Preschool-Aged Care Clinical Care Guidelines: Executive Summary
Lahiri T, Hempstead SE, Brady C, Cannon CL, Clark K, Condren ME, Guill MF, Guillerman RP, Leone CG, Maguiness K, Monchil L, Powers SW, Rosenfeld M, Schwarzenberg SJ, Tompkins CL, Zemanick ET, Davis SD. Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis. Pediatrics. 2016;137(4):e20151784. Epub 2016 mar 16. PMID: 27009033
In 2014, a multidisciplinary committee convened to develop preschool guidelines to fill in the gap between existing clinical care guidelines for infants 2 years and younger and individuals 6 years and older. Fifty-three recommendations were included on the following topics:
- Health maintenance
- Caregiver engagement
- Screening and monitoring
- Therapeutics
- Nutrition, behavior, and gastrointestinal (GI)
The purpose of these guidelines is to help make informed decisions in the care of preschool-aged children with CF. Research is needed to improve the strength of many of the recommendations in the preschool guidelines.
Since these guidelines were published, guidelines for enteral tube feeding in individuals with CF have been published that provide more detailed recommendations on the process of enteral tube placement and feeding.
Methodology
Fifty-three recommendations were approved by 80 percent or more of the committee. Reflecting the limited evidence in this age group, the majority of recommendations were made based on consensus only, and only three recommendations met the U.S. Preventive Services Task Force (USPSTF) Grade A criteria. Find additional information about USPSTF grading definitions.
Recommendations
The guidelines recommend the following for children with CF, ages 2 to 5 years:
Health Maintenance
| Recommendations |
Evaluation of the Evidence |
|
Routine well-child care according to American Academy of Pediatrics (AAP) guidelines, including routine immunizations.
|
Consensus
|
|
Children, family members, and caregivers should receive annual influenza vaccinations.
|
Consensus
|
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The first dose of pneumococcal polysaccharide vaccine (PPSV23) should be administered.
|
Consensus
|
Screening and Monitoring
| Recommendations |
Evaluation of the Evidence |
|
Parents and CF health care professionals should review treatment goals and care plans quarterly.
|
Consensus
|
|
Spirometry should be attempted as early as 3 years old and be used to monitor for exacerbations and response to therapy.
|
Consensus
|
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Chest X-rays should be obtained at least every other year.
|
Consensus
|
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A chest computed tomography (CT) scan may be considered every two to three years, to replace chest X-rays, using the lowest possible radiation dose.
|
Consensus
|
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Oropharyngeal cultures should be performed at least quarterly to monitor airway microbiology.
|
Consensus
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Therapeutics
| Recommendations |
Evaluation of the Evidence |
|
Daily airway clearance is recommended.
|
Consensus
|
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For children who are pancreatic insufficient, the maximum pancreatic enzyme replacement therapy dose is 2,500 lipase units/kilogram (kg)/meal and 10,000 lipase units/kg/day.
|
Consensus
|
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Hypertonic saline and dornase alfa may be selectively offered based on individual circumstances.
|
Grade: C
Certainty: Moderate
Benefit: Moderate
|
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Children persistently infected with Pseudomonas aeruginosa should be treated chronically with alternate-month inhaled antipseudomonal antibiotics.
|
Grade: C
Certainty: Moderate
Benefit: Moderate
|
|
Ivacaftor should be used for children with specific gating mutations and considered for children with a confirmed diagnosis of CF and an R117H mutation.
|
Consensus
|
|
For pulmonary exacerbations:
- Increase frequency and/or duration of airway clearance
- Use oral, inhaled, and/or intravenous antibiotics to treat
|
Consensus
|
Nutrition, Behavior, and GI
| Recommendations |
Evaluation of the Evidence |
|
Weight-for-age ≥10th percentile should be maintained.
|
Grade: A
Certainty: High
Benefit: Substantial
|
|
Use body mass index (BMI) percentile to assess weight-for-stature and maintain BMI ≥50th percentile.
|
Grade: B
Certainty: High
Benefit: Moderate
|
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CF team members should work with the family to set energy-intake goals and assess progress regularly.
|
Grade: B
Certainty: Moderate
Benefit: Substantial
|
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For those meeting optimal nutrition thresholds, calorie intake should be ≥90-110 kcal/kg/day, and protein intake should be ≥13g protein/day in 2- to 3-year olds and ≥19 g protein/day in 4- to 5-year olds.
|
Grade: A
Certainty: High
Benefit: Substantial
|
| Children at nutritional risk include those with BMI <50th percentile, weight gain <50th percentile expected for age, weight-for-age <10th percentile, or inappropriate weight loss. These children should be evaluated and receive more intensive management. |
Grade: B
Certainty: High
Benefit: Moderate
|
For children at nutritional risk, evaluate and intensify management:
- Re-evaluate within eight weeks for medical, behavioral, and nutritional assessments and education, and implement interventions aimed at achieving the patient's target goal for both weight-for-age and BMI
- Increase energy intake incrementally by 10-20 percent, as needed up to 200 percent of baseline
- Add oral nutrition supplements in addition to usual dietary intake
- Introduce the concept of enteral feedings early as a component of CF care
|
- Consensus
- Grade: B
Certainty: Moderate
Benefit: Moderate
- Grade: B
Certainty: Moderate
Benefit: Moderate
- Grade: B
Certainty: Moderate
Benefit: Moderate
|
|
For children at nutritional risk who are not responding:
- Evaluate causes of poor growth, including GI, endocrine, behavioral, and social causes
- If this evaluation does not lead to improvements, use enteral nutritional supplements
|
- Consensus
- Grade: B
Certainty: Moderate
Benefit: Moderate
|
|
Vitamin supplementation:
- Standard non-fat-soluble vitamins, and fat-soluble vitamins formulated for children with CF, are recommended
- Measure blood levels of fat-soluble vitamins annually and more frequently if abnormal
- Additional supplementation of vitamins A, D, E, and K is recommended if indicated, based on levels
- Manage vitamin D deficiency according to the Cystic Fibrosis Foundation vitamin D deficiency guidelines
|
Consensus |
| Add salt to meals and snacks, especially during summer months and in warm climates. |
Consensus |
| Regularly assess for mealtime behavior challenges and provide proactive assistance when needed. |
Grade: A
Certainty: High
Benefit: Substantial
|
| Provide behavioral therapy for children at nutritional risk or for children who exhibit challenging mealtime behaviors. |
Grade: A
Certainty: High
Benefit: Substantial
|
| Be aware of the presenting symptoms of constipation, gastroesophageal reflux disease, small bowel overgrowth, distal intestinal obstruction syndrome, and celiac disease. |
Consensus |
| Evaluate for abdominal pain at each visit, and investigate if persistent or recurrent. |
Consensus |
For children who are pancreatic sufficient:
- Obtain fecal elastase yearly, especially if mutations are associated with pancreatic insufficiency
- Measure lipase and amylase for severe abdominal pain, especially associated with vomiting
|
Consensus |
| With terminal ileal bowel resection, annually measure serum vitamin B12 concentration. |
Consensus |
Insufficient evidence to recommend for or against
| Recommendations |
Evaluation of the Evidence |
|
Routine pulse oximetry, assessment of bronchodilator responsiveness, or multiple breath washout
|
Grade: I
Certainty: low
|
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Chronic inhaled bronchodilators, high-dose ibuprofen, leukotriene modifiers, or azithromycin
|
Grade: I
Certainty: low
|
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Attempts to eradicate Staphylococcus aureus (S. aureus), including methicillin-resistant S. aureus (MRSA), in asymptomatic patients
|
Grade: I
Certainty: low
|
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Chronic use of oral antistaphylococcal antibiotics in children persistently infected with S. aureus
|
Grade: I
Certainty: low
|
Not to be used routinely
| Recommendations |
Evaluation of the Evidence |
| Bronchoscopy |
Grade: D
Certainty: Moderate
Benefit: Negative
|
| For children without asthma or recurrent wheezing, inhaled corticosteroids |
Grade: D
Certainty: High
Benefit: Low
|
| For children without allergic bronchopulmonary aspergillosis (ABPA), chronic use of systemic corticosteroids |
Grade: D
Certainty: High
Benefit: Low
|
|
Oral antistaphylococcal antibiotics
|
Grade: D
Certainty: Moderate
Benefit: Negative
|
Unanswered Questions
The authors note that additional research is needed in the following areas for preschool-aged children with CF:
- The optimal approach to monitoring and detecting lung disease (e.g., with magnetic resonance imaging [MRI], multiple breath washout)
- Efficacy of chronic respiratory medications, including any newly developed CFTR modulators
- Nutritional, behavioral, and GI studies to optimize nutrition and overcome behavioral challenges
- Timing of nutritional interventions and conversion from pancreatic sufficiency to pancreatic insufficiency
- Determining the prevalence of key GI disorders (e.g., constipation, reflux, bacterial overgrowth)
Further Reading
Schwarzenberg SJ, Hempstead SE, McDonald CM, Powers SW, Wooldridge J, Blair S, Freedman S, Harrington E, Murphy PJ, Palmer L, Schroeder AE, Shiel K, Sullivan J, Wallentine M, Marshall BC, Leonard AR. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cyst Fibros. 2016 Nov;15(6):724-35. Epub2016 Sep 3. PMID: 27599607. This article provides more guidance on the process of enteral tube placement and enteral feeding in individuals with CF.
This executive summary was prepared by:
Don B. Sanders, M.D., M.S. and Stephanie D. Davis, M.D. (Indiana University School of Medicine)