An Argument for Adolescent and Young Adult Cancer Registry: One Model

Abstract

Over the last several years, there has been increasing awareness around the unique challenges faced by adolescent and young adult (AYA) cancer patients. More cancer centers across the United States are introducing AYA-specific programs to help improve outcomes for these patients. However, given the nature of the United States health care system, there is little ability to track the efficacy of these programs and identify important variables with respect to both interdisciplinary interventions offered and medical and psychosocial outcomes. One program offers an argument as to why tracking these data is important, with a description of the registry they have developed.

Introduction

Approximately 70,000 adolescents and young adults (AYAs) between the ages of 15 and 39 are diagnosed with cancer each year in the United States.^1,2 AYAs experience a distinct set of challenges when presented with a cancer diagnosis, including access to medically appropriate care that takes into account their unique psychosocial needs. The complexity of dealing with a cancer diagnosis can overwhelm AYAs already grappling with social and financial obligations, including school, work, relationships, and family. While those who work closely with AYA patients may recognize the challenges in working with this population, there is still little quantitative research highlighting the clinical complexities (from both medical and psychosocial perspectives) that AYA cancer patients and survivors face. While countries with universal health care, and thus large population registries to pull data from, may be able to easily track some outcomes, the United States does not have a similar system in place. Additionally, some important variables related to AYA intervention and outcomes may not be considered essential data for national patient registries (e.g., results of neuropsychological assessment and participation in rehabilitation therapies). Establishing long-term data registries with AYA patients focused on their specific needs may be an important first step in better understanding how to improve medical outcomes and overall quality of life. This article will present an example of one such registry for an AYA oncology program embedded within a pediatric medical center.

Unique Challenges Faced by AYA Cancer Patients

For the AYA population, the crux of the cancer conundrum lies in where members of this age group belong. Simply knowing where to treat AYA patients poses a challenge as they may feel equally out of place in a stark adult cancer unit filled with elderly patients as in an outpatient waiting area equipped for young children.³ Even once the linear path of diagnosis, treatment, and recovery has been established, AYAs have many peripheral needs that are not always adequately addressed in either an adult or pediatric program, including interaction with peers, fertility preservation, access to child care, diet and nutritional interventions, and information about their diagnosis.⁴ Further complicating treatment is the knowledge that AYA patients have riskier health-related behaviors,⁵ including lower adherence to medical recommendations.^6,7 While it is not unique to the AYA population that patients who exhibit significant psychological symptoms have poorer outcomes, sometimes even halting treatment altogether,^7–10 AYA patients are at higher risk for developing psychological symptoms compared with younger pediatric or older adult patients.⁸

Medical challenges in the AYA population

Despite substantial strides in the diagnosis and treatment of cancer that have occurred in the past several decades, survival statistics in the AYA population significantly trail those of their older and younger peers.¹¹ While this may be attributed to myriad factors affecting this age range, including barriers to health care such as the lack of insurance or reluctance to seek medical attention for symptoms, a major obstacle to improving outcomes is the failure to enroll AYA patients in clinical trials.^11–13 The lack of access to clinical trials for AYAs may be compounded by the relatively small numbers of both patients and trials they qualify for. AYAs account for just 5% of the entire cancer population, and the types of cancers diagnosed in this small cohort of patients tend to be unique to their age group.¹⁴ Medical oncologists who rarely treat AYA patients or cancers particular to this age group may be unaware of current clinical trials for which their patients may be eligible.

A number of challenges also exist with respect to transitional care. Mulder et al.¹⁵ outlined a number of challenges faced by adolescent patients and practitioners taking care of these cancer survivors. They note that there are no guidelines for the transition from pediatric to adult clinics and that this time period (as evidenced in other adolescent medicine research^15,16) is a critical time period for AYAs in management of their long-term medical needs. Patients often have difficulty choosing between adult and pediatric providers as well as when to transition to adult care services for a variety of reasons (e.g., they are unclear about clinic rules around age of catchment and need for long-term follow-up and patient and parental anxieties^15,16). However, communication (both between providers and between patient and providers) and knowledge about care needs appear to be the most important measures of comfort and success in this transition.^17,18

Psychosocial challenges in the AYA population

Emotional

The ramifications of a cancer diagnosis in this group can cause delayed and/or prolonged adolescence for many patients.¹⁹ AYA patients and survivors of cancer are at a higher risk for depression, anxiety, post-traumatic stress disorder, low self-esteem, poor body image, and suicide when compared with younger children with cancer and their healthy controls.^19–24 Poor adjustment to cancer has led a small percentage of AYAs to halt treatment.¹⁰ As a result of their numerous and complex psychological symptoms, AYAs have a high need for psychotherapeutic services.²⁵ While individual treatment can be difficult to access for a number of reasons, group treatment is particularly effective in AYAs with cancer.²⁶

Social

Similarly to healthy peers, desire to fit in, form meaningful relationships, and identity development are prominent social concerns for AYAs.^25,27,28 AYAs in treatment for cancer often find themselves physically and socially isolated as a result of the need for hospitalization/isolation, low incidence rates of AYA cancer diagnoses (resulting in fewer people with similar experiences), and challenges of transitional care and hospital placement in this age range.^28–31 Pre-existing relationships that are crucial to reaching these developmental milestones and aid in coping can become strained due to lengthy absences from one's typical routine. AYAs also report feeling awkward around peers who have not suffered with cancer due to different perspectives on life, marginalization, and physical manifestation of their cancer.^25,28,31 Lack of ability to date or explore one's sexuality can also cause great distress,²⁹ including in lesbian gay bisexual transgender queer (LGBTQ) populations (an especially understudied population). Given the increase in sexual risk-taking behavior as a result of certain cancer diagnoses in AYAs, this population may be especially at risk.³²

Even though access to true peers leads to better outcomes for AYAs with a chronic illness,³³ not all AYAs have access to peer support and thus turn to their families.³⁴ However, these relationships are also strained as patients report frustrations with their new sick role, regression in parental treatment of them (e.g., being treated as if they are younger), feelings of guilt or desire to protect their families or partner from distress, and concerns about cultural acceptance of their illness.^24,25,31 The ability to parent, especially young children, is also significantly challenging even with the decision on when/how to disclose information to one's children.³⁵ Feelings of parental inadequacy or concerns about the impact of one's cancer on children, as treatment and survivorship progress, can lead to additional negative outcomes in all domains of functioning. A growing body of research suggests that family functioning, regardless of the parent/child role of the AYA, is an especially important factor as it relates to these outcomes.^36–38

Financial/occupational

Concerns about finances and education are significant stressors as the burden of medical care and uncertainty about the future can cause decision-making around these issues to become particularly complex.^25,30 As a result, life plans are delayed, which can increase distress and ambiguity as patients progress through treatment and enter into survivorship.³⁵ Research supports an early return to work or school for AYAs as having positive occupational, financial, and psychosocial outcomes in both short and long terms.³⁹ However, a significant portion of patients who do return to work have some kind of difficulty as a result of the physical, cognitive, and emotional impacts of their cancer.^40,41 Planning around the ability to return to work is crucial, but pediatric cancer programs typically do not offer resources in this area. A review of survivorship data from this hospital showed that while patients under the age of 18 are routinely provided with some form of treatment recommendations and support in school, adults are typically not provided with these resources (both for school and work).⁴² Lack of support in this area may be especially impactful when decisions around debt and health insurance (and thus follow through with medical appointments and treatment recommendations) are at stake. Some patients also may not be able to obtain health or life insurance or may be forced to pay high insurance premiums because of their cancer history.

Description of the Program

Our hospital is a suburban pediatric academic medical center and serves as the primary pediatric cancer center between two large urban areas in the region. Over 7000 ambulatory patient visits are conducted per year, of which 75–100 are new cancer diagnoses. Of patients at all levels of the treatment process, upward of 33% are AYAs. The hospital is also physically connected to a large adult hospital. Adult oncology patients are seen within a number of divisions, including providers within the surrounding community, and primarily followed as outpatients. All adult oncology providers see ∼1100 new diagnoses each year, although data were not available on the percentage of AYA patients.

Recently, our institution established an AYA program with a mission to:

“provide high-quality, evidence-based, coordinated care that promotes the physical, psychological and emotional health of adolescents and young adults with cancer during and after therapy by empowering them with the resources and knowledge they need to live an independent and fulfilling life.”

In accomplishing this mission, the AYA program staff (Fig. 1) set out to create a registry to focus on four main aims.

FIG. 1.

Adolescent and young adult oncology team.

Collect outcome data from AYA cancer patients and survivors diagnosed and/or treated within our hospital system.

Characterize the key adverse events for the AYA population with respect to the nature of the primary malignancy and specific details of their therapeutic protocol.

Identify treatment-related and demographic risk factors through a direct comparison of the patients and controls identified from the general public.

Identify unmet clinical, psychosocial, cognitive, and vocational/educational needs of the AYA cancer population.

These aims will support the AYA team's overall mission by both providing data to understand the long-term impact of treatment on AYA cancer patients and understanding the relationship that age-specific programming has on patient outcomes as the program continues to grow and evolve during the data collection period.

Enrollment process

As patients enter the AYA program, they are invited to participate in this registry. Based on our hospital census, we expect ∼50 patients per year to be enrolled across pediatric and adult services. We plan to collect data for 10 years (with patients being followed for 5 years after the end of their last treatment). Thus, we hope to gather data on 500 patients. Inclusion criteria are that they must be 14–39 years old at diagnosis and have a malignant tumor diagnosed and/or treated at either our pediatric or adult cancer center. There are no exclusion criteria. While referrals to the AYA team are made upon initial diagnosis, any patient with AYA-specific needs who began treatment before inception of the AYA program may also be referred and thus consented. Any member of the team can consent patients, but the medical social worker has been tasked with being the primary consenter on our team (other backup consenters are noted in Fig. 1). Consent forms cover all aspects of medical treatment and corresponding quality improvement-related questionnaires.

Demographic information is recorded, including information that is not often included in other research (e.g., sexual/gender preferences and family and career planning history/desires), in the hopes of identifying important factors that may be related to overall outcomes and medical decision-making.

Data variables

Markers for treatment such as important dates (diagnosis, completion of therapy, and relapse, etc.), disease and treatment history (stage and enrollment in clinical trials, etc.), and others are tracked. Psychological services are also included as important variables and while sensitive information (such as content of sessions) is not tracked, general information (such as level of participation and psychological assessment results) is tracked. Patients are routinely followed through survivorship care in this hospital and thus these data can continue to be tracked over the long term as future medical complications or diagnoses arise. Data collection takes place through chart review, with trainees responsible for coding these data (Fig. 1).

During the consenting process, patients are given a demographic form to complete. See Table 1 with a full list of variables. Quality improvement measures have also been included to enable continual process improvement within the AYA program. This brief questionnaire is open ended not only to explore the needs of patients as a structured needs assessment but also to provide the team with feedback around specific AYA initiatives that have been developed. This questionnaire allows the team to both inform clinical care and determine whether participation in AYA initiatives is beneficial to AYA patient outcomes.

Table 1.

Study Variables

Demographic data	All patients
	Date of birth
	Gender
	Sexual preference
	Family history
	Relationship status
	Dependent children
	Living arrangements
	Race
	Ethnicity
	Occupational/educational status
	Estimated annual household income
	All patients
Medical history	Primary diagnosis
	Date of diagnosis
	Disease stage/severity
	Date that therapy was completed
	Therapy received
	Status of ability to participate in the clinical trial
	Status of clinical trial enrollment
	Relapse history (if applicable)
	Secondary diagnos(es)
	Disease stage/severity
	Date that therapy was completed
	Therapy received
	Status of ability to participate in the clinical trial
	Status of clinical trial enrollment
	Date of bone marrow transplantation (bone marrow transplant recipients)
	Complications of cancer therapy
	Markers of severity level of complications
	Fertility preservation history
	Type of preservation offered
	Type of preservation chosen
	Date of preservation
	At study entry and repeated as clinically indicated
Psychosocial history	Level of participation in psychosocial service
	Individual psychotherapy
	Group psychotherapy
	Peer mentorship program
	Assessment results (if clinically applicable)
	Diagnosis
	Neurocognitive testing
	Questionnaires
	Program feedback
	Psychosocial services
	AYA program services
	Medical services
	If indicated
Wellness	Dietary/nutritional assessment
	Physical therapy/exercise assessment
	Occupational therapy assessment^a
	Speech therapy assessment^a

These are not services offered within our hospital system, but are important variables to consider.

Data analysis

The goal of this registry is to determine what factors influence the medical and psychosocial outcomes of AYA oncology patients and determine points of intervention that may help to improve the well-being of patients with a primary cancer diagnosed and/or treated at our hospital system.

Descriptive statistics, mean and standard, and number and percentage will be used to summarize patient demographics and clinical characteristics depending on continuous or categorical assessments. Long-term health events (e.g., secondary diagnoses, complications of treatment, and psychosocial functioning) will be reported. The severity of these long-term adverse health events will also be assessed with number and percentage. A cross-sectional analysis will be performed to determine the relationship between these key adverse events and demographics and clinical characteristics, as well as the nature of the primary malignancy and treatments. Analysis will be conducted to determine the impact of AYA-specific programming on patient outcomes.

Conclusions

Within our hospital system, we have struggled to identify ways to work collaboratively with adult oncologists in a number of areas. While the number one goal of a registry such as this may be to identify AYA patients who may have yet to gain access to (and thus increase their likelihood of enrolling in) clinical trials for AYA patients, it can also serve to foster increased interest in working across disciplines on patient care and research projects. A readily available set of data for analysis may also be an appealing draw for adult providers who have little buy in⁴³ to collaborate across the transitional care gap. However, until adult providers increase their buy in to such a program, there may be a disproportionate number of patients enrolled into the pediatric oncology service. This may cause a bias within the data, more toward the outcomes and needs of pediatric populations, and thus may not take into account the long-term impact of cancer on the older AYA patients who have a different set of needs than their pediatric counterparts (e.g., financial independence, caregivers to their own children, marital concerns, and different biologics as a result of the conclusion of puberty). While it is well known that AYA patients in adult care do not have access to as many clinical trials or supportive care and do not participate in as much oncology research,⁴⁴ other systemic issues within the adult cancer care community may also be missed.

Other local benefits of such a registry are related to the development of a hospital-specific program for AYA patients with cancer by engaging patients through quality improvement projects. Unsurprisingly, allowing AYAs to have a direct impact on the care they receive not only improves relationships with their medical team (which AYAs list as the most important relationship they have during cancer treatment) but improves outcomes as well.⁴⁵

Systematically gathering data on AYAs, both in the short and long terms, will lead to an important contribution in this burgeoning field of medicine as it relates to oncology patients with respect to better understanding of how to tailor treatment to this age group. Increasing the amount of data to also determine additional medical and public health factors will only improve medical outcomes for AYA oncology patients.

Footnotes

Acknowledgments

The writers would like to acknowledge Jessica Sampson and Gabrielle Piela for their help in preparing the manuscript as well as aiding in other study tasks.

Author Disclosure Statement

No competing financial interests exist.

References

Noone

, Howlader

, Krapcho

, et al. SEER Cancer Statistics Review, 1975–2015, National Cancer Institute. Accessed August 15, 2018 from: https://seer.cancer.gov/csr/1975_2015/

National Cancer Institute. A snapshot of adolescent and young adult cancers. 2012. Accessed February 22, 2013 from: http://www.thesamfund.org/samfund/wp-content/uploads/2012/10/NCI-on-AYA-2012.pdf

Ferrari

, Thomas

, Franklin

, et al. Starting an adolescent and young adult program: some success stories and some obstacles to overcome. J Clin Oncol. 2010; 28(32):4850–7.

Gupta

, Edelstein

, Albert-Green

, D'Agostino

. Assessing information and service needs of young adults with cancer at a single institution; the importance of information on cancer diagnosis, fertility preservation, diet and exercise. Support Care Cancer. 2013; 21:2477–84.

Mahalik

, Levine Coley

, McPherran Lombardi

, et al. Changes in health risk behaviors for males and females from early adolescence through early adulthood. Health Psychol. 2013; 32(6):685–94.

Packman

, Mehta

, Rafie

, et al. Young adults with MSUD and their transition to adulthood: psychosocial issues. J Genet Couns. 2012; 21(5):692–703.

Wysocki

, Hough

, Ward

, et al. Diabetes mellitus in the transition to adulthood: adjustment, self-care, and health status. J Dev Behav Pediatr. 1992; 13(3):194–201.

Betz

. Transition of adolescents with special health care needs: review and analysis of the literature. Issues Comprehens Pediatr Nurs. 2004; 27(3):179–241.

Oeffinger

, Mertens

, Hudson

, et al. Health care of young adult survivors of childhood cancer: a report from the Childhood Cancer Survivor Study. Ann Fam Med. 2004; 2(1):61–70.

10.

, Chin

, Haase

, et al. Coping experiences of adolescents with cancer: a qualitative study. J Adv Nurs. 2009; 65(11):2358–66.

11.

Roth

, O'Mara

, Seibel

, et al. Low enrollment of adolescents and young adults onto cancer trials: insights from the community clinical oncology program. Am Soc Clin Oncol. 2016; 12(4):323–94.

12.

Freyer

, Seibel

. The clinical trials gap for adolescents and young adults with cancer: recent progress and conceptual framework for continued research. Curr Pediatr Rep. 2015; 3(2):137–45.

13.

NIH Adolescent Young Adult Oncology Progress Review Group. Closing the gap: research and care imperatives for adolescents and young adults with cancer. Report of the Adolescent and Young Adult Oncology Progress Review Group. 2006. Accessed August 15, 2018 from: https://www.cancer.gov/types/aya/research/ayao-august-2006.pdf

14.

National Cancer Institute. Adolescents and young adults with cancer. National Cancer Institution at the National Institutes of Health, 2015. Accessed August 15, 2018 from: https://cancer.gov/types/aya

15.

Mulder

, van der Pal

HJH

, Levitt

, et al. Transition guidelines: an important step in the future care for childhood cancer survivors. A comprehensive definition as groundwork. Eur J Cancer. 2016; 54:64–8.

16.

Cramm

, Strating

MMH

, Sonneveld

, Nieboer

. The longitudinal relationship between satisfaction with transitional care and social and emotional quality of life among chronically ill adolescents. Appl Res Qual Life. 2013; 8:481–91.

17.

Jin

Y-T

, Chen

C-M

, Chien

W-C

. Factors influencing transitional care from adolescents to young adults with cancer in Taiwan: a population-based study. BMC Pediatr. 2016; 16:122.

18.

Sonneveld

, Strating

MMH

, van Staa

, Nieboer

. Gaps in transitional care: what are the perceptions of adolescents, parents and providers?. Child Care Health Dev. 2013; 39(1):69–80.

19.

List

, Ritter-Sterr

, Lansky

. Cancer during adolescence. Pediatrician. 1991; 18(1):32–6.

20.

Apter

, Farbstein

, Yaniv

. Psychiatric aspects of pediatric cancer. Child Adolesc Psychiatr Clin N Am. 2003; 12(3):473.

21.

Challinor

, Miaskowski

, Franck

, et al. Somatization, anxiety and depression as measures of health-related quality of life of children/adolescents with cancer. Int J Cancer. 1999; 12:52–7.

22.

Koocher

, O'Malley

, Gogan

, et al. Psychological adjustment among pediatric cancer survivors. Child Psychol Psychiatry. 1980; 21(2):163–73.

23.

Seitz

, Besier

, Debatin

, et al. Posttraumatic stress, depression and anxiety among adult long-term survivors of cancer in adolescence. Eur J Cancer. 2010; 46(9):1596–606.

24.

Drew

. Self-reconstruction and biographical revisioning: survival following cancer in childhood or adolescence. Health. 2003; 7(2):181.

25.

Clerici

, Massimino

, Casanova

, et al. Psychological referral and consultation for adolescents and young adults with cancer treated at pediatric oncology unit. Pediatr Blood Cancer. 2008; 51(1):105–9.

26.

Kent

, Arora

, Rowland

, et al. Health information needs and health-related quality of life in a diverse population of long-term cancer survivors. Patient Educ Couns. 2012; 89(2):345–52.

27.

Jones

, Parker-Raley

, Barczyk

. Adolescent cancer survivors: identity paradox and the need to belong. Qual Health Res. 2011; 21(8):1033–40.

28.

Kelly

. Developing age appropriate psychosexual support for adolescent cancer survivors: a discussion paper. J Sex Med. 2013; 10:133–8.

29.

Elwell

, Grogan

, Coulson

. Adolescents living with cancer: role of computer mediated support groups. J Health Psychol. 2011; 16(2):236–48.

30.

Hilton

, Emslie

, Hunt

, et al. Disclosing a cancer diagnosis to friends and family: a gendered analysis of young men's and women's experiences. Qual Health Res. 2009; 19(6):744–54.

31.

Suzuki

, Beale

. Personal web home pages of adolescents with cancer: self-presentation, information dissemination, and interpersonal connection. J Pediatr Oncol Nurs. 2006; 23(3):152–61.

32.

Klosky

, Foster

, Li

, et al. Risky sexual behavior in adolescent survivors of childhood cancer: a report from the childhood cancer study. Health Psychol. 2014; 33(8):868–77.

33.

Bomba

, Herrmann-Garitz

, Schmidt

, et al. An assessment of the experiences and needs of adolescents with chronic conditions in transitional care: a qualitative study to develop a patient education programme. Health Soc Care Commun. 2017; 25(2):652–66.

34.

Kazak

, Christakis

, Alderfer

, et al. Young adolescent cancer survivors and their parents: adjustment, learning problems, and gender. J Fam Psychol. 1994; 8(1):74–84.

35.

Fisher

, O'Connor

. “Motherhood” in the context of living with breast cancer. Cancer Nurs. 2012; 35(2):157–63.

36.

Gutiérrez-Colina

, Lee

, VanDellen

, et al. Family functioning and depressive symptoms in adolescent and young adult cancer survivors and their families: a dyadic analytic approach. J Pediatr Psychol. 2017; 42(1):19–27.

37.

Katz

Meeting the needs for psychosocial care in young adults with cancer. Pittsburg, PA: Oncology Nursing Society; 2015.

38.

Van Schoors

, Caes

, Knoble

, et al. Systematic review: associations between family functioning and child adjustment after pediatric cancer diagnosis: a meta-analysis. J Pediatr Psychol. 2017; 42(1):6–18.

39.

Mehnert

. Employment and work-related issues in cancer survivors. Crit Rev Oncol Hematol. 2011; 77(2):109–30.

40.

Duijts

, van Egmond

, Spelten

, et al. Physical and psychosocial problems in cancer survivors beyond return to work: a systematic review. Psychooncology. 2014; 23(5):481–92.

41.

Parsons

, Harlan

, Lynch

, et al. Impact of cancer on work and education among adolescent and young adult cancer survivors. J Clin Oncol. 2012; 30(19):2393–400.

42.

Braniecki

, Pereira

, Lawton

, et al. Comparing neurocognitive needs and referrals for school-age and young adult survivors of childhood cancer. Annual Meeting of the Society for Developmental and Behavioral Pediatrics. Cleveland, OH, October 13–16, 2017.

43.

Oris

, Luyckx

, Rassart

, et al. Illness identity in adults with a chronic illness. J Clin Psychol Med Settings. 2018; 25(4):429–40.

44.

Sanford

, Beaumont

, Snyder

, et al. Clinical research participation among adolescent and young adults at an NCI-designated Comprehensive Cancer Center and affiliated pediatric hospital. Support Care Cancer. 2017; 25(5):1579–86.

45.

Zebrack

, Chesler

, Kaplan

. To foster healing among adolescents and young adults with cancer: what helps? What hurts?. Support Care Cancer. 2010; 18(1):131–5.