For Preschoolers with Cystic Fibrosis, Clinicians Develop New Guidelines, Clinical Care Recommendations

For Preschoolers with Cystic Fibrosis, Clinicians Develop New Guidelines, Clinical Care Recommendations

A multidisciplinary team of clinicians and researchers created a comprehensive document featuring guidelines and recommendations for the clinical care of preschool children between the ages of 2 and 5 who have been diagnosed with cystic fibrosis (CF), according to a recently published article.

The document, developed by Thomas Lahiri, M.D., from the University of Vermont Children’s Hospital, and colleagues, compiles evidence-based and consensus recommendations in the areas of routine surveillance for pulmonary disease, therapeutics, and nutritional and gastrointestinal care.

The article is titled “Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis,” and was published in the journal Pediatrics.

Early monitoring of respiratory and gastrointestinal disease, as well as nutritional intervention, are key elements in the clinical care and outcome improvement in infant CF patients. Clinical care guidelines exist for infants, but only for those up to the age of 2 years, or older than 6 years, leaving an important gap in clinical care recommendations for children ages 2 to 5.

This age bracket is especially important, as airway inflammation, infection, and obstruction exist during these years, often in the absence of obvious respiratory symptoms. Moreover, with the development of newborn diagnostics tools, more and more children need specialized and targeted treatments.

Clinical care recommendations are essential for families and patients. In these important growth years, critical decisions regarding nutrition and exercise need to be made in order to promote the preschoolers’ normal growth and development, and to prevent pulmonary decline.

For the formulation of the present document, the CF Foundation gathered a committee of 16 cystic fibrosis pediatric experts and parents to develop the clinical care guidelines, based on Medline literature search that resulted in thousands of retrieved review articles, case reports, letters, and nonhuman studies related to specific medical questions.

Among the guidelines, it is established that a frequent collaboration between the family, primary care providers, and a CF Foundation-accredited care center is essential, with routine check-ups of the children. Moreover, it is recommended that spirometry, a common method of assessment of lung function, is attempted as early as age 3 to identify pulmonary exacerbation events and monitor the child’s response to treatment.

Treatment of pulmonary exacerbations should include oral, inhaled, or intravenous antibiotics, and daily airway clearance for reduction of exacerbations and improvement of lung function. Finally, it is recommended that caregivers pay special attention to weight-to-age rations, which should be maintained at or greater than the 10th percentile.

“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. We hope that these guidelines will help CF care teams and families make informed decisions regarding care of the 2- to 5-year-old children with CF,” the researchers said in a news release.

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