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)
Connect With Us
IMPORTANT NOTE ON ATTENDANCE AT FOUNDATION EVENTS:
To reduce the risk of COVID-19 the Foundation is taking steps to host safe events for our community. Please be advised that events may be subject to change at any time based on guidance from the Centers for Disease Control and Prevention and local health officials.
To help minimize the risk of COVID-19, we ask that attendees at CF Foundation events follow these steps:
Where mandated, practice physical distancing and maintain at least a safe 6-foot distance from persons outside of their household.
Face masks are encouraged and should 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 only attend outdoor CF Foundation events where they are able to distance and stay within their family group.)
Follow basic infection, prevention and control practices by regularly washing hands with soap and water or with an alcohol-based hand gel, and by 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.
For indoor events, it is CFF policy to invite only one person with CF to be in-person at the event at a time. For outdoor events, people with CF should maintain a safe 6-foot distance from persons outside of their household at all times.
FOR YOUR SAFETY AND THE SAFETY OF OTHERS:
For the health and well-being of the CF community, we strongly encourage that participants be fully vaccinated and have received the CDC recommended booster shot when joining us in-person for the event.
Please be advised certain localities and venues may require you to be fully vaccinated and show proof of vaccination. We will communicate these requirements in event communications.
We are counting on the fact that you appreciate the potential consequences of exposing members of the CF community to COVID-19 and will not attend an event if you are feeling ill or have been recently exposed to COVID-19.
We cannot guarantee that all people in attendance at the event, including participants, sponsors, volunteers, vendors, or others, have been fully vaccinated.