Cystic fibrosis is a recessive inherited condition that mainly affects the lungs, digestive system, and the exocrine glands. Malfunctioning genes cause the vital secretions produced by these organs to be thickened. These secretions normally have a much thinner consistency. This abnormal mucus clogs airways and other passageways, causing symptoms like coughing, wheezing, lung infections, salty sweat, failure to gain weight normally, intestinal blockage, and severe constipation. Cystic fibrosis cannot be transmitted to others, nor can it be cured. Patient life expectancy is limited. Most individuals with this disease do not live much beyond forty years. However, treatments are available that do extend life expectancy as much as possible. These treatments also improve quality of life.
Airway Clearance Techniques
The disease's name is somewhat of a misnomer. Cystic fibrosis has nothing to do with the formation of cysts, or fluid-filled sacs. The disease typically affects the lungs quite severely. The lungs normally have thin and watery mucus secretions, which help lubricate the airway passages and add moisture to the inhaled air. Cystic fibrosis causes this normally thin mucus to become so thick it clogs the airways, interfering with the normal movement of air within them. Coughing, wheezing, difficulty breathing and infections result from these thickened secretions. Airway clearance techniques help to loosen thickened mucus from the pulmonary airways. This may involve forced huffing and coughing. A therapist or trained caregiver may perform percussion, which is a type of controlled pounding on the back, chest, and sides. The vibrations produced help to loosen mucus. There are six basic body placement positions for the administration of airway clearance techniques.
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