Cystic Fibrosis Trivia Quiz

Answer: True

This is one of the earliest references to cystic fibrosis. Folklore from the Middle Ages, from Germany and Switzerland, made this reference. The earliest documented reference in the literature is from 1606, Alonso y de los Ruyzes de Fonteca, professor of medicine in Spain, wrote that it was known that the fingers taste salty after rubbing the forehead of the bewitched child.' This is a clear reference to the fact that children with cystic fibrosis have very salty sweat, due to the abnormal protein manufactured by the cystic fibrosis gene. This protein regulates the movement of sodium and chloride from the body's cells. Sodium and chloride are two components of salt. The sweat glands of a person with cystic fibrosis secrete too much salt, and the person tastes salty.
This is also a reference to the fact that in earlier days, people with cystic fibrosis did not live past infancy.
A good article on the history of cystic fibrosis is by Paul M. Quinton "Physiological Basis of Cystic Fibrosis: A Historical Perspective."

Answer: All of these

Cystic fibrosis primarily affects the glands that secrete. An abnormal protein is produced making secretions sticky. These thickened secretions are found in the body's sweat, the lungs' mucus, the pancreas' output, liver output, and reproductive organs.
For more information, go to www.cff.org

Answer: Mucovicidosis

Cystic fibrosis is often called mucovicidosis, especially in other regions of the world. This refers to the thick, sticky mucus secreted by the exocrine glands of someone affected by cystic fibrosis.
Cystic fibrosis was named that in 1938 by Dr. Dorothy Anderson, a pathologist in New York City. She described changes in the pancreas as cystic, or fluid-filled, and fibrotic, or scarred.

Answer: Sweat test

A small electrode is placed on the skin, which stimulates the sweat glands. Sweat is collected for a period of time and then analyzed. People with cystic fibrosis have an increased amount of chloride in their sweat, due to the abnormal protein manufactured by the cystic fibrosis gene.

Answer: A defective gene that leads to the making of an abnormal protein

Usually, the gene involved makes a protein called CFTR (Cystic Fibrosis Conductance Transmembrane Regulator). This protein regulates the movement of sodium and chloride in the body's cells. Disruption of this protein leads to thicker secretions due to the imbalance of salt and water in the cells. Too little salt and water are outside the cells, making everything thicker.

Answer: All of these

All of these are treatments for cystic fibrosis. Airway clearance techniques help people with cystic fibrosis move thickened mucus from the lungs. Pancreatic enzymes are taken to help digest the food. Special vitamins are needed to help replace vitamins A, D, and K which may not be absorbed well by the CF patient. There are more treatments, of course, but these are the main ones to help keep the person with cystic fibrosis healthy.

Answer: Better antibiotics and airway clearance treatments

Right now, 40% of the cystic fibrosis population in the US are adults. The current median age of survivial in the US is 36.5 years.
CF used to be a disease of childhood, as patients with it lived only a few months in the early 1900's. By the 1950's, they were living long enough to go to elementary school. By the 1980's, the average lifespan was 19. Huge leaps in care and antibiotics have led to this improvement. May it continue!

Answer: High calorie and high fat diet

The current recommendation is 120-150% of the recommended daily allowance of calories for children, and 40% fat. Adults with cystic fibrosis also need to eat high calorie food, with increased fat. This is because a person with cystic fibrosis doesn't absorb nutrients as well from their food, especially fat. A certain amount of fat is needed in your diet to keep you healthy.
While the person with cystic fibrosis can and should eat more fat, they still shouldn't eat junk food!

Answer: All of these

Autogenic drainage is a breathing technique aimed at moving mucus from the bottom parts of the lungs to the top, where it can be expectorated. It is intensive deep breathing. After exhaling as much as possible, the person takes normal sized breaths at the very bottom part of their lungs. In sequence, they take breaths moving up in the lungs. It is difficult to do unless you are properly trained. It is very effective.
High frequency chest wall oscillation is commonly called the vest. It looka like a life preserver vest, and is worn in a similar fashion. The vest has an air bladder in it, which is puffed up by a generator and vibrated. The effect of the lungs is to act as a series of mini-coughs, and moves mucus up the mucociliary elevator.
Chest physiotherapy is also called CPT, which is having the person (usually a small child) lie in different positions and tapping different parts of their chest. It is also known as the "catsup bottle" method of removing mucus.

Answer: False

A person with cystic fibrosis has inherited the defective gene from both their father and their mother, for two copies. If you have inherited one copy of the defective gene, you are a carrier. 1 in 31 Caucasian Americans are estimated to be carriers. It also affects other races to a lesser extent.

For more information on this disease, see www.cff.org. And please, help us fight CF!