Cystic fibrosis was one of the first diseases I was aware of. Rosemary Simpson had it, and our mothers were best friends. So, I played by myself on the days Rosemary lay on a slanted wooden board, and our mothers pounded her back to loosen the mucus in her lungs.
Once, when I had bronchitis, my mother did the same to me. Beat her fists up and down my back. Something she'd learned from Rosemary's mother. But, we were both aware of the difference.
Rosemary was the youngest of four children. Her oldest sister was the only child without cystic fibrosis. Her mother buried her two brothers when they were teenagers, and finally Rosemary. Before her 14th birthday.
Many people aren't very aware of this disease. Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that not only clogs the lungs and leads to life-threatening lung infections, but also obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. Since it's a gene defect, lung transplants are useless.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 40s and beyond, although there is still no cure.
Early diagnosis and treatment dramatically improve the prognosis for CF according to Dr. Joel Ledbetter, a pediatric pulmonologist at Children's Hospital. “Tennessee has been conducting newborn screenings for CF since 2008, and there is a great advantage when we can begin treatment before the child gets ill,” he says. If too much time passes before diagnosis, there can be lung damage, as well as malnutrition.
King Birnbaum has battled this disease his whole life. He is a warrior, no doubt. And he has a mighty army behind him. His parents, Ginger and Alex, and his sister, Emma Virginia, are the sort of folks you want on your team. Or in your battalion. They aren’t passive or accepting or making the best of this disease. They are making change. Difference makers, they are not strangers to Capital Hill. And major drug companies know their names.
Most folks don’t know about Trikafta, but a heck of a lot of folks do, thanks to the Birmbaums. It’s a new drug that promises to be life-changing for people with cystic fibrosis, and this family has done all in its power to bring it to fruition, including affecting change in Washington, D.C. With FDA approval of this drug, which should benefit over 90 percent of people with CF, cystic fibrosis is referred to as “the greatest story in medicine.” Trikafta is not a cure, but it’s an incredible milestone, one the Birnbaums have been praying for. And Ginger was desperate to get this tiny pill in her boy’s hands.
“King has tolerated Trikafta well so far! My prayers had been that not only would the medication be approved for those under the age of 12, that our insurance company would approve it, that King could get it in his hands relatively fast, but that he would also tolerate it physically and emotionally. We have a couple of hiccups to sort out, but overwhelmingly the pros outweigh the cons,” Ginger said. Grateful that her son finally has this long-awaited drug in his hands, she is not taking any sort of respite; her battle is far from over.
“King needs a cure, and there are still people (close to 10 percent) without effective treatment at the cellular level. Now it’s getting this drug in their hands I dream about!” Ginger said as she and her family readied themselves for a long distance hike to benefit CF.
The Cystic Fibrosis Foundation’s inaugural Xtreme Hike is underway, and the 12-mile hike took place at Prentice Cooper State Forest yesterday, raising funds for CF. Twelve miles of diverse elevation and terrain is challenging and a far way to go for sure, but it is no hill for a stepper. King’s team page is FIGHTCF.CFF.org/go.
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Ferris Robinson is the author of three children’s books, “The Queen Who Banished Bugs,” “The Queen Who Accidentally Banished Birds,” and “Call Me Arthropod” in her pollinator series. “Making Arrangements” is her first novel. “Dogs and Love - Stories of Fidelity” is a collection of true tales about man’s best friend. Her website is ferrisrobinson.com and you can download a free pollinator poster there. She is the editor of The Lookout Mountain Mirror and The Signal Mountain Mirror.