May: Cystic Fibrosis Awareness Month

by Elizabeth McDonald Blume and Sydney Hubbard, second year genetic counseling students

Ricky’s 65 Roses
In 1965 Ricky, a 4-year-old boy, inspired people for generations to come with one simple phrase, “65 roses.”¹

Ricky used to listen to his mother Mary make dozens of phone calls to friends, neighbors, and community members. Mary was a volunteer and advocate for the Cystic Fibrosis Foundation, and she spent her time calling various organizations to raise money for cystic fibrosis (CF) research. Mary’s three young sons, including Ricky, were all diagnosed with CF. In the 1960s, CF was a devastating genetic condition, it had just been discovered less than 20 years earlier and few doctors understood how to treat children with CF. Ten years earlier, in the 1950s, the life expectancy for a child with CF was only around 4-5 years 2. Mary, just like so many other families, was desperate for treatment for her children.

Ricky, not knowing about his CF diagnosis, would listen to his mother make these calls day to day. One day, he said to his mother, “I know what you are working for.” Mary was stunned, Ricky did not know what she was doing, nor did he know he even had CF. Ricky then said, “you are working for 65 roses.” The scary and tricky to pronounce diagnosis of “cystic fibrosis” seemed so simple and beautiful in the eyes of 4-year-old Ricky… 65 roses.

Since then, “65 roses” has been a term used by the CF community and its friends to inspire each other to keep fighting and to find a cure for all CF patients. CF research has exploded since the 1960s and now, in 2023, the life expectancy for CF patients is upwards of 48 years! Life changing medications and treatments have been developed that dramatically increase the quality of life for CF patients. But not everyone with CF has benefited from these discoveries. Some patients do not respond to medications or may have symptoms too severe for medication to help. So, the fight is not yet over.

Each May, for CF awareness month, CF patients, their families, and friends will celebrate the breaths they took this year and the progress made towards a cure for all. The CF community will come together to share experiences, educate others, and celebrate progress in CF treatment.

Join the Omaha community at the Cystic Fibrosis Foundation’s 2023 Great Strides Walk on May 20th in Turner Park Omaha, NE! Great Strides is a community walk to celebrate the great strides made in CF treatment. Great Strides walkers can participate in person or virtually. All participants can fundraise with tools in the Fight CF mobile app or with Facebook Fundraising. The CF Foundation is a national organization dedicated to ongoing research for CF treatments, with the hope of eventually curing this disease. In other words, no person is left behind and the work is not done until there is a cure for all patients with CF.

What is CF?
Cystic fibrosis (CF) is a rare genetic condition that affects about 30,000 people in the United States. About 1,000 babies are born with CF each year and there are around 270 people with CF in Nebraska. CF affects people of all racial and ethnic groups.

Some people may have heard of CF as being a condition that affects the lungs. What people may not know is that CF also affects other parts of the body like the digestive system and the pancreas.
People with CF often have thick sticky mucus in their lungs which makes it harder to breathe. This sticky mucus also makes it easier for bacteria to get trapped in the lungs. This increases the chances someone with CF will get lung infections throughout their life. This sticky mucus also clogs up the digestive system. The digestive system is made up of organs like the intestines, stomach, pancreas, and liver. The pancreas is important in making liquid that helps the body break down fats, sugars, and starches. Without this liquid, is it hard for people with CF to absorb the nutrients from the food they eat.

How is CF treated?
There is no cure for CF but there are many medications and treatments that help to improve the quality of life for those with CF. People with CF may have different breathing treatments, wear a vest that shakes up the mucus in their lungs, and take medicines. Until recently many people with CF were not living to adulthood. Now, thanks to increased research on CF treatments, many people are living into their 30s, 40s, and 50s, going to college, pursuing careers, and having children of their own.

Genetics of CF
CF is a genetic condition caused by changes in a person’s CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene.

Changes cause the CFTR gene to not work like it should. Genes are important pieces of our body’s genetic instructions. We have two copies of each gene, one from each parent. To have CF you must have a change in BOTH copies of the CFTR gene, one from each parent. According to the CF Foundation, there are 1700 known CF-causing changes in the CFTR gene – some are common, while others are only found in a few people. It can be hard to find medications that work for these different genetic changes.

When is CF diagnosed?
Since 2006, all babies born in Nebraska have been tested for CF through newborn screening (NBS). NBS is required in Nebraska and is done by collecting a few drops of blood from a newborn. Some people who do not show many symptoms might not be diagnosed until later in life.

What can we do now?
We think Mary would be happy to see that her work in raising money and awareness for CF research and treatment has been picked up by many others resulting in considerable progress. But she would also recognize that the work is not yet done.

So, this May when you see the roses start to bloom think of Ricky and the 30,000 other individuals across the U.S. with CF. We can celebrate the progress that has been made but continue to work towards a cure for ALL people with CF. We hope to see you at Turner Park on May 20th to learn more and celebrate the CF community!

To learn more about CF visit the Cystic Fibrosis Foundation website.

1. 65 Roses Story. Cystic Fibrosis Foundation. https://www.cff.org/about-us/65-roses-story
2. McBennett, K. A., Davis, P. B., & Konstan, M. W. (2022). Increasing life expectancy in cystic fibrosis: Advances and challenges. Pediatric pulmonology, 57 Suppl 1(Suppl 1), S5–S12. https://doi.org/10.1002/ppul.25733