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Diagnosing cancer early in teenagers and young adults

Lorna a Fern PhD MSc
Research & Development Co-ordinator
National Cancer Research Institute's Teenage and Young Adults Clinical Studies Group
University College London Hospitals NHS Foundation Trust

Susie Pearce MSc BSc   
Health Services Researcher for Young People with Cancer University College London Hospitals NHS Foundation Trust

Teenagers and young adults with cancer are experiencing poorer outcomes compared to children and some older adults, presenting challenges for early diagnosis in primary care.

A cancer diagnosis during an intense period of physical and psychological change presents a number of unique challenges that neither paediatric nor older adult cancer services are suitably equipped to deal with.

The unique psychosocial needs of teenagers and young adults (TYA) have gained considerable attention in healthcare policy in recent years and, in August 2005, the National Institute for Health and Clinical Excellence (NICE) issued 'Improving Outcomes Guidance for Children and Young People with Cancer'. The document serves a set of key recommen- dations, in particular, unhindered access to age-appropriate care. Early referral to specialist cancer services is advocated and much of the potential for this lies within primary care.

Cancer in young people
Cancer in TYA (13-24 years) is rare, with approximately 2,000 cases diagnosed annually in the UK. Despite this, cancer is the second leading cause of death for TYA, accounting for 11% of all deaths, preceded only by accidents.¹ Over the past 20 years dramatic improvements in survival for children with cancer are considered to be one of the most successful stories of modern cancer treatment. However, similar advances in survival for TYA have not been observed. Differences in cancer biology, poor recruitment to clinical trials and a prolonged period to cancer diagnosis or 'diagnostic delay' are all implicated.²

Delays in the diagnosis of cancer can be described as the time from the emergence of the first cancer-related symptom to the point of diagnosis and can be divided into patient delay, professional delay and system delays across primary, secondary and tertiary healthcare.

Cancer types
Young people present with a spectrum of cancer types. The most common are lymphoma (particularly Hodgkin's disease), leukaemia,bone and soft tissue sarcomas, germ cell tumours (ovarian and testis), brain and central nervous system, which account for approximately 70% of newly diagnosed cancers in those aged 13-24 years.³ The remaining 30% are melanomas and those described as 'early onset' adult carcinomas. However, carcinomas arising in young people differ from those observed in older adults where cancers of the breast, lung, colon and prostate account for around half of cases. Early onset carcinomas in TYA tend to be of the breast, colon, thyroid, cervix and head and neck.³

Why is early diagnosis important?
Improving the time to diagnosis is a national priority in the UK. The National Awareness and Early Diagnosis Initiative (NAEDI) was launched in 2008, a national campaign supporting activities aimed at raising cancer awareness and promoting early diagnosis. This was a consequence of the EUROCARE-4 report, which demonstrated poorer survival rates in the UK compared to our European counterparts.⁴ Poor survival, particularly one-year survival, can be partly explained by more advanced disease at presentation and this may be due to a longer diagnostic experience for UK patients.

Why prioritise early diagnosis in young people?
Young people report repeated visits to their GP before referral to a specialist. The recent National Cancer Experience Survey demonstrated that 70% of adults who visited their GP with cancer symptoms attended no more than twice before referral onto cancer services. A survey carried out annually by Teenage Cancer Trust (TCT) at their patient conference has shown, year on year, that only 30% of TYA are referred after their first or second visit, with a further 30% attending four times or more.

Young people themselves recognise the importance of early diagnosis, with 60% prioritising reducing delays in diagnosis as the most important area of research being carried out by the National Cancer Research Institute's Teenage and Young Adult Clinical Studies Group.⁵ Little research exists on the experiences of TYA during the diagnostic period. A recent study by Gibson has begun to describe the complexity of diagnosis in young people.⁶ Interviews with TYA as part of the Youth Health Talk project (see Resources) details the stressful, frustrating and emotional impact of delay in diagnosis, indicating that symptoms need to be taken seriously more quickly. The role young people have in delaying presentation as they focus on daily life, the crucial role of parents and friends in facilitating professional input is also emphasised in this, Gibson's work and other studies.⁶

Symptoms
Referral from primary care to secondary care is often triggered by 'alarm' symptoms that may be suggestive of cancer. In TYA potential cancer symptoms include unexplained lumps or swelling, unexplained pain, fatigue, weight loss, headaches, dizziness and changes in pigmented lesions. However, some symptoms may be vague and these patients present further challenges. Many TYA report having three or more of these symptoms at presentation. You can see TYA describing their diagnostic journey in the 'Sometimes its Cancer' DVD (see Resources).

One of the complexities of diagnosing cancer in primary care is that many of the symptoms, such as unexplained pain, are attributed to sporting injuries or 'growing pains', with headaches, fatigue and weight-loss assigned to exam stress.⁷

Symptoms unrelated to cancer
It is clear that young people do use primary care services, and a recent study looked at how often and why TYA consulted their GP in one year.⁸ Although nurse consultations were not included, it does go some way to defining primary care use by young people. Approximately 70% consulted their GP at least once; however, the majority of consultations were for contraception or pregnancy, infection or review of existing conditions. Inclusion of nurse consultations in this study would have increased the proportion of young people accessing primary care services, particularly those seeking contraceptive advice.

A key finding and important take-home message of the study was that 'alert' symptoms were rare, accounting for only 4% of over 6,000 consultations. The significance of this increases when taking into account that in the normal population 'alert' symptoms were reported in isolation. In stark contrast, TYA with cancer report having three or more 'alert' symptoms at presentation.

Awareness of cancer symptoms
Interventions to raise cancer awareness in adults are now in place. Those aimed specifically at TYA are somewhat fewer in number, but are beginning to emerge. Preliminary data from the north-west of England demonstrate a low level of cancer awareness among young people. Sam Smith, TCT Nurse Consultant and Lorraine Wright, TCT Youth Support Co-ordinator from Christie's Hospital in Manchester have developed a bespoke intervention designed by TYA who have had cancer, for young people. The DVD 'Sometimes Its Cancer' or 'SIC' features TYA detailing their diagnostic period (see Resources). Local dissemination and evaluation is in place, with national roll-out plans should the intervention demonstrate a sustained increased awareness of cancer symptoms in young people.

Raising cancer awareness in young people is also a priority for TCT, who have a dedicated nationwide Education and Advocacyteam that enters schools and colleges delivering talks about cancer symptoms and cancer in TYA (see Resources).

Opportunities for earlier cancer diagnosis in primary care
Avoiding teenage pregnancy, dealing with drug use and provision of smoking cessation advice are frequently the focal point of primary care services for young. However, primary care presents an ideal opportunity for raising cancer awareness and much of the potential for reducing diagnostic delay.

Understanding how and why young people use primary care services is an important step in developing educational tools and interventions to assist primary care services to refer TYA with suspected cancer appropriately and promptly. A recent NAEDI report highlights the complexity of diagnosing cancer in TYA and suggests vigilance of non-resolving alleged sports injuries, follow-up for ongoing musculoskeletal pain, and referral for neck and axilla lumps where there is no obvious infective cause, in which case the patients should be reviewed.⁹ In consultations where symptoms are unusual, referral on to appropriate specialists is advocated if the diagnosis is not clear. An appropriate youth-friendly consulting style is recommended and previous research has demonstrated a need for more youth friendly primary care services.

Primary care services have an important role as advocates for TYA where there are delays in other areas of the diagnostic journey. This includes promoting the timely communication of results, organisation of investigations and specialist care appointments; and the facilitation of good communication within and beyond primary healthcare. The opportunity to build trust and confidence in healthcare professionals in primary care services should not be underestimated and may influence treatment compliance in those later diagnosed.

Challenges
The challenges of diagnosing cancer in TYA in primary care are vast. These cancers are rare, and many GPs and practice nurses may only see one or perhaps two TYA with cancer in their working lifetime. Further, it is unlikely that a diagnosis or referral for a diagnosis would be made on the first visit and it is more likely the diagnosis may emerge after more than one visit, in which signs and symptoms are observed over a period of time, and investigations performed.⁸,¹⁰

Young people may misinterpret 'wait-and-see' advice and not return to primary care even if symptoms persist; further, the young person may feel he or she was not listened to properly or taken seriously and not engage with primary care again. Therefore, it is essential to capitalise on diagnostic opportunities when young people present. The recent NAEDI report suggested 'safety netting', or calling patients to enquire whether symptoms have resolved, rather than leaving the onus on the young person to return.

Improvements in outcomes for cancer in TYA have lagged behind those observed in children and older adults; delayed diagnosis is implicated. The rarity of cancer in TYA and the complexity of symptoms highlight the need for further research into the effectiveness of intervention tools to assist professionals in identifying and referring young people with suspected cancer promptly.

Acknowledgements
Lorna Fern is funded by the Teenage Cancer Trust; and Susie Pearce is funded by the National Institute for Health Research University College London Hospitals and University College London's Centre for Biomedical Research. Thank you to the Find Your Sense of Tumour Steering Committee and participants.

References
1.    Birch JM, Pang D, Alston RD et al. Survival from cancer in teenagers and young adults in England, 1979-2003. Br J Cancer 2008;99(5):830-5.
2.    Fern L, Davies S, Eden T et al. Rates of inclusion of teenagers and young adults in England into National Cancer Research Network clinical trials: report from the National Cancer Research Institute (NCRI) Teenage and Young Adult Clinical Studies Development Group. Br J Cancer 2008;99(12):1967-74.
3.    Birch JM, Alston RD, Kelsey AM, Quinn MJ, Babb P, McNally RJ. Classification and incidence of cancers in adolescents and young adults in England 1979-1997. Br J Cancer 2002;87(11):1267-74.
4.    Berrino F, De Angelis R, Sant M et al. Survival for eight major cancers and all cancers combined for European adults diagnosed in 1995-99: results of the EUROCARE-4 study. Lancet Oncol 2007;8(9):773-83.
5.    Fern L, Ashton J, Brooman K et al. Involving young people with cancer in research The National Cancer Research Institutes Teenage and Young Adult (TYA) Core Consumer Group (CCG) - a new and evolving model. Birmingham: National Cancer Research Institute; 2009.
6.    Pearce S, Gibson F, Eden TE, Glaser A, Hooker L, Whelan J, Kelly D. A narrative study of the experiences of young people from first symptoms to the diagnosis of cancer. European Journal of Cancer 2009;7(2):234.
7.    Smith S, Davies S, Wright D, Chapman C, Whiteson M. The experiences of teenagers and young adults with cancer - results of 2004 conference survey. Eur J Oncol Nurs 2007;11(4):362-8.
8.    Fern LA, Campbell C, Eden TO et al. How frequently do young people with potential cancer symptoms present in primary care? Br J Gen Pract 2011;61(586):223-30.
9.    Mitchell EMU, Rubin G. Report for National Awarenes and Early Diagnosis Initiative: Cancer in Primary Care. Durham: University of Durham; 2009:8-9.
10.    Hamilton W. Cancer diagnosis in primary care. Br J Gen Pract 2010;60(571):121-8.

Resources
Youth health talk project www.youthhealthtalk.org
National awareness and early diagnosis Initiative http://info.cancerresearchuk.org/spotcancerearly/naedi
National Cancer Research Institute's teenage and Young adult Clinical studies group http://ncrndev.org.uk/index.php?option=com_content&task=view&id=132&It emid=270
Teenage Cancer Trust www.teenagecancertrust.org/what-we-do/education
Find Your Sense of Tumour Patient Conference www.teenagecancertrust.org/get-clued-up/talk-to-other-young-people find-your-sense-of-tumour/
Sometimes it's Cancer
http://jimmyteens.tv/2011/08/sometimes-its-cancer-2/