Effects of Cystic Fibrosis

Question 1

what happens when there is too little water in the mucus in an unaffected person?

Answer 1

Too little water in the mucus is detected by the epithelial cells

CFTR protein channels open

Chlorine ions diffuse out of the epithelial cells into the mucus through open CFTR protein channels and sodium ions follow.

this increases the solute concentration of the mucus so water moves out of the epithelial cells by osmosis.

This increases the water content of the mucus

The mucus is no longer thick and sticky.

Question 2

what happens when there is too little water in the mucus in a person with CF?

Answer 2

Too little water in the mucus is detected by the epithelial cells

CFTR protein channel is absent or no functional.

Chlorine ions cannot diffuse out of the epithelial cells into the mucus so sodium ions do not move into the mucus.

No water enters the mucus by osmosis

Water content of the mucus remains too low.

Mucus is thick and sticky.

Question 3

How does having CF affect the respiration system?

Answer 3

cilia finds it hard to move the thick and sticky mucus 
blocks the airways 
prevents movement of air into alveoli 
reduces S.A. for gas exchange
concentration grad. reduces. 
diffusion is slower

Question 4

How does having CF affect the digestive system?

Answer 4

enzymes start to digest pancreatic cells
can lead to diabetes if insulin producing cells are damaged.

Thick and sticky mucus blocks pancreatic duct
enzymes cannot be secreted to small intestine
food not fully digested
fewer nutrients absorbed
leads to weight loss and malnutrition

Question 5

How does having CF affect the reproductive system in women?

Answer 5

Women have a reduces chance of pregnancy as thick and sticky mucus blocks the cervix.

Question 6

How does having CF affect the reproductive system in men?

Answer 6

Sperm ducts are blocked by thick and sticky mucus.
Sperm can’t leave testes.
Leads to infertility.