Table 142Summary of included studies

StudyIntervention/ComparisonPopulationOutcomesComments
Cochrane systematic reviews
Chinuck 2014
Cochrane SR
Appetite stimulants versus placebo (Eubanks 2002, Homnick 2004, Marchand 2000)Adults and children with CF
  • Change in weight (kg)
  • Change in weight z score
  • Change in FEV1 (% predicted)
  • Number of pulmonary exacerbations
  • Number of adverse effects
Goldbeck 2014
Cochrane SR
Comparison 1. Behavioural intervention versus usual care (Stark 1996)
Comparison 2. Behavioural management training plus educational intervention versus educational intervention alone (Powers 2003, Stark 2009)
Children or adults with CF
  • Change in weight (kg)
  • Change in weight z score
  • Change in BMI z score
  • Change in % ideal body weight
  • Change in weight % for age
  • Change in height (cm)
  • Change in height z score
  • Change in FEV1 % predicted
Morton 2015
Cochrane SR
Supplemental enteral tube feeding for 1 month or longer versus no specific intervention (No studies were included)People with CF of any ageNo studies were identified for inclusion in this review
Savage 2014
Cochrane SR
Nutrition education versus usual care (Watson 2008)Individuals of all ages with CF or family members or both
  • Change in weight
  • Change in FEV1 % predicted
Smyth 2014
Cochrane SR
Comparison 1. Oral calorie supplementation versus usual care (Hanning 1993, Poustie 2006)
Comparison 2. Oral calorie supplementation versus additional nutritional advice (Kalnins 2005)
People with CF
  • Change in weight (kg)
  • Change in weight centile (percentile points)
  • Change in weight z score
  • Change in BMI (kg/m2)
  • Change in BMI centile (percentile points)
  • Change in weight for height (percentage)
  • Change in height (cm)
  • Change in height centile (percentile points)
  • Change in height z score
  • Change in FEV1 (% predicted)
Primary studies included in SRs
Eubanks 2002
USA
RCT
Intervention: Appetite stimulant
  • Megestrol acetate 10 mg/kg/day (adjusted at subsequent visits)
Control: Placebo
N=17 participants (intervention: n=10; placebo: n=7)
  • Age: > 6 years
  • Inclusion criteria: pancreatic insufficiency, FEV1>40% growth failure defined as no weight gain in the preceding 6 months
  • Change in weight (kg)
  • Change in weight z score
  • Change in FEV1%
  • Number of pulmonary exacerbations
  • Number of adverse events:
    • Constipation
    • Decreased morning cortisol levels
Included in Chinuck 2014 SR
Duration: 6 months. After completion of the 6-month trial, the placebo group was offered MA for a further 6 months.
Population was considered as direct.
Hanning 1993
Canada
RCT
Intervention: oral calorie supplements Dietary supplements, drink powders, milk shakes, tinned puddings to achieve 25% of normal energy recommendations in addition to normal diet for 6 months
Control: usual care
N=20 children and young people with CF (20 randomised, 16 studied)
  • Age: 7 to 15 years
  • Inclusion criteria: not reported
  • Change in weight (kg)
  • Change in weight a s% expected for age and height
  • Change in height as % of expected for age
  • Change in FEV1 % predicted
Included in Smyth 2014 SR
Follow-up: 6 months
Population was considered as indirect.
Homnick 2004
USA
RCT
Intervention: Appetite stimulant Cyproheptadine hydrochloride 4mg 4 × daily
Control: Placebo
N=18 people with CF enrolled, 16 completed study (intervention: n=8; placebo: n=8)
  • Age: ≥5 years
  • Inclusion criteria: Ideal body weight for height <100%
  • Change in weight z score
  • Change in height (cm)
  • Change in BMI (kg/m2)
  • Change in BMI percentile
  • Change in % ideal body weight
Included in Chinuck 2014 SR
Follow-up: 3 months
Population was considered as indirect.
Kalnins 2005
Canada
Quasi-randomised controlled trial
Intervention: Oral calorie supplementation
  • High calorie drink to increase energy intake by 20% of predicted energy needs
Control: Nutritional counselling
  • Nutritional counselling to increase energy intake by 20% of predicted energy needs by eating high calorie foods.
  • N=15 participants with CF were enrolled but 2 dropped out
  • Participants were aged >10 years.
  • Mean (SD) age on entry to trial: advice group: 16.4 years (6.7); supplement group: 19.5 years (11.3).
  • < 90% ideal WFH or 5% reduction in ideal WFH over 3 months
  • Change in weight (kg)
  • Change in weight z score
  • Change in weight for height (%)
  • Change in % ideal body weight
  • Change in height (cm)
  • Change in height z score
  • Change in FEV1 (% predicted)
Included in Smyth 2014 SR
Interventions implemented for 3 months, follow-up: 3 and 6 months
Population was considered as direct.
Marchand 2000
USA
RCT
Intervention: Appetite stimulant
  • Megasterol acetate 10 mg/kg/day for 12 weeks
Control: Placebo
N=12 children with CF
  • Age: mean age 7.4 years. Range: 21 months to 10.4 years
  • Inclusion criteria were loss of weight or plateau in weight gain for more than 3 months, weight-for-height less than 85%, and a negative change in weight z score
  • Change in weight z score
  • Number of pulmonary exacerbations
  • Fasting blood glucose levels
  • Decreased morning cortisol levels
Included in Chinuck 2014 SR
Clinical assessment at week 0, 6, 12, 24 and 36
Population was considered as direct.
Poustie 2006
UK
RCT
Intervention 1: Oral calorie supplements
Intervention 2: Routine dietary advice (usual care)
N=102 children and young people aged 2 - 15 years with CF
  • Children with at least 1 of following criteria: BMI <25th centile but > 0.4th centile; or no increase in weight over the previous 3 months; or 5% decrease in weight from baseline over a period of < 6 months
  • Change in weight (kg)
  • Change in weight centile (percentile points)
  • Change in BMI (kg/m2)
  • Change in BMI centile (percentile points)
  • Change in height (cm)
  • Change in height centile (percentile points)
  • Change in FEV1 % predicted
Included in Smyth 2014 SR
Interventions implemented for 12 months.
Outcomes at 3, 6 and 12 months
Population was considered as direct.
Powers 2003
USA
RCT
Intervention 1: Behavioural management training plus educational intervention
  • Nutrition intervention with strategies for enhancing calorie intake
  • Behavioural management training for parents designed to encourage children to eat food consistent with CF dietary recommendations.
Intervention 2: Educational intervention only
N=12 infants and children with CF
  • (intervention 1: n=7, intervention 2: n=5).
  • Age: Less than 3 years old.
  • Pancreatic insufficiency.
  • Change in weight (kg)
  • Change in % ideal body weight
  • Change in weight % for age
  • Change in height (cm)
Included in Goldbeck 2014 SR
Both groups received 8 sessions (45 to 60 minutes) over 1 year: Sessions 1 to 4 (3 months) intensive education
Follow-up: 1 years
Population was considered as direct.
Stark 1996
USA
RCT
Intervention: Group behavioural intervention.
  • 7 weekly sessions - baseline assessment plus snack, breakfast, relaxation skills training, lunch, dinner and maintenance strategies targeted over following 7 sessions.
Control: Usual care (Wait list control)
  • Parent meeting and 7-day food diaries at times corresponding to baseline and last week of intervention
N=10 children with CF.
  • 1 withdrew from control group after randomisation.
  • Total sample n = 9 (intervention group: n=5, control group: n=4).
  • Age range: 5.3 years to 10.1 years; mean (SD) age: 7.3 years (1.7).
  • Change in weight (kg)
  • Change in weight (z score)
  • Change in height (cm)
  • Change in FEV1 % predicted
Included in Goldbeck 2014 SR
Duration of interventions: 6 weeks
Population was considered as indirect.
Stark 2009
USA
RCT
Intervention 1: Behavioural intervention Intervention 2: Nutrition education
  • Nutrition education in group setting
N=79 children and young people with CF
  • Number randomised, n = 79
  • Received the intervention, n=67 (behavioural intervention plus nutrition education: n = 33, nutrition education: n = 34)
  • Age: 4 to 12 years
  • With pancreatic insufficiency; and weight for age and height ≤ 40th percentile.
  • Change in weight (kg)
  • Change in BMI z score change
  • Change in height (cm)
  • Change in height z score
  • FEV1 change
  • Parent satisfaction (reported narratively)
Included in Goldbeck 2014 SR
Duration of interventions: 9 weeks. Timing of sessions: 7 sessions (each 90 minutes): pretreatment (session 1), 2 weeks later 5 weekly groups sessions (sessions 2 to 6), 2 weeks later post-treatment (session 7; follow up)
Follow-up: up to 2 years
Population was considered as direct.
Watson 2008
UK
RCT
Intervention: Nutrition education
  • General and disease-specific nutrition education (’Eat Well with CF’)
  • Content: knowledge on general and disease-specific nutrition topics; self-management skills on goal setting in small incremental steps to establish new behaviours
  • Mode of delivery: written material; supplementary workshops (introductory, weeks 5 and 10) and weekly telephone calls delivered by a dietitian
Control: Usual care
N=74 people with CF older than 16 years of age
  • participants were enrolled and stratified by disease severity into low or high risk disease.
  • Participants were randomly allocated into intervention (n = 37) and control (n = 37) group
  • 48 participants completed the study through to 12-month follow-up assessment (23 in intervention group, 25 in control group)
  • Age: intervention group 26.4 (17.2–43.2) years; control group 24.2 (16.9–38.1) years
  • Change in FEV1 (% predicted)
  • Change in weight
  • Quality of life (narrative reporting and p values only)
Included in Savage 2014 SR
Duration of the intervention: 10 weeks. Outcomes measured at 6 and 12 months
Population was considered as indirect.
Additional primary studies
Bradley 2012
United States
Cohort study
Intervention: Gastrostomy
Control: No gastrostomy (usual care)
N=40 people with CF (20 in the intervention group, 20 in the control group)
  • Age range: 2 to 20 years
  • Each child in the intervention group was pair-matched on age, sex, pancreatic status, BMI and lung function with a children from the control group
  • Children who had a gastrostomy for reasons other than nutritional supplementation were excluded
  • Change in weight z-score
  • Change in BMI z-score
  • Change in height z-score
  • Change in FEV1 % predicted
Outcomes measured at 6 and 12 months
Population was considered as direct.
Powers 2015
USA
RCT
Behavioural intervention
  • Individualized nutritional counselling targeting increased energy intake and behavioural child management skills
Control: education and attention control treatment
  • Education on general nutrition information and other topics (e.g. infection control and bicycle safety)
N=78 children with CF and pancreatic insufficiency (intervention: n=36, control: n=42)
  • Age: 2 to 6 years
  • Confirmed pancreatic insufficiency; no restrictions in consuming a high-fat diet.
  • Change in weight z score
  • Change in height z score
  • Number of adverse events (digestive system)
Both treatments were delivered in person or telehealth (via telephone)
Sessions occurred weekly for 8 weeks then monthly for 4 months (6 months).
The control arm served as a behavioural placebo controlling for attention and contact frequency.
Participants then returned to standard care for 1 year.
Follow-up: 18 months
Population was considered as direct.
White 2013
UK
Cohort study
Intervention: Enteral tube feeding
  • Supplemental enteral tube feeding administered over 3 years
  • Overnight enteral tube feed
Control: Usual care
  • N=21 adults with CF (intervention: n=15, control: n=6)
  • Mean (SD) age: intervention: 21.8 (3.6); control: 23.0 (5.7)
  • All study participants fulfilled the criteria for commencement of enteral tube feeding (CF Trust, 2002): BMI<19 kg/m2 and/or 5% acute weight loss over a 2 month period with a failure or oral nutritional supplements to adequately improve nutritional status.
  • Change in weight (kg)
  • Change in BMI (kg/m2)
  • Change in FEV1 (%)
  • Change in IV treatment days
Initially n=17 accepted enteral tube feeding. However 2 people died during the baseline year and subsequent analyses were conducted on the surviving participants.
Control group: people who declined enteral tube feeding
Follow-up: 1 year
Population was considered as direct.

BMI: body mass index; CF: cystic fibrosis; FEV 1: forced expiratory volume in 1 second; RCT: randomised controlled trial; SR: systematic review; WFH: weight for height

From: 10, Other monitoring, assessment and management

Cover of Cystic Fibrosis
Cystic Fibrosis: Diagnosis and management.
NICE Guideline, No. 78.
National Guideline Alliance (UK).
Copyright © NICE 2017.

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