10 Little-Known Weird Facts About Cystic Fibrosis

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by Brad Dell |

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After several decades of research into cystic fibrosis, there’s a wealth of weird information out there about the disease, its common bacteria, and medications. How many of these did you know already?

1. It is hypothesized that cystic fibrosis genetics have resistance to infectious diseases such as tuberculosis, cholera, and typhoid fever.

2. Pulmozyme is made of genetically engineered Chinese Hamster ovary cells. Some pancreatic enzymes, such as Creon, are made of pig pancreas.

3. Pseudomonas aeruginosa is said to smell like tortillas or sour grapes in the laboratory.

Pseudomonas aeruginosa

Pseudomonas aeruginosa (bastonete Gram negativo)

4. In 16 countries with large CF populations, the number of patients living into adulthood is expected to increase approximately 75 percent by 2025.

5. It’s common for people to not be diagnosed with CF until adulthood. But some have even lived into their 60s before being diagnosed.

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6. In the 1960s, it was popular to have patients with CF sleep in “mist tents,” with the belief that a fine mist would thin mucus in the lungs. This was also recommended by the National Cystic Fibrosis Research Foundation (now called the Cystic Fibrosis Foundation).

7. Gallium, a metal molecule similar to iron, is being tested in IV form to fight CF infections.

8. A natural antibiotic produced by Burkholderia bacteria in CF lungs could be used to fight tuberculosis.

9. While cystic fibrosis is primarily found in Caucasians, it affects other ethnicities as well: about one in 17,000 African Americans and one in 31,000 Asian Americans suffer from the disease.

10. Cystic fibrosis is considered a Mendelian disorder — a condition caused by the mutation of a single point in a gene. “Cystic” means something is characterized by “cysts.” “Fibrosis” is the scarring of connective tissue. Despite commonly being called a “lung disease,” “cystic fibrosis” refers to the cysts and scarring on a CF patient’s pancreas.

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