Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis

Abstract

Newborn screening for cystic fibrosis (CF) offers the opportunity for early medical and nutritional intervention that can lead to improved outcomes. Management of the asymptomatic infant diagnosed with CF through newborn screening, prenatal diagnosis, or sibling screening is different from treatment of the symptomatically diagnosed individual. The focus of management is on maintaining health by preventing nutritional and respiratory complications. The CF Foundation convened a committee to develop recommendations based on a systematic review of the evidence and expert opinion. These guidelines encompass monitoring and treatment recommendations for infants diagnosed with CF and are intended to help guide families, primary care providers, and specialty care centers in the care of infants with CF.

Figure 1.

For parents: Who to call and where to go to care for your infant with CF. Infants with CF need basic medical care as well as CF care. Your primary care provider will take care of some of your child’s health needs; the CF team will provide care specifically related to CF. Sometimes you will make a phone call with questions and other times you will need to bring your baby in to be seen. After you and your CF care team fill out this checklist, they will share it with your primary care provider so that everyone knows the care plan.

Figure 2.

Analysis of CF Foundation Patient Registry data from 2006: Forced expiratory volume in 1 second (FEV₁) in childhood in pancreatic insufficient patients with CF, stratified by their weight-for-length percentile at age 2 years. Reprinted with permission from Stallings et al, Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review, Journal of the American Dietetic Association, May 2008. 2008 American Dietetic Association. Published by Elsevier Inc.

Figure 3.

Evaluation of infants with weight loss or inadequate weight gain (based on consensus opinion).

Figure 4.

Enzyme dose by age, CF Foundation Patient Registry, 2005.

Figure 5.

Care of infants with a change in respiratory status (based on consensus opinion). Change in respiratory status identified: increased cough, tachypnea, wheezing, oxygen desaturation, crackles. Clinicians should assess whether these indicate a mild or moderate-to-severe exacerbation. In most cases, significant oxygen desaturation or the presence of crackles will indicate a moderate-to-severe exacerbation.