Pictured: Mandy Wagner, CF Survivor 1985-2012

For many people, Cystic Fibrosis (CF) is a condition they have never heard of. However, CF is the most commonly inherited disease in the United States, affecting over 30,000 people. In the U.S., 1 in every 3,700 people will be diagnosed with CF each year. On the plus side, early detection and treatment means that the median age has increased dramatically over the past decade alone. So what is CF?

CF is a lifelong disease caused by a defective gene and its protein product which affects chloride transport at the cellular level that triggers the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections, obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food, and damages the liver causing a CF-related form of diabetes. The defective gene also produces a salt imbalance in the body that impacts the sweat glands, respiratory tract (sinuses and lungs), gastrointestinaltract (notably the pancreas, intestines, and liver) as well as in both the male and female reproductive tract. The imbalance can also lead to electrolyte deficiencies and kidney issues. It is also believed that the resulting pH changes associated with the salt imbalances further challenge health maintenance.Symptoms in a person with CF include:

• very salty skin• persistent coughing, at times with phlegm• frequent lung infections• wheezing or shortness of breath• poor growth/weight gain in spite of a good appetite• frequent greasy, bulky stools or difficulty in bowel movements• high blood sugar• kidney stones• acid reflux

Nutrition plays a key role in the treatment of CF. See below how each characteristic of CF affects a person’s nutritional status:

Fat + Nutrient Malabsorption: someone with CF may need the appropriate dosing of pancreatic enzyme supplementation depending on the function of his or her pancreas. Having too many or too few of these supplements can lead to uncomfortable and sometimes painful GI symptoms (as mentioned above). These folks need education on balancing fat intake with the appropriate enzyme dosage, as well. Malabsorption of fats also leads to deficiencies of the fat-soluble vitamins A, D, E, K, as well as some minerals. Therefore, additional supplementation of these nutrients is essential. In addition, malabsorption and lung exacerbations can lead to low BMI and malnutrition; it is essential to recommend a high protein-high calorie diet that focuses on multiple meals and snacks throughout the day that include whole grains, fruits, vegetables, and healthy fats.

Reduced Appetite: Often times, shortness of breath, phlegm production, and even some medications suppresses the appetite. In this case, the patient may receive an appetite stimulant, nutritional supplement drink, or tube feeding to maintain/gain healthy weight. If a person with CF neglects to consume the right amount of nutrients over a long period of time, there is an increased risk of infection and reduction in lung function. Research shows that as BMI increases, so does lung function; no research exists yet that details the risks of overweight/obesity on CF as this is very uncommon.

Salt Imbalance: Because the person’s internal channels that contain sodium and chloride are affected, it is integral for the person with CF to eat foods that contain salt. A person may also choose to drink beverages that contain various electrolytes, such as Gatorade, Propel, or Pedialyte. This becomes essential after physical activity and in warmer weather.

Airway Clearance: Stressing the importance of physical activity is a must. Not only does cardiovascular activities such as running, kickboxing, etc. help to release phlegm (known as airway clearance), but strength training exercises such as weight lifting help increase lean body mass which may prevent occurrences of infection and lung exacerbations.

CFRD: When a person develops CF-related diabetes (CFRD), it is essential to cover a carbohydrate-counted diet with an appropriately dosed insulin regimen. Though calories should not be restricted, carbohydrate timing and regular intake can better maintain blood sugar control.

Every person with CF is unique and presents with his or her own set of symptoms. While some patients may experience a higher frequency of GI complications, others may have none. Individualizing holistic treatment methods in the CF population is necessary to ensure each person is reaching their optimal health potential. Most importantly, working in a multidisciplinary setting helps a person with CF maximize his or her nutritional potential. Understanding the patient’s socioeconomics, insurance coverage, genetic variance, lung function, and medical status is crucial to providing the best care possible. To learn more about CF and find out ways to help, please go to the website for the Cystic Fibrosis Foundation at www.cff.org.

Happy Eating, Living, and Breathe Easy.