Join our team and help add tomorrows!
To sign up to walk on our team click on the JOIN ADAM'S TEAM button.
There are approximately 30,000 Americans living with cystic fibrosis. They are moms, dads, sisters, brothers, daughters, sons, friends and co-workers who struggle every day just to breathe. We walk for them, and especially for Alaina and Ava. Will you join us? All we need you to do to become a member of our team is click on the "Join our Team" button. From there you can make a donation and start your fundraising.
By becoming a member of our team, Alaina and Ava's Angels, and making a donation, you are joining a growing group of people committed to finding a cure for cystic fibrosis. Together, we are adding tomorrows to the lives of people living with CF by supporting the search for a cure.
Great Strides is a fun, family-friendly event that raises awareness and support for people with CF and their families. Please consider walking beside our family in our fight for a cure for our daughters on Saturday, May 4th at Green Isle Park in Green Bay, WI.
Our family’s journey with cystic fibrosis (CF) began almost 10 years ago when Adam and I began participating in a yearly kickball tournament to benefit the CF Foundation. We had no idea this was only the beginning of our own personal journey with this genetic disease.
We beat the odds and despite our doctor's belief became pregnant with Alaina who arrived on November 2, 2013. Ten days later we received a call no parent should ever have to hear over the phone on a Friday afternoon that our daughter tested positive on the newborn screening for cystic fibrosis. We were left to go all weekend with an appointment scheduled the following Tuesday at American Family Children's Hospital. Alaina's cystic fibrosis diagnosis was confirmed. We were shocked as we had no prior family history of CF. To say we were overwhelmed first time parents is an understatement. We quickly learned how to give an infant enzymes in applesauce with a spoon, perform manual chest physical therapy, and how to administer nebulizer treatments.
On February 26, 2017 we were blessed with our second daughter, Ava. At 5 days old at a pediatrician checkup we were informed she too tested positive on the newborn screening for cystic fibrosis. Our fears were confirmed, despite only having a 6% chance of having two children with CF. In our hearts we already knew and saw the signs of malabsorption and weight loss. We were blessed with another precious angel, thus our team name rightfully changed to Alaina and Ava's Angels.
The bond the two girls share is heartwarming knowing they are both "the same". In the past month, Ava received her vest, a device that shakes her chest to loosen mucus from the lungs. Ava watched her sister get hooked up to the vest so many times that within the first week she was putting on the vest herself and getting the hoses hooked up. Since 22 months old, Ava has been taking her whole enzyme capsules in applesauce. The girls need to take enzymes before they eat any fat containing food, as their bodies are unable to digest the fat due to the sticky mucus CF causes in the body. Ava is already taking her pills now like an adult. No more applesauce being carried around with us everywhere we go has been an incredible accomplishment for a mere 2 year old little girl who swallows more than 10 pills a day.
Cystic fibrosis can be a lot to manage on a daily basis. CF never gets a vacation; it is something our family deals with 365 days a year. It requires 1 to 1.5 hours when "healthy" of time spent on breathing treatments with manual chest PT or vest and nebulizers in order to breathe easier. If cystic fibrosis has taught us one thing about life it is that time is precious. We try to maximize the time we do have each day and make the breathing treatments part of our normal routine. Speaking of routines, we are big on routines. When treatments need to be so many hours apart, the girls can't eat a certain amount of time around treatments, medications need to be given at certain times, and you can definitely see how we thrive on routine. And just when you think you have it down this disease can throw you another treatment or another medication and it all needs to be reconfigured.
Alaina and Ava have participated in many research studies through American Family Children's Hospital as we help doctors and researchers learn more about this rare genetic disease and the impact environment and nutrition play, better sweat testing methods, the lung infections that are associated with CF and how a person’s genetic makeup attributes to varying severities. When the Cystic Fibrosis Foundation was founded in 1955, a child born with the disease was not expected to live to elementary school. Because of the efforts of CF Foundation, children born today are living into their 30s, 40s and beyond.
Orkambi, the first FDA approved medication for our daughter's specific genetic mutation and only the second CF drug to treat the underlying CF defect, was approved for children 2 to 5 this past July. We are happy to say both Alaina and Ava are taking this medication which brings great hope for their future. It comes with blood draws every 3 months, eye exams, and a bitter tasting medication taken twice per day but we are hopeful this is only the stepping stone for the CF cure. There are many more medications in the CF pipeline that are showing promising results and this is only made possible through the generous donations the CF Foundation receives through such events as Great Strides.
Please join us for the Great Strides cystic fibrosis awareness walk at Green Isle Park in Green Bay on Saturday, May 4th to walk beside our family in our fight for a cure for CF for Alaina and Ava.
A Warm Thank You,
The Pfotenhauer Family