Chris & Emilia Worthey

Year five of Great Strides and we are full of hope and excitement for our favorite girlie! As of May 2023, Audrey has been lucky enough to take the revolutionary drug Trikafta. Serendipitously, Audrey was diagnosed on the same day (back in 2019) that Trikafta was approved by the FDA for adults. So we have been waiting roughly 3.5 years for it to be approved for young children. Trikafta comes in packaged granules and is shipped to us monthly from a specialty pharmacy. She takes two doses a day and it’s recommended to take with a fatty meal (don’t you wish you were ordered to eat a fatty meal twice a day?).

Simply put, Trikafta has been a game changer for Audrey. She’s been sick far less than prior to taking Trikafta. Additionally when she is sick, her cough is manageable and she’s most of the time she’s able to recover without antibiotics. We also have data to show that Audrey is responding positively to Trikafta. A sweat test (the gold standard of diagnosing CF and monitoring chloride in sweat) post Trikafta has shown that Audrey’s sweat chloride value has dropped dramatically. In fact, her sweat chloride value is now so low that it is below the threshold in which a person would be diagnosed with CF. Amazing, right?! That dramatic drop in her sweat chloride is all thanks to Trikafta! 

Audrey still sees her CF team at children’s hospital on a quarterly basis. The newest addition to these visits is a Pulmonary Function Test (PFT). This test measures the size of the lungs and how well the air flows in and out of the lungs. Audrey had to wait until she was about 4 years old to start PFTs, as it’s a test that requires correct technique to do with the right amount of inhalation and exhalation. Her PFT values are normal right now and will be another layer of information for us to gather about her lungs as she grows. 

Our Awesome Audrey will go to kindergarten in August! We’re so excited to be able to walk her to school every day (about an 8 minute walk)! We plan to work with her elementary school to create a 504 learning plan for Audrey that will ensure that she has every opportunity for success. 504 plans are unlike IEPs, in that they are not designed for kids with special needs, but really to level the playing field for kids with “disabilities”. We’ve already chatted with our CF team about her prospective 504 plan and learned that it usually includes things like unlimited bathroom breaks, self-administering medication, flexibility with absences due to illness and extended time on assignments due to illness/inpatient status. This was definitely not something I thought about as a parent of a child with CF, and also thankful such things exist. 

Audrey was named one of the amazing ambassadors for CFF Rocky Mountain this year. This means we will be sharing her story (and those gorgeous baby blue eyes) at Great Strides and other events throughout the year. We continue to believe in the mission of CFF and in the great, innovative work they are doing. On the horizon is CFF funding another ground breaking modulator (similar to Trikafta) to help those living with CF. I’ll also note that Trikafta is not available to all people living with CF. Around 10% of people do not have the correct gene mutation and/or cannot tolerate taking Trikafta. There’s more work to be done and there’s only one organization who can pave the way. We ask that you considering donating today so that CFF can continue its mission and make CF stand for Cure Found. Audrey is Worthey of a cure!