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Cystic Fibrosis Family Education Night Shares Hope and Help

Posted on Mar 23, 2018

Connor was a 3-month-old baby with a bad cough. His pediatrician suspected RSV, a common illness in young kids. Testing revealed the real culprit: cystic fibrosis (CF).

Now 13, he has been receiving care at the Cystic Fibrosis Center at Johns Hopkins All Children’s Hospital ever since that diagnosis. As his family began to learn about CF, they worried what Connor’s future would hold.

Each year, Connor’s mom, Heather, and his sister, Laura, attend the center’s family education event. It’s for family and friends only—not patients— to learn more about this chronic illness, find out about the newest research developments, meet other CF families, and check out new CF products and services.

In the early years, Heather would have to step away during the presentations. “I would escape to the restroom to cry,” she says. “The pictures were scary and the stories were sad. Thankfully, things are so different now.”

Precision medicine is bringing hope for a growing number of people with CF. The disease is caused by a faulty gene that contains more than 2,100 known mutations. In recent years, scientists developed two medications that can precisely target some of these mutations, and a third was approved by the Food & Drug Administration in February 2018. Additional personalized therapies that may help a greater number of CF patients are in the research pipeline.  

Connor began receiving targeted therapy last year. His condition has stabilized—it was the first year his lung function didn’t decline. He has been able to attend school for a few days a week, and is a straight A student.

At this year’s event, CF Center Director Deanna Green, M.D., reviewed the most common CF mutations and highlighted the role of these targeted medications. As a Cystic Fibrosis Foundation Therapeutic Development Network Center, the program can give patients early access to the latest therapies and clinical trials.

“We will fight to keep your child as healthy as possible while we fight for a cure,” Green promised.

Families also learned about an upcoming pilot program that will offer online counseling and education for teen patients with CF. 

Everyone at the event had looked forward to meeting Gunnar Esiason, a 27-year-old with CF. He’s president of the Boomer Esiason Foundation, which supports CF research, education and advocacy and provides grants and scholarships to patients and families. A health issue―something CF families understand quite well―meant Gunnar had to stay home in Boston. Via Skype video, he shared his story, from doctor visits at age 5 to his first boy-girl party, his last game as a high school quarterback, his years at Boston College and his active life today, and the impact of his parents’ and sister’s support throughout this journey.

Alexandria “Alex” Twigg, a CF patient, advocate and fundraiser, was also at the conference to share her story. She was diagnosed at age 9 and received care at the CF Center. While attending college out of state, she became critically ill with pneumonia and returned to Johns Hopkins All Children’s for a two-week hospital stay. “I wouldn’t be here today if it weren’t for Dr. Green,” she says.  After recovering from that scare Alex went on to graduate from college closer to home. Now she’s an active young professional who receives care at Tampa General Hospital’s adult CF Center.

Answering questions, Gunnar and Alex stressed the importance of encouraging teen patients to become responsible for their daily CF care routine, while being clear that skipping treatments is not an option. They discussed feeding tubes, nutrition, exercise, rest, mental health, and helping friends understand what it means to have CF. Most of all, they urged parents to give kids and teens with CF opportunities to succeed at life.

One parent spoke up with thanks instead of a question. After Hurricane Irma damaged the roof of the family’s home, CF Center Coordinator Megan Krygowski, R.N., encouraged them to apply to the Boomer Esiason Foundation for a grant to cover the deductible. The evening was an opportunity to personally thank Gunnar for that amazing gift.

It was clear that participants consider the CF Center team to be part of the family, too. A family that inspires parents to help kids and teens with CF to aim for the best possible health, and look forward to new discoveries and cures.

Learn more about our CF treatment and research programs.

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