Monitoring
There is no cure. Therefore, long-term care is a cornerstone of successful disease management.
Patients should be seen by a CF care team at a specialist centre at least once every 3 months.[33] At each visit, patients should have a history and physical examination performed by a specialist CF physician. They should also be evaluated by a registered dietician or nutritionist, ideally within speciality care in CF, who can guide all aspects of medical nutrition therapy, including nutrition screening, nutrition assessment, and dietary intake.[179]
If possible, patients should perform spirometry to monitor lung function. On a yearly basis and when clinical symptoms dictate, a chest x-ray, blood work (including full blood count, liver function tests, coagulation profile, vitamin A, D, and E levels), and full pulmonary function testing (including measurement of lung volumes and diffusing capacity) should be performed. FEV₁ and rate of FEV₁ decline are the main predictors of mortality.[180] Both computed tomography (CT) and magnetic resonance imaging (MRI) have utility for the routine monitoring of CF lung disease.[181] In particular, chest CT imaging is a better tool than chest x-ray for understanding radiographic changes in the lungs, despite the relative discomfort.
Patients with CF are treated with many ototoxic medications, including aminoglycosides, glycopeptides, macrolides, and non-steroidal anti‐inflammatory drugs.[152] Intravenous aminoglycosides are commonly used and may be given at higher doses and for prolonged periods, necessitating careful monitoring of serum aminoglycoside levels and at least annual hearing tests. Patients receiving nephrotoxic antibiotics should have their blood levels monitored regularly while receiving therapy, including weekly serum creatinine levels.
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