The Cystic Fibrosis (CF) Foundation has released new guidelines on caring for preschool-age children with the disease. Recommendations cover areas like surveillance, therapeutics, nutrition and gastrointestinal care.
“The care of the preschool aged child with CF includes complex, time-consuming treatment regimens and overcoming behavioral challenges common in this age group to maintain lung health and optimize growth,” the authors said in the report Clinical Practice Guidelines from the Cystic Fibrosis Foundation for Preschoolers with Cystic Fibrosis (Lahiri T, et al. Pediatrics. March 23, 2016, http://pediatrics.aappublications.org/content/early/2016/03/22/peds.2015-1784).
They say recommendations for 2- to 5-year-olds fill a gap as guidance previously addressed only older and younger children. The foundation convened a committee of experts and parents that came up with recommendations for specialists and primary care pediatricians.
The committee reviewed 344 articles while drafting recommendations, but acknowledged that for preschool-age children “evidence is lacking for most treatments and monitoring tools.” In such cases, it relied on evidence from other age groups and clinical experience.
“The creation of these guidelines was a good idea because this is the one age group for which we currently have none,” said Michael S. Schechter, M.D., M.P.H., FAAP, immediate past chair of the AAP Section on Pediatric Pulmonology and Sleep Medicine Executive Committee, who was not directly involved in writing the guidelines. “However, the committee had difficulty providing evidence-based as opposed to consensus-based recommendations, which is a reflection of the lack of adequate research and the need to learn more about the optimal approach to treat children with CF in this age group.”
The recommendations include:
- Attempt spirometry with children as young as 3 years.
- Perform chest radiographs at least every other year after diagnosis.
- Obtain oropharyngeal cultures to monitor respiratory microbiology at least quarterly.
- Use oral, inhaled or intravenous antibiotics to treat pulmonary exacerbations. Delivery method will depend on severity level.
- Use airway clearance therapy as maintenance treatment and encourage vigorous physical activity.
- Offer hypertonic saline or dornase alfa selectively for chronic use or acute pulmonary exacerbations based on individual circumstances.
- Do not use systemic and inhaled corticosteroids for routine CF care except to treat other issues like allergic bronchopulmonary aspergillosis. There was not enough evidence to make a recommendation on chronic use of inhaled bronchodilators.
- Provide interventions or specialty referral for those whose weight-for-age falls below the 10th percentile, body mass index falls below the 50th percentile or who exhibit challenging mealtime behaviors.
The committee suggests working with families to create a plan that addresses medical, behavioral and nutritional issues.
“Supporting preschoolers with CF and their families is critical to establish habits that promote normal growth and development and an active lifestyle (i.e., exercise) to prevent pulmonary decline,” the committee stated.