Cystic Fibrosis Awareness

Kiev Adair lives in Texas with her two children, Devon (age 22) and Leiki (age 11). Devon is a personal trainer at a local gym and pursuing the goal of opening his own gym and online stock trading company.

Kiev became aware of Cystic Fibrosis (CF) when Leiki was around 6 weeks old and wasn’t nursing well, plus having labored breathing. Kiev took her to the pediatrician, who diagnosed Leiki with pneumonia. They were sent home with an inhaler and antibiotics. Her symptoms improved slightly, but she was still having problems with her breathing that was causing more issues with nursing. After 2 rounds of pneumonia, Kiev spoke to her mother, Shannon Shumate, of Jackson, MS and explained Leiki’s symptoms and diagnosis. Shannon suggested that Kiev kiss Leiki on the forehead to determine if she tasted salty. Leiki indeed tasted salty!

After online researching which caused much distress in Kiev, she made multiple pleas to Leiki’s pediatrician and pulmonologist to hav e a sweat chloride test, where a technique called iontophoresis is employed in order to produce a volume of sweat. The technique requires the application of a tiny (painless) electrical current that medically stimulates maximum sweat production. The patient’s forearm is commonly used.

Prior to November 2009, the CF screening for newborns wasn’t a part of the heel-prick newborn screening done on babies. Leiki was born in April 2009. After a false-positive test and more probing of doctors to determine what was making Leiki sick, on August 18, 2009 @ 5 months old, Leiki was diagnosed with CF.

Shortly after the diagnosis on August 26, 2009, Leiki was hospitalized for CF complications. That’s when they found out what their future lives would

encompass. Initially, the doctors advised them to keep Leiki away from sick people, discouraged day care, provided a litany of breathing treatments and a daily schedule, and gave them numerous written resources for education on CF. The main goal was to continue her daily breathing treatments, keep her caloric intake high, and assure she was isolated from people that were or had been recently sick.

Kiev’s initial reaction to the diagnosis was FEAR of the unknown. “Not knowing if I was going to be able to keep her healthy, I was devastated and shocked. Then I went into what I like to call “Mama Bear” mode. How do I attack this disease and allow us to win the daily fight?”

Life with CF is a delicate balance of the necessary medical treatments and living a ‘normal’ life as much as possible. Leiki has 10 breathing treatments per day, 4 different inhaled medications, and takes up to 26 pancreatic enzymes with meals and snacks per day. The enzymes help her pancreas process fats, proteins and carbohydrates so she can absorb the maximum number of calories.

A daily routine of prescribed breathing treatments, snacks and meals high in calories and physical fitness has proven to be our best defense to combat CF. Sometimes the hospital can’t be avoided, since the common cold, flu, and airborne fungi can cause an infection that Leiki’s body is unable to combat. With the current Covid 19 virus, Kiev has treated the situation as she would during FLU season. Additional washing of hands, more sanitizing of surfaces and overall staying isolated from others is important. Our hometown went on “stay in place shelter” 2 weeks after Leiki and I had already been on self-isolation. The covid-19 situation is a HUGE learning experience for people to now understand what a CF person’s life is like when there’s a threat of viruses, common colds, flu, and strep throat.

Certain precautions are necessary for Leiki’s health since she is school age. We stay informed of the number of cases of FLU, strep, and various other viruses associated with the school, and Kiev works closely with Leiki’s teachers. “I have voluntarily pulled her from school and done intermittent homebound schooling with her to avoid catching anything. I educate her teachers every year on CF and the importance of hygiene and cleanliness, as well as volunteer at her school to assure Leiki still

Kiev has wise advice for other parents dealing with a new diagnosis of CF. “CF will seem overwhelming at first, and that it is ok. There are many resources through the CF Foundation to help guide you through the struggles and involve your trusted loved ones and family friends in knowledge and care of someone with CF. Build a ROUTINE for daily medical therapies, physical activity and

eating. I can’t stress that enough. Do not overwhelm yourself with personal research or the ‘what ifs’. Be kind to yourself, and all will fall into place.”

In the last 10 years since Leiki’s diagnosis, the CF Foundation has backed pharmaceutical companies in finding treatments and potentially a cure for the disease. Since 2009, 3 oral medications have been developed and prescribed to patients daily. In October 2019, a 4th oral medication that is showing the best and most positive results (TRIKAFTA, a combination drug of the 3 initial medications) was passed for production by the FDA. This newest drug is the only one that Leiki qualifies for the treatment of CF symptoms. She is eligible to start the physical testing and protocol to be prescribed the medication in the fall of 2020.