This is Jameson.
Jameson was diagnosed with cystic fibrosis (CF) at 2 weeks of age.
Mom and Dad were not aware they were CF carriers until they were pregnant with Jameson. His siblings, Anna and Jeremiah, do not have CF nor does anyone else in his family.
At its core, CF is caused by a mutation in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene. This gene is responsible for creating a protein that acts as a "gate" on the surface of cells, regulating the movement of salt and water.
In an everyday healthy body: Salt moves freely, keeping the mucus on our organs thin and slippery.
In a body with CF like Jameson: The "gate" is broken or missing. This causes mucus to become thick, sticky, and dehydrated.
Instead of acting as a lubricant, this thick mucus clogs various pathways throughout the body.
CF affects the lungs, pancreas, reproductive system, and digestive systems.
Jameson’s Personality
He is a true lover of life, finding joy and meaning in the simplest of things. He is rooted in his faith and love for gardening and farming, introduced by his Papa. He loves to be surrounded by his family and friends and being the social butterfly he is. Perhaps his most significant trait is infectious laugh - a sound that could instantly brighten a room. He also enjoys being a 6-year old boy; playing Minecraft, learning golf and tennis, obsessing over Dude Perfect (and many trickshots) and countless you-tubers, wrestling with his Dad, and hanging out with his brother and sister.
Love of Roses
In 1965 a woman named Mary G. Weiss, who became a volunteer for the Cystic Fibrosis Foundation after her three sons were diagnosed with the disease.
The Moment: Mary was tirelessly making phone calls to seek funding and support for CF research.
The Discovery: Her 4-year-old son, Richard, listened to her speak the words "Cystic Fibrosis" over and over again on the phone.
The Quote: After a while, Richard went to his mother and said, "I know what you are working for." When she asked what, he replied, "You are working for 65 Roses."
Since that moment, the rose has become the official symbol of the Cystic Fibrosis Foundation and the CF community worldwide. Jameson has adopted this love and symbol of hope for his story. He also enjoys saying he has “65 Roses”.
A Day In The Life Of Jameson
His healthy daily routine is 2-3 airway clearances (which includes a vest that shakes his body for approximately 30 minutes each session) and breathing treatments a day; 4-5 if he has lung exacerbations. Twice a day pills (including CF modulators) and twice a day steroid inhalers. All of this increases if he becomes ill.
Why This Matters
CF is an autosomal recessive condition. This means many people are "carriers" without ever knowing it because they don't have symptoms themselves. Knowing about CF helps you understand how a small group of dedicated parents and patients can literally change the life expectancy of an entire population through sheer willpower and organized fundraising.
In the 1950s, children with CF rarely lived long enough to attend elementary school. Today, because of early diagnosis and the rise of modulators (medications that help the "broken gate" protein) work more effectively at the cellular level.
The average age of someone living with CF is now 65 years of age! Cystic Fibrosis patients are living longer because folks are donating to research, donating to cause, donating for cure! Right now, the most important part of "knowing" about CF is understanding that the job isn't done. About 10% of people with CF have "nonsense mutations" that modulators can't fix.