Together, we have made remarkable progress in the fight against cystic fibrosis. The breakthrough drug, Trikafta, that targets the underlying cause of CF is showing promising lung improvements in many patients aged 12 and up who are eligible to receive such a miraculous drug. Specifically, this drug has the potential to help 90% of those with CF who have at least one dF508 mutation. But we can’t stop now – our work isn’t done until there’s a cure. We are so incredibly grateful for the love and support we've received from our community and hope we can keep the momentum going. Please join us in our fight to say "CYonara CF!"
Cy was born on 6/5/18. After telling everyone how perfect he was, we learned at 6 days old that he has cystic fibrosis. It was an emotional experience with periods of denial and depression for Jerry and me. We fluctuate between feeling overwhelmingly fearful and incredibly hopeful. Our heads were spinning after meeting with a gowned and gloved team of a doctor, nurse, respiratory therapist, dietician, and social worker at Nemours. We were handed a 3-inch binder describing CF, the complications, and treatments. We learned how to administer enzymes on applesauce before every feeding and how to do breathing treatments and chest percussions. One thing that I remember clearly from our first meeting with the CF clinic team was that although CF life expectancy used to be around 10 years old, it is now closer to 50 thanks to improved therapy.
Cy will be 2 years old in June and is doing well. He takes over 25 pills per day and 2 nebulizers. In the first 10 months of receiving his incourage vest, he has completed over 200 hours and 600 sessions of vest therapy, or as we call it "shakies". He has learned about 5 different ways to turn off his vest machine, but we perservere! He is a true CF warrior and amazes us everyday!
What is Cystic Fibrosis (CF)?
CF is a genetic disease that is inherited when both parents are carriers for a mutation in the CFTR gene. The CFTR protein regulates Chloride ion transport across epithelial cell membranes. When CFTR is mutated, the protein either gets degraded or doesn’t function properly. This causes extra sticky mucus to build up in the lungs, around the pancreas and in the liver bile ducts and excess salt loss. Although CF is commonly thought to be a lung disease, many areas of the body are affected by this devastating disease (described below).
What are the manifestations of CF?
Frequent respiratory infections, Coughing (can be bloody), Difficulty in breathing (lung transplants eventually needed due to scar tissue and decline in lung function)
Abdominal pain and discomfort, gassiness
Poor appetite, Malnutrition, Poor growth (poor absorption of fat-soluble vitamins and nutrients in general due digestive enzymes trapped in thick mucus around pancreas. Enzymes required prior to every meal. Feeding tubes are often necessary to administer extra calories. People with CF need a high fat, high protein, 2X Calorie diet)
A barrel-chested appearance
Sinusitis (inflammation of the nasal sinuses), Nasal polyps (fleshy growths inside the nose)
Clubbing (rounding and enlargement of fingers and toes)
Pneumothorax (rupture of lung tissue and trapping of air between the lung and chest wall)
Enlargement of the right side of the heart (called cor pulmonale, CF causes the heart to work extra hard due to low oxygen intake from blocked/scarred lungs)
Protrusion of the rectum through the anus (called rectal prolapse)
Liver problems (clogged bile ducts)
pancreatitis and gallbladder problems
Diabetes (insulin trapped in sticky mucus around pancreas)
Reproductive problems (vas deferens not developed properly, no exit for sperm-can’t reproduce naturally)
Salt depletion/Salty skin (Low salt levels in the body lead to fatigue, weakness, fever, muscle cramps, stomach pain, vomiting, dehydration, and heatstroke. People with CF need extra salt everyday because they sweat out excess salt)
How can I help?
Raise awareness! Walk with us! Learn about CF and share your knowledge with others.
Stop spreading germs! Wash your hands often, use hand sanitizer, and stay home when you’re sick.
Be an organ donor!
Raise money for CF research. All money donated to our Great Strides team will go to the Cystic Fibrosis Foundation, which supports research for improved CF therapy and hopefully one day...a cure!
Join our team and help add tomorrows!
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. Will you join us? To become a member of our team, just click on the "Join our Team" button. From there, you can make a donation and start fundraising.
By joining our team and making a donation, you are joining a growing group of people committed to finding a cure for cystic fibrosis. We will not rest until all those with cystic fibrosis have a cure. The time is now. Together, we can conquer this disease.
Great Strides is a fun event that provides a fantastic opportunity for family, friends, students, and colleagues to come together to make a difference in the lives of people with CF -- join our team today!
Important note on Attendance at Foundation Events:
Infections can exacerbate CF symptoms and worsen lung function, so we ask attendees at Foundation-sponsored events to observe the following best practices to reduce the risk of germ transmission:
Regularly wash hands with soap and water or with an alcohol-based hand gel.
Cover coughs and sneezes with a tissue or your inner elbow.
Maintain a safe 6-foot distance from anyone with a cold or infection.
To further reduce the risk of cross-infection, the Foundation’s attendance policy recommends that all people with CF maintain a safe 6-foot distance from each other at all times while attending an outdoor Foundation-sponsored event. All Great Strides walks are non-smoking events.