Amanda Noskowiak

The E Team 

Back in 2014 after Elliot was born, his newborn screen came back positive for cystic fibrosis. At the time, we had no idea what that meant or how our lives would change. We quickly started learning about what it would take to keep our new little nugget healthy. Breathing treatments twice a day, 4 times a day in times of illness. Airway clearance with manual clapping of the chest and later a vest machine that shakes and does the job instead. Digestive enzymes each time he ate because most people with CF have a pancreas that does not produce enzymes. At age 2 he had a gastrostomy tube, or g-tube, placed in his stomach so we could do some supplemental tube feedings and keep his growth on track. He continues to require overnight tube feeds to keep his nutrition on track. Elliot also routinely takes vitamins ADEK daily, culturelle to keep his bowels happy and the life changing modulator therapy called Trikafta. Trikafta helps his cells regulate sodium movement in and out of his cells and keeps his mucosal membranes much happier. This results in better nutrient absorption, increased lung function, more energy, better sleep and less illness overall. It has been a life saver and we are so grateful he is able to take it. It does not work for everyone with CF. 

Then in 2016 along came the twins. Evan and Everleigh were the last additions to our little family and while we were so disappointed to find out they both also had CF, we felt like we at least knew what we needed to do. They do all that Elliot does and a few extra things along the way. They have come with a few more hiccups in the road. Evan has more pancreatic issues and has had a few hospitalizations from that. Despite his g-tube he has trouble keeping weight on. He is also hovering on a CF related diabetes diagnosis. Everleigh’s liver has been giving her issues and she has had to cut her Trikafta dose by 2/3 to keep her liver enzymes in check. In the midst of continued elevated liver function tests we also discovered she has Alpha 1 Antitripsin deficiency which can also cause liver and lung disease. She also is borderline for CF related diabetes so we’ve been keeping a close eye on that. 

So that’s the nitty gritty. But the truth is, these kids are amazing.  They are happy, they are funny, they are adaptable to ever changing circumstances. They take each day in stride and they rarely complain. We could all learn a lesson about how to do life from them. 

If you’ve made it this far through our story make a donation to our team and please come join us on May 18th at St Johns University and help us walk for a cure! Trikafta has been a game changer but it isn’t the end. 

Nearly 40,000 people in the United States have cystic fibrosis: a progressive, genetic disease that affects the lungs, pancreas, and other organs. They are moms, dads, sisters, brothers, daughters, sons, and friends who face the sobering prospect of a shortened lifespan. I walk for them.

Working alongside the CF community, the CF Foundation has fostered the development of more than a dozen CF treatments and helped add decades of life for people with CF. Yet, many people with CF do not benefit from existing therapies. Our vision is a cure for every person with cystic fibrosis – a life free from the burden of this disease – and we will not leave anyone behind.

You can support me!

By supporting my fundraising goal, you have an opportunity in your lifetime to be part of ending this disease. Please consider joining us and help make medical history.