Cystic fibrosis is a disorder genetically passed on. In order for a child to be born with this disorder, both parents must possess the recessive gene causing cystic fibrosis. A mutation on chromosome number 7 is the cause for cystic fibrosis. When the CFTR protein is defective, the epithelial cells fail to regulate chloride passing over cell membranes. This causes the mucus lining of the lungs to become hypertonic, making the normally thin layer of mucus thick, and sticky. Being a fairly common disease in the United States, especially among Caucasians, it is manageable, but not curable and will worsen throughout the individual’s life. Those who are affected by cystic fibrosis can expect to live about 30- 35 years. If pancreatic function is of a high percentage, life expectancy will increase to about 40- 45 years. There are about 30,000 cases in the United States, with about 1,000 new cases every year.
For a short period of time, the disorder may be asymptomatic, usually only right after birth. Nagging cough with thick phlegm is a common symptom, demonstrating that the mucus is beginning to make breathing more difficult, along with shortness of breath or wheezing. Mucus is a habitat well suited for bacteria, causing the mucus in the lungs to have large amounts of bacteria. This will result in frequent lung infections. Multiple and frequent infections of the sinuses and lungs, such as sinusitis, bronchitis, and pneumonia are common among cystic fibrosis patients. Another common symptom, also evidence of hypertension, is salty tasting skin, due to the uncontrolled movement of chloride.
Other names for cystic fibrosis include: Cystic fibrosis of the pancreas, fibrocystic disease of the pancreas, mucoviscidosis, mucoviscidosis of the pancreas, pancreas fibrocystic disease, and pancreatic cystic fibrosis. (http://www.nhlbi.nih.gov). The pancreas is effected by cystic fibrosis because it causes the enzyme to become thick and fail to function as it should. This enzyme is important for production of insulin and for the digestive system to break down foods.
In order for treatments for cystic fibrosis to be effective, multiple treatments must take place. Therapy to trigger coughs to loosen mucus and allow it to exit the lungs is prudent for maintaining the ability to respire individually. Inhaled medications, implanted devices and antibiotics are common and effective treatments that maintain lung function in cystic fibrosis patients. Having established that their disease is not curable, these treatments only “hold off” the disease. About 90% of the people that have cystic fibrosis will die because of