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
Even though it's often a struggle getting a 3 year old to cooperate for 1 hour breathing treatments twice a day, we are so proud of him!
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:
Updated June 15, 2022. To reduce the risk of COVID-19 the Foundation is taking steps to host safe events for our community. Please be advised that events may be subject to change at any time based on guidance from the Centers for Disease Control and Prevention and local health officials.
To minimize the risk of COVID-19 infection, we ask that attendees at CF Foundation events follow these steps:
Where mandated, practice physical distancing and maintain at least a safe 6-foot distance from persons outside of their household.
Face masks are encouraged and should be worn in accordance with local guidelines. (Note, children under two years of age should not wear masks due to safety concerns and therefore should only attend outdoor CF Foundation events where they are able to distance and stay within their family group.) NOTE: For events hosted by the CF Foundation, masks will be made available on-site, when possible.
Follow basic infection, prevention and control practices by regularly washing hands with soap and water or with an alcohol-based hand gel, and by covering your cough or sneeze with a tissue or your inner elbow.
Persons with CF should consult their physician before participation in any in-person event as they may be at an increased risk for severe illness from COVID-19.
For indoor events, it is CFF policy to invite only one person with CF to be in-person at the event at a time. For outdoor events, people with CF should maintain a safe 6-foot distance from persons outside of their household at all times.
FOR YOUR SAFETY AND THE SAFETY OF OTHERS:
For the health and well-being of the CF community, we strongly encourage that participants be fully vaccinated and have received the CDC recommended booster shot when joining us in-person for the event.
Certain CF Foundation events may require vaccination to attend.
Participants are strongly encouraged to test within 24 hours prior of arrival at an event or meeting, or as close as possible to the time of departure (if traveling).
Please be advised certain events, localities, and venues may require you to be fully vaccinated and show proof of vaccination. We will communicate these requirements in event communications.
For events where the CF Foundation requires full vaccination, meeting participants must be fully vaccinated against COVID-19 at least two weeks before the date on which you will first attend the meeting. Registration and attendance at such events may require agreement to health and safety protocols and/or COVID liability waivers.
We are counting on the fact that you appreciate the potential consequences of exposing members of the CF community to COVID-19 and will not attend an event if you test positive, or have been recently exposed to COVID-19.
We cannot guarantee that all people present at the event, including participants, sponsors, volunteers, vendors, or others, have been fully vaccinated.