Case: Managing Cystic Fibrosis in a Patient With a Complicated History
Pediatric pulmonologist Nathan Kraynack, MD, reflects on a complicated case and looks ahead as they transition the patient’s care to the adult cystic fibrosis team.
Cleveland Clinic physicians “reinvented” a teenage patient’s cystic fibrosis (CF) care almost four years ago after he experienced more than a decade of limited access to comprehensive care, compounded by a rare infection. Now, with a good quality of life and well-managed disease, they’re getting ready to transition his care to the adult CF team.
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A 14-year-old male patient with CF first presented to Cleveland Clinic’s Pediatric Cystic Fibrosis Clinic in 2018 with a nontuberculous mycobacteria (NTM) infection. The infection was contributing to his deteriorating lung function.
In the United States, about 6%-7% of adults develop NTM lung disease, and though most people come into contact with the bacterial pathogen Mycobacterium avium complex (MAC) at some point, only few develop an infection. However, in patients with an existing respiratory illness, it can lead to severe and life-threatening disease.
The infection-causing bacteria are typically found in soil, dust, and water, and can also be found in natural and municipal water sources, according to the Centers for Disease Control and Prevention. NTM is related to tuberculosis and can be similarly challenging to treat, explains Nathan Kraynack, MD, a pediatric pulmonologist at Cleveland Clinic Children’s.
Dr. Kraynack, who also leads the Pediatric Cystic Fibrosis Program at Cleveland Clinic, says it remains unclear how many children develop the infection. One of its clinical hallmarks is the ability to produce mucous, which most children, even those with CF, are unable to do.
Dr. Kraynack has been treating the patient, now almost 18, since their initial encounter in 2018. The family had recently emigrated from South Africa and chose Cleveland Clinic as their destination for care. The initial CF diagnosis was made around age 9 months, but the patient and his family experienced difficulty accessing essential care for CF, which was later further complicated when he acquired NTM.
Dr. Kraynack recalls the initial encounter: “We immediately realized we needed to reinvent his CF care. He was not getting what we consider an adequate level of comprehensive care to manage the disease, and the organism had also become treatment-resistant.”
At this time, he had already transitioned off an intravenous antibiotic, which had been making him very sick, explains Dr. Kraynack, and can lead to hearing loss and kidney damage in some patients. “When he came to see me, he was on an inhaled form of that antibiotic, in addition to two other antibiotics, Biaxin and Cipro, and had received several rounds of treatment for a fungal infection,” he says.
“We adjusted his regimen based on the cultures and added standard airway clearance, inhaled mucolytic agents and azithromycin,” says Dr. Kraynack.
It took about two months to find the perfect cocktail of antibiotics to treat the infection and also avoid an adverse drug interaction; it took about two years until his lung function stabilized.
“He’s been stable for the last couple of years, and we haven’t had to treat him for the fungal infection recently; he’s been on maintenance therapy for the nontuberculous mycobacterial infection.”
He has also been taking cystic fibrosis transmembrane conductance regulator (CFTR) modulators, described as “groundbreaking” medications that improve the function of the CF-mediated protein. These have helped his lung function improve even more.
He says it’s a testament to the importance of delivering the gold standard approach to therapy even if it’s relatively late. “It could have implications for how well people feel but also for their long-term survival.”
As the patient turns 18 this year, Dr. Kraynack and his team will soon transition his care to the adult CF team. His care will remain under the same roof, his new care team can easily access his complicated medical and treatment history, and he will retain the same dedicated social worker.
The connectivity between adult and pediatric CF providers also helps facilitate this movement along the lifetime care continuum, which is still a relatively new paradigm in the CF community.
“Thankfully, many people are living with CF into their 40s, 50s, and 60s, which was almost unheard of during my training 25 years ago,” says Dr. Kraynack.
Dr. Kraynack and colleagues work alongside Elliot Dasenbrook, MD, who leads the Adult Cystic Fibrosis Program, to make sure the patient and family feel supported as they transition care.
“I’ve known Dr. Dasenbrook for 20 years; I know who he is as a person, a clinician, a researcher; I quite literally trust him with my own family members, which makes the hand-off much easier.”
“Whether it’s a patient from halfway across the world or a patient 20 miles down the road, providing a comprehensive standard of care, persistence in a treatment plan, and educating patients and families on the importance of adherence can really change their lives.”