1. Infant Feeding: Getting it Right From the Start
Julie Matel, MS, RD, CDE

2. Nutrition for Infants with CF
Does early growth matter?
Do I need a high calorie formula?
Should I drink breast milk or formula?
When can I eat real food?

3. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108: Stallings VA, et al.

4. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. Elizabeth H. Yen, MD; Hebe Quinton, MS; Drucy Borowitz, MD Aim: To evaluate the impact of nutritional status early in life on the timing and velocity of height growth, lung function, survival and CF complications through age 18
At last year’s NACFC, Dr. Elizabeth Yen, from Boston Children’s Hospital provided further evidence that the earliest years of nutritional status lay the foundation for future growth and optimal pulmonary health.
[Click]
The study used CF registry data to evaluate the impact of nutritional status early on in life as it related to height growth, lung function and survival and complications through the age of 18.
© Children’s Hospital of Wisconsin. All rights reserved.

5. Conclusions Greater weight at age four is associated with
greater height
better pulmonary function
fewer complications of CF
Greater weight for age in the peripubertal period is associated with improved growth
Greater weight at age four is associated with better survival through age 18

6. How do we achieve optimal growth during the first year of life?

7. Breastmilk vs Formula

8. Breastmilk vs Formula
Breast feeding has long been recommended as a first feeding for infants with Cystic Fibrosis
1992 Consensus statement promoted breast feeding
Current CF Nutrition Consensus continues to recommend breast milk

9. Breast Feeding Advantages
Lower rates of ear infections, colds, GE
Bonding between mom and baby
Maternal advantages
Saves money
Convenient
Less post-partum depression
Antimicrobial components of breast milk protect against respiratory infections in CF

10. Breast Feeding Disadvantages?
In High risk* infants with CF, breast milk may be inadequate in…
Calories
Protein
Essential fatty acids
Sodium
* High risk: Meconium ileus, pancreatic insufficient

11. Growth and Pulmonary Outcomes During the First 2 years of Life of Breast Fed and Formula Fed Infants with CF Through the Wisconsin Routine Newborn Screening Program
103 Wisconsin infants
born
diagnosed through newborn screening
53 infants were breast fed (BF) and 50 infants received only formula (FM)in the first year of life
Infants were classified into 3 categories
Diagnosed with meconium ileus [24 babies]
Pancreatic insufficiency [70 babies]
Pancreatic sufficient [9 babies]

12. Study Design Retrospective medical record review Data included:
Type and length of feedings
Introduction of solids and cow milk
Growth data
Respiratory cultures and infections; CXR
Genotype; age at diagnosis

13. Study Conclusions
Pancreatic insufficient infants who were exclusively BF < 2 mo:
achieved adequate weight gain
had fewer pseudomonas infection during the first 2 years than infants who were exclusively formula fed
Exclusively BF ≥ 2 mo:
associated with reduced weight gain
without an additional respiratory benefit

14. FIRST (Feeding Infants Right from the Start)
Prospective study to investigate the potential benefits and risks of breastfeeding in infants with CF
160 CF infants to be enrolled over 4 years ( )
5 sites:
Madison and Milwaukee, WI, Indianapolis, Boston, and Salt Lake City

15. Study design Follow up for 2 years Primary outcomes
Weight gain and Pa infections
Secondary outcomes
Nutritional: Length, head circumference, essential fatty acid and vitamin levels
Pulmonary: Inflammatory markers, other respiratory pathogens, chest x-ray scores, respiratory symptoms

16. Preliminary analysis of the FIRST Study
Aim is to identify optimal feeding for infant with Cystic Fibrosis
Focused on 82 infants
pancreatic insufficient, without meconium ileus
Evaluated feeding patterns for 6 months

17. Results: Feeding Patterns (82 infants)
Most infants were exclusively breast fed at 1 month
Most Infants were exclusively Formula Fed at 6 months

18. Results: Caloric Density of Feeds
More than half of infants received fortified breast milk or higher calorie formula by six months

19. Results: Growth patterns
In the first 2 months
all feeding groups experienced a decrease in weight for length percentiles
At 6 months
only the ex formula fed and (Breast + formula fed) groups had an improvement in their weight and weight for length percentiles (achieved the %ile)
Weight and weight for length percentiles remained low in the ex breast fed group
Exclusively breast feeding for 6 months was associated with lower weight gain at months compared to exclusively formula fed and (Breast + formula fed) groups

20. Essential Fatty Acid Content of Breast Milk and of Diets in Mothers of Infants with CF
Breast milk and diet content of 12 mothers with infants enrolled in the FIRST study (39 samples of breast milk were analyzed)
71% of breast milk samples would not be able to provide enough linoleic acid for infants with CF
57% of mothers’ linoleic acid intake was below the AI (adequate intake) for lactating women

21. Conclusions
Prolonged exclusive breast feeding was associated with lower weight percentiles and lower weight gain at age 4-6 months, compared to formula or mixed feedings (breast feeding plus formula)
Most breast milk samples appear to contain insufficient linoleic acid for CF infants

22. Starting Solid Food Feedings

23. Introduction of Solids
Infants with CF need complimentary foods between 4-6 months of age
Infants with CF have an advantage of practicing with enzymes for several months and are often “ready” to add solids to their feeding routine by this time.
Infants with CF need extra calories that can be included with solid food feedings
Added fats; carbohydrates; modulars
Selection of calorically dense foods (purees and finger foods)
More liberal inclusion of nutrient dense, high calorie solids compared to healthy infants

24. Introduction of Solids
Start with one meal per day (choose a time when infant is happiest)
Start with high calorie options such as…
Pureed meats
Infant cereals mixed with fortified breast milk or formula
Pureed vegetables with 1/4 to 1/2 tsp of oil, butter, or formula
Pureed avocado
Egg yolk
Full fat infant yogurt
Offer one new food at a time and wait a few days before
offering another

25. Don’t forget the salt!
Low sodium status in cystic fibrosis—as assessed by calculating fractional Na+ excretion—is associated with decreased growth parameters (Knepper et al; 2015)
Found lower weight, height, and BMI z-scores in sodium depleted children with CF compared to healthy controls

26. Summary
Track growth rates closely and work with family to develop a plan of action if infant is not meeting goals
Intervene early with high calorie supplementation to either breast milk or formula
Support family’s choice in type of feeding CF
Returning to work
Financial considerations
Fortify solids to provide adequate calories
Supplement with recommended salt

27. Thank you!

28. References
Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155:S73-S93.
Jadin SA, Wu GS, Zhang Z et al. Growth and pulmonary outcomes during the first 2 y of life of breastfed and formula-fed infants diagnosed with cystic fibrosis through the Wisconsin Routine Newborn Screening Program. Am J Clin Nutr May;93(5):
Lai HJ, Zhang Z. Exclusive breastfeeding and growth in young infants with CF. Pediatric Pulmonology Suppl
Ramsey BW, Farrell PM, Pencharz P. Nutritional assessment and management in cystic fibrosis: a consensus report. The Consensus Committee. Am J Clin Nutr Jan;55(1):
Stallings VA, Stark LJ et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc , May; 108 (5)
Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr Mar;162 (3):