JCF-01821; No of Pages 9

Journal of Cystic Fibrosis xxx (2019) xxx

Contents lists available at ScienceDirect

Journal of Cystic Fibrosis

journal homepage: www.elsevier.com/locate/jcf

Review

Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand
Natalie van der Haak a,⁎, Susannah J. King b,c, Tory Crowder d, Andrea Kench e,f, Catherine Painter g,
Nicole Saxby h,
the Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand Authorship Group and Interdisciplin-
ary Steering Committee 1
a
Nutrition Department, Women's and Children's Hospital, Adelaide, Australia
b
Nutrition Department, Alfred Health, Melbourne, Australia.
c
Department of Nutrition, Rehabilitation and Sport, LaTrobe University, Bundoora, Australia
d
Canterbury Cystic Fibrosis Service, Canterbury District Health Board, Christchurch, New Zealand
e
Department of Nutrition and Dietetics, The Children's Hospital Westmead, Sydney, Australia
f
Department of Respiratory Medicine, The Children's Hospital Westmead, Sydney, Australia
g
Nutrition and Dietetics, Central Adelaide Local Health Network, Royal Adelaide Hospital, Adelaide, Australia
h
Tasmanian Cystic Fibrosis Service, Paediatrics, Royal Hobart Hospital, Hobart, Australia

a r t i c l e i n f o a b s t r a c t

Article history: Optimal nutrition care is important in the management of cystic fibrosis (CF). This paper summarises the ‘2017
Received 5 October 2018 Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand (NZ)’. CF dietitians formulated 68 practice
Revised 5 March 2019 questions which were used to guide a systematic literature search and review of the evidence for nutrition in CF.
Accepted 11 May 2019
Identified papers underwent quality and evidence assessment using the American Dietetic Association quality
Available online xxxx
criteria checklist and the National Health and Medical Research Council of Australia (NHMRC) rankings. Evidence
Keywords:
statements, graded recommendations and practice points were developed covering core nutrition topics (assess-
Cystic fibrosis ment and nutrition interventions including oral, enteral and micronutrient supplementation); nutrition-related
Nutrition co-morbidities (including pancreatic insufficiency, CF-related diabetes, bone health and distal intestinal obstruc-
Nutrition therapy tion syndrome); and key new topic areas (genetic modulator therapies, overweight/obesity and complementary
Malnutrition therapies). This paper showcases highlights from the guidelines, focussing on new topic areas and geographic
Guidelines and climate considerations for vitamin D, salt and hydration.
Crown Copyright © 2019 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. All rights reserved.

Contents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2. Methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2.1. Guideline development groups . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2.2. Development of practice questions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2.3. Data sources and search strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2.4. Study selection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2.5. Literature appraisal. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2.6. Development of evidence statements, recommendations and practice points. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3. Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.1. Dietitian prescribing in New Zealand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.2. Nutrition assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.3. Nutrition interventions for undernutrition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.4. Overweight and obesity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.5. Routine dietary management: energy and macronutrients, including essential fatty acids . . . . . . . . . . . . . . . . . . . . . . . . . 0

⁎ Corresponding author at: Department of Nutrition, Women's and Children's Hospital, 72 King William Road, North Adelaide, SA 5006, Australia.
E-mail address: [email protected] (N. van der Haak).
1
Members listed in acknowledgements section.

https://doi.org/10.1016/j.jcf.2019.05.007
1569-1993/Crown Copyright © 2019 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. All rights reserved.

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
2 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx

3.6. Fat-soluble vitamins . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0

3.6.1. Vitamin D assessment and target levels. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.6.2. Supplementation of fat-soluble vitamins . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.7. Minerals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.7.1. Iron . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.7.2. Sodium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.8. Pancreatic enzyme replacement therapy (PERT) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.9. Genetic modulator therapies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.10. Complementary therapies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3.11. Cystic fibrosis-related co-morbidities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
4. Discussion and conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
Funding . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
Declarations of interest . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
Acknowledgements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0

1. Introduction Dietitians Association of Australia and Dietitians New Zealand. Roles of

the dietitian working group included:
The importance of nutrition is well recognised in cystic fibrosis (CF)
[1]. Studies consistently demonstrate that good nutrition is positively • Project chairs and facilitators - overarching responsibility for the plan-
associated with lung function and longevity [2,3]. Significant improve- ning and execution of the guideline development, coordination of
ments in the nutritional status of CF populations have been achieved chapter groups and compilation of the evidence base
over recent decades [4]. Despite these improvements, nutritional defi- • Chapter leads – responsible for overseeing evidence appraisal and de-
cits are still prevalent in CF, and can occur at a young age, persist velopment of evidence statements and practice recommendations for
throughout life, normalise with intervention, or emerge episodically their allocated chapter
[4]. The aetiology of undernutrition in CF is multifactorial, resulting • Chapter group members - provided input into the development of
from a combination of increased energy expenditure, nutrient malab- clinical questions and appraised research papers for quality and evi-
sorption, increased energy losses and inadequate dietary intake [4,5]. dence level
The use of clinical practice guidelines has been advocated as a strategy • Methodological experts - PhD qualified dietitians provided support
to improve the quality of health care by facilitating the application of and direction regarding literature review processes, writing evidence
consistent, evidence-based care [6]. A range of nutrition guidelines for statements, and grading of practice recommendations
CF have been published, targeting specific topics and/or countries/re-
gions [1,7–9].
The ‘Nutrition Guidelines for Cystic Fibrosis in Australia and NZ’
were released under the custodianship of the Thoracic Society of An interdisciplinary expert group, recruited via EOIs through the
Australia and New Zealand (TSANZ) in August 2017, as a planned up- TSANZ, Cystic Fibrosis Australia and Cystic Fibrosis New Zealand, was
date of a previously published clinical practice guideline [10]. Australia formed to provide specific context expertise. This group was comprised
and NZ are diverse countries with a CF population of approximately of respiratory physicians and other medical specialties related to CF care
4000 individuals, with dramatic climate variance ranging from tropical, including allied health professionals, nurse specialists, a consumer and a
hot and dry, through to cool temperate conditions [11]. Guidelines spe- medical librarian.
cific to the Australian and NZ context were considered salient as many
aspects of nutrition management are affected by geographical and cli-
matic conditions, including salt supplementation, hydration and 2.2. Development of practice questions
vitamin D.
The purpose of this paper is to showcase updated or changed recom- The dietitian working group identified and drafted the practice ques-
mendations for core components of nutrition care, along with highlights tions in PICO (Population, Intervention, Comparator, Outcome) format.
from the guideline recommendations and practice points. This paper The questions covered all aspects of the nutrition care process including
highlights topic areas that are new, unique, or impacted by climatic con- nutrition assessment and diagnosis, intervention, monitoring and eval-
siderations. The guidelines promote an interdisciplinary approach to uation and management of nutrition-related comorbidities. Further
management of nutrition in CF. Many of the guideline recommenda- input and refinement was sought from the interdisciplinary expert
tions will have relevance to those living in or travelling to other regions, group.
including those with similar climates, for example south-eastern
Europe, Asia, Africa and parts of South America.
2.3. Data sources and search strategy

2. Methods Systematic literature searches were conducted for each clinical

question with medical librarian support. Electronic databases (Embase,
The full methodology used to develop the ‘2017 Nutrition Guidelines CINAHL, PubMed, AustHealth, and Cochrane) were searched from Janu-
for Cystic Fibrosis in Australia and NZ’ is provided elsewhere [4,12,13]. A ary 2002 to June 2016. Key papers published between July 2016 and
summary is provided here. March 2017 were subsequently included if deemed to significantly in-
fluence practice or necessitate changes to recommendations or practice
2.1. Guideline development groups points.
Search terms were based on the Library of Medical Subject Headings
A working group was formed comprising over 40 Australian and NZ (i.e., MESH terms) and always included the terms “cystic fibrosis”, “nu-
CF dietitians. Expressions of interest (EOI) were circulated via the trition”, and “diet” (https://meshb.nlm.nih.gov/serarch).

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx 3

2.4. Study selection Table 1

Outline of guideline chapters.

All literature search results underwent a two-stage screening pro- Guideline Chapters
cess. Stage one included a review of titles and abstracts of all retrieved 1 Introduction
studies by a project chair, facilitator or chapter lead. All irrelevant, dupli- 2 Methods
cate and non-English publications were removed and full text articles 3 The Role of Nutrition in CF Care
for the remaining studies were sourced. In stage two, chapter leads 4 Service Delivery
5 Nutrition Assessment
and group members excluded articles that did not address any PICO
6 Nutrition Interventions
questions in that chapter. 6.1 – Undernutrition

- Behavioural Modification Strategies

- Appetite Stimulants
2.5. Literature appraisal
- Oral Nutrition Supplements
- Enteral Feeding
Two chapter group members independently appraised each study - Parenteral Nutrition
for level of evidence and quality rating. Each paper was assigned a
6.2 – Overweight and Obesity
level of evidence using the NHMRC criteria rankings [14] and a quality 7 Macronutrients
rating using the American Dietetic Association quality criteria checklist 8 Fat Soluble Vitamins
[15]. Where consensus regarding the level of evidence and/or quality 9 Minerals
rating was not reached between the two group members, a methodo- 10 Pancreatic Enzyme Replacement Therapy
11 Gastrointestinal and Hepatobiliary Considerations
logical expert was consulted to provide opinion. Consensus was reached
11.1 – Gastro-oesophageal Reflux Disease
through discussion. 11.2 – Distal Intestinal Obstruction Syndrome and Constipation
11.3 – Colon Cancer Screening
11.4 – Liver Considerations
2.6. Development of evidence statements, recommendations and practice 11.5 – Additional Considerations and the Role of the Gastroenterologist
points 12 Cystic Fibrosis Related Diabetes
13 Bone Health
14 Special Considerations
For each PICO question, consideration was given to the volume of ev- 14.1 – Pregnancy
idence, consistency of results and potential clinical impact. 14.2 – Genetic Modulator Therapies
Generalisability and applicability of the recommendation to Australian 15 Complementary Therapies
15.1 – Probiotics
and NZ healthcare contexts was also considered. The body of evidence
15.2 – Glutathione
was synthesised into evidence statements using the NHMRC evidence 15.3 – Coconut Oil
statement matrix [14]. Where sufficient evidence was available, practice 15.4 – Herbal Supplements
recommendations were developed, and each was assigned a grade ac- 16 Lung Transplantation
cording to the NHMRC grade of recommendation definitions for evi- 17 Implementing, Evaluating and Maintaining the Guidelines
18 Evidence Matrices
dence level: A (Excellent), B (Good), C (Satisfactory) or D (Poor) [14].
Appendices
Where insufficient evidence was available, consensus-based practice -Dietitians Association of Australia Cystic Fibrosis Role Statement
points were developed by the dietitian working group with comments -Comparison of WHO and CDC growth chart weight for age measurements in
sought from the interdisciplinary expert group. children birth to 24 months

3. Results

The guidelines are organised into eighteen chapters (Table 1). Sixty-
eight PICO questions were devised from which 41 graded recommenda- 3.2. Nutrition assessment
tions and a series of practice points were developed. These are available
in the guideline's Executive Summary https://www.thoracic.org.au/ Nutrition assessment is a cornerstone component of CF interdisci-
journal-publishing/command/download_file/id/44/filename/Preface. plinary care, with dietitians playing a key role. For outpatients, nutrition
pdf. Most chapters include a narrative background section (aetiology), surveillance is recommended at least four times a year, including a com-
as well as information on nutrition assessment, intervention, monitor- prehensive nutrition assessment undertaken at least annually [4]. The
ing and evaluation for that topic area. Since the previous guidelines guidelines recommend that inpatients undergo nutrition assessment
[10], four new topic areas have been added including dietitian prescrib- within 48 h of admission and a minimum of once per week thereafter.
ing in NZ, overweight/obesity, nutritional implications of genetic modu- Inpatient assessments should encompass acute changes in appetite,
lator therapies, and complementary nutritional therapies including the possible impact of hospital menu foods and quantification of intake
probiotics. All guideline chapters are freely available via the TSANZ during inpatient stay compared to usual intake. The guidelines provide
website www.thoracic.org.au. information to clinicians, including that which may be used to support
business case development for adequate dietetic services, food service
and nutrition support options.
3.1. Dietitian prescribing in New Zealand Table 2 outlines the criteria for classifying height, weight and BMI
status in CF. General population paediatric growth charts differ between
In New Zealand, Designated Dietitian Prescribers can prescribe countries, with Australia using the WHO growth charts for children 0–
subsidised items for therapeutic nutrition intervention, including fat- 2 years [16] and CDC Growth charts for children 2–18 years [17], and
soluble vitamins, pancreatic enzyme replacement therapy, zinc, iron NZ using the WHO growth charts for children 0–18 years [18]. The
and high dose vitamin D, along with oral and enteral supplements. criteria are informative for practitioners in other countries irrespective
This promotes timely access to nutrition-related medications and con- of which growth charts are used.
tributes to improved patient care. This information in the guidelines The guideline classifies optimal nutritional status with the same
may be useful for the development of extended scope of practice frame- targets as previously published for CF: BMI 50th percentile for chil-
works for dietitians. dren and adolescents / 22 kg/m2 in adult females and 23 kg/m2 in

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
4 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx

Table 2
Criteria for the classification of height, weight and BMI status and recommendations for intervention in cystic fibrosis⁎,1

Infants b 2 years⁎⁎ Children and Adults Recommendations for nutrition interventions

Adolescents 2–18 years⁎⁎

Optimal Weight-for-length ≥ 50th BMI 50th to b85th percentile Female BMI 22 to b27 kg/m2 Routine nutritional care and surveillance +/−
percentile⁎⁎⁎ using CDC growth chart Male BMI 23 to b27 kg/m2 education and preventative counselling
AND weight & length tracking (Australia)⁎⁎
AND within 2 percentile bands ofOR
each other BMI 50th to b91st percentile
using NZ-WHO growth
chart⁎⁎
Acceptable Weight-for-length 25th to b50th BMI 25th to b50th percentile Female BMI 20 to b22 kg/m2 Routine nutritional care and surveillance +/−
percentile AND weight & height Male BMI 20 to b23 kg/m2 education and preventative counselling
AND weight & length tracking tracking along previous AND no unintentional recent
AND within 2 percentile bands of percentiles weight loss
each other AND no recent weight loss
Suboptimal – at risk Weight-for-length 10th to b25th BMI 10th to b25th percentile BMI b20 kg/m2 Nutritional counselling, behavioural
of undernutrition percentile AND/OR AND/OR ≥ 5% unintentional weight management +/− oral nutrition supplements
AND/OR weight or length weight loss or plateau over loss over 2 months
decreasing N1 percentile band 2–4 months
AND/OR no weight gain
Persistent Persistent weight for length b BMI b10th percentile BMI persistently b18.5 kg/m2 As above, plus medical +/− psychological
undernutrition⁎⁎⁎⁎ 10th percentile AND/OR weight AND/OR weight falling N2 AND/OR ≥ 5% unintentional weight evaluation investigating factors contributing to
falling N2 percentile bands with percentile bands with loss over 2 months despite previous undernutrition. Consider intensive nutritional
stunting of growth stunting of growth nutritional interventions, regardless support via enteral nutrition
AND/OR failure of previous AND/OR failure of previous of starting BMI
nutritional interventions to nutritional interventions to
improve nutritional status improve nutritional status
High BMI Not applicable ⁎⁎⁎⁎⁎ Overweight: BMI ≥27 kg/m2 Diet and activity assessment, plus consideration
BMI 85th to b95th percentile AND/OR unintentional weight gain of medical and psychosocial contributing
Use growth chart to identify using CDC growth chart from previously acceptable BMI of factors. Consider goal-directed nutritional
rapid weight gain. (Australia)⁎⁎ N5 kg within a year. counselling
OR
BMI 91st to b98th percentile
using NZ-WHO growth
chart⁎⁎
Obese:
BMI ≥95th percentile using
CDC growth chart
(Australia)⁎⁎
OR
BMI ≥98th percentile using
NZ-WHO growth chart⁎⁎
High risk of developing
overweight or obesity
Unintentional weight gain
resulting in an increasing of
≥2 BMI centile bands
1
Source: Saxby N, Painter C, Kench A, King S, Crowder T, van der Haak N, et al. Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand. Bell S, editor. Sydney: Thoracic
Society of Australia and New Zealand; 2017.
⁎ Adapted from Australian population guidelines and previous Australasian and international recommendations for CF.
⁎⁎ WHO growth charts are used for all infants b2 years of age. For 2–18 years, at the time of writing, CDC growth charts are used in Australia, NZ-WHO growth charts are used in New
Zealand (http://www.health.govt.nz/our-work/life-stages/child-health/well-child-tamariki-ora-services/growth-charts). The overweight and obesity cut-offs for children and adolescents
are the same values used in each respective growth chart, reflecting the general population cut-offs in the absence of evidence to guide CF-specific thresholds.
⁎⁎⁎ See ‘Interpreting anthropometric measurements in children and adolescents’ in the full guideline document.
⁎⁎⁎⁎ Persistent undernutrition refers to undernutrition that is non-responsive to previous recommended interventions over a period of monitoring and evaluation that is individually
determined by the treating CF team.
⁎⁎⁎⁎⁎ While there are no evidence-based guidelines for treating overweight in infancy, recognition of rapid weight gain might identify if interventions to ameliorate the rate of weight gain
are indicated (Centers for Disease Control, https://www.cdc.gov/mmwr/pdf/rr/rr5909.pdf). Important to distinguish between catch-up growth after early deficit, and rapid weight gain
risking overweight or obesity in childhood.

adult males [1,8]. Consistent with other guidelines [1,19], a decline 3.3. Nutrition interventions for undernutrition
in nutritional status is recognised as a risk. This guideline augments
information in existing guidelines by including additional BMI tar- Interventions for managing undernutrition are consistent with those
gets (percentiles or thresholds) below which nutrition intervention outlined in other guidelines [1,7,8] and with subsequently published
should be escalated from routine anticipatory guidance. A 2008 standards of care [19]. Recommendations for managing undernutrition
study reported both a relative lack of specific criteria used to initi- cover behavioural interventions, oral nutrition support, enteral nutri-
ate and cease both ONS and enteral nutrition, and wide variation in tion and appetite stimulants. In this highlights paper, the topics of oral
the criteria being used [20]. The 2017 guidelines address these con- nutrition supplementation and enteral feeding were chosen to provide
siderations, by proposing a staged intervention based on nutrition a sample of how the PICOs and accompanying recommendations and
status category, outlined in Table 2. practice points appear in the full guideline document (Table 3).

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx 5

Table 3
PICOs, recommendations and practice points for Chapter 6.1 – Interventions for Undernutrition in CF.1

Chapter 6–6.1 Undernutrition

Behaviour Modification Strategies

Q 6.1.1 Compared to standard nutritional care, do behavioural interventions around food and mealtimes improve behaviours, diet variety, and weight or nutrition
status in children with CF?
R 6.1.1 GRADE B. Offer behavioural modification strategies to children at risk of/or with identified undernutrition. Conduct behavioural modification strategies in
combination with nutrition education.
Q 6.1.2 When should behavioural interventions around food and mealtimes be considered for children with CF?
R 6.1.2 GRADE C. Commence behavioural modification strategies early in life (i.e. during infancy or toddlerhood) and potentially continue throughout childhood.
Offer the following strategies:

• Differential attention (praise and ignoring)

• Contingency management (child only receives a desired reward after they have eaten their meal and/or performed desired mealtime behaviours)
• Self-monitoring of food intake (parents and/or child)
• Parental limit setting (establishing clear expectations and consequences)
PP 6.1.1 and Behavioural modification strategies are a valuable component of standard paediatric CF care
6.1.2 Strategies should be considered at a young age, before disruptive eating and mealtime behaviours become an ongoing issue.
For best results, strategies should be conducted with nutrition education.

Enteral Feeding
Q 6.1.6 Should enteral feeding be considered to improve nutrition outcomes for people with CF?
R 6.1.6 GRADE B. Consider enteral feeding as a means of improving markers of nutritional status (including weight, BMI and BMI z-score) in children and adults with
CF who have been assessed as being undernourished.
Q 6.1.7 Should enteral feeding be considered to improve pulmonary status in people with CF?
R 6.1.7 GRADE C. Practitioners should refrain from commencing supplementary enteral feeding for the sole purpose of improving or stabilizing pulmonary outcomes.
PP 6.1.6 and The decision to commence either short or long term enteral nutrition support should be made by an interdisciplinary team and in consultation with the
6.1.7 individual and their family, including discussion of risks and benefits.

• Benefits on nutrition outcomes, particularly weight and BMI are well documented
• There is no conclusive evidence to support beneficial effects on pulmonary function
The decision can be emotionally challenging for some people with CF. Where possible, appropriate psychosocial support should be provided and the
individual's decision should be respected. An anaesthetist should be consulted prior to surgical or endoscopic gastrostomy tube insertion in people with
moderate to severe CF lung disease.
Q 6.1.8 When should enteral feeding be introduced for people with CF?
R 6.1.8 Ungraded. There is insufficient evidence to make a recommendation regarding when to introduce enteral nutrition in CF. Evaluate appropriate timing on an
individual basis.
PP 6.1.8 No evidence to support best timing for enteral nutrition support in CF. The following considerations should be noted in regards to timing of enteral nutrition:

• Whilst many patients will have had a trial of oral nutritional supplements (ONS) prior to the need for enteral nutrition being assessed, there is no evidence
that favours assessing the impact of ONS first, over proceeding to enteral nutrition. Evaluate whether to trial ONS prior to considering enteral nutrition on
an individual basis
• Enteral nutrition should be commenced prior to the onset of significant disease progression and FEV1 decline for more favourable nutritional outcomes
Q 6.1.9 What is the ideal enteral feeding regimen for people with CF?
R 6.1.9 Ungraded. There is insufficient literature to suggest the ideal enteral formula or regimen in the CF population. Select enteral formulas and devise enteral
feeding regimens on an individual basis.
Q 6.1.10 What are the risks associated with enteral feeding in CF compared to the general population?
R 6.1.10 GRADE C. There is no evidence that people with CF are at increased risk of major complications and mortality as a result of enteral feeding. Manage minor side
effects of enteral feeds, including stoma site issues and GORD, as for the general population.⁎

PICO = Population Intervention Comparator Outcome.

Q = Question.
R = Recommendation.
PP = Practice Points.
1
Source: Saxby N, Painter C, Kench A, King S, Crowder T, van der Haak N, et al. Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand. Bell S, editor. Sydney: Thoracic
Society of Australia and New Zealand; 2017.
⁎ see Chapter 6 in the full guideline document for additional practice points.

3.4. Overweight and obesity and adolescents, the guideline proposes use of general population over-
weight and obesity criteria for children and adolescents (Table 2). For
Nutrition care in CF has historically been focussed on managing un- adults, the general population cut-offs to define the upper end of the
dernutrition. To the authors' knowledge, the Australian and NZ CF Nu- healthy weight range (BMI 25 kg/m2 in adults) are unlikely to be appli-
trition guidelines are the first CF nutrition guidelines to include cable in chronic conditions such as CF which are associated with alter-
information on the identification, assessment and management of ations in body composition including normal weight obesity, and it
high BMI/BMI percentiles (Table 2). With newborn screening, lifetime cannot be assumed that weight loss is predominantly fat mass loss
centre-based multidisciplinary care, improved survival, the advent of [23,24]. The guidelines suggest that for adults, a BMI ≥27 kg/m2 or unin-
genetic modulator therapies and other treatment improvements and in- tentional weight gain of N5 kg from a previously acceptable weight
creasing CF diagnoses later in life associated with milder mutations [21]; merits attention (Table 2). Regular monitoring of individuals with
nutritional status of CF populations in many countries has improved high BMI / BMI percentiles is important, and it is suggested that inter-
[21,22]. With this has come the recognition of a growing proportion of ventions aimed at reducing weight be instituted only after assessment
the CF population with high BMI or BMI percentiles [21,22]. of diet, body composition, physical activity and contributing medical
The clinical significance of overweight and obesity in CF is unclear. and psychosocial factors, and be regularly monitored to ensure mainte-
There is a plateau in the association between BMI and FEV1% predicted nance of lean body mass [4].
in the range traditionally defined as overweight (BMI 25–30 kg/m2 for The Nutrition Intervention section of the guidelines provides guid-
adults) [8,21]. To date, there are no universally accepted cut-offs for ance for assessing and managing individuals with high BMI, including
overweight or obesity (high BMI/BMI percentiles) in CF. For children consideration of weight history, body composition, dietary and lifestyle

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
6 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx

factors, lung function, the risk of metabolic disease and individualisation Table 4
of dietary energy and macronutrient targets. Further research is needed Recommended daily doses of fat soluble vitamin supplementation for people with cystic
fibrosis and pancreatic insufficiency.1
to articulate risks associated with overweight and obesity, and to iden-
tify best practice management approaches. In the intervening period, Age Vitamin A Vitamin D Vitamin E Vitamin K
the 2017 guidelines provide a starting framework for evaluating over- (IU) (IU) (IU) (ug)

weight/obesity in CF, against which practices can be measured and Infants 1500–2000 400–1000 40–80 300–1000
monitored. They are likely to have applicability in other regions of the Young Children 1500–5000 800–2000 50–150 1000–10,000
Older children 2500–5000 800–4000 150–300 1000–10,000
world which are also seeing increases in overweight and obesity in CF
Adolescents and adults 2500–5000 800–4000 150–500 1000–10,000
[3,25].
1
Source: Saxby N, Painter C, Kench A, King S, Crowder T, van der Haak N, et al. Nutrition
Guidelines for Cystic Fibrosis in Australia and New Zealand. Bell S, editor. Sydney: Thoracic
3.5. Routine dietary management: energy and macronutrients, including Society of Australia and New Zealand; 2017.
essential fatty acids

Recommendations for energy intake in the 2017 guidelines are con- clinicians are guided by recent European recommendations for vitamin
sistent with other guidelines, namely 110–200% of the population- K supplementation (Table 4) [1].
based energy requirements. The inter- and intra-individual variation
in energy requirements is emphasised, underscoring the importance 3.7. Minerals
of regular individualised assessment and monitoring of nutritional in-
take, status and goals. There was insufficient evidence to make CF- The minerals section of the guidelines provides a comprehensive
specific protein intake recommendations, however, guidance on an overview of those considered to be at risk of deficiency in CF: calcium,
upper limit of 25% of energy intake from protein in line with recommen- zinc, magnesium, iron and sodium. This highlights paper will focus on
dations for the general population is provided [26]. iron and sodium.
The guideline provides a comprehensive overview of the aetiology of
essential fatty acid (EFA) deficiency in CF; plant and animal-based 3.7.1. Iron
sources of EFAs; signs and symptoms of EFA deficiency; the evidence People with CF are thought to be at an increased risk of iron defi-
for dietary supplementation with omega-3 fatty acids in CF; safety con- ciency due to the chronic inflammatory nature of CF, potential for inad-
siderations and monitoring of omega-3 fatty acid supplementation. In equate dietary intake, gastrointestinal co-morbidities such as
summary, while there is Grade C (satisfactory) evidence that dietary malabsorption, small intestinal bacterial overgrowth or gastroesopha-
supplementation with omega-3 fatty acids may improve some bio- geal reflux, haemoptysis and chronic bacterial airway colonisation
chemical and inflammatory indices for people with CF, such as im- [29]. The guidelines recommend annual assessment of iron status
proved fatty acid profiles, the evidence is insufficient to recommend using a combination of serum iron, transferrin and ferritin, together
their routine use for improving overall clinical outcomes. with a marker of inflammation such as C-reactive protein [4], and fea-
ture information on interpreting biochemical markers of iron status. In
the presence of inflammation or infection, serum ferritin may be falsely
3.6. Fat-soluble vitamins
elevated. Therefore if CRP is high, serum iron is low but ferritin is nor-
mal, iron deficiency should not be overlooked. Measurement of soluble
The fat-soluble vitamins section of the guidelines provides informa-
transferrin receptor level can assist interpretation as it is unaffected by
tion on food sources of each vitamin, aetiology of deficiency and toxicity,
inflammation [30]. Information on dietary targets, animal and plant-
assessment of vitamin status, supplementation of vitamins A, D, E and K
based food sources of iron, and treatment of iron deficiency, together
and monitoring of status [4].
with guidance on oral supplementation using liquid or tablet prepara-
tions, is provided. Iron infusion should be considered when oral iron is
3.6.1. Vitamin D assessment and target levels not tolerated or fails to improve iron status. There was insufficient evi-
The guidelines recommend a target for serum vitamin D of dence to support the contraindication of iron supplementation in
≥50 nmol/L, if measured at the end of winter. When levels are measured those chronically colonised with Pseudomonas aeruginosa [4].
at other times of the year, a target of 10-20 nmol/L higher is recom-
mended [4]. This approach takes into account the geographic diversity 3.7.2. Sodium
of Australia and NZ, where due to distance or other factors it may not It is well known that people with CF are at increased risk of sodium
be feasible for every patient to have annual blood testing at the end of deficiency [10] and this is compounded for people living in hot and/or
winter. This information provides a pragmatic approach for other coun- humid climates. The primary factor contributing to sodium deficiency
tries if similar constraints on the season of measurement exist. is the CFTR gene defect which affects the flow of sodium and chloride
ions and water in and out of cells, resulting in higher sodium and chlo-
3.6.2. Supplementation of fat-soluble vitamins ride losses in sweat. There is a lack of research available to guide specific
There is satisfactory evidence to support the routine supplementa- individual sodium requirements for people with CF and as a result, rec-
tion of vitamins K and E in individuals with CF [4]. However, evidence ommendations vary in international consensus and review documents.
to support routine supplementation of vitamins A and D is lacking, Recommendations for sodium requirements in Australia and NZ are
favouring individualised supplementation guided by biochemical as- outlined in the 2017 guidelines: 500-1000 mg for infants, 1000-
sessment and previous response to supplementation. In the absence of 4000 mg for children and up to 6000 mg for adolescents and adults
new guiding evidence, recommendations for vitamin A and E dosing re- [4]. Individual requirements may vary and are best guided by signs
main unchanged from the 2006 guidelines (Table 4) [10]. At the time of and symptoms of sodium depletion, dietary intake, exercise level and
guideline preparation, there were no studies investigating the optimal sweat rate. A range of strategies for the administration of sodium sup-
routine dosing of vitamin D to prevent deficiency in CF. The guideline plementation, for example administration in apple puree, infant for-
points clinicians to recent US and European recommendations [1,27] mula or syringing a salt solution in divided doses across the day for
(Table 4). infants, are available in the guidelines. The guidelines provide a useful
In recent years, there has been an increase in recommended vitamin reference for other countries with areas of hot or humid climates
K supplementation doses in international consensus documents, based where local CF guidelines may not exist and for clinicians in temperate
on evidence of its role in bone health [28]. It is recommended that climates whose patients are travelling to hot or humid climates.

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx 7

Prescribed PERT dose

Inadequate Response
Adequate Response
(Steatorrhoea/poor weight gain)

No Action Required
Good Adherence Poor Adherence
Appropriate Inappropriate
distribution of PERT distribution of PERT
to fat content to fat content

Check storage and use-by date

Consider strategies to
Consider quantifying steatorrhoea
reinforce adherence
Consider referral to a gastroenterologist for consideration of role
of gastric emptying rate
Review of PERT dosing to ensure appropriate
Consider changing timing of PERT to either before, during or after
distribution of PERT to fat content
the meal depending on current pattern

Adequate Response Inadequate Response

No Action Required Increase dose by small

increments to a maximum of
10,000 IU lipase/kg/day

Adequate Response Inadequate Response

No Action Required
Consider PPI trial ± referral to
gastroenterologist

Adequate Response Inadequate Response

Monitor and review and Gastroenterology review to

consider trial off PPI after a consider investigations for
period of time - e.g. 3-6 other causes for
months malabsorption

Fig. 1. Flowchart for the monitoring and evaluation of pancreatic enzyme replacement therapy (PERT)1 1 Source: Saxby N, Painter C, Kench A, King S, Crowder T, van der Haak N, et al.
Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand. Bell S, editor. Sydney: Thoracic Society of Australia and New Zealand; 2017

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
8 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx

Strategies to meet sodium requirements are provided in the guidelines 3.11. Cystic fibrosis-related co-morbidities
and include commercial salt supplementation, encouraging salty foods
and adding salt to food and cooking. The guidelines address key nutrition-related co-morbidities (CF-re-
lated diabetes, CF-related liver disease, gastro-oesophageal reflux; distal
3.8. Pancreatic enzyme replacement therapy (PERT) intestinal obstruction syndrome, bone health); and other key topics, in-
cluding pregnancy, lung transplantation and colon cancer screening,
The guidelines provide information on the aetiology of pancreatic in- outlining evidence-based recommendations and practice guidance on
sufficiency, PERT preparations available in Australia and NZ and their nutrition and dietary management [4]. The guidelines promote an inter-
mechanism of action, dosing, administration with enteral feeds, the im- disciplinary approach to nutrition care and provide a useful resource to
pact of genetic modulator therapies on PERT and phthalates in PERT [4]. support a team approach for nutrition management for CF physicians
The practice points provide advice for clinicians to consider when dos- and other clinicians in specialities including gastroenterology,
ing PERT including the maximum upper limit. The guidelines give an hepatology and endocrinology.
overview of tests available to assess pancreatic function, including the
three-day faecal fat balance test and the highly sensitive and specific
faecal elastase-1 test. Information on the strengths and limitations of 4. Discussion and conclusion
the most common tests is also provided. The guidelines acknowledge
the lack of universal consensus on a single test to determine pancreatic The ‘2017 Nutrition Guidelines for CF in Australia and NZ’ offer a
status [4]. number of significant points of difference from other published nutri-
The complex nature of CF means that the response to PERT can often tion guidelines, such as the inclusion of information on overweight/obe-
be suboptimal, relating to adherence, timing, type and dose of PERT, and sity, genetic modulators and complementary therapies. While some
small intestinal pH. Fig. 1 provides a flowchart for monitoring and eval- recommendations and practice points, such as vitamin D and salt sup-
uating PERT efficacy [4]. plementation, were prepared specifically for the Australasian context,
they can be generalised to other countries with a wide latitude variation
and varied climate conditions. The guidelines also include valuable, up
3.9. Genetic modulator therapies to date information on core nutrition topics, such as growth assessment,
pancreatic enzyme replacement therapy, nutrition interventions, mi-
One of the key advances in CF care has been the introduction of CF cronutrient supplementation and nutrition-related co-morbidities.
genetic modulator therapies. These have had significant impact on the The rigorous process undertaken to develop these evidence-based
nutritional status of CF patients, resulting in improved weight status, recommendations and practice points, underpinned by peer review in-
improved pancreatic function and normalised sweat chloride [31,32]. volving both consumers and local and international clinical experts, en-
To the authors' knowledge, no nutrition guideline to date has provided sures the 2017 nutrition guideline content reflects the needs of the CF
information on the nutrition care of patients on CFTR modulator thera- community. The full guideline document contains rich narrative text
pies. There is excellent evidence to suggest that ivacaftor therapy leads which summarises the evidence that informed the recommendations.
to significant improvements in weight and BMI in children over two Where the quality of evidence was insufficient to make graded recom-
years of age and adults. The guidelines provide insight into how genetic mendations, practice points were developed, which provide practical
modulator therapies may change the nutritional needs of people with material to support guideline implementation. The framework used
CF together with guidance for nutritional management, including for the guidelines will allow for revision of evidence statements and
weight, energy intake targets, PERT and salt. upgrading of practice points into graded recommendations as addi-
The evidence available at the time of writing was derived largely tional evidence emerges. These attributes render the guidelines particu-
from studies involving ivacaftor, although the principles may translate larly unique in that they are an integrated resource providing both
to other genetic modulator therapies. Clinicians are encouraged to pro- recommendations and guidance for translation into practice for CF clini-
actively monitor weight patterns during genetic modulator therapy to cians providing nutrition care.
ensure nutritional recommendations are tailored to individual needs The guideline acknowledges gaps in the evidence base and suggests
and changing body composition. This information is relevant world- areas for future research including, but not limited to, the optimal
wide, and provides clinicians with practical advice for managing pa- timing and regimen for enteral feeding; management of overweight/
tients on genetic modulator therapies. Subsequent research will obesity in CF; optimal supplementation of fat-soluble vitamins includ-
contribute to further understanding of the mechanisms underlying nu- ing the use of high dose vitamin D; long term nutritional considerations
tritional status changes and the impact of genetic modulators on related for people on genetic modulator therapies; and the use of probiotics and
CF treatments including insulin, which in turn will guide the evolution other complementary therapies.
of practice advice and future guidelines. To ensure the best quality nutrition care for people with CF, it is sug-
gested that nutrition guidelines be implemented in conjunction with
3.10. Complementary therapies local standards for CF care, a number of which have been published
[19,33], emphasising the importance of nutrition and growth monitor-
To our knowledge, this is the first nutrition guideline in CF to include ing as integral components of quality CF. The full guideline document
information on complementary therapies, highlighting the interest in contains a framework for implementation and evaluation.
the area which emerged from consumer feedback at the guideline de- In conclusion, the ‘2017 Nutrition Guidelines for Cystic Fibrosis in
velopment stage. Specific complementary therapies reviewed included Australia and NZ’ provide comprehensive guidance for the management
probiotics, garlic, glutathione, coconut oil and herbal supplements. of nutrition and nutrition-related co-morbidities in CF. They comple-
Probiotics is an emerging area of research in CF. The guidelines provide ment and augment other recently published guidelines through the
a summary of the available evidence at the time of guideline develop- use of systematic appraisal of the literature which was synthesised
ment. While Lactobacillus genus probiotic specifically may provide into evidence statements; graded recommendations, and practice
some health benefits for people with CF, there is insufficient evidence points guiding translation of evidence into clinical management of peo-
to support routine or targeted supplementation in CF [4]. The guidelines ple with CF. The incorporation of geographic and climatic considerations
include information on safety and potential adverse effects of these for issues such as vitamin D, salt and hydration, together with the inclu-
therapies, and recommends that clinicians encourage patients to discuss sion of novel topics such as the nutritional implications of genetic mod-
complementary and alternative therapy use with their treating teams. ulator therapies, overweight/obesity and complementary therapies

Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007
 N. van der Haak et al. / Journal of Cystic Fibrosis xxx (2019) xxx 9

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Please cite this article as: N. van der Haak, S.J. King, T. Crowder, et al., Highlights from the nutrition guidelines for cystic fibrosis in Australia and
New Zealand, Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2019.05.007