Paige Ella Christensen, a 14 year old from Blaine, MN, is one of the kindest and gentlest souls I have ever known. She loves her friends, being outside, going on adventures, doing gymnastics, playing or listening to music, and bossing her little sister, Brooke, around. Paige also happens to have a chronic disease called cystic fibrosis (CF).
CF is a genetic disorder Paige inherited from Paul and me (Jill). Turns out we were both unknowingly carriers of a CF mutation (as 1 in 25 Caucasians are). The CF mutation makes certain cells lining Paige's sinuses, lungs, pancreas, liver, intestines and reproductive organs not function properly. Instead of these cells producing a thin, protective mucus, they produce a thick, oppressive mucus the consistency of toothpaste.
This sticky mucus leads to life-threatening infections in Paige's lungs, and causes malnourishment as her clogged pancreas is unable to digest food. But CF does not stop there. CF impacts almost every organ in its victim's body; leaving a host of other conditions like intestinal obstructions, osteoporosis, sterility, scarring of the liver, and diabetes in its wake.
As we adapted to the overwhelming treatment regimen designed to keep CF at bay, our fear of the unknown turned into stubborn determination. Feelings of injustice fueled our commitment to stop CF from delivering its final and devastating blow. Our family and friends came together to start With One Breath more than a decade ago in an effort to help find a cure, and show Paige she would never have to face CF alone.
In the past 12 years, countless individuals and businesses have helped us raise more than $500,000 for CF research. Thanks to your steadfast love and support, 6 new life-lengthening therapies have been made available to the 30,000 people in our nation battling CF. With each new drug discovery you make possible, you give hope to the entire CF community that CF will one day stand for Cure Found.
Emma Jo Willcox is a “Daddy’s girl” from Fridley, MN. What father wouldn’t be charmed by her smile, sense of humor, and caring nature? While she may be all girl, she still loves to fish with her Dad, Curtis, and get dirty from time to time with her Mom, Judy.
Emma’s arrival into the world 16 years ago was laced with fear of the unknown as she had been diagnosed with cystic fibrosis (CF) in utero through amniocentesis. Thankfully, Emma came out of the womb a fighter.
Since birth, Emma has spent more than 250 days in a lonely, boring, and sometimes scary hospital room stuck to an IV pole. By age 4, she had a feeding tube permanently placed to help nourish her underweight frame, and an IV port surgically implanted inches below her collar bone so IV antibiotics could be administered at home to treat her frequent lung infections. Emma spent the next decade of life slowly but surely drowning from the inside out as CF destroyed her lungs. On July 26, 2016, after spending 65 long days on the transplant list, the Willcox’s received a call that would forever change their lives. In the midst of tragedy, an anonymous family decided to donate their daughter’s lungs to save Emma’s life.
A team of 20 medical professionals opened Emma’s chest to remove the lungs they called “junk” and left beautiful, pink, CF-free lungs in their place. Within 2 weeks of surgery, Emma had dozens of IVs, 5 chest tubes, a central PICC line, 2 pain blocks, and a port removed, and returned home pain-free.
Recovery from transplant was long and arduous. Emma could not be left alone or travel more than an hour away from the hospital for 3 months. She spent multiple days each week at doctor visits and hours at therapy appointments for months to improve her lung capacity. While Emma no longer needed airway clearance or vest treatments, she continues to take pancreatic enzymes (to digest her food), insulin (for CF-related diabetes), and immunosuppressant medications (to suppress her immune system and prevent rejection) for a lifetime. Since Emma’s immune system is “turned off” by immunosuppression, she must also avoid getting sick (EVER!)
As is typical of kids battling chronic disease, Emma takes her fight for life in stride.
For her loving parents, however, watching their little girl suffer is unbearable. With each setback, they wonder if they will be given the opportunity to walk their daughter down the aisle one day. But instead of letting despair take hold, Curtis and Judy force themselves to move forward and be strong for the sweet girl that has taught them so much about tenacity and appreciating each day they are given.
They relentlessly and passionately work to raise funds for cure-finding research and provide support to CF families undergoing hospitalization. In addition, they encourage each of us to become an organ donor! KATIE
Katie Rose is a 13-year old from Arden Hills, MN. As the youngest of 8 children, Katie is described by her loving parents, Todd and Sara, as a sweet, motivated perfectionist that never gives up and is willing to try anything (except new foods!). The first thing I noticed about Katie during my interview were her remarkably beautiful eyes. They were the kind that render onlookers speechless, so I quickly looked down at the paper before me and began asking questions.
When asked what she loves most, Katie easily replied dance. She spends much of her time practicing jazz, ballet, and lyrical routines with her older sister, Heather, but her passion is tap. Pride sparkled in Katie’s eyes as her mom told me about the tap scholarship she had recently won.
After dance, Katie’s priority is spending time with her family. Katie knows first hand that togetherness is an important ingredient to family life. Nothing could have driven this lesson home more than the recent loss of her two beloved sisters, Anna and Nicole. Anna tragically died in a motor vehicle accident at age 23, and months later, her sister Nicole lost her life-long battle against CF at age 13.
With Anna, Katie lost a big sister that loved to dote on her with secret trips to the mall or nail salon. With Nicole, Katie lost her best friend. Together, Katie and Nicole made silly videos, giggled about nothing, and fought CF side by side. From airway clearance treatments to medications to PICC lines to feeding tubes to hospitalizations—CF was simply another thing the girls shared amidst playing, learning, caring, forgiving, laughing, hugging, helping, dancing, and healing.
While Katie politely answered my inquiries about adversity and loss, it was clear she had not been crippled by the pain and suffering she has known. In awe, I chanced one last look into her eyes and caught a glimpse of steely determination and resilience. Like a willow that bends in a storm, Katie has withstood the obstacles placed in her path without breaking. In doing so, she teaches those around her what it takes to get up off the ground when life knocks us down: tenacity, grit, and a willingness to be flexible.
One look at 5 year old Ava Beyer’s wide, vibrant smile conveys her boundless energy, stubborn determination, and loving nature. Try telling unstoppable Ava she can’t do something, and she’ll prove you wrong!
Ava is the picture of health, but don’t be fooled, she has fought CF since her very first day. Ava was diagnosed with CF in utero when prenatal ultrasound imaging revealed meconium ileus. Essentially, Ava’s first stool (meconium) was blocking the last part of her small intestine (ileum) because CF made her stool thicker and stickier than normal.
Immediately after birth, Ava underwent abdominal surgery to have the obstructed stool and her appendix removed. During the procedure, a gastrostomy tube (G-tube) was inserted through her abdominal wall to deliver nutrition directly to her stomach during her lengthy recovery. Once settled at home, first-time parents Kate and Dj learned how to administer medications and perform manual percussion or clapping treatments to keep Ava’s lungs clear.
In an effort to learn more ways to help Ava, Kate and Dj attended an annual CF Education Conference put on by the University of MN CF Center. While listening to others affected by CF speak, Kate unexpectedly broke into tears. The fear of the unknown, the horror of seeing tubes jutting out of Ava’s infinitely small body, the added stress of following an intense treatment regimen each day, and the feelings of shear helplessness cascaded down her cheeks one drop at a time. A nurse at the conference approached Kate and gently said, “You’re going to suffer the first 18 months, and then a light bulb will go off and you’ll get this.”
In the months that followed, Kate and Dj squared their shoulders, embraced hope, and refused to allow CF to define their family’s future. While they were committed to giving Ava the best care, chronic disease would never be an excuse to not live life to the fullest. Ava would receive no leniency or babying, and would never be introduced as a person with CF. Kate and Dj, on the other hand, would not let CF keep them from expanding their family and welcoming beautiful Mia into the world.
In their journey with CF, the Beyer’s know fearless is living in spite of the things that scare you to death.