imagine it’s 1950, and your son’s or daughter’s doctor walks into an exam room with test results. “Your child has Cystic Fibrosis,” he says. “He may not live through elementary school.”
Now imagine trying to explain to your child that they have a disease with a name that can be difficult for even adults to pronounce. But thanks to a sweet 5-year old boy misunderstanding his mom, Cystic Fibrosis has become known as “65 roses” for children all over America for nearly a half century.
Around 1,000 families a year have to explain “65 roses” to their children, but due to funding of scientific breakthroughs, doctors can now say, “Your daughter may live into her 30s, 40s or beyond.”
Cystic Fibrosis is an inherited chronic disease that affects the lungs and digestive system. The body of someone with CF produces unusually thick, sticky mucus — often described as honey-like in consistency — that can clog the lungs and cause infections or obstruct the pancreas, making it difficult to absorb food.
Taste of the Finest is a second annual fundraiser for the Arkansas Chapter of the Cystic Fibrosis Foundation. In addition to an evening of music, dancing and food/beverage tastings, 24 outstanding young professionals under the age of 40 are honored for their role in raising funds and awareness of CF throughout the community. This year’s honorees are a diverse group lending their names and influence to a good cause.
“These individuals were selected based on their professional success and commitment to bettering the central Arkansas community,” says MacKenzie Horrell, co-chair along with Kyle Holmes for Taste of the Finest. “This exceptional group has committed to helping raise awareness and funds for CF.” Horrell further explains that each honoree is asked to raise $1,500 for the CF Foundation and that many have personal connections to the disease through friends and family.
In 2012, Horrell was part of the group honored, and during that time became aware of the disease and the goals of the Cystic Fibrosis Foundation.
“I didn’t realize what a devastating disease it was until I became involved. Most people diagnosed with CF are young children, and there is a life expectancy placed on someone diagnosed with the disease,” Horrell says. “At the time of the event I was just turning 25, about 10 years shy of the CF life expectancy. It really helped put things in perspective for me.”
Horrell says she feels good about being part of an organization that is on the brink of major medical breakthroughs for their patients. Patients like 6-year-old Aven Emery, the 2013 Arkansas Cystic Fibrosis Ambassador.
Aven’s mom, Brittany Emery, says her pregnancy was normal and after the birth of Aven the only thing odd was that Aven vomited after every feeding.
“We figured this was a normal baby thing — just spitting up or a possibility she was allergic to her formula,” says Emery. “But within 48 hours of being born, Aven’s stomach had become distended and she had not [passed stool] at all since she was born. The doctors soon realized that her condition was a lot worse than they had originally thought.”
Newborn Aven was rushed to Arkansas Children’s hospital where all efforts to remove the blocked stool failed; emergency surgery became the only option.
“The surgeon mentioned to us that about 80 percent of children that have this kind of blockage have a disease called Cystic Fibrosis,” Emery said. “We immediately went to the Internet and looked up Cystic Fibrosis. This definitely wasn’t the best idea with our emotions running high.”
Aven spent three weeks in the ICU after surgery, during which her parents spent hours educating themselves about Cystic Fibrosis just in case this was Aven’s diagnosis.
“As we were getting ready to be discharged from ACH, the lab results came back positive for Cystic Fibrosis. At that moment, we knew it was real,” Emery says. During the next five days, they remained at ACH to learn how to care for their daughter, including how to administer medications and breathing treatments.
“Because CF affects such a small population, it is considered an ‘orphan disease’ and the CF Foundation does not receive any federal funding. We rely strictly on private donations,” says Hannah Herriman, director of development at the Arkansas Cystic Fibrosis Foundation. “The CF Foundation is the world’s leader in research and care for the CF population.”
Herriman continues with pride, saying that nearly 90 cents of every dollar goes directly to drug development and research, along with the foundation’s other areas of focus, advocacy, education and patient care.
“We have a network of over 120 care centers across the country,” Herriman says. “There are three care centers in Arkansas, and the Arkansas chapter helps fund them.”
The Care Center for Children is located at Arkansas Children’s Hospital, then there is the Care Center for Adults at UAMS and a satellite care center located in Northwest Arkansas at the Center for Children in Lowell.
As part of their outreach, the foundation works to spread awareness and educate lawmakers. Thanks to their lobbying efforts, today all 50 states test for CF at birth. Arkansas began newborn screening on July 1, 2008.
Perhaps most exciting is the significant advancements the CF Foundation has made regarding drug research.
“Because CF affects such a small population, there is not much incentive for pharmaceutical companies to invest in the research and development of new drug therapies,” Herriman says.
Because of this, the CF Foundation has partnered with several pharmaceutical companies to create new treatments. Nearly every approved CF drug available today was made possible because of funding provided by the CF Foundation, including the groundbreaking new drug Kalydeco, just released to the public in January 2012. This first drug of its kind actually corrects the defective gene and how it works in the body.
“Those patients taking Kalydeco are experiencing life-changing results,” Herriman says. “We consider Kalydeco a ‘daily cure’ because it is a pill patients will take for the rest of their lives, but it neutralizes their symptoms — meaning if a CF patient on Kalydeco were to test for CF, their test results would come back negative. It is truly amazing.”
Right now, Kalydeco is only effective for about 4 percent of the CF population (about 1,600 people). There are over 1,000 different mutations of CF that someone can carry, and Kalydeco is only effective for one of those mutations. While this is incredible progress, this leaves over 95 percent of the CF population waiting for a cure.
In just over 60 years, the survival rate for someone with Cystic Fibrosis has risen dramatically thanks to research. Funding that research is key to finding a cure. The CF population invested over $75 million into research for Kalydeco alone, and is currently putting more than $120 million toward finding “daily cures” for other mutations of CF. One such mutation, Delta F508, affects Over 80 percent of the CF population. There are several drug therapies in trial phases that could be effective for this larger CF population.
“We are hopeful that in the next five years, we will have a [version] of Kalydeco for the Delta F508 population,” Herriman says.
In addition to medication, little Aven spends one and a half hours a day wearing a vest that vibrates to help break up the thick mucus. She also takes approximately 6,000 pills a year just to help her absorb fat and fat-soluble vitamins, since the mucus in her digestive tract is sticky and it is very hard to absorb nutrients.
“Our hope is that one day we can say that CF stands for ‘Cure Found,’ ” Emery says. “Until then, we will continue our fundraising efforts and help spread awareness about this awful disease.”
Imagine a day when Aven has a child and she does not have to fear passing Cystic Fibrosis to her little one. That’s what Taste of the Finest hopes to do through continued fundraising and awareness.
“Last year was our first year … and we were blown away by the success,” says Horrell of the event, which raised more than $100,000. “We had over 400 people in attendance and we anticipate that number to be even larger this year. Guests can expect a lot of fun, food, drinks and dancing. With tickets starting at $50, this would make a perfect date night or night out with your friends. I know it was a big hit with all of my friends last year.”
A Day in Aven’s Life
• Wake Up
• Breakfast: Pediasure for extra calories along with any other food she consumes
Pancreatic Enzymes — three pills
Nexium — helps absorb enzymes
Claritin — allergies
Flonase — steroid
AquaDEK — Fat soluble vitamin
• First Vest Treatment: 30 minutes, Albuterol, Flovent
• School (Aven attends the Arkansas Virtual School Academy): playtime, physical exercise. Physical exercise is very important for CF patients because it is a natural form of Chest Physiotherpy.
• Snack time: Pancreatic enzymes (number of pills depends on how much and what she eats)
• School: playtime, physical exercise
• Lunch: Pediasure along with any other food she consumes
Pancreatic Enzymes — three pills
• School — playtime, physical exercise
• Second Vest Treatment — 30 minutes, Albuterol, Flovent
• Snack time: Pancreatic enzymes
• School: playtime, physical exercise
• Dinner: Pediasure for extra calories along with any other food she consumes
Pancreatic Enzymes — three pills
Nexium — helps absorb enzymes
Hydroxizine — allergies
Flonase — steroid
• Third Vest Treatment: 30 minutes, Albuterol, Flovent
• Tobi: inhaled antibiotic to treat Psuedomonas, a very harmful bacteria for CF patients
• Bedtime
Taste of the Finest
When: Friday, Aug. 23 | 6 p.m., VIP | 6:30 p.m. general admission
Where: Metroplex Event Center/Team Summit, 10800 Colonel Glenn Road
Tickets: $50 for general admission, $75 for VIP
Info: Arkansas.CFF.org/tasteofthefinest
Honorees
Chris Bray, Bray Gourmet; Haley Burks, Rose Law Firm; Danielle Crafton, Regions Bank; Amanda Elrod, Coldwell Banker Rector Phillip Morse; Rick Gillham, Crews & Associates; Payne Harding, 1620 Savoy; Sarah Hays, Elite Chiropractic and Acupuncture; Trevor Holbert, Merrill Lynch; Chris Howe, Iberia Bank; Andrew Huett, Unified Systems; Jennifer Imbro, Stephens; Courtney Little, ACE Glass; Justin Long, Stephens; Jeff Marcussen, Baldwin & Shell Construction; Drew Mentzer, Flake & Kelley Commercial; Elizabeth Meyer, OneBanc; Michael Neathery, BKD, Inc; Jimbo Ramsay, The Cashion Company; Chris Robinson, Delta Trust Mortgage, Inc.; Brent Salter, Salter Construction; Jennali Shook, Arvest; Chris Stewart, Stewart Law Firm; Mickey Willett, The Social Type; Ashley Younger, Mitchell Williams
As co-chair of Taste of the Finest, MacKenzie Horrell helps raise awareness of the Cystic Fibrosis Foundation and its mission to find a cure for CF. Dress and accessories from Barbara/Jean Ltd.; hairstyling by Carrie Parsons; makeup by Dustye Helms.
The 2013 Arkansas Cystic Fibrosis Foundation Ambassador, 6-year-old Aven Emery of North Little Rock, poses in the Soirée studios. In a year’s time, Aven takes approximately 6,000 pills and spends at least 40 hours a month wearing her vibrating vest, which helps to break up the sticky mucus in her lungs.