About CYSTIC FIBROSIS
WHAT IS CYSTIC FIBROSIS ?
Cystic Fibrosis also known as “CF” is genetic disease that causes persistent lung infections and progressively limits the ability to breathe. CF also slowly impair the lungs and other bodily such as fertility in young adults.
People living with CF, have a defective gene that causes a thick, buildup of mucus in the lungs, pancreas and other organs. In the lungs, the mucus builds up and traps bacteria leading to infections, extensive lung damage and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.
People with CF can have a variety of symptoms, including:
- Very salty-tasting skin
- Persistent coughing, at times with phlegm
- Frequent lung infections including pneumonia or bronchitis
- Wheezing or shortness of breath
- Poor growth or weight gain in spite of a good appetite
- Frequent greasy, bulky stools or difficulty with bowel movements
- Male infertility in young adults
DIAGNOSIS AND GENETICS
Cystic fibrosis is a genetic disease inherited from both parents. People with CF have two copies of the defective CF gene — one copy from each parent. Both parents must have at least one copy of the defective gene for their child to be both with CF.
Children born with only one copy of the defective gene are called carriers, they are however not affected by the gene in the same way a person with two copies of the defective gene. Each time two CF carriers have a child, the chances are:
- 25% (1 in 4) the child will have CF
- 50% (1 in 2) the child will be a carrier but will not have CF
- 25% (1 in 4) the child will not be a carrier and will not have CF
There are current more than 1,800 known mutations of the disease.
Cystic fibrosis is a complex disease and the severity of symptoms can differ widely from person to person. There so called CF’s
- Physiotherapyhelps loosen and get rid of the thick mucus that can build up in the lungs. Some of these techniques require help from family members, friends or respiratory therapists. Some people living with CF use an inflatable vest that vibrates the chest at a high frequency to help loosen and thin mucus.
- Inhaled medicines to open the airways or thin the mucus. These are liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer. These medicines include antibiotics to fight lung infections and therapies to help keep the airways clear.
- Pancreatic enzyme supplement capsules to improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. People with CF also usually take multivitamins.