Cystic Fibrosis: Cause, Progression, and Treatment

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  • 0:03 A Sticky Mess
  • 0:51 What is Cystic Fibrosis?
  • 1:49 Why Does Cystic…
  • 3:56 Clinical Signs,…
  • 5:51 Treatments for Cystic Fibrosis
  • 6:33 Lesson Summary
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Lesson Transcript
Instructor: Artem Cheprasov

Artem has a doctor of veterinary medicine degree.

This lesson will discuss a genetic disorder called cystic fibrosis as well as it how it affects the respiratory and digestive system, the ways by which it can be diagnosed, and treated.

A Sticky Mess

During the summer, I enjoy hooking up the slip-and-slide I have to the hose, turning on the water, and then having a blast. The reason this is all fine and dandy is because the water makes it easier for my body to slide across the, well, slide!

But imagine if, instead of water, the slide was chock full of gooey and sticky honey. I can tell you for sure that, I, instead of sliding, I'd just be slopping in one place on those hot summer days.

Sadly, certain fluids in your body can also transform into gooey and sticky messes that impede proper organ function, resulting in a person's eventual death. We'll be learning about a famous condition that causes precisely this problem.

What Is Cystic Fibrosis?

This problem is known as cystic fibrosis. This is an autosomal recessive genetic disease that affects proper exocrine gland function, resulting in multiple organ dysfunction, including that of the lungs, pancreas, liver, and intestines.

Exocrine glands are the glands in your body that excrete something such as digestive juices, mucus, or sweat via a duct into an external environment. For example, the pancreatic duct serves as a conduit for pancreatic digestive enzymes to be excreted from the pancreas and into the duodenum.

Or as a more familiar example, your sweat glands are exocrine glands because they secrete sweat out into the environment as opposed to dumping it into the bloodstream, which is what endocrine glands do with the hormones they produce.

Why Does Cystic Fibrosis Occur?

Cystic fibrosis occurs as a result of a mutation in the CFTR, or cystic fibrosis transmembrane conductance regulator protein. This protein is actually a channel that transports an ion called chloride. To remember that chloride transport is coded for by the CFTR gene associated with cystic fibrosis, note how all three of them begin with the letter c, there to remind you of chloride.

When it comes down to the lungs, if a mutation in the CFTR gene occurs, this channel does not allow for chloride to exit out of the cells that produce the mucus in the lungs. This, by extension, will cause sodium and water to move into these cells, thereby reducing the amount of water in the mucus. This causes the mucus in the lungs to become more viscous and sticky.

Think about this. I'm more than sure that at one point you spilled some sugar filled coffee, tea, or some fruit juice onto a countertop. If you left it there long enough, the water in the liquid that you drank slowly evaporated. As the water disappeared, the fluid became gooier and stickier. This is what happens to the exocrine gland secretions that do not have enough water contained within them.

The big problem here is that this sticky mucus clogs the airways, is difficult to expel, and thereby traps bacteria in the lungs. This causes persistent or recurrent infections, inflammation as a result of infection, destruction of the lungs as a result of the long standing or recurring inflammation, chronic obstructive pulmonary disease, and eventually, end stage lung disease as the major cause of death in people with cystic fibrosis.

Clinical Signs, Symptoms, and Diagnostics

Furthermore, these pulmonary infections result in a person coughing, wheezing, and having difficulty breathing.

Terribly, this isn't where this condition ends. Besides causing chronic infection of the airways, people with cystic fibrosis can also experience:

  • Exocrine pancreatic insufficiency, causing problems digesting food
  • Infertility, especially in men
  • Meconium ileus, which is where a newborn's first stool (called meconium) is unable to be passed due to its stickiness, thereby creating a blockage in the portion of the small intestines called the ileum.

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