Clinicians say a combination of factors have led to improved therapy adherence and disease management in young cystic fibrosis patients since the outset of the novel coronavirus pandemic.
By Lisa Spear
Cystic fibrosis, a rare and progressive disease, leads to the buildup of mucus in the lungs. Under normal circumstances, cystic fibrosis patients are encouraged to use airway clearance techniques and pharmacological interventions to avoid infections, but many pediatric patients struggle with the burden of daily therapy routines. Since the COVID-19 pandemic began, some cystic fibrosis centers throughout the country have seen an uptick therapy adherence among young people.
“What we are seeing from all of our patients is that they actually have been much more diligent with using their treatments,” said pediatric pulmonologist Deanna Green, MD, MHS, the medical director of the Cystic Fibrosis Care Center and the CF Therapeutic Development Center at Johns Hopkins All Children’s Hospital in Saint Petersburg, Fla.
Overall, there are 30,000 cystic fibrosis patients in the nation. Recent data suggests that these individuals may have a lower incidence of COVID-19 than the general public. According to a recent manuscript, this is potentially due to this community’s strict adherence to physical distancing and other infection control practices. At the time of publication, the rate of infection for people with cystic fibrosis in certain countries was about half the levels of the general population.
An unanticipated positive outcome of the pandemic is that many of these patients managed to avoid, not just COVID-19, but other viral infections by staying home more, avoiding exacerbations, and staying out of the emergency room, according to several clinicians.
“What we have seen, which is really interesting, is that our patients have not been sick in general recently,” said pulmonologist Elizabeth Burgener, MD, who treats pediatric patients at Lucile Packard Children’s Hospital at Stanford in Palo Alto, Ca.
She said there have been fewer hospital admissions among her patients since March as well as fewer sick calls. “I think that cystic fibrosis patients are very good at isolating and avoiding people who are sick. The community as a whole has been sheltering in place. It’s really decreased the transmission of all viruses,” said Burgener.
Early data from European countries, published in The Lancet Respiratory Medicine, suggests that few patients with cystic fibrosis, mainly adults, are becoming infected with COVID-19, “without apparent effect on cystic fibrosis disease severity.” The researchers say that from the limited data, it is not possible to identify factors that could be protective, for instance use of long-term antibiotics.
Pediatric pulmonologist Green said she has also noticed a trend in declining hospital admissions among these patients during the pandemic. At her center, clinicians take care of 180 cystic fibrosis patients in the Tampa Bay area. Typically, the affiliated hospital will have about 4 of those patients admitted during a typical week. After March, hospital admissions of these patients dipped down to one patient every three weeks. “Absolutely, we’ve had a significant decline in the number of [hospital] admissions for patients,” she said.
The reason for the decline in hospital admissions is twofold, said Green. Heightened precautions and better therapy adherence are factors, she said, but another reason for the drop in adverse outcomes is a new drug called Trikafta, a combination of three medications that target a common gene defect in cystic fibrosis patients. The US FDA approved the drug in October of 2019 and clinicians are already seeing positive outcomes.
“That medication was predicted to reduce the number of admissions by two thirds. For us, we definitely saw that because we were already down to having an admission once a week pre-COVID,” said Green.
Trikafta is approved for patients 12 years and older with cystic fibrosis who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which is estimated to represent 90% of the cystic fibrosis population. Two trials demonstrated that Trikafta could improve lung function in these patients, according to a statement from the FDA.
“I think that you cannot downplay, for our patients, the impact that the newest medication has had with them. We all feel very strongly that if we could not have had Trikafta here in 2020. This could be a very different scenario for our patients,” said Green.
Since the first cases of COVID-19 appeared in the United States in March, Green’s patients have ramped up measures to control their conditions, by increasing their use high-frequency chest wall oscillation, also known as vest therapy, to shake the secretions free from their lungs to avoid infections. This airway clearance technique is important for maintaining lung function for patients with cystic fibrosis, but it can be time-consuming and cumbersome for some. One Respiratory Care study that collected vest usage data over a 12-month period found that among adolescent subjects age 13- to 19-years-old, average adherence to daily vest therapy was only 44.0%.
“We are finding out that school, their lifestyles, got in the way. Many of them were non-compliant,” said Green, who was not involved in the study.
The treatment regimen that these patients are supposed to follow depends on the severity of their disease but could involve at least three different inhaled medications and the use of a high-frequency chest wall oscillation or a high-frequency chest wall compression vest, which lasts for 30 minutes each session. This routine typically takes patients an hour to perform, twice per day. Many young patients find it challenging or too time consuming to do all the steps involved, and many would skip a treatment session to save time, said Green.
There is also the potential issue of not using the therapy devices correctly. Another treatment to help these patients clear excess secretions from their lungs, oscillating positive expiratory pressure. The therapy involves breathing with an active expiration against expiratory resistance through a device. Despite standardized instruction, there is vast variation in the technique. Incorrect use of the device can lead to poor airway clearance, according to research published in Respiratory Care.
Before the pandemic, Green said that many of her adolescent patients tended to only do their treatments about once a day. Post-COVID, and “especially since they are not going to school, they can’t really use that as an excuse.” With more time at home, patients have found more opportunities to use their therapy devices.
“Some of our patients are a little bit more adherent because they are home more. I think a lot of the difficult for teens and young adults is that it is hard to do your treatments when you are not home,” said Burgener.
Other safety measures, including the use of personal protective equipment, came naturally to this population. “They were very used to having to wear masks, so when we talk to many of our patient, they were actually very happy about the mandates across the country for people to wear masks because now basically everybody else in the nation caught up with them,” said Green. “These patients are much more used to infection control practices and policies.”
Since cystic fibrosis centers in the hardest hit regions of the country have pivoted to telemedicine, patients have been forced to take a more active role in their care. While in-person spirometer readings and throat swab cultures stalled, a program through the Cystic Fibrosis Foundation enabled some patients to have home spirometers to continue to monitor their lung function.
While cystic fibrosis patients are doing better during the pandemic than initially anticipated, clinicians say, there are still many unknowns about how COVID-19 impacts this population.
According to the Centers for Disease Control and Prevention, people with cystic fibrosis might be at an increased risk for severe illness from COVID-19, but since this virus is a new disease there is currently a limited amount of information about the impact of underlying medical conditions. Currently, providers continue to recommend that their patients continue to stay at home and pursue virtual education.
Lisa Spear is associate editor of RT. For more information, contact [email protected]
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