Naomi Staats

As may or may not know, our cuddly, affectionate and silly Naomi was unexpectedly diagnosed with Cystic Fibrosis at 26 days old through the newborn heel prick test. Showing really no symptoms of anything being wrong and having no family history of CF that we are aware of, we were completely shocked by her diagnosis. It has taken us time to process and share this news and we hope to continue to share more of her story to her friends and family so she knows how many people love her and are fighting for her to have a long, lovely life! 

We are so grateful you're here! We're asking for support of this cause that is fighting so tirelessly for our sweet little Naomi to live a long, healthy and happy life! 

CF is a complex, hereditary, genetic 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!) 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 you normally 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 making it hard to breathe over time. 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 open her airways and thin the mucus. Because the mucus clogs her pancreas it 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 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. Two key reasons for the frequency are to do 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. She has been doing all of this since months old and is amazing at taking her medications, going to the doctor and knowing so much about her body already! She is truly an earthly angel! 

CF is progressive because of what the thick sticky mucus does to the body over time. It can create irreversible lung damage by accumulating bacteria that can be very difficult to eradicate causing hospitalizations to try to treat it with IV antibiotics, cause diabetes, among many other complications. Up until recently, the treatments for CF were able to only treat the symptoms of CF rather than help fix the actual defect. 

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. 

In 2019, 8 months before Naomi was born, a life-changing medication called Trikafta was approved for adults with CF. In a span of months it worked so well to treat the underlying cause of CF that many CF patients were taken off of lung transplant lists and have had very few hospitalizations since, whereas they may have spent months each year in the hospital trying to treat lung infections among other things. However, this medication doesn't work for all CF patients and the long-term effects are unknown. 

On April 28, 2023 we expect the FDA to approve this medication for CF patients for Naomi's age group (2-6 years old) with Naomi's gene mutations. Trikafta will work at the cellular level to correct the defect, thus helping reduce the build up of mucus in her lungs and organs amongst other things. The hope is that with an improved flow of salt and water throughout her body the mucus will not be able to accumulate and cause as many complications/progression of the disease. It is truly the most recent miracle in the world of CF. 

She will hopefully start it this May and we're so grateful for the tenacious efforts of parents, CF patients, doctors and scientists who paved the way for us to be able to benefit so soon in Naomi's life. It is expected that the earlier a patient starts these groundbreaking medications, the better the chance of living a long, healthy life because it will help prevent damage before it happens. 

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 assisting those whose mutations won't work with this new medication. Most of all, they are working relentlessly towards a cure, which includes gene therapy. 

When Naomi was born the life expectancy of a patient with CF was just a few years older (38) than we were at the time we had her and we hope with the new medications on the horizon and a cure that CF patients will live normal lifespans.

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! 

We're so grateful Naomi was born at a groundbreaking time for CF research and that she will benefit from this breakthroughs so early in her life. We're on the cusp of a cure and hope that Naomi's life will be the start of the new history books for CF, with CF standing for Cure Found! 

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 

We invite you to walk with us in Santa Clarita on Saturday, May 6th in honor of Naomi! We have chosen not to have her attend, to keep her away from the risk of being around other people with CF, but we will be there walking in her name) If you want to join us, reach out and we'll give you the details! 

Want to learn more?

Watch on Netflix the movie "Five Feet Apart" https://www.imdb.com/title/tt6472976/

On instagram follow: @thelittledreameruk, @thecandcdiaries @lovetobreathe  @cf_foundation @rosielifewithgrey

The movie Salt in My Soul on youtube - A story of a young woman with CF who documented her life for her parents to turn into a film after she passed

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.