Table III.
Routine monitoring and care recommendations for the infant diagnosed with CF
| AGE AT VISIT → | Day of Sweat test |
24–48 hrsof dx |
1 wk later or age 1 mo |
2 mo* | 3 mo* | 4 mo* | 5 mo* | 6 mo | 8 mo | 10 mo |
1 year |
Every 2–3 months in the second year of ife |
24 mo | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ↓INTERVENTIONS | Note: Some Centers may plan additional routine visits at 7, 9 and 11 months |
||||||||||||||
| DATE DONE→ | |||||||||||||||
| CARE ISSUES | |||||||||||||||
| Discuss diagnosis | Either visit | C | C | C | |||||||||||
| Encourage human milk feeding | Either visit | ||||||||||||||
| Start PERT1 | Either visit | C | C | C | C | C | C | C | C | C | C | C | |||
| Start salt supplementation | 1/8. tsp salt | ¼ tsp | |||||||||||||
| 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 | C | All 10 sibs | |||||||||||||
| Pancreatic functional status testing4 | At one of these visits | C | C | C | C | C | c | C | C | C | C | C | |||
| Respiratory culture 5 | |||||||||||||||
| Chest x-ray | At one of these visits | ||||||||||||||
| Vitamin levels A, D, E 6 | At one of these visits | ||||||||||||||
| Serum electrolytes BUN7, creatinine | |||||||||||||||
| Complete blood count | |||||||||||||||
| AST/ALT/GGT/ bili , abumin, ALP 7 | |||||||||||||||
| EDUCATION | |||||||||||||||
| Infection Control | |||||||||||||||
| Fill out “Who to call - where to Go” sheet | Either visit | ||||||||||||||
| CFF Patient Registry consent | Either visit | ||||||||||||||
| Discuss clinical research | C | C | C | C | C | ||||||||||
| Feeding Behavior Anticipatory Guidance | Either visit | Either visit | At 2 of these visits | ||||||||||||
| Referrals to community food resources | C | C | C | C | C | C | C | C | C | C | C | C | |||
| Review ACT technique | |||||||||||||||
| Tobacco smoke exposure avoidance education | |||||||||||||||
| Genetic counseling | At one of these visits | C | C | ||||||||||||
CF, cystic fibrosis; PERT, pancreatic enzyme replacement therapy; PCP, primary care provider; X, do at this visit; C, consider doing at this visit.
In some circumstances, care may be shared with PCP; infants growing poorly may need to be seen more often; some stable infants can be seen every 6 weeks.
Start PERT if patient has symptoms, fecal elastase <200 μg/g, coefficient of fat absorption <85%, or 2 CFTR mutations associated with PI.
Many centers include oximetry; pulse oximetry should be performed in infants with acute respiratory symptoms.
Routine immunizations should be given by the primary care provider; Palivizumab may be given in appropriate season (see text); influenza vaccine should be given in the appropriate season after 6 months of age.
Recheck a measure of pancreatic phenotype, such as fecal elastase, if PS pts have weight loss or GI symptoms.
Respiratory cultures may be performed more frequently if patient has symptoms.
Vitamin levels are optimally checked 1 to 2 months after starting supplements; ensure that fluoride intake is adequate or is supplemented.
BUN, blood urea nitrogen; AST, alanine aminotransferase; ALT, aspartate aminotransferase; GGT, gamma-glutamyl transferase; bili, bilirubin; ALP, alkaline phosphatase.