Infant Care Clinical Care Guidelines

These guidelines were developed by consensus based on expert opinion and a medical literature review to guide the monitoring and care of infants who have an abnormal cystic fibrosis newborn screening result, but do not meet the full criteria for a CF diagnosis.

14 min read
In this article
Summary
  • Although many treatment issues are the same for individuals with cystic fibrosis regardless of when they are diagnosed, some are unique to the population of newborn infants.
  • These guidelines contain detailed recommendations regarding care of infants beginning at the time of diagnosis, and discuss topics such as feeding, nutrition, airway clearance, diagnostic testing, treatment of infection, and chronic pulmonary therapies.

Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants With Cystic Fibrosis

Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009 Dec; 155(6 Suppl):S73-S93.

Although many treatment issues are the same for individuals with cystic fibrosis regardless of when they are diagnosed, some are unique to the population of newborn infants who may not have overt symptoms of the disease before being referred to a CF care center.

In 2009, the Cystic Fibrosis Foundation convened a committee of experts to develop guidelines for care based on available evidence for this distinct population of newly diagnosed infants. These guidelines contain detailed recommendations regarding care of infants beginning at the time of diagnosis, and discuss topics such as feeding, nutrition, airway clearance, diagnostic testing, treatment of infection, and chronic pulmonary therapies.

Methodology

The 2009 Evidence-Based Guidelines for Management of Infants With Cystic Fibrosis summarize recommendations from a multidisciplinary expert committee. These guidelines were created based on a systematic review of the evidence and expert opinion. The process for developing these guidelines included an expert committee, specific questions, systematic literature review, recommendations drafted, committee vote, and final recommendations.

The final recommendations were graded using the U.S. Preventative Task Force (USPSTF) Scheme. Find additional information about the USPSTF grading definitions.

Recommendations

Initial Diagnosis

Recommendations Evaluation of the Evidence
1. Treatment for infants diagnosed with CF by newborn screening (NBS) should be done at an accredited CF care center, with the goal of initial visit within 24-72 hours of diagnosis (1-3 working days in absence of overt symptoms). Consensus recommendation

Nutrition Recommendations

Pancreatic Function and Pancreatic Enzymes
Recommendations Evaluation of the Evidence
2. For infants with CF under 2 years of age, pancreatic functional status should be measured by fecal elastase or coefficient of fat absorption (CFA) in all individuals. Certainty: Low
Benefit: Substantial
Consensus recommendation
3. For infants with CF under 2 years of age, pancreatic enzyme replacement therapy (PERT) should be initiated at a dosage of 2,000-5,000 lipase units at each feeding. Adjustment should not exceed 2,500 lipase units/kg at each feeding with a maximum daily dose of 10,000 lipase units/kg. Certainty: Low
Benefit: Substantial
Consensus recommendation
Recommended in the CF Foundation Consensus Report on Nutrition for Pediatric Patients and the European Consensus on Nutrition in Patients with CF
4. For infants with CF under 2 years of age, PERT should be started:
  1. In all infants with two cystic fibrosis transmembrane conductance regulator (CFTR) mutations associated with pancreatic insufficiency (PI)
  2. In all infants with fecal elastase <200 μg/g or CFA <85 percent (in infants <6 months of age), or other objective evidence of PI
  3. In infants with unequivocal signs or symptoms of malabsorption, while awaiting confirmatory test results
Certainty: Low
Benefit: Substantial
Consensus recommendation
5. For infants with CF under 2 years of age, PERT should not be started in infants with one or two CFTR mutations associated with pancreatic sufficiency, unless:
  1. An objective testing indicates fat malabsorption; or
  2. The infant has unequivocal signs or symptoms of malabsorption, while awaiting confirmatory test results
Consensus recommendation
6. For all patients with CF, including those under 2 years of age, generic non-proprietary PERT should not be used. Certainty: Low
Benefit: Moderate
Consensus recommendation
Recommended in the CF Foundation Evidence-Based Practice Recommendations for Nutrition
Feeding, Vitamins, and Micronutrients
Recommendations Evaluation of the Evidence
7. For infants with CF under 2 years of age, human milk should be the initial feeding type. Certainty: Moderate
Benefit: Substantial
Grade: B
8. For infants with CF under 2 years of age who are fed formula, standard infant formula (rather than hydrolyzed protein formulas) should be used. Certainty: Low
Benefit: Moderate
Consensus recommendation
9. For infants with CF under 2 years of age, calorie-dense feedings should be used if weight loss or inadequate weight gain is identified. Certainty: Low
Benefit: Moderate
Consensus recommendation
10. For infants with CF under 2 years of age, encourage positive feeding behaviors, such as by the provision of educational resources. Certainty: Low
Benefit: Moderate
Consensus recommendation
11. For infants with CF under 2 years of age, prescribe multivitamin supplements with recommended levels of vitamins A, D, E, and K for patients with CF shortly after diagnosis. Certainty: Low
Benefit: Moderate
Consensus recommendation
Recommended in the CF Foundation Consensus Report on Nutrition for Pediatric Patients
12. For infants with CF under 2 years of age, blood levels of fat-soluble vitamins should be measured approximately two months after supplement initiation and annually thereafter, with more frequent measurements if values are abnormal Consensus recommendation
13. For infants with CF under 2 years of age, a trial of zinc supplementation (1 mg/kg/day of elemental zinc in divided doses for six months) may be given to some infants who are not adequately growing despite adequate caloric intake and PERT. Certainty: Low
Benefit: Moderate
Consensus recommendation
Recommended in the CF Foundation Consensus Report on Nutrition for Pediatric Patients
14. For infants with CF under 2 years of age, supplement with 1/8 teaspoon table salt per day starting at diagnosis, increasing to 1/4 teaspoon of table salt per day by 6 months of age. Certainty: Low
Benefit: Moderate
Consensus recommendation
15. Patients aged 6 months to 2 years whose community water supply contains less than 0.3 ppm should be supplemented with fluoride 0.25mg/dl. Consensus recommendation
Recommended in the Center for Disease Control and Prevention Guidelines
16. For children 1-12 years of age with growth deficits, intensive treatment with behavioral intervention in conjunction with nutritional counseling should be used to promote weight gain. Consensus recommendation
Recommended in the CF Foundation Evidence-based Practice Recommendations for Nutrition

Pulmonary Recommendations

Recommendations Evaluation of the Evidence
17. For infants with CF under 2 years of age, a smoke-free environment should be provided and all caregivers be informed that cigarette exposure harms children with CF. Consensus recommendation
Airway Clearance
Recommendations Evaluation of the Evidence
18. For infants with CF under 2 years of age, airway clearance therapy should be initiated in the first few months of life. Certainty: Low
Benefit: Moderate
Consensus recommendation
19. For infants with CF under 2 years of age, use albuterol before percussion and postural drainage. Certainty: Low
Benefit: Moderate
Consensus recommendation
20. For infants with CF under 2 years of age, do not use the head-down position for percussion and postural drainage. (Percussion and postural drainage is recommended as the form of airway clearance for most infants in the Airway Clearance Guidelines.) Certainty: Low
Benefit: Moderate
Consensus recommendation
Infection Control, Surveillance, and Treatment

Recommendations

Evaluation of the Evidence
21. For infants with CF under 2 years of age, newly diagnosed patients should be separated from other patients cared for in CF clinics until adequate infection control education has been provided to, and is understood by, the caregivers. Certainty: Low
Benefit: Moderate
Consensus recommendation
22. For infants with CF under 2 years of age, infection control measures should be implemented in compliance with CF Foundation recommendations to minimize transmission of bacterial infection to infants. Consensus recommendation
Recommended in the CF Foundation Consensus Conference on Infection Control
23. Annual influenza vaccination is recommended for infants with CF ≥6 months of age, as well as all household members and health care providers caring for these infants. Household contacts and out-of-home caregivers for children with CF <6 months of age should receive annual influenza vaccination. Consensus recommendation
Recommended in American Academy of Pediatrics Guidelines and the CF Foundation Consensus Conference on Infection Control
24. For infants with CF under 2 years of age, the use of palivizumab should be considered for prophylaxis of respiratory syncytial virus. Certainty: Low
Benefit: Moderate
Consensus recommendation
25. For infants with CF under 2 years of age, oropharyngeal cultures should be performed at least quarterly. Certainty: Low
Benefit: Moderate
Consensus recommendation
26. For infants with CF under 2 years of age, bronchoscopy and bronchoalveolar lavage should be considered in infants with signs or symptoms of lung disease, particularly with failed response to appropriate intervention. Consensus recommendation
27. For infants with CF under 2 years of age, the CF Foundation recommends against the prophylactic use of oral anti-staphylococcal antibiotics in asymptomatic infants. Certainty: Low
Benefit: Zero-negative
Recommended in the CF Foundation Pulmonary Guidelines on Chronic Medications
28. For infants with CF under 2 years of age, the CF Foundation recommends against the use of chronic antibiotics for prophylaxis to prevent Pseudomonas aeruginosa. Certainty: Low
Benefit: Zero-negative
Consensus recommendation
29. For infants with CF under 2 years of age, new acquisition of Pseudomonas aeruginosa, defined as initial acquisition or new acquisition after “successful” eradication therapy, should be treated with anti-pseudomonal antibiotics and increased airway clearance in the presence or absence of symptoms. Certainty: Low
Benefit: Moderate
Consensus recommendation
30. Infants with CF under 2 years of age who remain persistently colonized with Pseudomonas aeruginosa after two attempts at eradication should be treated chronically with alternate month tobramycin solution for inhalation. Certainty: Low
Benefit: Moderate
Consensus recommendation
31. For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against active attempts to eradicate Staphylococcus aureus in asymptomatic infants. Certainty: Low
Benefit: Unknown
Grade: I
32. For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against active attempts to eradicate methicillin-resistant Staphylococcus aureus (MRSA) in asymptomatic infants. Certainty: Low
Benefit: Unknown
Grade: I

DIAGNOSTIC TESTING

Recommendations Evaluation of the Evidence
33. For infants with CF under 2 years of age, pulse oximetry measurements should be obtained with acute respiratory symptoms. Certainty: Low
Benefit: Substantial
Consensus recommendation
34. For infants with CF under 2 years of age, a baseline chest X-ray should be obtained within the first 3-6 months and again within the first two years of life. Certainty: Low
Benefit: Moderate
Consensus recommendation
35. For infants with CF under 2 years of age, the CF Foundation recommends against the use of chest computed tomography (CT) scans for routine surveillance. Certainty: Low
Benefit: Zero-negative
Consensus recommendation
36. For infants with CF under 2 years of age, chest CT scans should be considered in infants with signs or symptoms of lung disease who fail to respond to appropriate interventions. Consensus recommendation
37. For infants with CF under 2 years of age, infant pulmonary function tests (PFTs) should be considered as an adjunctive tool to monitor respiratory status.  Certainty: Moderate
Benefit: Small
Grade: C
38. For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against pulse oximetry routinely as an adjunctive tool to detect lung disease. Certainty: Low
Benefit: Small
Grade: I

Chronic Pulmonary Therapies

Recommendations Evaluation of the Evidence
39. For infants with CF under 2 years of age, dornase alfa (recombinant human DNase) may be used in symptomatic infants. Certainty: Low
Benefit: Moderate
Consensus recommendation
40. For infants with CF under 2 years of age, 7 percent hypertonic saline may be used in symptomatic infants. Certainty: Low
Benefit: Moderate
Consensus recommendation
41. For infants with CF under 2 years of age without airway reactivity or asthma, the CF Foundation does not recommend the use of inhaled corticosteroids to improve lung function or reduce exacerbations. Certainty: Low
Benefit: Zero-negative
Consensus recommendation
42. For infants with CF under 2 years of age, there is insufficient evidence to recommend for or against chronic azithromycin in patients colonized with Pseudomonas aeruginosa. Certainty: Low
Benefit: Unknown
Grade: I

Unanswered Questions

  • What is the role of CFTR modulator therapy among infants with CF?
  • What is the role of multiple breath washout in monitoring lung disease?
  • What is the role of chest CT in monitoring lung disease?
  • What is the most effective method of Pseudomonas aeruginosa eradication?
  • What is the optimal dose of PERT for infants with CF, particularly in early infancy?

Further Reading

Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.

Borowitz D, Gelfond D, Maguiness K, Heubi JE, Ramsey B. Maximal daily dose of pancreatic enzyme replacement therapy in infants with CF: a reconsideration. J Cyst Fibros. 2013 Dec; 12(6):784-5. doi: 10.1016/j.jcf.2013.05.011. Epub 2013 Jun 26.
Freitas DA, Dias FA, Chaves GS, et al. Standard (head-down tilt) versus modified (without head-down tilt) postural drainage in infants and young children with cystic fibrosis. Cochrane Database Syst Rev. 2015 Mar 10;(3):CD010297. doi: 10.1002/14651858.CD010297.pub2.
Jain K, Wainright C, Smyth AR. Bronchoscopy-guided antimicrobial therapy for cystic fibrosis. Cochrane Database Syst Rev. 2016 Jan 21;(1):CD009530. doi: 10.1002/14651858.CD009530.pub3.
Langton Hewer SC, Smyth AR. Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis. Cochrane Database Syst Rev. 2017 Apr 25;4:CD004197. doi: 10.1002/14651858.CD004197.pub5.
Ramsey KA, Rosenow T, Turkovic L, et al. Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis. Am J Respir Crit Care Med. 2016 Jan 1;193(1):60-7. doi: 10.1164/rccm.201507-1409OC.
Reix P, Metecki S, Fayon M. Early Lung disease in infants with cystic fibrosis. Diagnostic tools and possible therapeutic pathways. Rev Mal Respir. 2016 Feb; 33(2):102-16. doi: 10.1016/j.rmr.2015.06.017. Epub 2016 Feb 2.
Robinson KA, Odelola OA, Saldanha IJ. Palivizumab for prophylaxis against respiratory syncytial virus infection in children with cystic fibrosis. Cochrane Database Syst Rev. 2016 Jul 20;7:CD007743. doi: 10.1002/14651858.CD007743.pub6. Review.
Rosenfeld M, Ratjen F, Brumback L, et al. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA. 2012 Jun 6;307(21):2269-77. doi: 10.1001/jama.2012.5214.
Sanders DB, Emerson J, Ren CL, et al. Early Childhood Risk Factors for Decreased FEV1 at Age Six to Seven Years in Young Children with Cystic Fibrosis. Ann Am Thorac Soc. 2015 Aug;12(8):1170-6. doi: 10.1513/AnnalsATS.201504-198OC.
Subbarao P, Stanojevic S, Brown M, et al. Lung clearance index as an outcome measure for clinical trials in young children with cystic fibrosis. A pilot study using inhaled hypertonic saline. Am J Respir Crit Care Med. 2013 Aug 15;188(4):456-60. doi: 10.1164/rccm.201302-0219OC.
Smyth AR, Rosenfeld M. Prophylactic anti-staphylococcal antibiotics for cystic fibrosis. Cochrane Database Syst Rev. 2017 Apr 18;4:CD001912. doi: 10.1002/14651858.CD001912.pub4.
Visca A, Bishop CT, Hilton S, Hudson VM. Oral reduced L-glutathione improves growth in pediatric cystic fibrosis patients. J Pediatr Gastroenterol Nutr. 2015 Jun;60(6):802-10. doi: 10.1097/MPG.0000000000000738.

This executive summary was prepared by:
Sylvia Szentpetery, MD, (Children's Hospital of Pittsburgh) and Margaret Rosenfeld, MD, MPH, (Seattle Children's Hospital)

The guidelines were published in December 2009, they were reviewed in April 2019 and it was determined that no update is needed at this time.

Share this article
Topics
Clinical Care Guidelines
Was this information helpful?
Downloads
Clinical Care Schedule for Newborns to 5-Year-Olds with CF Download PDF
Have questions? We’re here to help. Call us at 1-800-FIGHT CF

(1-800-344-4823)
Mon - Thu, 9 am - 7 pm ET
Fri, 9 am - 3 pm ET

 

More Ways To Get Help