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:
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.
|AGE AT VISIT||day of sweat test||24-48 hours
|1 wk later
|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|
|Discuss diagnosis||Either visit|
|Start PERTc||Either visit|
|Start salt supplementation||1/8 tsp. salt||1/4 tsp. salt|
|Start vitamins designed for
|History and physical with weight,
|Teach / initiate P&PD|
|Assess weight gain, caloric intake and PERT dose|
|Sweat test||All 1° sibs|
|Pancreatic functional status testingᶠ||At one of
|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|
|Fill out “Who to call–where to go” sheet||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|
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.
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