Posted by Brigham and Women's Hospital May 30, 2012
2012 is the year that, for the first time, more adults than children will live with cystic fibrosis (CF) – a chronic, inherited disease that affects the lungs and digestive system of about 30,000 U.S. patients. Just 50 years ago, most CF patients didn’t live past elementary school. But modern medicine works wonders, and various treatments have helped most CF patients live into adulthood – often into their 30s, 40s, and beyond.
It’s tempting to end the story there – on a positive note. But for CF patients, the story is complicated – because the triumph comes at a cost.
Beth Peters is a 31-year-old CF patient, a high school teacher, and a theater director. With much of her lung function restored through a recent lung transplant, she considers herself lucky. She’s vibrant, smart, funny, and wears a bright smile. She looks great.
And that gets confusing for those around her – because often she doesn’t feel great.
“Anybody with CF – whether they are healthy or not, before or after a transplant – the amount of energy they have to put in just to be status quo – it requires hours of ‘stuff’ every day,” Beth explains. “Even if they look good, it’s still so much work.”
The “stuff” is a daily routine that can absorb hours of every CF patient’s day:
- Oral medications – about 30 pills per day, spread throughout the day
- Infusions (intravenous therapies) – ranging from 15 minutes to three hours per day, depending on the situation
- Inhaled medications – three hours every day, stuck in a chair with a machine on your face
- Airway clearance – can be handled through special vests that beat on the chest, particular breathing techniques, or machines; daily commitment varies
- Exercise – about 30 minutes (or more) of sustained exercise per day
Then there’s the ongoing string of diagnostic tests – ultrasounds, MRIs, bone density scans, and so on. Even after all that, CF patients remain susceptible to sicknesses that sometimes land them in the hospital for week- or month-long stays.
These therapeutic rituals become a lifestyle for both children and adult CF patients. But adult CF patients face unique challenges: They’re trying to get jobs, maintain friendships, enter the dating scene, live their lives – all while managing their treatment.
In another life Beth might have pursued a more rigorous career in theater. “But with CF, I could never be a starving artist.” The health benefits and physical demands of a job are fundamental concerns. And remember all that treatment “stuff?” In the past, Beth had to take off every Wednesday to manage her health. The school hired a substitute to fill her mid-week absences.
Through all of life’s ups and downs – some health-related and some not – Beth, like many CF patients, has a close circle of very loyal friends. She also treasures her online community, in which CF patients process their various anxieties and joys.
And now – in an event she’s helping to organize through the Adult Cystic Fibrosis Program at Brigham and Women’s and Boston Children’s Hospital – she’s trying to help that community take its stories to a broader audience.
On June 17, “The Power of Two in You: Staying Healthy, living with CF and lung transplant” will feature a special screening of “The Power of Two,” a documentary offering an intimate look into the lives of twin sisters living with CF after double lung transplants. The event, which also includes breakout sessions on balancing a busy schedule with cystic fibrosis therapies and maintaining health through exercise, will convene CF patients, families, and friends with cystic fibrosis specialists to discuss issues important to the CF and transplant communities.
For family, friends, doctors, and nurses who witness only specific “moments” in the CF patient’s life, the event and documentary will provide a rare window into the ongoing struggle – and triumph – of living every day with cystic fibrosis.
– Linda W
You are invited to attend “The Power of Two” in person or online (either way, please RSVP online). To follow Cystic Fibrosis Foundation Infection Control Guidelines, patients with CF aren’t able to attend the event in person; however, the event will be streamed live, allowing CF patients to participate remotely – adding comments or asking questions through programs like Ustream (streamed live at childrenshospital.org/CF) or Facebook. Limited access to the documentary also will be provided on iTunes to those who RSVP.