CYonara CF

Our Story

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 4 years old in June and is doing well.  He swallows over 25 pills, 2 nebulizers, and does 1-2 hours of vest therapy every day. He also takes Orkambi twice a day, one of the first CFTR modulators. He is a true CF warrior and amazes us everyday!

• At the age of 2, he learned how to swallow 6 enzyme pills in one gulp.
• He holds his own nebulizer and knows how to start and pause his vest machine during his breathing treatments.
• He learned how to take deep breaths from his inhaler.
• He knows how to wrap his finger and all the right buttons to push to check his oxygen with the respiratory therapist.
• He bravely gets his blood drawn for labs every 3 months and does a "big cough" for his throat culture
• He is a self-taught ninja-in-training and makes the best fighting sound effects
• He will try just about any food because he knows he needs extra fat and protein
• He is the most loving big brother to his baby sister

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)
• Delayed puberty
• 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!