At Mercy Northwest Arkansas, a team dedicated to providing care for those with cystic fibrosis is excited about recent medical advancements that will bring a better prognosis for their CF patients.
As one of only two adult cystic fibrosis clinics in Arkansas, Mercy NWA provides care close to home for Northwest Arkansas patients. Robbie Land, cystic fibrosis coordinator with Mercy NWA, explained the main issue CF patients face is respiratory distress. As symptoms worsen, bacteria often develop in the lungs leading to hospitalization, often for up to two weeks at a time.
Cystic fibrosis is a chronic illness caused by a genetic defect that involves the respiratory, digestive and reproductive systems. Symptoms for the disorder can range from mild to severe. Some of the more prevalent symptoms include:
- Thick, sticky mucus.
- Coughing or wheezing.
- Recurrent lung (respiratory) infections with resistant organisms.
- Recurrent sinus infections.
- Delayed or poor growth.
- Enlargement or rounding of the fingers or toes.
Many CF patients may begin to experience new symptoms as they get older. The treatment for cystic fibrosis varies based on the individual and may include airway clearance, medications and nutritional support. At Mercy, patients receive care from a team of experts, including physical and respiratory therapists, dietitians, pharmacists and others.
The Mercy NWA Cystic Fibrosis Clinic was created five years ago after the Cystic Fibrosis Foundation reached out on behalf of the growing population of adult CF patients in Northwest Arkansas. Dr. Jason McKinney, pulmonologist and critical care director at Mercy NWA, said the disease is rare but common enough that the Mercy team had training with it.
“It's a little bit different than a traditional, regular pulmonary clinic in that it's a complex disease and it affects multiple systems, not just the lungs,” he said. “But it really impacts the patient in various ways – from nutrition to psychological, from malnutrition to lung disease and multiple infections, all sorts of different things. We really try to focus the clinic in a way we can really give each patient 360-degree care in one instance.”
Taking on cystic fibrosis is a team effort at Mercy NWA, Dr. McKinney said.
“The patient meets with a pulmonologist, followed by a physical therapist, a dietitian and a social worker to make sure they have access to everything they may need. There’s also a respiratory therapist and a clinical pharmacist involved, as the patients are on multiple medications and might have issues tolerating them or making sure they're dosed appropriately,” he said. “We want to provide that service for them within our own community.”
Amy Hickman, whose daughter Azaylee suffers from cystic fibrosis, has always found ways to find hope despite her daughter’s diagnosis.
“When she was born, my husband and I, we did not even know what cystic fibrosis was,” Hickman said. “It was a lot of information at once, very overwhelming. But as we learned more and more, of course it was scary, and it was just really unknown. But getting involved with the Cystic Fibrosis Foundation, and of course her doctors who are wonderful, we became hopeful for the future.
“We’ve never been given any ‘doom and gloom’ prognoses; it's always been hope for a cure, hope for medications that will help her and be life-changing.”
With the advent of Trikafta, many CF patients have seen great improvement, Land said. Patients on the maintenance medication are showing better pulmonary function and less secretion into their lungs. The drug recently was approved for use in patients ages 6 and older who qualify.
“The patients feel so much better,” Land said. “I'm so excited that I'm able to be involved in that. I have one patient who first arrived in a wheelchair and on oxygen with really low lung function. He has been on Trikafta for a year and now he walks into our clinic for visits. So, it's just such a blessing; it's just amazing.”
Dr. McKinney agreed, “It's been probably one of the most remarkable things that I've seen so far in my career where people who are extremely debilitated, extremely sick, then go through this kind of transformation process as they start on this medication to where they feel almost normal.”
The Mercy team has watched some patients who had very impaired lung function normalize their lung function over the course of treatment.
“It’s really been remarkable to see people who have been chronically ill their entire life, since they were infants, and now they feel normal,” McKinney said. “Just to see what that means for those people, to feel like what a lot of us take for granted every day, it's been very impressive.”
Diane Byram, associate executive director of the Arkansas chapter of the Cystic Fibrosis Foundation, said the foundation has accelerated research over the past 15 years and is excited about what Trikafta has done for patients.
“It's been life-changing, and it's been really exciting to see the older population and hear from them, and hear how miraculous and life-changing this drug has been in their lives and how their lung capacity has increased, their weight gain, just their entire quality of life,” she said. “So, the younger population, they’re so excited by what’s happened, knowing the progression of lung disease can be prevented through this drug and they can live a typical life.”
Land said they will do more studies to see whether at some point time there may be treatments that patients don't have to do. Land said further studies could allow patients to receive treatment that require less work and are easier to maintain.
“It's really rigorous for patients – they get up in the morning and do nebulizer treatments, they’re on enzymes, there are many different medications taken several times a day and repeat the process over and over it really consumes their life. Even if they're able to cut back on some of that treatment down the road will be a blessing,” she said.
Dr. McKinney said he is pleased with the progression of treatments for CF patients in recent years and is grateful for the community support that’s been shown.
“The thing about cystic fibrosis is that while it's rare, it's not as rare as we think,” he said. “It's also a disease that shows us when any disease process gets the attention it deserves, often in the form of focused support, it can come full circle. We’ve seen what happens when people invest dollars into a disease and industry invests time and scientific energy – patients gain hope and often what feels like a cure in some instances. Now, they're not cured, but this will translate into years and decades of increased life expectancy and improved quality of life for these patients.”
Hickman said she is grateful for the huge support system the family has in providing help for her daughter, including the Cystic Fibrosis Foundation and the Mercy doctors who treat her daughter.
“Even though it's a rare disease and there aren’t a lot of patients, they still want to help every person possible,” she continued. “I know that even as she's gotten older, life expectancy for CF patients has increased, and I know that it will continue to increase. She's at the perfect age that maybe CF won’t even affect her life expectancy that much.”
Land added that she hopes one day “CF” stands for “Cure Found” instead of “cystic fibrosis.”