Eating management as a part of medical nutrition therapy for children with cystic fibrosis

Ellyn Satter, MS, MSSW, Inés Anchondo, Dr PH, RD, LD, CSP, MPH; Margot Cleveland, mother of a child with cystic fibrosis

Nutrition therapy: integral to cystic fibrosis management

Nutrition therapy is an integral part of the management of Cystic Fibrosis (CF). The goals of nutrition therapy for the child and adolescent with CF are to:

1. Improve or maintain the quality of life, nutritional status, and physiological and emotional health.
2. Provide adequate nutrients, including calories and supplementary fat-soluble vitamins, to support optimum growth and a strong immune system, thereby preventing as much as possible the acute and long-term complications of CF and associated co-morbid conditions.
3. Support parents in feeding well, including making wise use of oral supplements and tube feedings.
4. Support children and adolescents in growing up to be Competent Eaters.
5. Support children in taking stage-appropriate responsibility for managing their own condition.

Medical care requires supporting the child’s self-mastery

Medically, to take care of their child and adolescent with CF, parents and caregivers must take kind and firm leadership with treatment, and the child must achieve positive self-mastery with compliance. To help the child achieve such self mastery, see Medication and the Division of Responsibility in Feeding. Such positive self-mastery comes into play when the child and adolescent:

• Voluntarily take parent-administered and eventually self-administered oral pancreatic enzymes.
• Voluntarily participate in and eventually perform pulmonary therapy and exercise.
• Ingest prescribed and needed vitamins, courses of laxatives, antibiotics, and other medicines and supplements.
• Cooperate with necessary medical examinations and procedures, including throat cultures, blood draws, x-rays, IV placement, and others.

Nutritional care requires supporting the child’s self regulation and autonomy

With eating, the issue for the child is self regulation, not compliance. To support their child in achieving optimum nutrition, parents must follow the Satter Division of Responsibility in Feeding (sDOR). Parents provide leadership with feeding and give the child autonomy with eating. Rather than being expected to follow dietary prescriptions, children who are given autonomy with eating are trusted to eat as much or as little as they want at the regular meals and snacks that parents provide. In combination with treatments for CF, applying sDOR begins at birth and continues throughout the growing-up years. For children with CF, the same as for other children, sDOR can and must be applied in a developmentally appropriate fashion.

• For infants, choose breastmilk or formula, then calm and organize the infant by feeding smoothly, following infant cues for when, how often,how fast, and how much.
• During the transition to family food at family meals, parents remain responsible for the what of feeding at the same time as they increasingly become responsible for the when and where. Parents build on the child’s increased oral-motor capabilities and regularity with eating to introduce solid foods and make the transition to including the child in family meals and structured snacks.
• From the toddler stage onward, parents manage the what, when, and where of family meals and sit-down snacks. Then parents let children determine how much and whether they will eat of what parents provide at those structured meals and snacks.

Parents also observe a division of responsibility in activity by providing structure, safety, and opportunities to move, and letting children determine how much and whether to move and the manner of moving.

 Children with CF need to achieve eating competence

Positive parenting with food allows children with CF to achieve and maintain eating competence. That is, they:

• Feel positive about eating and about food;
• Are relaxed around unfamiliar food, eat some foods, ignore others.
• Are relaxed about getting enough to eat, have a sense of hunger and fullness.
• Enjoy family meals and behave well at mealtime.
• Grow and gain weight consistently, in a way that is right for them.

Throughout the growing-up years, all children, including those with CF, maintain their ability to internally regulate food intake and gain weight consistently. Depending on their temperament, mastery opportunities, and challenges of the CF regimen, children gradually learn to eat a greater variety of foods and take increasing responsibility for self- care of CF. During the high school years, in preparation for living independently, children with CF develop food-management and CF-management skills. At the same time, the adolescent living at home benefits greatly from consistently participating in family meals.

Structured meals and snacks are essential

Parents’ regularly offering structured meals and snacks that include all the food groups and an excellent fat source supports the child’s Eating Competence and therefore nutritional status and weight gain. To help parents sustain the considerable task of maintaining meals and snacks, they must be encouraged to choose food that they find rewarding to plan, prepare, and eat. To help parents avoid pressure tactics such as forcing the child to eat and catering to the child’s food preferences, parents must be helped to understand (with both in-clinic education and encouragement to study Ellyn Satter’s books and other sDOR-consistent publications) that children’s normal eating patterns are erratic and inconsistent. What children like and eat enthusiastically one time, they ignore the next; they eat a lot one day, a little the next; they are likely to eat little for many meals, then, periodically eat an astonishing amount of food.

To support children’s erratic eating patterns as well as to allow them to get enough calories to compensate for CF-related malabsorption and gain weight consistently, parents can be considerate without catering with meal-planning. Menus can match unfamiliar with familiar foods and include ample high-fat foods (butter, salad dressing, gravy, avocado) to allow children to increase their calorie intake when needed.

Children with CF can regulate food intake

Properly attended to and supported, the internal regulation cues of hunger, appetite and satiety of children with CF are trustworthy. Those cues are in dynamic equilibrium with tendencies for movement and the broader environment. This equilibrium combines to produce, where possible given the reality of disease progression, a consistent growth trajectory that reflects each child’s genetically determined weight gain and growth potential. A weight trajectory below the 50th percentile is appropriate as long as it is consistent and the child has adequate fat stores to withstand illness. On the other hand, abrupt weight flattening or faltering is unlikely to be appropriate, needs to be promptly investigated, and may have as much to do with disease-management issues as food consumption.

Attempting to apply prescriptions for calorie intake and growth undermines children’s eating competence, distorts their sensitivity to internal regulatory cues, and competes with metabolic homeostasis. Energy regulation is based on the amount of calories and other nutrients that actually get into the blood stream and the cells, not the amount of calories that go through the gastrointestinal tract. Thus, an eating competent child who loses calories through the GI tract instinctively eats more to compensate. Since metabolic regulation is long term, with cycles that last weeks, months, seasons, even years, children with CF who eat little during illness do catchup eating when they feel better. Moreover, the nutritional goal with CF or any other condition is to support optimal nutrition, not promote over-nutrition. Once nutritional needs are satisfied, additional calories and other nutrients do not further enhance health, medical status, or the immune system.

When parents follow sDOR and feed in a developmentally appropriate fashion, children with CF are Eating Competent: they enjoy eating and voluntarily eat as much as they need to gain weight and grow consistently. Within that positive context, children’s showing a loss of interest in eating over several days, filling up quickly, having regular stomach aches, and showing a change in stool patterns can signal GI issues and possibly even warn of intestinal obstruction. When children are not competent with eating, that is, when they don’t enjoy eating and require coercion to eat, the eating dysfunction can mask medical issues. These issues include constipation, DIOS, bacterial overgrowth, or a need to adjust enzyme dosing.

The table below compares the Satter Feeding Dynamics Model (fdSatter) with the conventional approach in managing childhood CF.