Understanding Cystic Fibrosis

What is Cystic Fibrosis (CF)?

Cystic fibrosis (CF) is a complex genetic disease that affects the lungs, digestive system, reproductive tract, and sweat glands. In the United States, roughly 30,000 people are living with cystic fibrosis, and another 1,000 are being diagnosed with the disease each year. Most CF patients are diagnosed by age two.

Cystic fibrosis is a progressive condition, involving body’s mucus glands¹. Despite its widespread effects on the body, the majority of patients in United States suffer from lung complications with CF. These patients cannot removes excess mucus from their lungs which can lead to the accumulation of thick, viscous secretions¹. Mucus accumulation is often a breeding ground for bacterial growth. Children and adolescents with CF often have decreased growth, which could be caused by a combination of malabsorption, decrease in appetite, and increase in energy expenditure due to this condition². Some CF patients do not make adequate pancreatic enzymes, which are needed to help the body absorb the fat soluble vitamins A,D, E, and K. These vitamins are essential for body growth, immune function, and reproductive health.

Dietary Supplementation

It is important for CF patients to eat a proper diet. They often require a higher caloric intake than other people. Pancreatic enzymes should be replaced if the patient is diagnosed with pancreatic insufficiency. These are some examples of targeted nutrients and/or pharmacological agents that are used in practice:

Non-Pharmacological Nutrients in Cystic Fibrosis^3,5

• Omega 3 fatty acids to lower inflammation.
• Probiotic supplement to improve digestion.
• Anti-inflammatory foods, such as extra virgin olive oil, avocadoes, walnuts, and flaxseed oils.
• Sodium – patients with CF are prone to sodium loss. However, they should carefully monitor their blood pressure if their doctor recommends a high sodium diet.
• Fluoride – vitamins formulated for the CF patient do not contain fluoride. It is essential to feed them fluoride supplement.
• Zinc – CF patients under the age of two, who have inadequate growth despite the proper nutrient support, should be evaluated for zinc deficiencies.

Treatments for Cystic Fibrosis

Cystic fibrosis treatment strives to help patients reach a better quality of life by improving breathing and lung capacity. Devices, like oscillatory positive pressure, remove the mucus and secretions of the lungs. Hypertonic saline can be used to increase air flow into the lungs and break up mucus. Breathing exercises and physical therapy can help dislodge the mucus in the chest and promote better breathing⁴.

Symptoms of Lung Complications in CF Patients

Force expiratory volume (FEV1) is a measure of how much air a person can exhale in a forced breath, and is a good indicator of lung function. It’s an easy, convenient method for monitoring lung function at home. FEV1 below individual goal is the indication of reduction in pulmonary function³. In young children, viruses are the cause of acute exacerbations leading to a decline in pulmonary functions. Diagnosis of pulmonary exacerbations is based on decline in individual health condition with pulmonary symptoms, as compared to recent baseline health status³. Symptoms that are commonly present include:

• New or increased cough
• Increase in sputum production or chest congestion
• Increased fatigue
• Decreased appetite
• Changes in sputum appearances

Pharmacological Treatments

CF patient are at a severe risk for influenza infection. Prophylaxis or treatment with oseltamivir (Tamiflu) or zanamivir (Relenza) is often recommended under certain circumstances⁶. Annual vaccination against viral influenza is recommended to all patients with CF six months and older⁶.

Many patients with CF have chronic bacterial infection of lungs because of the thick viscus mucus accumulation. Systemic antibiotics are indicated to all patient with CF if they experience new or increased cough, and changes in the color of their mucus, which can indicate bacterial presence due to unnecessary mucus accumulation in the lungs. Antibiotic selection will depend on the results of a sputum culture.

Patients with the CF should focus on the type of food they consume to ensure they are getting proper nutrients. Daily use of the FEV1 is important, which helps to clear the mucus and prevent possible infections.

References:

1. Cohen, T. S., & Prince, A. (2012). Cystic fibrosis: a mucosal immunodeficiency syndrome. Nature medicine, 18(4), 509-519.
2. Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002; 35:246.
3. Reilly JJ, Edwards CA, Weaver LT. Malnutrition in children with cystic fibrosis: the energy-balance equation. J Pediatr Gastroenterol Nutr 1997; 25:127.
4. Rosenfeld M, Emerson J, Williams-Warren J, et al. Defining a pulmonary exacerbation in cystic fibrosis. J Pediatr 2001; 139:359.
5. Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H, Clinical Practice Guidelines on Growth and Nutrition Subcommittee, Ad Hoc Working Group J Am Diet Assoc. 2008;108(5):832.
6. https://www.uptodate.com/contents/cystic-fibrosis-overview-of-the-treatment-of-lung-disease?sectionName=Influenza%20vaccine&anchor=H20&source=see_link#H20

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