Cystic Fibrosis — a condition many people are unfamiliar with — is the most commonly inherited disease in the United States, affecting more than 30,000 people. In the U.S., one in every 3,700 people will be diagnosed each year. On the plus side, early detection and treatment means that the median age for survival has increased dramatically over the past decade from mid-early 20s to late 30s-40s. So what is CF?
CF is a lifelong disease caused by a defective gene’s protein product, which triggers the body to produce unusually thick, sticky mucus, clogging the lungs and leading to life-threatening lung infections. It also 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 that can lead to electrolyte deficiencies and kidney issues. Symptoms 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 treatment. Here’s how each CF characteristic affects nutritional status:
Fat + Nutrient Malabsorption: Someone with CF may need pancreatic enzyme supplementation depending on the function of the pancreas. However, having an incorrect dose 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. Malabsorption of fats also leads to deficiencies of the fat-soluble vitamins A, D, E and K, as well as some minerals, so additional supplementation of these nutrients is essential. In addition, malabsorption and lung exacerbations can lead to low BMI and malnutrition; it is important 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, shortness of breath and phlegm production suppress the appetite. In this case, the patient may need 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.
Salt Imbalance: Because CF affects internal channels that contain sodium and chloride, it is integral for the CF patient to eat foods that contain salt or consume electrolyte-containing beverages such as Gatorade, Propel or Pedialyte, particularly after physical activity and in warmer weather.
Airway Clearance: Physical activity is a must. Not only do cardiovascular activities such as running, kickboxing, etc. help to release phlegm (known as airway clearance), but strength-training can help increase lean body mass, which may help prevent infection and lung exacerbations.
CFRD: When a person develops CF-related diabetes (CFRD), it is crucial 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.
Each CF patient is unique, with his or her own set of symptoms. While some patients experience more frequent GI complications, others have none. Individualizing holistic treatment methods in the CF population is necessary to ensure each person’s optimal health. 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, including ways to help, visit the Cystic Fibrosis Foundation website.