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

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?

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;108:832-839. Stallings VA, et al.

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.

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

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

Breastmilk vs Formula

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

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

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

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 1994-2006 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]

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

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

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 (2012-15) 5 sites: Madison and Milwaukee, WI, Indianapolis, Boston, and Salt Lake City

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

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

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

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

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 55-60 %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 4-6 months compared to exclusively formula fed and (Breast + formula fed) groups

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

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

Starting Solid Food Feedings

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

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

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

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

Thank you!

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. 2011 May;93(5):1038-47. Lai HJ, Zhang Z. Exclusive breastfeeding and growth in young infants with CF. Pediatric Pulmonology Suppl. 45. 2016. Ramsey BW, Farrell PM, Pencharz P. Nutritional assessment and management in cystic fibrosis: a consensus report. The Consensus Committee. Am J Clin Nutr. 1992 Jan;55(1):108-16. 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. 2008, May; 108 (5) 832-9. 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. 2013 Mar;162 (3):530-535.