A Fundraiser for Cystic Fibrosis

For the month of May I will be selling a set that we have named "Ava's Set"  This is to honor and help fundraise for a special little girl who is near and dear to my heart.  100% of the money earned from this set will be donated to the Cystic Fibrosis Foundation.  We are helping Ava's family persevere through this disease and won't stop until CF stands for CURE FOUND!

Build a Set

Visit my order form for Ava's Set to build your own set.  Select your favorite shade of purple, select your accent nail design, and help raise awareness for cystic fibrosis!

    Meet Ava

Ava is my little buddy, and the daughter of my good friend, Liz!  I will never forget getting the phone call from Liz just days after Ava was born and learning of her diagnosis.  I have watched Ava and her family endure so much uncertainty with grace, faith and  perseverance.  Please help me, to help them keep working toward finding a cure for Ava and all patients with CF!

Below you can learn more about Ava's story as told by her mom, Liz.

 

 

 

 

 

 

Ava's Story...

Cystic Fibrosis (CF) is a genetic, progressive disease affecting many organs including the lungs, kidneys, pancreas, and liver among others. It is a rare disease affecting only about 40,000 children and adults in the United States, and roughly 105,000 people in 94 countries around the world. CF causes the body to produce thick, sticky mucus which then traps bacteria in the lungs causing infections and other complications. It also prevents the pancreas from releasing digestive enzymes important for food and nutrient absorption. Thick, sticky mucus in the liver can cause blockages of the bile duct which leads to liver disease.

Prior to Ava’s birth, we had no reason to suspect that our second child, Ava, would be born with such a debilitating disease. There was no family history to our knowledge and our first child, Emma, was born without any signs or symptoms of CF. So it was a shock
to us when the pediatrician called a few days after her birth and said, “Ava has Cystic Fibrosis. There is a CF specialist in Syracuse and they want to see her right away.” Having just turned 8 years old on March 13th , Ava has endured much more than the
average child. She was hospitalized at 3 weeks old after she briefly stopped breathing due to a cold, something that is challenging for any newborn under the age of 1 month. With routine specialist visits every 3 months, Ava has had blood draws since she was 6
months old, as well as throat swabs. She has also had annual chest x-rays, ultrasounds of the liver and a liver biopsy and an endoscopy. Managing her care can be difficult and
involves daily airway clearance through the use of a vest, in addition to inhalers and specialized inhaled medicines to maintain healthy lungs. She also takes digestive enzymes, specialty vitamins, liver and GI medicines, appetite stimulants, and
modulators designed specifically to treat the underlying cause of CF. Treatment for cystic fibrosis has come a long way since it was first discovered in 1938. And yet, even with the advancements in technology and medicine, there is still no cure.

If nails aren't your thing, but you would still like to make a donation, please consider donating directly to the Durling's fundraiser:

In 2019, the FDA approved a new modulator, Trikafta, a medicine that treats CF so well it’s almost like a cure…ALMOST. But patients still need to take Trikafta twice daily and unfortunately, about 10% of the cystic fibrosis population are ineligible to take Trikafta
for one reason or another. What’s tricky about CF is that there are many “classes” of genetic mutations depending on what part of the CFTR gene is “defective.” Ava was lucky to have been born with the most common CF mutation, meaning more treatment
options would be readily available to her; but her luck only goes so far. For years, Ava’s liver levels have followed a path similar to that of a rollercoaster –
constant ups and downs. We finally learned in the fall of 2024 that Ava is a carrier of another genetic mutation known as Alpha-1 Antitrypsin (A1AT) deficiency. Although she is only a carrier of this deficiency she is still at a higher risk for lung and liver issues. We
simultaneously learned that Ava also has CF-related liver disease. She also has gallstones which don’t seem to be causing an issue for now, but do require close monitoring. Different doctors have different opinions based on their specialty; however,
due to the liver disease and A1AT, Ava is ineligible for Trikafta.
Compared to the experience of other CF patients Ava has done remarkably well all things considered. We’re still waiting for that “miracle drug” that will one day be the cure for Ava and all other CF patients. Until then, we must work diligently and relentlessly to
make that dream a reality. Thank you for helping us make that dream come true.