Table 5b. [Treatment of Manifestations in a Newborn with Cystic Fibrosis]. - GeneReviews®

Manifestation/Concern	Treatment	Considerations/Other
General	Refer to CF specialist / CF care center.
Pulmonary	Teach parent/caregiver airway clearance (typically postural drainage & percussion) to aid in mucus clearance.
Infection	Encourage feeding w/breast milk. Routine vaccinations
Use contact precautions (gown & gloves) for every encounter by every clinician.	Assume all people w/CF have transmissible bacteria.
Antibiotics (oral, inhaled, or IV) for chronic suppression & to treat pulmonary exacerbations Treat identified bacteria in affected person & bacteria common in CF. ¹	Eradication attempts are recommended for Pseudomonas aeruginosa. ²
Exocrine pancreatic insufficiency	Consult nutritionist w/experience in CF. Pancreatic enzyme replacement therapy Nutrient-dense food & supplements Fat-soluble vitamin supplements
Meconium ileus & distal intestinal obstructive syndrome	If obstructed, consult surgery for intervention. If not obstructed, treat w/laxatives.
Salt loss syndromes / Dehydration	Salt supplementation Extra salt & water for hydration & salt loss in situations w/salt loss (e.g., ↑ heat/humidity, exercise)

Manifestation/Concern

Treatment

Considerations/Other

General

Refer to CF specialist / CF care center.

Pulmonary

Teach parent/caregiver airway clearance (typically postural drainage & percussion) to aid in mucus clearance.

Infection

Encourage feeding w/breast milk.
Routine vaccinations

Use contact precautions (gown & gloves) for every encounter by every clinician.

Assume all people w/CF have transmissible bacteria.

Antibiotics (oral, inhaled, or IV) for chronic suppression & to treat pulmonary exacerbations
Treat identified bacteria in affected person & bacteria common in CF. ¹

Eradication attempts are recommended for Pseudomonas aeruginosa. ²

Exocrine pancreatic
insufficiency

Consult nutritionist w/experience in CF.
Pancreatic enzyme replacement therapy
Nutrient-dense food & supplements
Fat-soluble vitamin supplements

Meconium ileus & distal
intestinal obstructive
syndrome

If obstructed, consult surgery for intervention.
If not obstructed, treat w/laxatives.

Salt loss syndromes /
Dehydration

Salt supplementation
Extra salt & water for hydration & salt loss in situations w/salt loss (e.g., ↑ heat/humidity, exercise)

From: Cystic Fibrosis

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Adam MP, Feldman J, Mirzaa GM, et al., editors.

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