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The optimal approach to nutrition and cystic fibrosis: Latest evidence and
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Article in Current Opinion in Pulmonary Medicine · November 2017

DOI: 10.1097/MCP.0000000000000430

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REVIEW

CURRENT
OPINION The optimal approach to nutrition and cystic
fibrosis: latest evidence and recommendations
Francis M. Hollander a, Nicole M. de Roos b, and Harry G.M. Heijerman c

Purpose of review
Cystic fibrosis (CF) is a progressive genetic disease that affects multiple organ systems. Therapy is directed
to maintain and optimize nutritional status and pulmonary function, as these are key factors in survival. In
this review, the most recent findings regarding nutritional management associated with pulmonary function
and outcome will be explored.
Recent findings
Evidence-based and expert-based guidelines emphasize the need for adequate nutritional intake to improve
nutritional status. For infants and young children, the aim is to achieve the 50th percentile of weight and
length for a healthy same-age population up to age 2 years. For older children and adolescents 2–18
years, the target is a BMI of at or above the 50th percentile for healthy children. For CF adults of at least
18 years, the target is a BMI of at or above 22 kg/m2 for women and at or above 23 kg/m2 for men.
Recently, new drugs are developed with the aim to treat the malfunction of the cystic fibrosis
transmembrane conductance regulator gene. This potentiator/corrector therapy improves lung function and
nutritional status and decreases the number of infective exacerbations. As survival is improving and the CF
population is aging, it is important to focus on micronutrient and macronutrient intake of CF patients in
different age and disease stages.
Summary
Recent evidence-based nutritional guidelines and improved medical treatment support the nutritional
monitoring and interventions in CF patients. Nutritional care should be personalized and provided by a
specialized CF dietitian because patients’ care needs may change dramatically during their disease
progress.
Keywords
cystic fibrosis, cystic fibrosis transmembrane conductance regulator, evidence-based guidelines, nutrition,
pulmonary function

INTRODUCTION New Zealand and the majority of states in North

Recent decades have shown a great improvement in America. Major improvements in both nutritional
survival of individuals with cystic fibrosis (CF) [1]. status and lung function in young children, adoles-
Registries in Europe, the USA and Canada now cents and adults as a result of NBS have been reported.
demonstrate that median survival for CF is around Early diagnosis of CF by NBS and early intervention,
40–50 years (UK 45.1, USA 41.1 and Canada 49.7 including nutritional supplementation to prevent
years). The median age at death worldwide is around
30 years [1,2] because of health inequalities with the a
Department of Dietetics, Internal Medicine and Dermatology, University
dramatic variation in the survival of CF individuals Medical Center Utrecht, Utrecht, bDivision of Human Nutrition and
across Europe [1]. The increased survival can be Epidemiology, Wageningen University and Research, Wageningen and
c
Division Heart and Lung, Pulmonology, University Medical Center
explained by implementation of centralized care,
Utrecht, Utrecht, The Netherlands
newborn screening (NBS) programmes and evi-
Correspondence to Francis M. Hollander, RD, Department of Dietetics,
dence-based guidelines including better nutritional Internal Medicine and Dermatology, University Medical Center Utrecht,
care and the introduction of drugs aimed at the G01.111, P.O. Box 85 500, 3508 GA Utrecht, The Netherlands. Tel: +31
modulation of cystic fibrosis transmembrane con- 88 75 566 09; fax: +31 88 75 556 09;
ductance regulator (CFTR) protein. e-mail: [email protected]
NBS programmes for CF have been widely Curr Opin Pulm Med 2017, 23:556–561
adopted in several European countries, Australia, DOI:10.1097/MCP.0000000000000430

www.co-pulmonarymedicine.com Volume 23  Number 6  November 2017

 Nutrition and cystic fibrosis Hollander et al.

Moreover, nutritional status is positively associ-

KEY POINTS ated with pulmonary function and survival in patients
 Recent evidence-based and expert-based nutritional with CF. This strongly supports the nutritional strate-
guidelines provide important guidance for the optimal gies and the efforts to obtain normal growth in CF
treatment of each individual with CF in different age children, and the importance to maintain adequate
and disease stages. nutritional status in adults with CF. Careful nutri-
tional monitoring as recommended in European
 More experiences of CFTR modulation and its effects on
nutritional status and lung function will offer challenges Society of Parenteral and Enteral Nutrition (ESPEN)-
to adapt dietary interventions in CF care in the ESPHAN-European Cystic Fibrosis Society (ECFS)
&&
near future. guidelines [5 ] should continue to apply current early
growth recommendation, with goal weight-for-length
 Future research needs to focus on perfecting bedside
(WFL) at or above the 50th percentile on growth charts
monitoring of body composition and energy – and &&

protein requirement in CF to improve nutritional status. before age 2 [6 ,7]. Timely and focused interventions
to improve nutritional status are of great importance
 Nutritional care is an important part of personalized to improve prognosis and survival in CF.
medicine provided by a specialist CF dietitian. The standard nutritional care for CF has been a
 Nutritional management of CF is a continuously high caloric diet with pancreatic enzyme replace-
evolving field and warrants rigourous nutritional ment therapy (PERT) and fat-soluble vitamin sup-
scientific research. plementation to achieve an adequate nutritional
&&
status [5 ]. The nutritional status can also be
improved by using CFTR modulators, including
malnutrition, mucolytic therapy and aggressive correctors and potentiators. The effect of CFTR
treatment of infection with Pseudomonas aeruginosa, potentiation on clinical outcome and subsequently
may have contributed to these improvements improved nutritional status is well described by
[3,4].
&&
Borowitz et al. [8 ] (Table 1). A CFTR potentiator,

Table 1. Potential mechanisms through which Ivacaftor might contribute to improved nutritional status
Effect of CFTR potentiation Associated clinical outcome that could lead to improved nutritional status
In the sinuses and lower respiratory tract
" Fluidity of respiratory tract mucus
# Sinus mucus " Sense of smell ! improved appetite
# Airway mucus ! # Work of breathing !# energy expenditure and improved appetite
# Airway obstruction ! fewer pulmonary exacerbations
In the gastrointestinal (GI) tract
" Bicarbonate and fluid flow through pancreatic duct,
Brunner’s glands, and biliary tract
. . .Leads to improved fat/nutrient absorption Lipolytic: improved pH optimum for pancreatic enzymes,
possible unplugging of pancreatic ductules and ‘rescue’
of acinar function
Postlypolytic: improved micelle formation
# Thickness of gut mucus layer
# Inflammation ! improved enterocyte absorption
. . .and increase fluid in GI tract
# Obstipation " Appetite
# Small intestinal bacterial overgrowth # Competition for intestinal nutrients
Less inflammation ! appetite
. . .and better action of defensins Change in fecal microbiome
Less inflammation !improved appetite, #energy expenditure
Systemic
Improved insulin secretion # Loss of calories through glycosuria
" Protein anabolism
Less inflammation ! improved appetite, #energy expenditure

&&
Reproduced from [8 ].

1070-5287 Copyright ß 2017 Wolters Kluwer Health, Inc. All rights reserved. www.co-pulmonarymedicine.com 557
Cystic fibrosis

&&
known as Ivacaftor and commercially available as predicted and nutritional status [6 ,29]. Children
Kalydeco, has produced results as respiratory func- with CF who achieve higher WFL at age 2 years have
tion gain, and pulmonary exacerbation reductions, improved pulmonary and survival outcomes into
and several other achievements, such as better con- adulthood. CF care providers should be using
trol of diabetes, improvement of pancreatic func- growth charts appropriate to the nationality and
tion, increase of body weight, improvement of BMI ethnicity of the patients. If these are not available,
&
and BMI z scores and quality of life [9–12,13 ,14]. the WHO growth charts should be used www.who.
&&
Other studies show an improvement in lung func- int/childgrowht/standards/en/ [5 ]. Growth and
tion [12], a slower rate of lung function decline [10] nutritional status should be monitored as part of
&&
or no significant changes [11]. With more knowl- routine CF care, including pubertal status [6 ]. For
edge about genetic modifiers of the disease, the CF adults over the age of 18 years, the target is a BMI
individual prognosis can be better defined and indi- of at or above 22 kg/m2 for women and 23 kg/m2 for
&& &
vidualized therapy can be optimized. men [5 ,30 ]. Methods and timing of assessment
As survival has increased, the number of adults and monitoring of nutritional status in age-related
with CF is expanding, as the majority of children with CF people are well described in the ESPEN-
&&
CF now live into adulthood. Evidence-based nutri- ESPGHAN-ECFS guidelines [5 ]. In adults, assess-
tional guidelines for CF have been developed recently ment of body composition is becoming clinically
with more attention to adequate treatment of nutri- important as depleted or low fat free mass (FFM)
tional-related complications in CF such as CF-related is associated with significant lung disease and
diabetes (CFRD) and malnutrition [15,16]. impaired pulmonary function [15] and independent
&
of BMI level [30 ]. In children, bone mineral content
(BMC) is a more sensitive indicator of nutritional
NUTRITIONAL MANAGEMENT OF CYSTIC deficit than low BMI; low values of BMC are corre-
FIBROSIS; AN UPDATE FOR ADEQUATE lated with impaired pulmonary function in children
&&

TREATMENT ACROSS THE LIFESPAN with CF [5 ]. Furthermore, total body potassium

counting is a body composition assessment method
Recently, excellent evidence-informed and practice-
that measures the body cell mass (BCM). The BCM is
based guidelines on nutrition care of infants, chil-
the metabolically active component of the FFM and
dren and adults with CF have been developed by the
reflects the functional cellular components of the
ESPEN-European Society of Paediatric Gastroenter-
body involved in biochemical processes and energy
ology, Hepatology and Nutrition (ESPGHAN)-ECFS
&& metabolism. BCM is not influenced by hydration. In
[5 ], and also the Cystic Fibrosis Foundation (CFF)
contrast to the interpretation of total FFM, which
evidence-informed guidelines on enteral tube feed-
can be affected by hydration changes with growth
ing (ETF) [17]. In addition, the Australian evidence-
&& and disease [31]. Therefore, BCM measurements
based guidelines are expected for October 2017 [18 ].
are an important reflection of nutritional status in
Also (systematic) reviews on oral calorie supple-
growing children and those with clinical conditions.
ments for CF, vitamin D, vitamin E, omega-3 poly
However, this method may not be widely available
unsaturated acids, probiotics, insulin and oral
and other methods such as bioelectrical impedance
agents for managing CFRD endorse the importance
analysis and dual energy X-ray absorptiometry can
of adequate nutritional treatment at different age
&& provide information on fat mass and FFM and should
and disease stages in CF [19–24,25 ,26–28].
be used where possible. Future research needs to
focus on perfecting bedside techniques to assess
body composition, which will assist in improving
IMPORTANCE OF ADEQUATE GROWTH nutrition-related outcome measures [31].
AND NUTRITIONAL ASSESSMENT
BMI and BMI percentile for age are important mea-
sures of nutritional status in both adults and chil- ENERGY BALANCE IN CYSTIC FIBROSIS
dren with CF. The goal is a WFL of at or above Optimal energy intake is critical to the overall
the 50th percentile in children less than 2 years health of people with CF. Insufficient nutritional
of age and a BMI of at or above the 50th percentile intake is common in the CF population and
for children older than 2 years, meaning that is caused by poor appetite, malabsorption and
nutritional status is comparable with that of well- disturbed body image. This, in combination with
&& &&
nourished healthy children [5 ,6 ,7]. The rationale increased caloric expenditure, makes it often diffi-
&&
for this goal is that a positive association exists cult to attain an appropriate nutritional status [6 ].
between pulmonary function, generally measured The energy balance is not only determined by
by forced expiratory volume in 1 s (FEV1) percentage energy intake (food), energy expenditure (activity,

558 www.co-pulmonarymedicine.com Volume 23  Number 6  November 2017

 Nutrition and cystic fibrosis Hollander et al.

maintenance and increased demands with inflam- supplements have increased energy intake and
&&
mation) and losses (diarrhea and vomiting). In CF, a weight in undernourished patients [5 ], but further
variety of factors contribute to individual energy research should investigate the long-term effect of
needs, including nutrient maldigestion and/or mal- supplement use.
absorption, presence of pulmonary exacerbation, The CFF recommends the use of ETF in people
pulmonary function, FFM, sex, pubertal status, with CF who are unable to attain caloric require-
CFTR mutation, age and other medical complica- ments to meet growth/weight maintenance goals
&&
tions, such as liver disease and CFRD [6 ]. despite evaluation by a multidisciplinary team [17].
Loss of energy due to malabsorption is a problem Using this intervention, it is important to monitor
in CF patients with exocrine pancreatic insuffi- for complications such as glucose intolerance and
ciency. PERT is essential to improve fat malabsorp- glucosuria. Gastrostomy tubes are commonly used
&
tion in pancreatic insufficient patients [32 ]. Eighty- in patients using ETF, and jejunostomy tubes are
&
five percent to 90% of people with CF have pancre- placed in patients with gastro paresis [30 ].
atic insufficiency, leading to malabsorption of Although CF is commonly associated with mal-
nutrients, especially fat and fat-soluble vitamins nutrition, the proportion of overweight and obese
&& &
[6 ,32 ]. Addition of proton pump inhibitors may individuals is increasing. In one US pediatric CF
&
improve the effectiveness of PERT [32 ]. Current center, 23% of patients with CF aged 2–18 years
guidelines recommend lipase intake by age of the were found to have an average BMI percentile
patient, by body weight and by grams of fat ingested greater than 90. Surprisingly, 88% of the overweight
&&
per day [5 ], but a large variability and inconsis- patients and 69% of the obese patients had CFTR
tency with new guidelines on nutrition and PERT- mutations associated with pancreatic insufficiency.
use was found in six European pediatric CF centers In a longitudinal cohort study that spanned from
[33]. The MyCyFAPP Project has been started to 1985 to 2011, the proportion of overweight or obese
develop educational and self-management tools adults in a Canadian CF center increased from 7 to
for patients’ better adherence to therapies [33]. 18%. The benefit of increasing BMI greater than
Increased caloric expenditure is mainly caused 25 in adults seems to be small as improvement in
&
by increased work of breathing due to chronic lung pulmonary function seems to be blunted [30 ].
infection and loss of pulmonary function. In addi- Moreover, recent studies show a higher proportion
tion, chronic pulmonary infection and inflamma- of obese individuals with CF having elevated serum
&& &
tion lead to cytokine-induced catabolism [6 ]. In triglycerides and total cholesterol [30 ]. Further-
patients with end-stage CF, predication equations more, a cross-sectional study in 32 adults with CF
for energy needs underestimate resting energy and a reference group of 20 adults without CF
expenditure (REE) [34]. showed that a normal-weight BMI with elevated
There is a wide variation in the energy require- percentage body fat is associated with reduced lung
&
ments of people with CF. To achieve a normal function [35 ]. Dietary recommendations should
growth and nutritional status, energy intake targets therefore focus on a balanced diet and a healthy
for age may need to be increased in children with lifestyle with good exercise habits to achieve an
&&
CF, although obesity should be avoided [5 ]. For adequate body composition with normal fat and
this reason, recommendations for energy needs are FFM percentages.
110–200% of those required by the healthy popula-
&&
tion of the same sex and age [5 ,15]. Improvement
in weight gain can be achieved using energy dense SPECIALIZED NUTRITION-RELATED
diets, and additional oral nutritional supplements TREATMENTS IN CYSTIC FIBROSIS
(ONS), behavioral interventions, ETF or parenteral For CF patients with pancreatic insufficiency, recent
&&
nutrition [5 ,15]. guidelines recommend the evaluation of plasma
A recent systematic review of ONS in three levels of fat-soluble vitamins after the start of
randomized clinical trials (total of 131 patients) enzyme and vitamin supplementation 3–6 months
found that ONS did not promote additional weight after initiation or change in vitamin therapy; and
&&
gain in moderately malnourished children with CF annually thereafter [5 ]. A review of Li et al. [36]
over and above the use of dietary advice and moni- compared recent reports of actual dietary intake and
toring alone [19]. In contrast with children, the use nutritional status of micronutrients such as miner-
of ONS in adults with CF has not been adequately als, trace elements and vitamins with relevant
&&
studied [5 ]. Although the Cochrane review con- dietary recommendations for CF. They conclude
cluded that there is not enough evidence to support that although dietary intake and nutritional status
the use of ONS, practice-based evidence has shown in CF have improved significantly in recent decades,
that in clinical practice, individually prescribed micronutrient status seems to have diverged.

1070-5287 Copyright ß 2017 Wolters Kluwer Health, Inc. All rights reserved. www.co-pulmonarymedicine.com 559
Cystic fibrosis

Recommendations for different age groups differ 30% predicted or if pulmonary hypertension is pres-
between countries [36]. The optimal dosages of ent [40]. Pregnant women with moderate pulmo-
long-term micronutrient supplementation require nary function increase the probability of an optimal
further investigation, so that safety and effect on outcome [41]. Management includes adequate
reducing lung disease severity and CF-related com- nutrition with PERT and fat-soluble vitamins, man-
plications are balanced [20,36]. Vitamin D is under agement of the chest infections with antibiotics and
investigation for its potential role in gut microbiota monitoring of maternal diabetes [40]. The third
modification [37], intestinal calcium absorption trimester may be associated with increasing dyspnea
&&
and bone health [5 ,22], recovery from pulmonary that may necessitate bed rest with supplemental
exacerbations [22] and improvement of lung func- oxygen and nutritional supplements [41].
tion [23]. The positive results observed in CF and Lung transplantation (LTx) is a well-established
non-CF trials of vitamin D supplementation provide treatment option for CF patients with end-stage
a strong rationale for larger, randomized control lung disease. Ongoing weight loss despite aggressive
trials of long-term, high-dose vitamin D3 supple- nutritional supplementation is one of the criteria for
mentation in CF patients [23]. listing for LTx [42]. It is a challenge for dietitians to
CF patients usually have abnormal intestinal optimize energy requirements for improving the
microbiota because of exposure to antibiotics. Pro- nutritional status in pre-LTx patients with CF. Mea-
biotics could modify the gut microbiota. Several surement of REE can be a helpful tool to optimize
reviews examined the use of probiotics in the treat- nutritional intervention as prediction equations
ment of CF pulmonary exacerbation and intestinal seem to underestimate REE in end-stage CF patients
&&
inflammation [25 ,26,28,38]. Unfortunately, they [34]. After LTx, patients with CF are at risk of devel-
lack the scientific quality that is needed for a rec- oping diabetes, and both tacrolimus and systemic
ommendation. Until there is more robust evidence steroids are known to increase the risk of diabetes
supporting the safety of probiotics in clinical care, posttransplant [43].
they should be used with caution with high-risk
patients such as those with acute exacerbations or
&&
those with severe respiratory function [25 ].
Also the evidence for dietary supplementation CONCLUSION
of fatty acids to improve lung function or anti- Nutritional care should be closely adapted to the
inflammatory effects [39] in children or adults with various stages and complications of CF across the
CF is too limited for recommendations in daily lifespan to extend survival and to improve quality of
&&
practice [5 ,21]. life. Moreover, new therapies, such as CFTR modu-
Treatment of CFRD includes education on dia- lators and transplantation techniques, change the
betes self-management, insulin therapy and aerobic needs for nutritional intervention. Recent evidence-
exercise. Although some CF centers use oral medi- based and expert-based guidelines provide up-to-
cations to help control diabetes, the CFF clinical date information for optimal nutritional care of
practice guidelines support the use of insulin ther- the general patient, but for treatment of individual
apy and this remains the most widely used treat- CF patients personalized treatment by a specialized
ment method [24]. The achievement of adequate CF dietitian is recommended.
blood glucose levels would be best to prevent
decline in pulmonary function [27]. Patients need
Acknowledgements
to learn to adjust their insulin dose to the carbohy-
drate content of the meal, sip feeds or ETF. CFRD F.M.H. is a research dietitian, Department of Dietetics,
patients can benefit from being seen periodically by Internal Medicine and Dermatology, University Medical
a specialized team with expertise in both diabetes Center Utrecht, N.M.d.R., PhD, is an assistant professor,
&&
and CF [5 ]. Division of Human Nutrition, Wageningen University,
For CF women planning to become pregnant, a and H.G.M.H., MD, PhD, is a pulmonologist, Head of
daily supplement of 400 mg of folic acid in the Department, Division Heart and Lung, Pulmonology,
preconception period and throughout the first tri- University Medical Center Utrecht, The Netherlands.
mester to prevent neural tube defects is recom-
&&
mended [5 ], similar to non-CF women. A variety Financial support and sponsorship
of complications may occur in pregnant CF women, There is no funding to disclose.
including impaired airway clearance, chronic respi-
ratory tract infections, diabetes mellitus, pancreatic
insufficiency and nutritional deficiencies. Preg- Conflicts of interest
nancy is advised against if the FEV1 is less than There are no conflicts of interest.

560 www.co-pulmonarymedicine.com Volume 23  Number 6  November 2017

 Nutrition and cystic fibrosis Hollander et al.

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