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 18th!
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)
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IMPORTANT NOTE ON ATTENDANCE AT FOUNDATION EVENTS:
Updated July 5th, 2023
The CF Foundation is committed to ensuring the health and wellbeing of individuals attending Foundation events. Individuals attending CF Foundation events must abide by the Foundation's Event Attendance Policy www.cff.org/attendancepolicy, which includes guidance for event attendees living with cystic fibrosis.