Nutrition For Cystic Fibrosis

We are pleased to have an overview on the nutrition needed for children with cystic fibrosis from Elizabeth (Betsy) Britt, RD and Edith Pilzer, MD who are members of the Cystic Fibrosis Care Team at Children's Healthcare of Atlanta.

                                    Dr. Stan

Cystic fibrosis (CF) is an inherited disease. For CF to develop, both parents contribute a gene so that the two genes together alter the salt content of many of the body's fluids. As a result, children with CF taste salty when you kiss them and thick mucus builds up in the lungs, digestive tract, and other areas of the body. The buildup in the lungs causes coughing and wheezing making it difficult to breathe. The mucus also blocks the ducts in the pancreas, preventing the enzymes that normally digest fat, protein and starch from working effectively—causing bulky, foul bowel movements. Together these 2 main features use enormous amounts of energy and waste the calories being eaten.   

Fortunately, treatments are now available that helps those with CF live longer and have productive lives. As a result, more focus has been placed on better nutrition, because it improves lung function and is essential in increasing the growth and quality of life for children with CF.

Healthy Nutrition

In those with CF, a healthy body weight is associated with good lung function, according to the Cystic Fibrosis Foundation. As a result, it is recommended that children with CF over the age of 2 years have a weight-for-length (or when they're older, a BMI) that is at least at the 50th percentile for their size and age.  

It can be difficult to meet growth goals due to increased calorie and protein expenditure in this population. A high calorie, high protein diet without fat restriction is usually recommended—1.5 - 2 times what an otherwise healthy child would require. Many children with CF utilize high calorie nutritional supplements to meet these recommendations. Appetite stimulants and tube feedings can also be utilized in children who have difficulty with weight gain <<link>>.

Children with CF also lose extra salt in their sweat. So eating a high salt diet is recommended for all patients with CF. Older children and adults are encouraged to add extra salt to their foods. For babies, parents should add 1/8 teaspoon salt to 32 ounces of formula, since babies also need more salt .

Vitamins

Children with CF are at risk for nutrient deficiencies, particularly of fat soluble vitamins A, D, E, and K. This is because thick mucus blocks the digestive enzymes from doing their job to break down fats so they can be absorbed. As a result, children with CF should take an additional CF specific multivitamin daily.

Specific vitamin recommendations are re-evaluated regularly with the table providing recent ones. The Vitamin D (the most likely to change soon) can be obtained from sunlight as well as from the diet, though those in the northern US and those with darker skin may have less skin absorption.

Vitamin

Amount Needed

A

 10,000 international units daily

D

 400 international units daily   

E

 200-400 international units daily

K

 2.5 - 10 mg weekly, the higher numbers if liver disease is present

 

Special CF multivitamins have been formulated to contain adequate A, D, E and K to meet the current recommendations for those with CF. The ones that are water soluble are most easily absorbed for those with CF because they don't have depend on fat absorption.

Bottom Line

  • Good nutrition is a necessary part of CF care. Recommendations include:
  • A high-calorie, high-protein diet
  • Maintaining a weight-for-length or BMI that is greater than or equal to the 50th% for age 
  • Extra salt intake
  • PERT for those who have pancreatic insufficiency
  • Vitamins A, D, E, and K
  • Careful monitoring 

Resources

Leonard, A., & Schindler, T. (July 2014). Cystic Fibrosis Nutrition 101: Getting Started. 6th edition.

Cystic Fibrosis Foundation - Patient Registry Annual Data Report 2013. (2014). 

Dr. Stan Cohen21 April 2016

Dr. Stan Cohen is one of our founders and our CEO as well as the Chairman of our Medical Advisory Board. Dr. Stan is an internationally recognized expert in Read more

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