What is Cystic Fibrosis?

 

The following has been taken directly from the Cystic Fibrosis Foundation. (www.cff.org)


Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.


In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s, and beyond.


Symptoms of Cystic Fibrosis


    * very salty-tasting 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


Treatment for CF Patients


Currently, there is no cure for cystic fibrosis. However, specialized medical care, aggressive drug treatments and therapies, along with proper CF nutrition, can lengthen and improve the quality of life for those with CF. Individuals with CF not receiving proper care leads to early death.


The best treatments and therapies for CF vary from person to person. CF caregivers at a CF Foundation-accredited care center work closely with CF patients and their families to create individualized treatment plans.


Since cystic fibrosis affects the lungs of most patients, a large part of the treatment routine is to clear mucus from the airways by using different airway clearance techniques. These techniques use vibrations to help loosen the mucus in the lungs so it can be coughed out.


Because the severity of CF differs widely from person to person, and CF lung infections flare up from time to time, there is no “typical” day. However, each day most people with CF:


    * Take pancreatic enzyme supplement capsules with every meal and most snacks (even babies who are breastfeeding may need to take enzymes).

    * Take multi-vitamins.

    * Do some form of airway clearance at least once and sometimes up to four or more times a day.

    * Take aerosolized medicines—liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer


Statistics


    * About 1,000 new cases of cystic fibrosis are diagnosed each year

    * More than 70% of patients are diagnosed by age 2

    * More than 40% of the CF patient population is age 18 or older

    * The predicted median age of survival for a person with CF is more than 37 years


CF Foundation
www.cff.org

CF Living
www.cfliving.com

Cystic Fibrosis
www.cysticfibrosis.com


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