Purpose[edit source]

This guideline was developed to address the need for a systematic, evidence-based approach to the management of communication and swallowing in children diagnosed with childhood brain tumour or leukaemia (CBTL). It aims to assist health professionals to provide and advocate for evidence-based care and management of communication and swallowing in children diagnosed with CBTL. It also aims to educate patient consumers (parents of children with CBTL, survivors of CBTL) and support them to advocate for best practice management of communication and swallowing difficulties.

The intended outcome of this guideline is to improve quality of life for children surviving brain cancer and leukaemia. This has involved translating evidence from the research and clinical/consumer expertise into recommendations that will guide improvements in cancer services and quality of clinical care for this population across Australia and worldwide.

The implementation of the evidence-based recommendations presented here will support a systematic and equitable approach to clinical management for communication and swallowing in CBTL, including long-term follow-up. This guideline will also form the basis for targeted early intervention program development and survivorship surveillance planning. These recommendations will support children to keep healthy and lead a fulfilled life, not only during cancer diagnosis and treatment, but critically after cancer survival.

Clinical problem[edit source]

Child survivors of brain cancer and leukaemia - the top two most common childhood cancers in Australia and developed countries worldwide - often face a new challenge during and after their cancer treatment is completed.[1][2][3][4][5][6] While incidence rates continue to rise, so do survival rates and the size of this rapidly growing population of survivors, due to advancements in medical care and treatments.[3][6][7][8] Effects from the cancer and treatments can have significant and often severe impact to quality of life in the areas of communication and swallowing; affecting a child or adolescent’s development of new skills, the ability to communicate their needs, succeed at school, make friends, engage in family mealtimes or eat-out socially, use social media, successfully date, or achieve social and financial independence in adulthood.[9]
To date, an equitable and systematic approach to management for communication and swallowing has not been established in Australia or worldwide; despite children diagnosed with CBTL remaining at-risk throughout development and into adulthood if untreated or lost to follow-up.

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Clinical questions[edit source]

To guide the evidence review for this guideline, two clinical questions were developed. The questions were developed by the Chair and Project Co-ordinator with opportunities for feedback from the Steering Committee. The questions are consistent with the PICOTS (population, intervention, comparison, outcome, timing, setting) format.[10] The clinical questions are shown in Box A below.
Box A Clinical questions used to develop this guideline

Communication Outcomes What are the communication outcomes associated with childhood brain tumour or leukaemia?*
Swallowing Outcomes What are the swallowing outcomes associated with childhood brain tumour or leukaemia?*
*PICOTS format – Population (P): Children with aged 1–16 with brain tumour or leukaemia; Intervention (I) – Any; Comparison (C) – Any; Outcome (O) – Communication/Swallowing; Timing (T) – At diagnosis prior to cancer treatment, during the oncology treatment phase, during the oncology follow-up phase, during the survivorship phase; Setting (S) - Both inpatient and outpatient settings.

The population[edit source]

The guideline focuses on children diagnosed with any type of childhood brain tumour or leukaemia aged 0–16 years of age.

Brain tumour or leukaemia[edit source]

A brain tumour in childhood is an abnormal mass of cells growing in the brain. Tumours can start to grow before birth in the foetal period, or anytime during childhood. They can grow slowly over time, causing a slow onset of symptoms, or quickly with a faster onset of more obvious symptoms. Brain tumours can be treated with a combination of surgery, radiotherapy and/or chemotherapy, depending on the type and malignancy. There are no clear known causes. Leukaemia is a cancer of the white blood cells, where more leukocytes (or immature white blood cells) are produced and suppress normal blood cells. If leukaemia is diagnosed as acute, the disease progresses more quickly and requires more aggressive treatment. Treatments can include monitoring, chemotherapy, radiotherapy, and/or stem-cell transplant.

The population of CBTL is inherently diverse, due to a range of presentation characteristics. Examples of diversity include differences in cancer diagnoses, cancer treatments, cancer treatment effects (during and after), age at diagnosis, as well as progression of disease, periods of admission, medical complications (e.g. increased intracranial pressure, infections), family circumstances and values.

In this guideline, brain tumour and leukaemia have been considered as one population (i.e. childhood brain tumour or leukaemia; CBTL) due to the similarities in central nervous system (CNS) targeted cancer treatments and outcomes for these groups. Both cancer groups often receive CNS applied chemotherapy and/or radiotherapy that are reported to impact the developing brain and CNS.[4] However, a majority of the literature evidence on which the guideline recommendations are based was noted to be more largely represented by reports of children with brain tumour, with a relatively recent increase in the amount of leukaemia studies (see Administrative & Technical report, Table 2 & 3).

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Age[edit source]

The scope of the guideline focuses on children aged 0–16 years. The 16-year age limit represents the upper age limit commonly applied to patients receiving paediatric services in the majority of Australian hospitals/cancer centres. The current available evidence base does not support further sub-grouping of recommendations into separate age groups. However, the key practice points presented in this guideline about assessment consider age of the child to ensure that age-appropriate assessment procedures are applied. It is, however, anticipated that survivorship experiences will extend beyond this age group into upper adolescence and early adulthood. The guideline discusses the implications for future application and services throughout survivorship and into adulthood for child survivors.

Timing and setting in childhood brain tumour or leukaemia: A framework[edit source]

Even after cancer diagnosis and treatment, children with CBTL face a long road ahead, with regular monitoring of their medical and cancer status. This is necessary due to risk of cancer recurrence for some cancer types.[11][12] It is also important because of the potential ongoing impact of the cancer and its treatment on health and development.[13][14][15][16][17][18][19]

Therefore, the course of CBTL needs to be conceptualised over time, as well as adopting a long-term approach to health and well-being. Literature focusing on communication and swallowing skills in this population have examined outcomes at one or more points in time across a child’s cancer journey, from diagnosis through to survivorship. To provide consistency in describing these time points, the authors of this guideline have developed a framework (see Figure 2). This framework identifies four key paediatric oncology phases:

  1. At diagnosis/pre-treatment: at cancer diagnosis, prior to the start of cancer treatment
  2. Oncology treatment phase: during or, in the weeks after, cancer treatment
  3. Oncology follow-up phase: <5 years since cancer treatment has finished
  4. Survivorship phase: ≥5 years since cancer treatment has finished

These oncology phases are closely linked with setting. While some studies examine outcomes while children diagnosed with CBTL are still in hospital, others do so in community settings such as clinics, schools and at home. For simplicity, in the framework, setting has been binary classified into: “hospital - inpatient” and “after hospital - outpatient”.

It is important to recognise that due to the possibility of cancer recurrence, secondary cancer/s, or multiple primary cancer/s, a child may return to an earlier phase in this framework. For example, a child who is cancer-free for six years and considered to be in the survivorship phase may experience cancer recurrence and therefore return to oncology treatment phase.

Figure 2 Framework of timing and setting in childhood brain tumour/ leukaemia

CBTL Figure 2.png

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Intended end users of the guideline[edit source]

This guideline has been developed to provide evidence- based recommendations for Speech Pathologists and multidisciplinary health professionals involved in the management of communication and swallowing difficulties for children diagnosed with childhood brain tumour or leukaemia. It is to be used alongside clinician judgement and patient preferences. It is based on the best evidence available at the time of publication. Additional relevant health professionals may include, but are not limited to, Oncologists, Rehabilitation Physicians, Nurses, Occupational Therapists, Physiotherapists, Dietitians, Child Life and Music Therapists. It is also intended that education providers in educational settings will also access the guideline and recommendations in order to support CBTL survivors to transition back into the classroom and school community for educational services.

These recommendations will equip parents and families as lifelong advocates in seeking optimal quality of life outcomes for their children, by providing knowledge about issues their child may likely experience and what these might look like. This will ensure families can be connected with timely management, early intervention services and appropriate referral services. It is important that parents do not feel isolated or lost to follow-up, or do not feel inadequately prepared for their child’s future.[20] This cohesive source of information about long- term communication and swallowing management will serve to guide survivors and their families to partner with health professionals where necessary to ensure improved quality of life outcomes for communication and swallowing.

Cultural and socio-economic considerations[edit source]

Aboriginal and Torres Strait Islander people and people from culturally and linguistically diverse (CALD) communities as well as families from socio-economically disadvantaged backgrounds face unique challenges in regard to accessing cancer care services. A rapid review commissioned by the Cancer Institute NSW[17] highlighted the importance of practical, educational and social support in improving cancer outcomes and achieving increased satisfaction for CALD populations. This review specified that the development of culturally appropriate interventions and programs should consider individual and cultural barriers to accessing health services.[17] A lower prevalence has recently been reported for admissions of children with chronic conditions from socio-economically disadvantaged or regional and remote areas, including cancer diagnoses.[9] This has been attributed to reduced access to hospital services due to location, as well as cultural barriers for some population groups.[9] However, presentation of children from these areas were more highly represented in emergency admission rates, suggesting that visits were less likely to be planned.[9]

Australians now come from nearly 200 countries and represent more than 300 ancestries.[21][22][23] Based on the most recent available Australian census data in 2016, 3.3% of the total Australian population is represented by Aboriginal and Torres Strait Islander people (798,400).[24] One in four people in Australia (26%) are born overseas with over 300 separately identified languages spoken at home.[21][22][23] While English is the main language spoken, the most recent population data reports that 21% of Australians speak a language other than English at home.[21] For example, the most commonly spoken language in Sydney is Arabic (4.8% of the total population), closely followed by Mandarin (3.6%), Cantonese (3.5%), Vietnamese (2.3%), Greek (2.1%), and Italian (2.1%). Of the overseas-born people who had arrived in the 25 years prior to 2016, 11% either did not speak English well or at all.[21]

In these guidelines, the cultural diversity of Australians was considered in several ways. In the systematic review of evidence that informed development of the recommendations, the search strategy for the population concept was purposefully broad (i.e., brain cancer or leukaemia AND child) so as to capture studies across all potential population/cultural subgroups (see Administrative & Technical report, p. 15-16). Many studies in the systematic review, however, were found to focus on English-only language speakers and noted to be an eligibility criterion of most reported studies.

Issues relevant to Aboriginal and Torres Strait Islander people and CALD populations were also considered through the evidence from the Health Professional and Consumer Group and Steering Committee members. Several members identified as CALD and/or currently work directly with clinical populations. They incorporated their experience and knowledge of Aboriginal and Torres Strait Islander people and CALD families when providing their input. Important considerations for implementation of the guideline for Aboriginal and Torres Strait Islander people and CALD populations can be found in the Dissemination & Implementation Plan.

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References[edit source]

  1. Australian Institute of Health and Welfare. Cancer in Australia 2019. Canberra, ACT: AIHW; 2019. Report No.: Cancer series no. 119. Cat. no. CAN 123. Available from: https://www.aihw.gov.au/reports/cancer/cancer-in-australia-2019/summary.
  2. Australian Institute of Health and Welfare. Australia’s children. Canberra, ACT: AIHW; 2020. Report No.: Cat. no. CWS 69.. Available from: https://www.aihw.gov.au/reports/children-youth/australias-children/contents/executive-summary.
  3. 3.0 3.1 Australian Institute of Health and Welfare. Cancer Data in Australia. Canberra, ACT: AIHW; 2020. Report No.: Cat No.: CAN 122.. Available from: https://www.aihw.gov.au/reports/cancer/cancer-data-in-australia/contents/summary.
  4. 4.0 4.1 Chang E, Goldsby R, Mueller S, Banerjee A. Late effects of treatment and palliative care. In: Gupta N, Banerjee A, Haas-Kogan D, eds. Pediatric CNS Tumors Berlin, Heidelberg: Springer; 2017. p. 365-387.
  5. Ostrom QT, Gittleman H, Xu J, Kromer C, Wolinsky Y, Kruchko C, et al. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2009-2013. Neuro Oncol 2016 Oct 1;18(suppl_5):v1-v75 Available from: http://www.ncbi.nlm.nih.gov/pubmed/28475809.
  6. 6.0 6.1 Youlden DR, Baade PD, Green AC, Valery PC, Moore AS, Aitken JF. The incidence of childhood cancer in Australia, 1983-2015, and projections to 2035. Med J Aust 2020 Feb;212(3):113-120 Available from: http://www.ncbi.nlm.nih.gov/pubmed/31876953.
  7. Smoll NR. Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs). Cancer 2012 Mar 1;118(5):1313-22 Available from: http://www.ncbi.nlm.nih.gov/pubmed/21837678.
  8. Youlden DR, Aitken JF. Childhood cancer in Australia, 1983-2015.; 2019 [cited 2020 Jun 6] Available from: https://cancerqld.blob.core.windows.net/content/ docs/Childhood-Cancer-in-Australia-1983-2015.pdf.
  9. 9.0 9.1 9.2 9.3 Bell J, Lingam R, Wakefield CE, Fardell JE, Zeltzer J, Hu N, et al. Prevalence, hospital admissions and costs of child chronic conditions: A population-based study. J Paediatr Child Health 2020 Sep;56(9):1365-1370 Available from: http://www.ncbi.nlm.nih.gov/pubmed/32502332.
  10. Riley RD, Moons KGM, Snell KIE, Ensor J, Hooft L, Altman DG, et al. A guide to systematic review and meta-analysis of prognostic factor studies. BMJ 2019 Jan 30;364:k4597 Available from: http://www.ncbi.nlm.nih.gov/pubmed/30700442.
  11. Amid A, Keene DL, Johnston DL. Presentation of central nervous system tumors. In: Scheinemann K, Bouffet E, eds. Pediatric Neuro-oncology New York, NY: Springer; 2015. p. 3-8.
  12. Lanzkowsky P. Central nervous system malignancies. In: Lanzkowsky P, ed. Manual of Pediatric Hematology and Oncology London, UK: Academic Press; 2011. p. 647-670.
  13. Vetsch J, Wakefield CE, Robertson EG, Trahair TN, Mateos MK, Grootenhuis M, et al. Health-related quality of life of survivors of childhood acute lymphoblastic leukemia: a systematic review. Qual Life Res 2018 Jun;27(6):1431-1443 Available from: http://www.ncbi.nlm.nih.gov/pubmed/29372438.
  14. Janzen L, Mabbott D, Guger SL. Neuropsychological outcomes in pediatric brain tumor survivors. In: Scheinemann K, Bouffet E, eds. Pediatric Neuro-Oncology. New York, NY: Springer; 2015. p. 267-276.
  15. Pogorzala M, Styczynski J, Kurylak A, Debski R, Wojtkiewicz M, Wysocki M. Health-related quality of life among paediatric survivors of primary brain tumours and acute leukaemia. Qual Life Res 2010 Mar;19(2):191-8 Available from: http://www.ncbi.nlm.nih.gov/pubmed/20077142.
  16. Vinchon M, Baroncini M, Leblond P, Delestret I. Morbidity and tumor-related mortality among adult survivors of pediatric brain tumors: a review. Childs Nerv Syst 2011 May;27(5):697-704 Available from: http://www.ncbi.nlm.nih.gov/pubmed/21409425.
  17. 17.0 17.1 17.2 Phillipson L, Larsen-Truong K, Jones S, Pitts L. Improving Cancer Outcomes Among Culturally and Linguistically Diverse Communities: A Rapid Review of the Literature. Australia: The Sax Institute; 2012. Sponsored by Cancer Institute NSW. Available from: http://www.saxinstitute.org.au.
  18. von der Weid N, Mosimann I, Hirt A, Wacker P, Nenadov Beck M, Imbach P, et al. Intellectual outcome in children and adolescents with acute lymphoblastic leukaemia treated with chemotherapy alone: age- and sex-related differences. Eur J Cancer 2003 Feb;39(3):359-65 Available from: http://www.ncbi.nlm.nih.gov/pubmed/12565989.
  19. Walsh KS, Paltin I. Neuropsychological effects of pediatric brain tumors and associated treatment. In: Mucci G, Torno L, eds. Handbook of Long Term Care of the Childhood Cancer Survivor. New York, NY: Springer; 2015. p. 249-262.
  20. Feraco AM, Brand SR, Mack JW, Kesselheim JC, Block SD, Wolfe J. Communication Skills Training in Pediatric Oncology: Moving Beyond Role Modeling. Pediatr Blood Cancer 2016 Jun;63(6):966-72 Available from: http://www.ncbi.nlm.nih.gov/pubmed/26822066.
  21. 21.0 21.1 21.2 21.3 Australian Bureau of Statistics. Australian Bureau of Statistics. Census of Population and Housing: Australia Revealed, 2016. Canberra, ACT: ABS; 2017 Sep 1 [cited 2020 Jun 7]. Report No.: cat. no. 2024.0. Available from: https://www.abs.gov.au/ausstats/abs@.nsf/mf/2024.0.
  22. 22.0 22.1 Australian Bureau of Statistics. Census of Population and Housing: Reflecting Australia - Stories from the Census, 2016. Canberra, ACT: ABS; 2018 Jul 12 [cited 2020 Jun 7]. Report No.: cat. no. 2071.0. Available from: https://www.abs.gov.au/ausstats/abs@.nsf/mf/2071.0.
  23. 23.0 23.1 Australian Government – Cancer Australia. Cancer and culturally and linguistically diverse communities. Canberra, ACT; 2010 [cited 2020 Jun 7].
  24. Australian Bureau of Statistics. Estimates and Projections, Aboriginal and Torres Strait Islander Australians, 2006 to 2031. Canberra, ACT: ABS; 2019 Jun 11 [cited 2020 Jun 7]. Report No.: cat. no. 3238.0. Available from: https://www.abs.gov.au/ausstats/abs@.nsf/mf/3238.0.

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