We are so grateful you're here! We're asking for support of this cause that is fighting so tirelessly for our little Naomi to live a long, healthy and happy life!
CF is a heredity, recessive (meaning you must inherit two defective genes to have a disease) complex-whole body disease caused by a mutation in the gene that is responsible for regulating the flow of salt and water throughout the body (we never knew the importance of salt until now!) There are over 1,400 types of defects in this gene that can prevent it from working properly and Naomi carries two copies of the most common defect (1 in every 29 Americans is a carrier). Because the flow of salt and water is disrupted in her cells, a very thick, sticky mucus builds up in her organs, including her lungs, pancreas and digestive tract where normally you would find a thin, lining of mucus that helps organs function properly and flushes out germs. However, this sticky mucus (think rubber cement) catches germs and bacteria and is very hard to get rid of. To try to help loosen the mucus from her lungs so she can cough it up, Naomi wears a "shaky vest" two times a day for 30 minutes each. She inhales 3 separate breathing treatments daily (15 minutes each) to help open her airways and thin the mucus. Because the mucus clogs her pancreas her pancreas can't release enzymes to process the nutrients from her food, so she takes enzymes every time she eats, so she can benefit nutritionally from the food she is eating. She goes to CF Clinic appts at Children's Hospital every three months for a 3-5 hour appt, where she sees between 5-8 doctors/nurses to check on every element of her disease. One of the key reasons for the frequency, is to a swab test to check if any dangerous bacteria got into her lungs that would need to be treated right away and to get blood panels done.
Just a few short years ago, a new medication called Orkambi was released helping to treat CF at the cellular level. Naomi started this medication at 2 and although it is known to help, a newer-life changing medication was recently released.
This is a very high-level overview of CF and doesn't go into the ways bacteria found in our every day environment and germs can hospitalize CF patients and cause irreversible damage. Nor does it address the cost of the medications and lack of access to them in other countries. There are so many ways in which the CF Foundation is helping to develop more effective medications for eradicating bacteria, for helping CF patients with advanced lung disease, and helping those whose mutations won't work with this new medication. Most of all, they are working relentlessly towards a cure, which includes gene therapy.
CF can be an isolating disease for patients (They can't be around each other or ever meet because of the risk of spreading bacteria that live within their lungs and creating/catching dangerous superbugs from one another) and caregivers since it is such a unique disease and constant balancing act of creating a lifestyle that prolongs her health but allows her to live it to the fullest. So we thank you for being here to support us, to support her and every other family impacted by this disease!
Please visit this page to learn about all of the amazing ways in which your donation will be used to extend and protect lives! https://apps.cff.org/trials/pipeline
If you're into reading - Breathe from Salt: This inspiring book is the discovery of CF, the fight to find a cure, the science behind the disease and path to a cure.
- On Netflix, the movie "Five Feet Apart" https://www.imdb.com/title/tt6472976/
IMPORTANT NOTE ON ATTENDANCE AT FOUNDATION EVENTS:
Updated July 5th, 2023
The CF Foundation is committed to ensuring the health and wellbeing of individuals attending Foundation events. Individuals attending CF Foundation events must abide by the Foundation's Event Attendance Policy www.cff.org/attendancepolicy, which includes guidance for event attendees living with cystic fibrosis.