CYSTIC FIBROSIS

CYSTIC FIBROSIS - A DISORDER OF MEMBRANE TRANSPORT




A great variety of proteins have roles in moving molecules and ions across cell membranes. Passive transport proteins permit certain substances to diffuse down concentration gradients by moving through the protein's interior. Active transport proteins use ATP energy to pump substances across the membrane against their concentration gradients. To investigate the importance of transport proteins, we will consider the effects of cystic fibrosis, a genetic disorder in which there is a defect in a transport protein..



Activity



Part 1.



In this part of the activity you will visit the Cystic Fibrosis Foundation’s web site to learn about the causes and symptoms of cystic fibrosis.



Use your browser to go to http://www.cff.org/home/



Use the information provided in the “About cystic fibrosis” section to answer the following questions:



1. What are the signs and symptoms of cystic fibrosis?

The symptons of cf of are salty tasting skin, caughing at time with plegm, frequent lung infections, shortness of breath, poor growth/weight gain in spite of good appitite, frequent greasy bulky stools or difficult bowel movements.

2. How common is this disorder?

About 1000 new causes are diagnossed each year.

3. How is cystic fibrosis diagnosed?

A sweat test can be used to diagnose a patient with cf. a high level of salt indicated cf.

4. How is cystic fibrosis inherited? Does everyone who has a mutant gene for the protein have cystic fibrosis?

Cf is inheretied through a mutant protein gene. No there is a 25% chance that they will not inherat cf.

Part 2.



In this part of the activity you will read an article to learn more about cystic fibrosis.



Use your browser to go to:



http://resources.schoolscience.co.uk/MRC/3/page3.html



Use the information in this article to answer the following questions:



1. Explain the normal function of the protein that is defective in cystic fibrosis.

There are channels in these lining cells through which ions can pass. Normally, the movements of ions brings water to the surface of the airway and keeps the mucus moist.

2. What happens to this protein in CF patients and what are the consequences for the health of these individuals?

The defective gene acts to block the channels, which causes the mucus to dry out. It is then difficult for a person to shift the mucus, which then becomes prone to infection by bacteria.



Part 3.



In this part of the activity you will read about how cystic fibrosis is treated.



Use your browser to go to:



http://www.mayoclinic.com/health/cystic-fibrosis/DS00287



Use the information in the “Treatment” and “Self-care” sections to answer the following questions:



1. Explain at least 3 treatments for the symptoms of cystic fibrosis.

One treatment they can give cf patients in medication. there are antibotics, mucus thinning drugs and Bronchodilators. they help stop lung infection, thins the mucus and makes it less sticky, and keeps the airway open.

Another treatment is Therapy. it physically loosens the mucus in the chest.

The last treatment is Surgical. like feeding tubes lung transplants and bowel surgery.

2. Discuss at least 3 ways for parents to help their children who have cystic fibrosis.

Parents should talk openly about there feelings. also the should join a support group for the child and last they should get there kid up and moving get them to loosen the mucus in there chest.