Cystic Fibrosis

F. Alvarez, L. Bauer, S. Getahun, L. Hernandez, and E. Simpson

BSCI 440 Discussion Tu 2pm

CF Group Research Paper

Cystic Fibrosis is an inherited chronic disease that affects the lungs and digestive system of

approximately 30,000 children and adults in the United States and approximately 70,000 people

worldwide. A defective gene and its protein product cause the body to produce unusually thick,

sticky mucus that causes two main issues: 1) it clogs the lungs, leading to life-threatening lung

infections and 2) it obstructs the pancreas and stops natural enzymes from helping the body break

down and absorb food.1 Approximately 1,000 new cases of cystic fibrosis diagnosed each year.

Over 70% of patients are diagnosed by the age of two and over 45% of the CF patient population is

18 or older.2 In the 1950’s, the life expectancy of people with CF was elementary school age

however with the treatments offered today, the median age of survival has increased to about 37.4

years old.3 CF affects all races however is primarily seen in Caucasians. A typical case study

would be of a baby or small child presenting with a history of poor growth and a chronic cough.

CF patients typically have some sort of respiratory distress, such as asthma and have trouble

eating, causing a failure to thrive due to frequent vomiting and loose, greasy, bowel movements.

The earliest record of cystic fibrosis (CF) date back to before the Middle Ages in eastern

Europe. Medieval folklore taught that symptoms of the disease, in particular that the salty taste of a

child’s skin, were indicators that the child was hexed and doomed to an early death. A doctor’s

description of a deceased child as being “meager with a swollen, hardened, gleaming white

pancreas” in 1595 was one of the earliest accurate descriptions of pancreatic lesions resulting from

CF.2 Other anecdotal records from the mid-1600s report an excess of fat in the feces, arising from

pancreatic insufficiency, which is a...