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girl with cystic fibrosis getting breathing treatment

The role of long-chain fatty acids in cystic fibrosis inflammation

girl with cystic fibrosis getting breathing treatment

A recent study sheds light on the microbiologic triggers for lung inflammation and pulmonary exacerbations in cystic fibrosis.

Cystic fibrosis is an autosomal recessive disease that affects more than 70,000 people worldwide and results in a progressive decline of lung function. Patients with cystic fibrosis experience intermittent episodes of acute worsening of symptoms, commonly referred to as pulmonary exacerbations. While Staphylococcus aureus and Pseudomonas aeruginosa are thought to contribute to both lung inflammation and pulmonary exacerbations, the microbiologic trigger for these events remains unknown. Andrea Hahn, M.D., M.S., and her colleagues at Children’s National Hospital recently shed light on this matter by studying the changes in bacterial metabolic pathways associated with clinical status and intravenous (IV) antibiotic exposure in cystic fibrosis patients.

The researchers found increased levels of long-chain fatty acids (LCFAs) after IV antibiotic treatment in patients with cystic fibrosis. LCFAs have previously been associated with increased lung inflammation in asthma, but this is the first report of LCFAs in the airway of people with cystic fibrosis. This research indicates that bacterial production of LCFAs may be a contributor to inflammation in people with cystic fibrosis and suggests that future studies should evaluate LCFAs as predictors of pulmonary exacerbations.

Additional authors from Children’s National include: Hollis Chaney, M.D., Iman Sami Zakhari, M.D., Anastassios Koumbourlis, M.D., M.P.H. and Robert Freishtat, M.D., M.P.H.

Read the full study in Pediatric Research.

asthma medication delivery

School’s in for asthma medication adherence

asthma medication delivery

A research team from Children’s National tried to reduce missed doses of daily medications, improve asthma control and tamp down on schoolchildren’s asthma attacks by outsourcing morning delivery of inhaled corticosteroids to the school nurse.

Doctors and researchers have long known that the level of stress patients experience is inversely linked to how adherent they are with taking medications: The higher the stress, the less likely patients are to take doses of their medication correctly, on time or at all. For families of school-aged children, there are few times more stressful than mornings, when parents or caregivers need to get kids ready for their school day, pack everything they need and get them out the door on time.

These stressful mornings, says Stephen J. Teach, M.D., M.P.H., chair of the Department of Pediatrics at Children’s National Health System, can spell danger for children with persistent asthma. This chronic condition is typically treated with nightly and morning doses of inhaled corticosteroids (ICS), medications that decrease lung inflammation to prevent asthma attacks. When children miss a morning dose because their families are too busy, their asthma symptoms can exacerbate, causing them to miss school, be unable to participate in activities like sports or lose sleep at night.

But Dr. Teach and colleagues had a simple idea to bypass the morning struggle for many families: Instead of trying to fit delivery of ICS into an already packed schedule, why not outsource it to the school nurse?

“We thought that if we could have those morning doses administered by these medically trained individuals with great technique and regularity, then maybe we would see some improved outcomes in kids,” Dr. Teach says. “And we did, in a striking way.”

Dr. Teach and colleagues recruited 46 children to participate in a pilot study, published online June 8, 2017 in the Journal of Asthma. To be eligible, these participants had to be in grades kindergarten through eighth in the Washington, D.C. public school system and on Medicaid, demonstrating the type of financial need that can add to the cumulative stress a family already faces. The children were scattered across 18 schools.

“We thought that if we could have those morning doses administered by these medically trained individuals with great technique and regularity, then maybe we would see some improved outcomes in kids,” Dr. Teach says. “And we did, in a striking way.”

Twenty-one of these participants received morning doses of ICS (the intervention group), which the researchers provided to school nurses along with an asthma action plan. The rest (the control group) remained on their prescribed morning and evening doses at home.

After 60 days, the researchers followed up with schools and families. Through electronic records kept by each school, the researchers found that the intervention group received more than 90 percent of their prescribed morning doses—about the same number reported by parents of the control group. However, the two groups demonstrated impressive differences in quality-of-life measures:

  • While about 24 percent of the intervention group missed one or fewer days of school due to asthma during the 60-day trial, about 44 percent of the control group did.
  • About 43 percent of the intervention group reported functional limitations due to their asthma, compared with 74 percent of the control group.
  • The intervention group reported only 1.7 nights with asthma-related sleep loss in the previous two weeks, compared with 4.1 nights in the control group.
  • Additionally, only about one-quarter of the intervention group required adjustments in family life to accommodate their asthma, compared with more than one-half of the control group.

The reasons for these differences aren’t clear, says Dr. Teach. But he and colleagues suggest that they might be due to over-reporting of how many doses were delivered at home in the control group or improper administration of these drugs at home.

Regardless, he says, the results show that this type of school-based intervention was not only feasible for children, school nurses and families, but also led to numerous positive health outcomes for the participants who received it. Based on the results of this study, Dr. Teach and colleagues have started to prescribe school-based administration of morning ICS doses to families interested in receiving them as a new standard of care.

“These data, combined with data from similar studies at other institutions, suggest that school-based therapy is increasingly becoming a very real and proven option for clinicians and families when adherence is a struggle,” he says.