Paeds: CF Flashcards
Main dietetic aims for Cystic Fibrosis (PAEDS):
Main dietetic aims for Cystic Fibrosis (PAEDS):
* Bone health maintenance (at risk of osteoporosis due to digestive issues)
* Optimum vitamin levels
* Support growth & development
* Control malabsorption symptoms
* Normal eating behaviours
* Maintenance of blood sugar in cystic fibrosis related diabetes
Main nutritional concerns in Cystic Fibrosis
Main nutritional concerns in CYSTIC FIBROSIS:
* Malnutrition risk is HIGH (undernutrition)
* Rise of obesity & overweight
* Malabsorption
* No enzymes from pancreas: Pancreatic enzyme replacement
* Salt: Higher requirements
* Fluid: Needed to loosen secretions
What is Cystic Fibrosis?
Cystic Fibrosis is an autosomal recessive inheritance disease caused by genetic mutation which results in a faulty CFTR gene that causes a build-up of thick, sticky mucus on cell surfaces due to impaired chloride ion transportation. The effects of the disease vary for Cystic Fibrosis sufferers, but they often experience issues with their respiratory and digestive systems
What are common nutritional problems in Cystic Fibrosis?
Common nutritional problems in Cystic Fibrosis:
* Malabsorption
* Weight loss
* Undernutrition
* Excess gained weight (older children and adults)
What IS NOT recommended for Cystic Fibrosis sufferers with pancreatic enzyme insufficiency?
It IS NOT recommended for Cystic Firbrosis sufferers with pancreatic enzyme insufficiency to have a fat restriction because this may lead to reduced energy intake in a group of individuals that are already at risk of malnutrition. Fat restriction is only recommended in the minority of patients who are taking PERT but steatorrhoea cannot be controlled.
Infants with CF
Infants with CF:
* May require sodium supplementation: serum-creatinine ratio <17-52: 1-2mmol na/kg needed.
* Good weaning practice needed
* F&v as first foods may help if CREON is (spoon use)
* CREON micro/ Creon 10000 best
* If growth reduces, increase energy
* GORD?: Feed thickeners. H2 antagonists & PPIs
* Lower strength PERT needed
When estimating energy and nutrient requirements for paediatrics, what is advised?
When estimating energy and nutrient requirements for paediatrics it is advised to base this on actual weight. In some cases it is appropriate to estimate requirements based on height age instead of chronological age to provide a more accurate measure of the child’s body size. This may be more achievable for a malnourished child.
Underweight and stunting diagnosis
Underweight: weight for age < -2 SD or BMI <5th percentile, <-2 SD (¼ 2nd percentile) of the median reference value for age and sex.
◦ Stunting: height for age < -2 SD of the mean reference
value for age and sex.
(ESPEN & ESPGHAN)
What are the clinical consequences of Cystic Fibrosis?
Clinical consequences of cystic fibrosis
* Recurrent respiratory infections, inflammation, bronchial damage → respiratory failure (↑ requirements)
* Exocrine pancreatic dysfunction → 84% pancreatic insufficient → malabsorption → malnutrition
* Bowel obstruction ~15% meconium ileus at birth, DIOS in older patients
* Impaired glucose tolerance → CFRDM (19% of adolescents, ~50% of adults)
* Liver disease and portal hypertension,
* ↓ BMD,
* gut motility problems,
* infertility in males,
* CF arthropathy
* behavioural and psychological problems
What are the main nutritional concerns for Cystic Fibrosis?
The main nutritional concerns for CF:
* Malabsorption: Pancreatic Enzyme Insufficiency
* High risk of undernutrition because of issues with absorption
* Weight gain
* Poor blood glucose control
* Osteoporosis risk
Which ethnicities does CF commonly affect?
CF commonly affects people who are White, from the Indian subcontinent and Middle East.
Cystic Fibrosis Historical Data
Cystic Fibrosis Historical Data:
* Most children have a normal birth weight now
* Historical data showed slow prepubertal growth (Mearns 1980)
* Conflictin evidence about Genetic potential:Mearns 1980: similar height achieved to those without CF. Soutter 1986:CF children failed to meet growth potential
* Historical data shows strong links between improved nutritional status (taller, heavier) and survival (N America – low fat vs high fat diets, Corey et al 1998)
* Growth failure and wasting are both independent prognostic indicators in survival – patients with ht percentile <5th at 5 years of age →↑ risk of death (Beker et al 2001)
* Patients with IBW >85% had better prognosis at 5 years of age (Sharma et al 2000)
* Good nutritional status has +ve impact on lung function (Peterson et al 2003)
* A BMI ≤20th centile associated with reduced lung function and a BMI ≥50th centile with better lung function (Stallings et al, 2008)
Cystic Fibrosis Historical Data: Gastrostomy, Jejunostomy, NG/Gastrostomy
Cystic Fibrosis Historical Data: Gastrostomy, Jejunostomy, NG/Gastrostomy
Levy 1985
* Feeding route: Gastrostomy
* Age: 5-22years
* Duration: 1.1years
* % Wt for Ht: Increase
* % Body fat: Increase
* Lung function: 0
Boland 1986
* Feeding route: Jejunostomy
* Age: 6-21years
* Duration: 1.6years
* % Wt for Ht: Increase
* % Body fat: -
* Lung function: 0
Shepherd 1986
* Feeding route: NG/Gastrostomy
* Age: 3-13years
* Duration: 1.0years
* % Wt for Ht: Increase
* % Body fat: -
* Lung function: Increase
Limitations: none of the studies were RCTs.
Recent data are also not RCTs.
What causes issues with meeting energy requirements in CF?
The following causes issues with meeting energy requirements in CF:
* Increased requirements
* Increased losses
* Malabsorption
For wt gain or maintenance: intake needs to > than requirements.
Why does weight loss in Cystic FIbrosis matter?
Weight loss in Cystic Fibrosis matters because:
* Respiratory muscle strength is reduced
* Lungs are weakened> increased infections
* Infection risk is increased
* Poor growth
* Poor body image
* Deriorating lungs leads to reduced appetite, more medication and reduced survival
*
Reasons for poor intake in CYSTIC FIBROSIS
Reasons for poor intake in CYSTIC FIBROSIS
* Iatrogenic fat restriction
* Poor appetite: Chest infections > anorexia, nausea, vomiting
* Abdo distension/pain, flatulence, constipation, diarrhoea
* Psychological: Peer group pressure
* Impact of professional/medical interventions
* Depression
* Feeding disorders
* Other: Timing difficulties, poor parenting
* If PEI: symptoms relates to malabsorption: bloating, abdo pain
Reasons for INCREASED Energy needs in CYSTIC FIBROSIS
Reasons for INCREASED Energy needs in CYSTIC FIBROSIS:
* Malabsorption: Pancreatic Insufficiency: reduced enzymes & bicarbonate, increased losses
* Altered bile salts
* Excess/abnormal mucus
* Raised REE: FEV1, Salbutamol use, Treating infections, Fever
Energy requirements in CF
Energy requirements in CF (DRVs)
* EAR + 20-50%
* Poor wt gain: actual kcal intake + 20-30%
Infants 0-3months
* Normal: 100-115kcal/kg
* 120%: 120-140kcal/kg
* 150%: 150-170kcal/kg
7-10 Y Males
* Normal: 1970 kcal/day
* 120%: ~ 2400kcal/day
* 150%: ~3000kcal/day
7-10Y Females
* Normal: 1740kcal/day
* 120%: ~ 2100 kcal/day
* 150%: ~2600kcal/day
CF population & median death age
Approximately 60% of the CF population are now adults. The median death age is 31 years and it is expected to increase.
The genotype of CF causation affects ?
The genotype of CF causation affects severity
Which classes of CF are likely to be severe or atypical?
Classes of CF & severity
Classic-Severe: I, II, III
Atypical likely: IV, V
How is CF diagnosed?
CF diagnosis:
* Heel prick test: 5th day
* Sweat test & identification of CF mutation: </= 4 weeks
Why are CF sufferers at risk of chronic respiratory diseases?
CF sufferers at risk of chronic respiratory diseases because:
* abnormal secretions
* chest infections
* inflammation
Chest treatment for CF sufferers
Chest treatment for CF sufferers: aim to reduce infections & lung damage
* Chest physio
* Antibiotics
* Bronchodilators
* Active lifestyle/exercise
* Separation from other CF sufferers
Factors associated with poor nutrition in CF
Factors associated with poor nutrition in CF:
* Losses via stool: PEI
* Untreated PEI: fat malabsorption, reduced fat soluble vits, pro malabsorption, CHO malabsorption
* Reduced bicarb secretion
* Reduced bile acid
* Reduced gastric/intestinal motility
* Infections/inflammation> reduced lung function: increases energy requirements
* Severity of disease influences requirements
* Infection: anorexia, sputum causes: nausea, abdo pain, v, GORD, disordered eating behaviour
* Co-morbidities e.g. CF related diabetes: polyuria losses
* Timing difficulties of PERT
* Poor parenting
Poor Intake and CF (lecture slides)
Poor Intake and CF (lecture slides)
* Iatrogenic fat restriction
* Poor appetite: Chest infections > anorexia, nausea, vomiting
* Abdo distension/pain, flatulence, constipation, diarrhoea
* Psychological: Peer group pressure
* Impact of professional/medical interventions
* Depression
* Feeding disorders
Other: Timing difficulties, poor parenting
Nutritional requirements in CF
Nutritional requirements in CF:
Energy: EAR + 20-30%
Poor wt gain: Actual E intake + 20-30%
Protein: 120% of EAR
Fat soluble vitamins:
* A 1500-10000 IU/day (age dependent)
* D 400-5000 IU, up to 10000 in adolescents
* E 40-500 IU (age dependent)
* K 300ug/kg/d for infants, 5mg for 2-7 yrs, 5-10mg >7 yrs
* Sodium: Infants, hot weather, exercise (1-7 yrs 1g Na/day, >7 yrs 2-4g/day)
* Essential fatty acids: ? Not enough evidence to advocate routine supplementation
What is important to note about the use of BMI & wt?
BMI & wt should not be used in isolation.
They do not distinguish between fat and fat free mass. Anthropometric measures of body composition are important to avoid reliance on BMI (ESPEN & ESPGHAN)
What do ESPEN & ESPGHAN advise for infants that are breast fed?
ESPEN & ESPGHAN advice for infants that are breast fed:
* fortify breast milk or supplement with formula milk
Critical analysis for CF
Critical analysis for CF:
- ESPEN/ESPGHAN: suggestion of EAR as cannot make evidence-based recommendations specifically for CF sufferers.
- Use of EAR: not individualised enough, use of indirect calorimetry would give more accurate indication of energy needs. However not readily available in practice.
- BMI/weight: not to be used in isolation as does not distinguish between fat and fat free mass. May mask stunting.
- BIA (validated equation): more appropriate than BMI, more accessible less expensive than DEXA. A validated equation for FFM has been found for paediatric CF sufferers: French study, 54 patients, 5-21y (no toddlers), age + sex not included in final model (not significant), ? comparable to DEXA, age + sex have an effect on growth pattern, there are variations in body composition between sexes. Repeat: greater sample size.
- Circumference measures + BMI: improve estimation of nutritional status (ESPEN & ESPGHAN)
- Levy, Boland, Shepherd ENT studies: highlight improvement %Wt for Ht in patients >3y (what about<3y), Shepherd: ↑ lung function. But none were RCTs.
- 2000-2019 (US study): relative decrease in underweight status by ∼40%, simultaneously with a > 300% increase in overweight status, and >400% increase in obesity.
Key points for CF
Key points for CF:
- Aim for normal growth and development
- >50th centile to support optimal lung function (ESPEN & ESPGHAN)
- Caused by faulty CFTR gene that regulates chloride ion transport> results in thick sticky mucus
- Underweight historically prevalent, overweight/obesity on rise
- Inadequate intake to meet requirements is common: high losses & symptoms
- Energy: EAR +20-30%, Protein 120% of RNI.
- Treatment must be individualised
- Establishing healthy eating behaviours/environment important
- Pancreatic function should be monitored in ALL: damage occurs progressively
- Adolescences more likely to have disordered eating/not comply
- Risk of osteoporosis/ low BMD
- A: Don’t just rely on weight & BMI
- B: FE1, Fat soluble vitamin status
- C: Severity of CF, Signs of malabsorption (PEI is common), infections, antibiotics, other conditions: Crohn’s, Coeliac disease
- D: Nutritional requirements & intake, intake must be greater than requirements, Ca,
- E: Parental attitudes to feeding/condition
- F: Cultural/religious beliefs: PERT contains PORK
Cystic Fibrosis Interventions
Cystic Fibrosis Interventions: should be healthy and not promote weight gain
- PEI: PERT
- Inadequate intake: energy dense foods, increased snacks,
- Poor wt gain: unsaturated fat
- Poor growth: fortification (breast milk fortification preferred in breast fed), little and often, ONS
- Not reaching growth goal (despite interventions): Tube feeding
- Na supplementation: infants, exercise, hot weather
- Feeding behaviour: limit distractions, encourage messy play, ensure weaning is done within advised timeframe and includes variety of foods.
- Establish healthy weaning
