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Anyone commit to a New Year’s resolution? Possibly saving more or paying down debt, living a healthier lifestyle or being more charitable? How are you doing on that commitment? Alexa and Presley could be the poster-children for dedication and follow-through.

Can you think of something that you do every single day? If so, it is probably something you enjoy like drinking coffee, reading the newspaper, or scrolling Facebook or Twitter. Now try to think of something that you do every single day that isn’t for enjoyment. This is much harder because we usually take a break from obligation at least a few days a year. Does someone with the stomach flu drink their obligatory 8 glasses of water a day? Does a person with influenza get out of bed to exercise?

That’s why people with cystic fibrosis live a life unlike the rest of us. Alexa and Presley spend 30 minutes in their vests doing airway clearance twice a day. No exceptions. They have done vest therapy in the car, at the airport, with friends over, on their birthdays and on Christmas morning. Even when they are ill and don’t want to leave their beds, they get up and shake for 30 minutes to clear mucus from their lungs. Alexa and Presley never take a day off. Not from vest and nebulizer treatments. Not from a strict high-calorie diet. Not from medications. We saved Alexa and Presley’s empty medication containers last year and they filled a 30-gallon trash bag! (see photos). And yet they smile and laugh and find joy in every single day.

In January, Alexa started Orkambi, a drug newly approved by the FDA for her age group. Orkambi is the only medication currently available that targets the underlying cause of CF for her mutation. Alexa got a cold several weeks after starting Orkambi and suffered considerable sinus congestion and a concerning cough. We only recall one time in her 11 ½ years when she has recovered from a cold without an antibiotic. This time, Alexa was insistent on seeing if Orkambi would help her body work well enough to fight the cold off on its own. We were hesitant and watchful, but held off on calling the CF clinic. Remarkably, we watched Alexa’s symptoms improve, and after a week, her symptoms disappeared. We were in awe and it gave us a small glimpse of a light at the end of the tunnel. And we watched our little girl get a glimpse of “normal.”

However, Orkambi is not without side effects. It has caused elevated liver enzymes in some patients which is concerning given Alexa’s pre-existing CF liver disease. Her liver enzymes will be monitored closely but thankfully, her recent blood work does not indicate any liver complications from the medication at this time.

Presley continues to thrive as well, despite CF. Much like Alexa, she often requires antibiotics to fight off colds, but she has avoided any serious illness this past year. Presley has also started to do pulmonary function tests at the CF clinic and her results are very good. Presley is excited to start kindergarten in the fall and will be eligible to start Orkambi when she turns six on November 18^th!

Research funded by the Cystic Fibrosis Foundation resulted in the discovery of Orkambi. While we rejoice in this discovery, more promising drug combinations are on the horizon with even better results and fewer side effects.  The CFF remains steadfast in their commitment to finding a cure for all CFers and we remain dedicated to doing our part in making sure that happens. The ultimate reward for Alexa and Presley’s daily commitment to fighting CF would be a cure.

We all may waver on our financial or fitness goals, but might you consider staying committed to finding a cure for CF alongside our family?

You can make a donation to Team Hall’n for CF by clicking on the "Donate Now" button at the top of the page.

Thanks so much for your ongoing care and concern for our girls!

The Hall Family

Steve, Wendy, Alexa (11), Noah (9) and Presley (5)