Sunday, May 21, 2017

CF Awareness Month Day 21 - What is CF?

It strikes me as a little silly that this topic would come up 3/4 of the way through the month! Haha.  Hopefully if you're reading these daily posts, by now you have some kind of idea what CF is.  But just in case...

CF stands for cystic fibrosis.  It is a recessive genetic disease that primarily affects the lungs and digestive system, though other systems are often affected as well.  The main problem with CF has to do with a defective protein in our cells that doesn't function properly and results in the production of thick, sticky mucus throughout our bodies.  This mucus causes various problems, but the most prominent is that bacteria get stuck in it, and the mucus itself doesn't clear out like it's supposed to because it is so thick and sticky.  Therefore, the bacteria thrive in the mucus that is more or less trapped in our bodies, which causes multiple issues.  Typically, this infectious bacteria is found in our lungs and sinuses, making it difficult to breathe.  Eventually, the recurring infections cause scarring in our lungs, making it even harder to breathe, and resulting in the loss of lung function.  The thick mucus also oftentimes blocks the production of enzymes in our pancreas that are necessary for digesting and absorbing fat.  This means that without pancreatic enzyme supplements, many CF patients are unable to properly digest fat, which leads to malabsorption and nutritional deficiencies.  CF and its required care is often very strenuous on the body, and as people with CF age, other health issues can arise from their CF complications, such as CF-Related-Diabetes and CF-Related-Arthritis.

Most CF patients must do breathing treatments and some form of airway clearance to keep their lungs as clear as possible from that sticky mucus.  Most patients also take some kind of pancreatic enzyme supplementation to help digest their food.  From time to time, most patients will also need course of antibiotics to treat lung infections.

There are many new treatments available these days to help fight the effects of CF, and even some new drugs that are meant to address the root problem are correct the defective protein that causes the thick mucus in the first place.  It's an exciting time for CF Research.

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