The following guidelines are treatment recommendations for managing CF in the first 2 years of life and are intended to help guide healthcare professionals and CF center staff members.¹

During routine appointments, a caregiver can anticipate their loved one may undergo certain procedures, tests, and scans, like below¹:

image

Airway clearance techniques1,2

Assists with moving mucus away from the lungs, which may reduce infection and improve lung function

image

Routine vaccinations¹

  • The American Academy of Pediatrics recommends that all standard vaccinations apply for individuals with CF
  • Annual influenza vaccine is recommended for all infants over 6 months of age with CF and their household members
image

Oropharyngeal cultures¹

Routine surveillance of respiratory microbiology is necessary, including eradication regimens, regardless of presence or absence of symptoms

image

Lung clearance index (LCI)³

LCI is a sensitive measure of overall ventilation inhomogeneity assessed by multiple-breath washout that is based on tidal breathing techniques

image

Chest X-rays¹

Used to detect early abnormalities, including hyperinflation, peribronchial cuffing, or streakiness

Monitoring and Care for Infants With CF Up to Age 2¹

The table below provides an overview of CF care recommendations and considerations up to age 2.

Download Chart Expand
RecommendConsider
AGE AT VISIT day of sweat test 24-48 hours
of dx
1 wk later 
or age
1 mo
2 MOa 3 MOa 4 MOa 5 MOa 6 mob 8 mob 10 mob 1 yr every 2-3 mo. in the 2nd yr of life 2 yr
care issues
Discuss diagnosis Either visit                      
Encourage human
milk feeding
Either visit                      
Start PERTc Either visit                      
Start salt supplementation 1/8 tsp. salt           1/4 tsp. salt          
Start vitamins designed for
CF patients
Either visit                      
History and physical with weight,
length, OFC
Either visit                      
Teach / initiate P&PD                          
Assess weight gain, caloric intake and PERT dose                          
Diagnostic testing
Sweat test     All 1° sibs                  
Pancreatic functional status testingᶠ At one of
these visits
                   
Respiratory cultureᵍ                          
Chest X-ray       At one of these visits          
Vitamin levels A, D, Eh     At one of these visits                
Serum electrolytes, BUN, creatinine                    
Complete blood count                    
AST/ALT/GGT/bili, albumin, ALP                    
education
Infection Control                          
Fill out “Who to call–where to go” sheet     Either visit                  
CFF Patient 
Registry consent
    Either visit                  
Discuss clinical research                          
Feeding Behavior Anticipatory Guidance           Either visit   Either visit   At 2 of these visits  
Referrals to community food resources                          
Review ACT technique                          
Tobacco smoke exposure avoidance education                          
Genetic counseling At one of these visits                  
DATE DONE                          

aIn some circumstances, care may be shared with a primary care physician; infants growing poorly may need to be seen more often; some stable infants can be seen every 6 weeks.

bSome centers may plan additional routine visits at 7, 9, and 11 months.

cStart PERT if patient has symptoms, fecal elastase <200 mg/g, coefficient of fat absorption <85%, or 2 CFTR mutations associated with pancreatic insufficiency.

dMany centers include oximetry; pulse oximetry should be performed in infants with acute respiratory symptoms.

eRoutine immunizations should be given by the primary care provider; Palivizumab may be given in appropriate season; influenza vaccine should be given in the appropriate season after 6 months of age.

fRecheck a measure of pancreatic phenotype, such as fecal elastase, if patients have weight loss or GI symptoms.

gRespiratory cultures may be performed more frequently if patient has symptoms.

hVitamin levels are optimally checked 1 to 2 months after starting supplements; ensure that fluoride intake is adequate or is supplemented.

You may want to discuss:

Weight assessments and caloric intake¹
Weight assessments and caloric intake¹
Pancreatic functional status testing and pancreatic enzyme replacement therapies¹
Pancreatic functional status testing and pancreatic enzyme replacement therapies¹
Salt supplements as well as age-appropriate non-fat-soluble vitamins, and vitamins A, D, E, and K¹
Salt supplements as well as age-appropriate non-fat-soluble vitamins, and vitamins A, D, E, and K¹

Click here for additional care considerations based on age.

Ultimately, careful monitoring and medical intervention from an early age will encourage better outcomes for a child with CF.¹

ACT, airway clearance therapy; ALP, alkaline phosphatase; ALT, aspartate aminotransferase; AST, alanine aminotransferase; bili, bilirubin; BUN, blood urea nitrogen; CFF, cystic fibrosis foundation; CFTR, cystic fibrosis transmembrane conductance regulator; Dx, diagnosis; GGT, gamma-glutamyl transferase; GI, gastrointestinal; MO, month; OFC, occipitofrontal circumference; PERT, pancreatic enzyme replacement therapy; P&PD, percussion and postural drainage; WK, week; YR, year.

References: 1. Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(suppl 6):S73-93. doi:10.1016/j.jpeds.2009.09.001 2. Airway Clearance. Cystic Fibrosis Foundation. Accessed June 1, 2021. https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/ 3. Gustafsson PM, De Jong PA, Tiddens HA, Lindblad A. Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2008;63(2):129-134. doi:10.1136/thx.2007.077784 4. Elborn JS. Cystic fibrosis. Lancet. 2016;388(10059):2519-2531. doi:10.1016/S0140-6736(16)00576-6

CF Management in the First 2 Years of a Child’s Life