Management

Whereas in prior decades CF typically resulted in early childhood or adolescent death from end-stage pulmonary disease, aggressive antibiotic therapy with modern agents, supplemented with medical and physical therapies to clear the viscous bronchial secretions, has led to a steady increase in life expectancy, currently extending into the mid- to late 30s (although some patients still die early of meconium ileus or intractable pulmonary infections). More heroic measures, such as lung or heart-lung transplantation, have also become available, and there is great hope for the future (although scant success to date) for gene-replacement therapy.

Because there is still no cure and the disease has a high morbidity, significant mortality, and economic burden, a major thrust of management efforts has been toward prevention. This takes the form of carrier screening to identify couples at risk, followed by prenatal diagnosis for those who wish to avoid the birth or recurrence of an affected child.

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