Ten Surprising Facts About CF

Even after living with CF for 31 years, it seems like everyday I learn something new about this disease. CF is such a weird, unique, and frankly, surprising disease when you really look at it. There aren’t many people who realize this, but after reading about the history, development of treatments, and lifestyle of someone with CF, it’s quite obvious.

CF is tough to deal with, but it really is a cool and unique illness!

Did you know?

1. Pulmozyme’s weird ingredient

Pulmozyme, an inhaled CF medication that cuts up extracellular DNA to thin mucus, was developed from a bizarre source: Chinese hamster ovary cells. In fact, people with a hypersensitivity to hamster ovary cells are instructed not to take Pulmozyme. Pulmozyme was first developed in 1993 and was the first drug designed specifically for CF.¹

2. CF was first identified by a woman

In 1938, Dr. Dorothy Andersen made an astounding discovery when she identified cystic fibrosis after performing autopsies of children who were believed to have died from Celiac disease. She noticed the unique scarring of the pancreas and was able to determine CF was a completely separate disease. From her research, she created a test to diagnose cystic fibrosis.²

3. Sweet as grapes

Pseudomonas aeruginosa is a common bacteria found in the mucus of people with CF. It has a bizarre and distinct smell resembling a sweet fruit: grapes.³ (Honestly, this grosses me out because it's so true.)

4. The legend of CF

CF folklore can be traced back to the Middle Ages. Stories warned, “Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon must die.”⁴ Many scholars believe this is a description of CF even back then.

5. A different type of tent sleeping

Back in the 1960s and 70s, CF treatments were limited. People with CF slept in “mist tents” that circulated cool, moist air to moisten and loosen the sticky mucus.⁵ The tents looked much like a shower curtain surrounding the head of the bed.

6. Cystic fibrosis is a nickname

The term “cystic fibrosis” is actually a nickname for another name of the disease, mucoviscidosis. The name combines two Latin based words “mucus” and “viscidus”  which means “viscous mucus”.⁶

7. CF carriers are more at risk of severe COVID-19

CF carriers have been found to be more at risk of severe COVID-19 than non-CF carriers. Researchers recorded a higher inflammatory response in carriers than controls and an increase in the risk of 14-day death rate according to a study conducted in Italy.⁷

8. Famous composer Chopin died from CF

Frederic Chopin, Polish-born composer and pianist, was believed to have died from CF at age 39. Although his death certificate states tuberculosis, Chopin suffered from lifelong lung infections with hemoptysis, chronic cough, poor weight gain, infertility, and fatigue. Upon his autopsy, no classic signs of tuberculosis were found but he did have extreme lung damage and an enlarged heart.⁸

9. CF doctors integral in development of heparin lock

A heparin lock is when heparin, a blood thinner, is pushed through an IV line and clamped to keep the line open and free of clots. This practice allows the patient to move freely instead of being bed bound with an IV pole with continuous medications or fluids. Today, this practice is almost universal with IV infusions. Coincidentally, it was a team of CF doctors who pioneered the development of the heparin lock in the 1960s. It allowed their patients to receive antibiotics and participate in airway clearance.⁹

10. Salt water is a natural CF treatment

Many people with CF have reported feeling better after visiting or moving to the beach. It’s true that saltwater is a natural treatment for people with CF.¹⁰ It thins and rehydrates the sticky mucus. It’s so effective that many people inhale salt water through a nebulizer for the same effect.