1. Nestlé Health Science
  2. Health management
  3. Chronic medical conditions rare diseases
  4. Cystic Fibrosis
CYSTIC FIBROSIS

Cystic fibrosis (CF) is most commonly considered a disease that affects the lungs, but cystic fibrosis also affects the pancreas and digestive system, among other body systems. For patients with cystic fibrosis, getting adequate nutrition is essential for quality of life.

There are varied symptoms of CF. The respiratory system is largely affected where mucus build-up can cause persistent coughing, wheezing and shortness of breath, combined with an increased risk of respiratory infections, and chronic inflammation of the lungs. Mucus can also affect the digestive system, causing a deficit of enzymes released by the pancreas that aid in digestion. The result is reduced absorption of nutrients, greasy and bulky stools, potential intestinal blockage, as well as poor weight gain and delayed growth in infants and children. People with CF are also at higher risk of diabetes. Other challenges include reproductive problems in both men and women, excessive salt content of a person’s sweat, and lower bone density.

People with CF need to consider important lifestyle changes to protect their health, including up to date vaccines that help prevent respiratory infections, avoiding smoke, frequent hand washing, and increased calorie and nutritional intake. As mucus buildup can prevent proper absorption of nutrients, eating a high-calorie, high-fat and high-protein diet can help prevent decreased weight gain and slow growth in infants. Taking prescribed enzymes with food has been an important means for improving digestion and absorption in patients with CF. Staying well hydrated and getting sufficient levels of salt are both important, as the changes in the secretory function cause more salt to be excreted in the sweat.

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Cystic fibrosis is one of the most common genetic disorder.


If parents are known to be carriers of the disease, there is a 25 percent chance of passing cystic fibrosis to their children.


Source:http://www.nhlbi.nih.gov/health/health-topics/topics/cf/

Accessed December 2014.

<h2>In the U.S., newborn screening is common in all 50 states and babies can be diagnosed early</h2>

IN THE U.S., NEWBORN SCREENING IS COMMON IN ALL 50 STATES AND BABIES CAN BE DIAGNOSED EARLY

Since the genetic cause of cystic fibrosis is known, a blood test is all that is needed to detect the presence of the defective gene. Prenatal screening and testing to see if an otherwise healthy parent is a carrier of the defective gene are also possible.


Source: http://www.nhlbi.nih.gov/health/health-topics/topics/cf/.

Accessed December 2014

<h2>Physical therapy for respiratory problems</h2>

PHYSICAL THERAPY FOR RESPIRATORY PROBLEMS

Due to the buildup of mucus in the lungs, cystic fibrosis is associated with respiratory problems like shortness of breath, and acute and chronic lung infections. Breaking up and clearing this mucus is a primary goal of chest physical therapy.

<h2>Ways to manage digestive problems</h2>

WAYS TO MANAGE DIGESTIVE PROBLEMS

Cystic fibrosis can limit the absorption of nutrients in the digestive tract. Besides the oral intake of digestive enzymes, nutritional management including supplementation of vitamins A, D, E, and K, and high-calorie, high-fat, and high-protein diets can help to make up for nutritional deficits.


Source: http://www.nhlbi.nih.gov/health/health-topics/topics/cf/.

Accessed December 2014.

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