Cystic fibrosis (CF) isn’t the same disease it was just a few short years ago. Thanks to recent medication advancements, many children are experiencing improved health outcomes. As a result, it’s increasingly important to address their educational and mental health needs.

In recent years, Cincinnati Children’s pulmonologists have launched efforts through the CF Wellness and Education Learning Laboratory (WELL) to improve support for families who care for patients with this chronic, disabling disease.

“We know that when families don’t thrive, the care of children with cystic fibrosis suffers,” says Thomas Boat, MD, founding director of CF WELL. “Within CF WELL, we recognize that we must be more aware of and responsive to family needs, particularly as educational and mental health concerns for patients are changing.”

The New Face of CF

In October 2019, the landscape changed for most children with CF when a combination of three drugs—elexacaftor, tezacaftor and ivacaftor—received FDA approval. The mixture of medications, called Trikafta®, targets protein defects caused by the most common CF genetic mutation, F508del. This medicine improves lung function and quality of life for nearly 90% of patients.

Consequently, life expectancy for children born with CF has risen dramatically. Today, experts project many patients will live well into their 50s. It’s an improvement that’s significantly changed clinical care.

"With this greater life expectancy, we now need to focus more on how we’re going to promote wellness for these patients,” says Michelle Hjelm, MD, current CF WELL director.

Screening for Educational Achievement

Now that children with CF are living longer, ensuring their educational achievement is vital, Boat says. Most children will outlive their parents, so they will need the skills to manage their chronic illness, as well as be financially independent.

But the high school graduation rate for these children is dropping even as their health improves. It’s currently unclear why, and Cincinnati Children’s multidisciplinary team is searching for the answer. In the meantime, though, CF WELL is helping families navigate the school environment better.

Since 2017, Sarah Strong has served as the program’s school liaison specialist. She uses her knowledge of school culture to help parents advocate for their children and assist schools in better understanding families’ needs.

Strong works with 180 families with children in schools across Ohio, Kentucky and Indiana. Using a specially designed screening tool, she can determine whether a child is on track educationally and where they may need more help.

"Sarah administers this tool to families as a way to ensure a child is meeting the desired educational milestones,” Hjelm says. “And if they’re not, how do we encourage advocacy from within our program, as well as with their caregivers or parents?”

The CF WELL team published results that showed the importance and positive impact of having a school specialist in a recent Journal of Pediatrics article.

Based on results from these screenings, Strong counsels parents about a variety of topics, including how to help their children manage school accommodations, illnesses, peer relationships and extracurricular demands. She also helps them obtain and fill out any paperwork associated with the necessary accommodations for their children. For schools, she conducts virtual and in-person visits with staff and students to explain CF and the unique needs of these children, such as more frequent bathroom breaks or extra protections against viruses.

Expanding the Screening Process

This screening works so well that others have reached out for guidance. To expand the process, Cincinnati Children’s has secured grant funding from the Cystic Fibrosis Foundation (CFF) to create a formal collaborative to improve the available educational support and ultimately disseminate the Cincinnati Children’s process across other CF centers.

“We surveyed CF centers about where they needed help, and roughly 93% said they need more assistance with schools and school needs,” Hjelm says. “We must get centers more information because that means families invariably get more help.”

The goal, she says, is to create a standardized process that most centers can tailor to the specific needs of their patient population and area schools. The CF WELL team has also worked with the CFF to update their website with information around school needs through the COVID-19 pandemic, remote learning access, back-to-school routines and self-advocacy strategies.

Addressing Mental Health

Alongside providing resources to improve educational achievement, CF WELL is tackling mental health concerns for children with CF.

“We know that people with CF have a two to three times greater risk of having mental health symptoms compared to the general population. And children’s rates of anxiety and depression are higher if their parents have the same symptoms,” Hjelm says. “As providers, we’re investigating how we can be more mindful in our interactions with families and patients.”

In a study recently published in the Journal of Cystic Fibrosis, Hjelm and her colleagues highlighted the consistency in mental health screening at Cincinnati Children’s throughout the COVID-19 pandemic and introduction of Trikafta®.

Ultimately, CF WELL is actively invested in improving quality of life for children with CF, as well as making it easier for healthcare providers and schools to support and meet these children’s needs.

“We’re trying to provide comprehensive care for patients with CF. We don’t want to create more work for people with CF and their care teams,” she says. “We want to integrate this into their current work and build upon the great job they’re already doing.”