When Megan Carey was pregnant with her son, Josh, an amniocentesis revealed he carried the mutation for cystic fibrosis (CF), a progressive, genetic disorder that causes frequent lung infections and can impair breathing. Questioned by her doctors about whether she wanted to continue the pregnancy, Megan says there was never a doubt.
“I had half-sisters who were twins who had CF,” she said. “I knew what I was signing up for.”
Megan says although her sisters, Tanya and Tiffany died at ages 25 and 30, she knew advances in medications and treatments had come a long way.
“Josh has had a very different life than my sisters,” she said. “He’s much healthier because we are strict about following treatment protocols.”
At 17 years old, Josh is a junior in high school who plays saxophone in the marching band, and enjoys basketball, riding his bike, fishing and painting cars—something he does through an auto collision program he is enrolled in at Jefferson County Vocational School. His days begin and end with hour-long treatments for his cystic fibrosis. He also takes medication to treat his cystic fibrosis-related diabetes (a unique type of diabetes that is common in people with CF). In all, Josh takes 34 pills a day.
“I get up between 5-5:30 a.m. and do 3 separate breathing treatments (albuterol, hypertonic saline and Pulmizyme®) followed by wearing a battery-powered inflatable vest that vibrates to help loosen and thin my chest mucus,” he said.
Over the course of his life, Josh has been hospitalized approximately 10 times for respiratory infections. During his 1- to 2-week hospital stays, he enjoys building Lego sets (his record for completing a 500-piece set is about 10 minutes!), playing Minecraft on the Xbox and mini nerf basketball.
“I love getting admitted to the hospital because the food is amazing,” he said. “I order the same thing every time—a bowl of chicken noodle soup, 3 hash browns, 6 slices of bacon, sweet tea, a red and blue slushie, hot chocolate and a Rock’N Refuel drink.”
A patient of pulmonologist Dr. Heather Strawbridge, Josh is known affectionately for naming his IV pole (Ivan) and drawing a picture with a face on it to entertain staff. During COVID, Josh avoided being hospitalized for the better part of a year—one of the silver linings of the pandemic.
“With everyone masking and social distancing there were less opportunities for Josh to pick up an infection,” Megan said.
Prior to COVID, Megan was actively involved in CF advocacy and quality improvement work. She participated in surveys and conversations from the parent perspective about what can be done to improve the patient experience for CF patients. She also attended a national CF conference in Denver.
“I want to stay up to date on all the new things happening in the CF world,” she said. “There are some exciting things on the horizon that one day may help to find a cure. I hope I can get involved again now that we are getting through the pandemic.”
Josh has the most common mutation of CF and may never require a lung transplant.
“CF is different for every person,” Josh said. “Your lung function has to be at about 30% to be eligible for a transplant. Luckily, my lungs are still between 90-100%.”
“It’s scary at first to find out your child has CF,” said Megan. “But looking at Josh now and how far he’s come, I am confident he will have a normal life just like any other kid.”