Advances in CF Nutrition Past, Present and Future

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Advances in CF NutritionPast, Present and Future

• Susannah King
• BSc (Hons), M Nut Diet, MPH, APD
• Dietitian, Alfred Hospital
• PhD candidate, Monash University

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Nutrition in CF

• Integral component of overall care
• Influences function and survival
• Encompasses a complex range of issues

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Key focus of adequate nutrition

• Adequate intake of energy to meet bodys needs
• Energy kilojoules calories
• Dietary energy fats, protein, carbohydrates
• Energy fuel for
• Bodys functions
• Growth
• Fighting illness and infection
• Storage (fat)

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Nutritional imbalance in CF
Energy Expenditure (lungs)
Energy Intake (diet)
Energy Losses (poor digestion)

• Energy imbalance
• Growth failure in children
• Underweight / weight loss in adults

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Common goals

• Optimise growth and development
• Optimise nutrition into adulthood
• Adequate body tissue stores for function

High energy diets ? higher intakes of fat,
protein, carbohydrates
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Scope of nutrition issues in CF
Nutritional supplementation
High energy intake
Enzymes
Infant feeding
Enteral feeding
Nutritional adequacy
Vitamin supplementation
Growth monitoring
Reflux
Bone health
Shopping and cooking
Behavioural issues
Diabetes
Transplant preparation
Liver disease
Pregnancy
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Role of the dietitian
Nutrition science
Individual food and supplements plan
Dietitian
Individual health status needs
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Historical treatment of CF

• Malabsorption 20 pancreatic insufficiency treated
  with low fat diet
• Inadequate energy intake
• Endemic malnutrition
• Poor survival
• Median survival 16 years in 1970

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Pancreatic enzymes
Enzymes for foods containing fat /- protein
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Impact of an unrestricted fat diet on nutrition
and survival in CF
Corey M et al, J Clin Epidemiol, 1988, 41583-591
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Progress in Australian CF adults

• Nutritional status of CF adults attending Alfred
• 1983 vs 1997
• Change from low fat to high fat diet
• 1997 Significant improvements in nutritional
  status
• Energy intakes 20 higher
• Males 9cm taller, 12 kg heavier
• Females 5cm taller, 12 kg heavier
• Mean heights close to Australian average

Richardson et al, Nutrition, 2000, 16 255-259
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Modern CF Nutritional care

• Endemic malnutrition prevented with
• High energy, high fat diets
• Pancreatic enzyme replacement therapy
• Specialised centre-based care
• Interventions to treat malnutrition
• Scope of nutritional care
• Diabetes
• Gastrointestinal issues
• Bone health

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Nutrition advances throughAdvocacy and research

• Home enteral nutrition funding
• Standards of care for CF
• Best practice guidelines
• Consumer consultation
• Clinical research

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Best practice guidelines for nutrition management
in CF

• International consensus documents 2002
• Australasian Guidelines for Nutrition in CF
• Development began 2003
• CF dietitians in Australia and New Zealand
• Stakeholder involvement
• Comprehensive document
• Nearing finalisation
• Aiming for endorsement from DAA

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CF nutrition guidelines
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Current state of nutrition in CF

• Improvements in nutritional status over time
• US data for children and adolescents 1990 vs
  2004

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Current state of nutrition in CF

• Improvements in nutritional status over time
• Mean BMI of adult patients attending Alfred
  Hospital

Source Alfred CF database
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Despite improvements, malnutrition is still
prevalent

• At risk, or malnourished
• 17 children/adolescents
• 29 adults

Source Australian CF Data Registry 2002
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Nutrition ? Pulmonary disease

• Nature of CF recurrent chest infections
  progressive lung disease
• Fluctuations in nutritional status
• Potential for progressive deterioration if
  nutritional status not restored
• Body composition changes may occur

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Investigating the causes of malnutrition

• Are weight and BMI the most appropriate markers
  of nutritional status?
• How does body composition assist us with
  understanding malnutrition and its causes?

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Traditional measures of nutrition

• Height
• Weight
• Weight percentiles
• Body mass index (BMI) Weight/Height2
• BMI percentiles

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Body composition
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Role of body composition measurement in chronic
disease

• Fat-free mass
• Strength skeletal and muscle
• Cell / organ structure function
• Immune function
• Depleted FFM ? mortality in COPD
• Schols et al, AJCN 2005

• Fat mass
• Energy store

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Why body composition is important

• Weight and BMI do not distinguish
• Fatness from Muscle bulk
• Different types of malnutrition
• Weight and BMI of limited value in
• Assessing nutritional depletion
• Evaluating response to nutritional supplementation

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Malnutrition remains common

• Body mass index in adults Fat-free mass

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Longitudinal follow-up of 58 adults with CF
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So where are we today?

• Recognition of the importance of adequate
  nutrition in optimising outcomes for CF
• Recognition that normal nutrition is achievable
• Resources for achieving normal nutrition
• skilled dietitians
• access to services and provisions
• team care
• Challenges ahead.

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New insights into the role of inflammation in
nutrition

• How does inflammation influence
• development of malnutrition?
• response to nutritional interventions?
• Can anti-inflammatory therapies modulate
  nutritional status?
• Can diet influence the inflammatory environment?
• Omega 3 fatty acids

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What about overnutrition?

• Overweight 3 children, 13 of adults
• Very few obese
• Known cases of morbidly obese CF adults in USA
• Importance of individualisation of care
• e.g. high fat diet may not be necessary for all
  CF individuals to achieve normal nutritional
  status
• Monitoring for complications with increasing age

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Dietary issues on the horizon

• Omega 3 fatty acids
• Anti-oxidants
• Probiotics
• More evidence needed for CF-specific
  recommendations
• Encouragement of varied food intake within a
  diet adequate in energy for individual needs

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The key importance of EARLY nutritional status
and intervention

• Studies in general population
• Programming of body composition
• Delay in adequate dietary intake early in life
• Affects adult nutritional status

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The key importance of EARLY nutritional status
and intervention

• Evidence for programming of body composition in
  CF
• Wisconsin screening project, USA
• If catch-up growth not achieved by 2 years of
  age gt children were unlikely to catch up in the
  next 8 years
• Ratifies importance of early diagnosis
• treatment of maldigestion
• adequate dietary intake absorbed

Schoff et al, Pediatr Pulmonol, Suppl 28, 347,
2005
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How can we best detect nutritional abnormalities?

• Adequate dietetic staffing in CF centres
• Routine surveillance of nutritional status
• Use of sensitive assessment methods
• Guidelines and algorithms
• Australasian Clinical Practice Guidelines for
  Nutrition in CF

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When and how should we intervene?

• Early
• With defined objectives
• With adequate supports
• With close monitoring of outcomes

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Acknowledgements

• CF adults
• Supervisors
• A/Prof John Wilson
• Ibolya Nyulasi
• Co-investigators
• Dr Tom Kotsimbos
• A/Prof Duncan Topliss
• Michael Bailey
• Alfred Radiology
• Dianne Payne
• Monash University
• Medicine Tiffany Bamford
• Immunology Judith Field

• CF Service
• Dr Brenda Button
• Drs Robert Stirling Alan Young
• Felicity Finlayson
• Libby Francis
• Nutrition Department
• Colleen Ash
• Indi Swan
• Royal Melbourne Hospital
• A/Prof Peter Ebeling
• Funding
• NHMRC Dora Lush Scholarship
• Australian CF Research Trust
• Monash University Grants
• The CASS Foundation
• Cystic Fibrosis Victoria