diseases in adolescents and young adults. Blood, 117(22), 5803-5815.
• 17. Erickson, J. M., MacPherson, C. F., Ameringer, S., Baggott, C., Linder, L., & Stegenga, K. (2013). Symptoms and symptom clusters in adolescents receiving cancer treatment: A review of the literature. International journal of nursing studies