Despite the extreme amounts of hand sanitizer, social distancing, and vaccinations, Audrey has had a banner year. Here are just a few of her CF related highlights:
• Right around when Audrey was 16 months old, her chest circumference was large enough to use a High-Frequency Chest Wall Oscillation vest (AKA "the vest"). This machine is made up of two pieces, an air-pulse generator and an inflatable vest that is connected to the generator by hoses. The generator sends air through the hoses to the vest causing it to vibrate quickly to loosen mucus in her lungs. Audrey does her vest twice a day for 15 minutes at a time. We are as surprised as you that we can get a very active toddler to sit still for 30 minutes a day, but she is in a good routine at the moment. She sits, brushes her teeth and watches her favorite TV shows. It has been a nice way to ease into an early morning and signal that it is nearly time for bed.
The vest has been such a game changer for us. Prior to the vest, Audrey's caretakers (parents and grandparents) were doing manual chest physiotherapy (PT) multiple times a day. We would use a small cup like item to simulate the feeling of wearing a vest and getting her used to having her lungs vibrating. When she was a tiny immobile infant, manual chest PT was pretty simple. But as she continued to grow along with her independence, it became very difficult. We are very thankful for this modern convenience! Copy and paste following link into your browser to watch Audrey practice her scales while wearing her vest: https://www.youtube.com/watch?v=1dTRjhBs9E8
• Additionally, Audrey is now enrolled in the BEGIN Research Study. This is a three year, two-part observational study that will look at the effects of modulators on grown in infants and young children with CF. A modulator is a medication, such as Trikafta, that works to correct the faulty CF gene and treat at person's CF symptoms. Audrey is in the first part of this study, which evaluates information about children's height and weight prior to taking modulators. Trikafta is not yet approved for children under the age of 12. Clinical trials have finished for ages 6-11 and their evidence has been submitted to the FDA for approval by June 2021. Recruitment for clinical trials for children ages 2-5 is underway. Audrey's wonderful CF Team at Children's Hospital is confident that Audrey will be eligible to take Trikafta around age 3.
If you are a person with CF, it is fairly inevitable that you will participate in a research or clinical trial at some point in your life. Keep in mind that there are only about 30,000 people in the United States living with CF. That is a pretty small sample size to draw from at any given point! We felt that this was a good opportunity to get Audrey involved in CF research and it requires no more effort or work on her part other than attending her quarterly CF visits. A win for Audrey and a win for CF research!
Great Strides matters more than ever this year. Not only because the Foundation has not been immune to the financial strains of COVID-19 and donations are down 40%, but more importantly because of every single facet of Audrey’s care mentioned above. If we did not have access to health insurance or the ability to pay the copay on Audrey’s vest, the Cystic Fibrosis Foundation would provide resources and assistance to navigate these psychosocial challenges. Trikafta’s funding and research were financially supported by the CF Foundation. And Trikafta represents just a small sliver of the research the Foundation makes possible. From studying difficult to treat lung bacteria to varying disease trajectories based on genetic mutation to gene editing cures, the Foundation is involved in every aspect of a CF person’s life. The Foundation has certainly been involved in Audrey’s life since day one and we know they will continue to be present until CF stands for Cure Found. To support Audrey and the CF Foundation, please click the bright yellow button to the left saying “Donate to Chris & Emilia” or “Join Chris and Emilia’s Team”. There’s no doubt Audrey is Worthey of a cure!
Due to the COVID-19 pandemic, indoor events sponsored by the CF Foundation are strictly prohibited at this time. Scheduled outdoor events must adhere to strict requirements to minimize the risk of COVID-19 infection. Events may be subject to change at any time based on guidance from the Centers for Disease Control and Prevention and local health officials.
IMPORTANT NOTE ON ATTENDANCE AT OUTDOOR FOUNDATION EVENTS:
To minimize the risk of COVID-19 infection, attendees at CF Foundation events must adhere to the following requirements:
Practice physical distancing and maintain at least a safe 6-foot distance from persons outside of their household at all times
Face masks strongly encouraged and expected to be worn in accordance with local guidelines. (Note, children under two years of age should not wear masks due to safety concerns and therefore should not attend CF Foundation events where there is risk of interacting with someone outside of their household.)
Follow basic infection, prevention and control practices by regularly washing hands with soap and water or with an alcohol-based hand gel, covering your cough or sneeze with a tissue or your inner elbow.
Persons with CF should consult their physician before participation in any in-person event as they may be at an increased risk for severe illness from COVID-19. People with CF should maintain a safe 6-foot distance from persons outside of their household at all times.