Cystic Fibrosis

Cystic fibrosis is a disease that is hereditary. It causes thick, sticky mucus to build up in the lungs, digestive tract, and other areas of the body. It is one of the most common chronic lung diseases in children and young adults. It is a life-threatening disorder. It is caused by a defective gene which causes the body to produce abnormally thick and sticky fluid, called mucus. This builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food. The collection of sticky mucus results in life-threatening lung infections and serious digestion problems.

An estimated 1 in 29 Caucasian Americans have the CF gene. The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It’s more common among those of Northern or Central European descent. Most children with CF are diagnosed by age 2. A small number, however, are not diagnosed until age 18 or older. These patients usually have a milder form of the disease.

Symptoms:

  • Persistent cough
  • Wheezing
  • Repeated lung infections
  • Repeated sinus infections
  • Salty taste to the skin
  • Foul-smelling, greasy stools
  • Poor weight gain and growth
  • Distended abdomen from constipation
  • Intestinal blockage, particularly in newborns
  • Child isn’t growing properly

**One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Parents often can taste the salt when they kiss their child.

**Cystic fibrosis is one of the leading causes of bronchiectasis, a condition in which damaged airways widen and become flabby and scarred.

**Repeated lung infections damage the lungs, making it more likely for the lung to collapse.

**Because the lining inside the nose is inflamed and swollen, it’s more likely to develop large or multiple polyps — soft, fleshy growths inside your nose.

**The pancreas also produces insulin, which your body needs to use sugar. Cystic fibrosis increases your risk of developing diabetes.

Nutritional Deficiencies: Thick mucus blocks the tubes that carry digestive enzymes from your pancreas to your intestines. Without these enzymes, your body can’t absorb protein, fats or the fat-soluble vitamins — A, D, E and K.

CF Diet – In CF, a high-calorie, high-fat diet is vital for normal growth and development in children, and offers adults a way to maintain optimal health. People with cystic fibrosis may need extra calories to compensate for the malabsorption of nutrients. These extra calories also help to meet the greater energy needed for breathing.

Cystic fibrosis patients loose on average 10 times more salt than other people. Salt is important, as it controls the water balance in the body. A lack of the mineral causes the body to dehydrate, which happens frequently with this genetic disorder. It’s therefore normal for patients to eat salty foods or add salt to their dishes.

Adding more fat to the diet—

  • Simply adding one or more spoons of oil to meat or fish adds a lot of calories without adding enormous amounts of food. Examples are olive oil, sunflower oil, mayonnaise, béarnaise…
  • Butter is also a good source of fat which can be spread on a toast or sandwich. Cooking butter can also be used in large quantities when cooking meat and fish.
  • Cheese is also a great source of fat which can be eaten with bread, potatoes, soup and dishes prepared in the oven.

Sources:

http://www.cff.org/LivingWithCF/StayingHealthy/

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/

http://www.aboutcysticfibrosis.com/cystic-fibrosis-diet.htm

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