BENEFITING: Cystic Fibrosis Foundation
ORGANIZER: Cystic Fibrosis
EVENT: 2018 Pittsburgh Marathon
EVENT DATE: May 06, 2018
Emma, my granddaughter, is 15 years old and a 9th grader at Penn Trafford High School. Emma has Cystic Fibrosis and the reason I "Run to Cure CF.” What Is Cystic Fibrosis? 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 clogs the lungs and leads to life-threatening lung infections, and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. The first year of Emma’s life was a very horrific and terrifying journey, not only for her but for her family as well, particularly her mom, Kris. Emma received multiple breathing treatments daily as well chest PT several times a day. Emma was frequently on antibiotics to help keep her lungs clear. At age 5, Emma was diagnosed with CF related diabetes; she is now insulin pump dependent. Emma is a very intelligent active teenager who plays tennis, loves to be on stage and is very involved in her church youth group. Emma does all of that in spite of her daily CF regimen that includes nightly gtube feedings, 1 hour of treatments and vest 3-4 times per day. Emma also takes enzymes with every meal to help her digestive system work properly. She has been getting IV antibiotics thru a Mediport for 2-3 week courses three to four times a year for more than a few years. Emma's Lung functions (PFTs) have struggled to stay at least 60%. Healthy PFTs are 120% so her lungs are working at only 50% or less. I asked her how she is able to run in tennis and she said it depends on how much running her opponents do. I guess that wasn’t the answer I expected but to her it made perfect sense; she goes with life as is. Emma has double delta f508 mutation. Orkambi and Symdeko are the CFTR Modular meds for her mutation. She tried Orkambi for 3 months but had too many negative side effects that outweighed the positive. CF Research will continue until the negative side effects are minimal or non-existent.