May is Cystic Fibrosis Awareness Month, bringing to light a rare disease that affects just over 30,000 Americans. Chances are you don’t know much, if anything about Cystic Fibrosis (CF), and I can’t blame you. I didn’t know about CF either, until my son Levi (now three) was diagnosed with it at just three weeks old. Three years later, I’ve learned a lot and want to share the awareness. Here are 10 things you probably don’t know about Cystic Fibrosis:
- Cystic Fibrosis is a genetic disease that primarily affects the lungs and digestive system. People with CF have thicker mucus in their bodies which clogs the airways and other organs leading to lung infections and problems with the pancreas (nutrient absorption and digestion). CF is very complicated and can also affect other organs, but is most commonly associated with the respiratory system.
- Most babies today are flagged for CF through newborn screening. Since 2008, Tennessee tests babies at birth for CF along with a number of other genetic conditions through a quick blood draw. Most parents never hear anything more about the test unless results come back abnormal, which is what happened to us when Levi was just 8 days old.
- People with CF release more salt when they sweat. CF causes a defective sweat chloride channel in the body, which leads to the issues I described above. In fact, measuring the amount of salt in a person’s sweat is how people with CF are diagnosed.
- There are more than 1,800 mutations that cause cystic fibrosis, making it hard to find a “one size fits all” cure for the whole population of people living with the disease.
- People with Cystic Fibrosis require extra maintenance to stay healthy. Levi’s daily routine includes inhaled medication and vest therapy (a machine that helps break up the mucus in his lungs through vibration) for 30 minutes twice a day, taking pancreatic enzymes before he eats to ensure he absorbs his food and gains weight, and vitamins. If he gets sick, which for him, can mean a runny nose or a cold, he does his vest treatments four times a day. Many adults with CF can’t work because they must dedicate a large part of their day to treatments. Outside of his daily routine, Levi visits a specialty CF clinic several times a year, where he is seen by a team of specialists to monitor his health.
- A little dirt can hurt. Even though Levi has a normal immune system, we are super cautious about germs and keep hand sanitizer companies in business. I can hear someone coughing from the other side of Target and sometimes have to say no to social situations where there may be runny noses. Like I mentioned, a cold can mean doubling treatment time, so we try to prevent illness as much as possible. Additionally, harmful bacteria can be found in soil and water, so we wash hands constantly.
- People with CF don’t look sick. You would never know by looking at Levi that he has cystic fibrosis. Instead, you’ll see a wild haired, blue eyed little boy who loves music, swimming and Paw Patrol. He goes to a Parents’ Day Out program, and we love attending sporting events, festivals and traveling, we just always make treatments a priority and pack that Purell.
- You could be a carrier for CF and never know it. About 1/25 people are symptomless carriers for the disease. If two carriers have a baby, there is a 1 in 4 chance the child will have cystic fibrosis. Most women never think to be screened for CF until after they are pregnant, but a simple blood test can tell you if you or your partner carries the most common CF mutations. We had no idea either of us were carriers until Levi was diagnosed.
- People with CF can’t be around each other. Due to CF specific bacteria in the lungs that can be harmful to another person with CF, care guidelines mandate that people with CF not be in the same room together and be at least six feet apart at outside events to prevent cross infection. I’ve met so many wonderful parents of children with CF, but unfortunately, our children will never meet, making it a pretty lonely disease.
- There is a tremendous amount of hope for the future of people with CF. In the 1950s, children with CF were not expected to live to grade school. Today, the life expectancy is 41 and rising. What’s more exciting though, is the amount of research and science pointing toward lifesaving medicines and therapies, including drugs that correct CF at the genetic level. Some of these drugs are on the market to a select group of patients now, with better, stronger drugs in the pipeline that will hopefully benefit a much larger population. We are confident Levi will have access to miracle drugs, if not a cure in his lifetime.
To learn more about Cystic Fibrosis, visit the Cystic Fibrosis Foundation website, where you can also make a donation to support research for drugs and therapies.