65 Roses

From Jane’s World

When my grandson was just a kid, he'd tell people that his sister, Helena, had “65 roses.” Ethan is an adult now and knows how to say cystic fibrosis

Cystic fibrosis (CF) is a progressive genetic disease that causes persistent lung infections and, over time, limits the person’s ability to breathe. 

Wisconsin screens newborns for 45 different conditions, including cystic fibrosis. Helena was diagnosed with CF at birth. It can be heartbreaking to hear the misconceptions about CF. It’s not contagious, nor does it come from eating white bread, drinking soda, or watching too much television. Although I’m certain none of those support a healthy lifestyle, CF is due to a mutation in a gene on chromosome 7. 

For as long as I can remember, Helena has had to use a special vest and an airway-clearing device. When well-meaning friends and family learned of her diagnosis, they worried she wouldn’t be seen as normal, but if you saw Helena without her medical tools, you wouldn’t know she had CF. She’s a normal kid who recently graduated from high school. 

Staying active helps people with cystic fibrosis keep their lungs strong and clear. Helena is good at staying active: She’s been involved in karate, basketball, hip-hop and other kinds of dance, and track. But she’s not as compliant when it comes to using her vest or taking her medication now that she is older. What teen who generally feels good wants to slow down long enough for that?

In the 1950s, a child with CF rarely lived long enough to attend elementary school. Today, more than half of the CF population is over 18. The life expectancy of people with CF has been extended by breakthrough treatments: Now, the median predicted survival age is close to 40.

That doesn’t mean there aren’t challenges. As I write this, Helena has been admitted to Children’s Hospital of Wisconsin due to a cold that left her lungs functioning at only 65 percent of their capacity. The medical team inserted a PICC line, started her on antibiotics, and will treat her aggressively to fight the infection. Helena is no stranger to Children's Hospital. As a baby and a young child, she spent one day of every three months there, and she was hospitalized twice before she was even a year old. 

When I called Helena today at the hospital to check on her, she said, “I’m doing okay, Grandma.”

I have no doubt that she is. Helena is tough and has worked hard at keeping herself healthy. She has a bright future ahead of her. Although cystic fibrosis will always be a part of Helena’s life, her plans are to “kick its butt!”

I’ve been proud of how Helena has not allowed CF to rule her life. There is no cure and yet we can be thankful the treatments keep improving. I hope that someday the life expectancy for people with cystic fibrosis, like Helena, reaches 80 or even 90 years old. 

For more information on CF, please go to https://www.cff.org/