CYSTIC FIBROSIS AND NUTRITION IN THE FIRST YEAR

The Basics

Nutrition and CF

            Normal growth and development are important goals for your child throughout his or her childhood. Excellent growth and good nutritional habits from the very beginning are extremely beneficial in CF. As you will learn, cystic fibrosis can affect nutrition in many ways. The recommended diet is high in calories and protein with liberal amounts of fat and salt.  This “Dream Diet” is contrary to the health recommendations for the general public!  Your CF clinic will incorporate growth and nutrition monitoring at every visit, and nutritionists are available at many clinics for each visit.  

Formula or Breast Milk?

Breast milk is excellent nutrition for babies with CF and we encourage families who wish to breastfeed.  Commercial infant formula can also support good growth and nutrition.  Whether your baby is breastfed or bottle fed, the CF team will ask about his or her feeding pattern, stool habits, and sleep and nap schedules as part of our overall nutrition assessment.  

Pancreatic Status:  Pancreatic Insufficiency versus Pancreatic Sufficiency

            By far, most individuals with CF are “pancreatic insufficient”.  This means that the digestive enzymes that are made by the pancreas are not able to help with digestion.  If untreated, this would result in malnutrition.  Fortunately, these individuals can take digestive enzymes in the form of a medication that is commonly referred to as simply “enzymes”. 

            The stool elastase test can tell us if your child is pancreatic insufficient or pancreatic sufficient.  This requires a quarter-sized sample of stool; results are known in about 7-10 days. The test may be repeated later in infancy if the first test was borderline or normal, because pancreatic function can change over time.  

Pancreatic Enzyme Replacement Therapy, also known as “enzymes”

            What are enzymes?  There are several brands of prescription enzymes used to treat pancreatic insufficiency (Creon^®, Zenpep^®, and Pancreaze^®).  Never accept a generic substitution for name brand enzymes because they don’t work as well. Enzymes come as capsules and are available in different strengths.  The dose depends on many factors and will be determined by your care team.  Inside the capsules are the digestive enzymes in the form of tiny beads.  Each bead is coated to protect the enzymes from being destroyed by the strong acid in the stomach.  The beads are designed to release their activity in the small intestine where digestion and absorption occur. 

            How do they work?  When the enzymes are in contact with formula, breast milk or food in the body, they start to break down the protein and fat that were eaten.  Enzymes should be given at the start of each formula feeding or breastfeeding session, so they can travel with the food as it passes through the intestine.  For this reason, enzymes are given every time your baby eats.  The only exception is a meal or snack that does not contain protein or fat (for example, a jar of baby fruit by itself).  Because infant formula and breast milk contain a lot of fat, your baby will need to have enzymes with it in order to benefit from it.  

Giving enzymes to a newborn baby:  Open the capsule and pour the beads onto a small amount of an acidic food, such as baby applesauce, and immediately feed it to your baby on a spoon, at the start of every bottle feeding or nursing session.  Even newborns can safely take enzymes this way. Your CF team will provide you with additional guidance and instruction.             

Additional tips for using enzymes:  Don’t allow the beads to sit in the applesauce – exposure to the moisture will start to activate the enzymes too early.  Also, take care to store your enzymes properly. 

CF Vitamin Therapy

            Individuals with CF have trouble absorbing the fat-soluble vitamins A, D, E and K.  To prevent vitamin deficiencies, special vitamins designed for CF are prescribed (AquADEK^®, SourceCF^®or Vitamax^®).  Because enzymes help the body absorb these vitamins, it is best to give vitamins at the time of a meal or snack when enzymes are also given. 

Salt

            People with CF lose more salt through sweat than people without CF.  Salt depletion can lead to serious health problems. Your nutritionist will recommend adding a specific amount of table salt to your baby’s diet every day to prevent this.  The salt is usually added to formula; for breast fed babies, it can be added to the applesauce given with the enzymes.  Liberal salt intake is encouraged through life and it should be added to baby foods and table foods as your baby gets older.  All people with CF should increase their salt intake during the summer months and when sweating from exercise.  

Fluids

            Generally, babies that drink enough breast milk or formula to grow well are getting enough fluid.  For most infants with CF, we do not recommend routinely giving water or juice because the nutrition content is poor. Occasionally, there are situations that require more attention to fluid intake, such as vacationing in hot climates.  Your nutritionist will guide you.  

Introducing Solid Foods

            For a baby with CF, the guidelines for starting baby food and table food may sometimes be different from the child without CF.  In general, babies without health concerns are ready to start solids between 4-6 months of age; soft table foods between 6-8 months; and whole cow’s milk at 12 months. The timing for babies with CF is individualized.  The factors to consider are your baby’s growth, appetite and interest in eating.  Although your pediatrician may talk to you about normal infant feeding stages, your CF nutritionist will guide you on the timing of solids, enzyme and vitamin dose adjustments, and high calorie strategies as needed. 

Reaching Nutrition Goals

            Calorie and protein needs are generally higher in those with CF than the non-CF population.  Despite this, many babies grow well with regular infant feeding practices and proper dosing of enzymes.  If a baby has trouble gaining weight, there are many strategies you can try with the guidance of your CF Team.  These include:

• Add more calories to formula, breast milk, baby food or table food.
• Fine-tune enzymes and absorption.
• Adjust times of feedings, meals and/or snacks.
• Review common causes of slow weight gain.  

            Sometimes, even with the best nutrition plan, there can still be a large “calorie gap”. That is, the calories consumed are not enough to match the calories used, and growth slows.  In this case, tubefeeding can be used to supplement the food a child eats. Tubefeeding is a method of delivering liquid nutrition through a tube directly into the stomach. In CF care, tubefeeding is not a “last resort” treatment, nor a sign of failure to feed a child well.  Instead, by supporting robust growth, it is one of many “tools” available to fight the disease.   

Living the Dream Diet, not the Heart Healthy Diet

            Dietary fat is a concentrated source of calories and it plays a very important role in CF nutrition.  It may be hard to get used to the idea of feeding your child a diet that is high in fat and calories when other parents are focused on avoiding childhood obesity and heart disease!  You may find it necessary to explain to family members, friends and other caregivers that restricting fat intake can actually be harmful to your baby.  For individuals with CF of all ages, we encourage full fat yogurts and ice creams, whole milk or even half and half, and generous amounts of butter or margarine, just to name a few! 

What to Expect from Your Nutritionist     

            You will find that the CF team asks a lot of questions at each visit to know how your baby is doing.  Feeding patterns, digestive symptoms, appetite, enzymes and vitamins are part of the nutrition picture. Your nutritionist is here to partner with you through any situations or obstacles that may arise in the area of nutrition. 

This information was kindly provided by The Cystic Fibrosis Center at Children’s Memorial Medical Center in Chicago. It is based on the Current Clinical Practice Guidelines as established by the CF Foundation for accredited care centers as of 2010 and is subject to revision. Check with your CF Team for the most current guidelines.