Internal Medicine

0521779407-C04 CUNY1086/Karliner 0 521 77940 7 June 14, 2007 20:37

454 Cystic Fibrosis

management

What to Do First

■Assess oxygenation, adequacy of ventilation, need for admission (as

for COPD)

General Measures

■Initiate antibiotics (usually 2–3 wks), nutritional assessment and res-

piratory therapy

■Hospitalization may be more for nutrition, respiratory therapy than

antibiotics

specific therapy

Indications

■Combination of increased symptoms, weight loss, hemoptysis, wors-

ening spirometry

■Patients usually know when inpatient care necessary

Treatment Options

■Bronchodilators: as for COPD

■Inhaled steroids: often tried; high doses may be more effective; titrate

down for safety

■Antibiotics: base on recent organism and sensitivities; higher than

standard doses are required; use central line (ie, percutaneous or

indwelling port):

➣S aureus: usually penicillin resistant

➣Pseudomonas: double drug therapy required (2 of 3 classes)

Beta lactam: eg, ceftazadime, piperacillin, meropenem

Quinolone: ciprofloxacin

Aminoglycoside: gentamicin or tobramycin

■Inhaled tobramycin: useful for chronic Pseudomonas colonization

■Inhaled DNAse: prevents exacerbations; no benefit for acute exac-

erbation

■Respiratory therapy: patient preference important:

➣Traditional: chest physiotherapy with postural drainage

➣Mechanical devices: flutter valve, PEP mask, mechanical percus-

sor, chest vest, positive pressure oscillation (IPV)

■Ventilatory support:

➣Partial: nasal mask ventilation useful as temporizing maneuver

or bridge to lung transplantation

➣Mechanical ventilation: used for acute respiratory failure when

reasonable hope of extubation