Last updated March 15, 2022

Preschoolers With Cystic Fibrosis

Recommendations

Health Maintenance

For children with CF, ages 2 through 5 y, the CF Foundation recommends routine well-child care at PCP following AAP guidelines. (CR)
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The CF Foundation recommends that children with CF, ages 2 through 5 y, receive all routine immunizations, following the recommended vaccination schedule per the AAP. (CR)
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The CF Foundation recommends that children with CF, ages 2 through 5 y, family members, and caregivers should receive annual seasonal influenza vaccination. (CR)
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The CF Foundation recommends that children with CF, ages 2 through 5 y, receive the first dose of the pneumococcal polysaccharide vaccine (PPSV23), given at least 8 wk after last pneumococcal conjugate (Prevnar) vaccine dose. (CR)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends that a smoke-free environment be provided and that all caregivers are informed that cigarette smoke exposure harms children with CF. (CR)
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Caregiver Engagement

For children with CF ages, 2 through 5 y, the CF Foundation recommends that parents and a CF health care professional review treatment goals and individualized care plans quarterly to assess and address barriers to CF care. (CR)
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Screening and Monitoring: Pulse Oximetry

For children with CF, ages 2 through 5 y, the CF Foundation concludes that there is insufficient evidence to recommend for or against the use of pulse oximetry routinely as an adjunctive tool to detect lung disease. (I)
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Screening and Monitoring: Spirometry

For children with CF, ages 2 through 5 y, the CF Foundation recommends that spirometry should be attempted as early as age 3, depending on the developmental stage of the individual child. (CR)
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For children with CF, ages 3 and older, the CF Foundation recommends the use of spirometry for identifying pulmonary exacerbations and monitoring response to therapy in those children able to perform acceptable and reproducible maneuvers. (CR)
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Screening and Monitoring: Bronchodilator

For children with CF, ages 2 through 5 y, the CF Foundation concludes that there is insufficient evidence to recommend for or against routine monitoring of bronchodilator responsiveness. (I)
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Screening and Monitoring: Multiple Breath Washout

For children with CF, ages 2 through 5 y, the CF Foundation concludes that there is insufficient evidence to recommend for or against routine monitoring of multiple breath washout. (I)
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Screening and Monitoring: Chest Imaging

For children with CF, ages 2 through 5 y, the CF Foundation recommends chest radiographs be obtained at a minimum every other year to monitor progression of lung disease. (CR)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends consideration of chest CT as an alternative to chest radiograph to monitor progression of lung disease. If chest CT is performed, it should replace chest radiograph, be performed every 2–3 y, and use the lowest radiation dose possible. (CR)
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Screening and Monitoring: Microbiology

For children with CF, ages 2 through 5 y, the CF Foundation recommends against routine use of bronchoscopy to obtain lower airway cultures. (D)
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Therapeutics: Exacerbations

For children with CF, ages 2 through 5 y, the CF Foundation recommends the use of oral, inhaled, and/or intravenous antibiotics to treat pulmonary exacerbations. (CR)
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Therapeutics: Airway Clearance

For children with CF, ages 2 through 5 y, the CF Foundation recommends the use of daily airway clearance to improve lung function and reduce exacerbations. (CR)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends increasing frequency and/or duration of airway clearance treatments for children diagnosed with pulmonary exacerbations. (CR)
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Therapeutics: Bronchodilators

For children with CF, ages 2 through 5 y, the CF Foundation concludes that the evidence is insufficient to recommend for or against the chronic use of inhaled bronchodilators to improve lung function and quality of life or reduce exacerbations. (I)
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Therapeutics: Hypertonic saline

For children with CF, ages 2 through 5 y, the CF Foundations recommends that hypertonic saline be selectively offered to patients based on individual circumstances. (C)
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Therapeutics: Dornase alfa

For children with CF, ages 2 through 5 y, the CF Foundation recommends that dornase alfa be selectively offered to patients based on individual circumstances. (C)
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Therapeutics: Inhaled Corticosteroids

For children with CF, ages 2 through 5 y, and without asthma or recurrent wheezing, the CF Foundation recommends against the routine use of inhaled corticosteroids to reduce exacerbations, airway inflammation, or improve lung function or quality of life. (D)
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Therapeutics: Corticosteroids

For children with CF, ages 2 through 5 y, and without allergic bronchopulmonary aspergillosis, the CF Foundation recommends against the chronic use of systemic corticosteroids to reduce exacerbations, or improve lung function, or quality of life. (D)
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Therapeutics: Ibuprofen

For children with CF, ages 2 through 5 y, the CF Foundation concludes that there is insufficient evidence to recommend for or against chronic high-dose ibuprofen use to slow rate of decline of FEV1, reduce exacerbations and hospitalizations, or improve quality of life. (I)
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Therapeutics: Leukotriene Modifiers

For children with CF, ages 2 through 5 y, the CF Foundation concludes that the evidence is insufficient to recommend for or against the routine chronic use of leukotriene modifiers to improve lung function or quality of life or reduce exacerbations. (I)
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Therapeutics: Azithromycin

For children with CF, ages 2 through 5 y, the CF Foundation concludes that there is insufficient evidence to recommend for or against the chronic use of azithromycin. (I)
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Therapeutics: Chronic Pseudomonas Infection

For children with CF, ages 2 through 5 y, the CF Foundation recommends that children who remain persistently infected with P. aeruginosa be treated chronically with alternate-month inhaled antipseudomonal antibiotics. (B)
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Therapeutics: S. aureus

For children with CF, ages 2 through 5 y, the CF Foundation recommends against the prophylactic use of oral antistaphylococcal antibiotics. (D)
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For children with CF, ages 2 through 5 y, the CF Foundation concludes that there is insufficient evidence to recommend for or against active attempts to eradicate Staphylococcus aureus, including methicillin-resistant S. aureus, in asymptomatic patients. (I)
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For children with CF, ages 2 through 5 y, and with Staphylococcus aureus persistently present in cultures of the airways, the CF Foundation concludes that the evidence is insufficient to recommend for or against the chronic use of oral antistaphylococcal antibiotics to improve lung function or quality of life or reduce exacerbations. (I)
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Therapeutics: Ivacaftor

For children with CF, ages 2 through 5 y, the Preschool Guidelines Committee recommends the routine use of ivacaftor in those with specific gating mutations* and a consideration for those with a confirmed diagnosis of CF and a R117H mutation. (CR)
*The mutations are G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, and S549R.
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Nutrition, Behavior, and Gastrointestinal: Nutrition

For children with CF, ages 2 through 5 y, the CF Foundation recommends that weight-for-age be maintained at ≥10th percentile. (A)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends weight-for-stature assessments use the BMI% method on the Centers for Disease Control and Prevention growth charts and a BMI ≥50th percentile be maintained. (B)
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For children with CF, ages 2 through 5 y, who are meeting optimal nutritional thresholds, the CF Foundation recommends ≥90–110 kcal/kg per day and protein intake based on dietary reference intakes and dietary guidelines recommendations: ≥13 g protein/d 2–3 y old, ≥19 g protein/d 4–5 y old. (A)
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Nutrition, Behavior, and Gastrointestinal: Nutritional Risk

For children with CF, ages 2 through 5 y, the CF Foundation recommends evaluation and more intensive management of children demonstrating any of these criteria of nutritional risk: 
• BMI <50th percentile, or rate of weight gain <50th percentile expected for age (≥6 g/d), or weight-for-age <10th percentile, or inappropriate weight loss.
(B)
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For children with CF, ages 2 through 5 y, and at nutritional risk, the CF Foundation recommends patients be seen in 8 wk or sooner. These visits should include medical, behavioral, and nutritional assessment; education; and intervention. Nutritional intervention should aim at achieving the patient’s target goal for both weight-for-age and BMI. (CR)
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For children with CF, ages 2 through 5 y, and at nutritional risk, the CF Foundation recommends energy intake 10% to 20% above baseline with continued incremental upward adjustments of 10% to 20% as needed up to 200% to achieve weight gain. (B)
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For children with CF, ages 2 through 5 y, and at nutritional risk, the CF Foundation recommends the use of oral nutrition supplements, in addition to usual dietary intake, to improve rate of weight gain. (B)
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For children with CF, ages 2 through 5 y, at nutritional risk who do not respond to previously described nutritional interventions, see Figure 2, the CF Foundation recommends an expanded evaluation to consider other causes of poor growth, including gastrointestinal, endocrine, behavioral, and social causes. Subspecialty consultation may be considered. (CR)
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For children with CF, ages 2 through 5 y, at nutritional risk who do not respond to standard nutritional intervention and who have not responded to the evaluation and management plan of the multidisciplinary team, the CF Foundation recommends the use of enteral nutritional supplements via a feeding tube to improve the rate of weight gain. The concept of enteral feedings should be introduced early as a component of CF care. (B)
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Nutrition, Behavior, and Gastrointestinal: Vitamins

For children with CF, ages 2 through 5 y, the CF Foundation recommends standard, age-appropriate non–fat-soluble vitamins and the recommended levels of vitamins A, D, E, and K by using a fat-soluble vitamin supplement formulated for children with CF and if indicated based on levels, additional supplementation of vitamins A, D, E, and K. (CR)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends that blood levels of fat-soluble vitamins be measured annually. If values are abnormal, more frequent measurements after dose adjustment are recommended. (CR)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends that management of vitamin D deficiency follow the treatment outlined in the CF Foundation Vitamin D guidelines: An Update on the Screening, Diagnosis, Management, and Treatment of Vitamin D Deficiency in Individuals with Cystic Fibrosis: Evidence-Based Recommendations from the Cystic Fibrosis Foundation, 2012. (CR)
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Nutrition, Behavior, and Gastrointestinal: Salt

For children with CF, ages 2 through 5 y, the CF Foundation recommends adding additional salt to meals and snacks, especially during the summer months and for those who live in warm climates. (CR)
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Nutrition, Behavior, and Gastrointestinal: PERT

For children with CF and PI, ages 2 through 5 y, the CF Foundation recommends that PERT be adjusted up to a dose of no greater than 2500 lipase units per kg per meal with a maximum daily dose of 10 000 lipase units/kg. (CR)
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Nutrition, Behavior, and Gastrointestinal: Behavior

For children with CF, ages 2 through 5 y, the CF Foundation recommends that the CF team members, working in concert with the family, set energy-intake goals and assess progress on a regular basis. (B)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends that all families are regularly assessed for the presence of mealtime behavior challenges and are provided with proactive behavioral assistance when needed. (A)
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For children with CF, ages 2 through 5 y, who are at nutritional risk, or exhibiting challenging mealtime behaviors, or not meeting energy-intake goals, behavioral therapy provided by knowledgeable team members should accompany nutritional therapy. (A)
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Nutrition, Behavior, and Gastrointestinal: Gastrointestinal

The CF Foundation recommends that all providers be aware of the presenting symptoms of the following gastrointestinal tract disorders: constipation, gastroesophageal reflux disease, small bowel overgrowth, distal intestinal obstruction syndrome, and celiac disease. (CR)
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For children with CF, ages 2 through 5 y, the CF Foundation recommends that children and their parents be questioned regarding abdominal pain at each visit, and that pain is investigated if persistent or recurrent. (CR)
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For children with CF, ages 2 through 5 y, and who are PS, the CF Foundation recommends that children are reevaluated annually for the conversion to PI with fecal elastase measurement, particularly if genetic testing reveals 2 mutations potentially associated with PI. (CR)
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For children with CF, ages 2 through 5 y, and who are PS with severe abdominal pain, particularly if associated with vomiting, the CF Foundation recommends measurement of lipase and amylase to determine if pancreatitis is present. (CR)
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For children with CF, ages 2 through 5 y, who had terminal ileal bowel resection, the CF Foundation recommends annual measurement of serum vitamin B12 concentration. (CR)
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Recommendation Grading

Overview

Title

Preschoolers With Cystic Fibrosis

Authoring Organization

Publication Month/Year

June 1, 2020

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Target Patient Population

Preschool children between the ages of 2 and 5 years

Inclusion Criteria

Child, Infant

Health Care Settings

Emergency care, Hospital, Outpatient

Intended Users

Respiratory therapist, nurse, nurse practitioner, physician, physician assistant

Scope

Counseling, Management, Treatment

Diseases/Conditions (MeSH)

D003550 - Cystic Fibrosis

Keywords

cystic fibrosis, airway clearance therapy

Source Citation

Thomas Lahiri, Sarah E. Hempstead, Cynthia Brady, Carolyn L. Cannon, Kelli Clark, Michelle E. Condren, Margaret F. Guill, R. Paul Guillerman, Christina G. Leone, Karen Maguiness, Lisa Monchil, Scott W. Powers, Margaret Rosenfeld, Sarah Jane Schwarzenberg, Connie L. Tompkins, Edith T. Zemanick, Stephanie D. Davis. Pediatrics Mar 2016, peds.2015-1784; DOI: 10.1542/peds.2015-1784