There is no cure for cystic fibrosis at this time. However, there are both clinical and at-home treatments available.
The Lehigh Valley Cystic Fibrosis Center at Lehigh Valley Reilly Children’s Hospital has cared for Aria and Jack from day one. The siblings are currently receiving care from Dr. Logan, who the Hespells say is “wonderful.” “We know our children are in good hands with Dr. Logan and we really appreciate her,” Adelina says.
Aria and Jack’s extensive treatment plan consists of the siblings returning to the CF center every three months for 3.5 hours of treatment. To maintain their best possible health, the siblings also have a daily at-home treatment regimen that consists of taking inhaled medications two times a day as well as chest physiotherapy for 20 minutes, twice a day. With chest physiotherapy, the siblings have to wear a vest that’s designed to shake up the mucus that they may have in their chest and help them expel it.
“CF is an everyday battle for Aria and Jack,” Adelina says. “Their at-home treatment is a daily regimen that they have to do every single day, no exceptions. That includes holidays, birthdays, special events – there are no days off with their treatment.”
Aria and Jack also both take Trikafta, a gene modifier therapy, which Adelina believes is “really transforming how we think about CF.” And with the positive impact this gene modifier drug has had, the Hespells feel incredibly fortunate for this medical advancement and others that have been made over the last 11 years.
“Trikafta is one of several highly effective modulator therapies (HEMT) that we now have available for people with CF,” Dr. Logan says. “HEMT medications, like Trikafta, are the first medications that work to correct the underlying problem with CF to effectively improve the lives of people with CF.”
Because of the CFF’s continuous investment in finding a complete cure, Dr. Logan says “so many people with CF are now healthier than ever” and that with new therapies on the horizon, “the future looks very bright for people with CF.”
“We see the difference CFF is having with the medications and treatments that are available today that weren’t around when Aria was born,” Adelina says.