Pathophysiology of Cystic Fibrosis Implications for Critical Care Nurses

Grossman, Sheila; Grossman, Lisa C.
August 2005
Critical Care Nurse;Aug2005, Vol. 25 Issue 4, p46
Academic Journal
Even though a specific gene associated with cystic fibrosis has been identified, patients who have this disease are still experiencing life-threatening problems. Understanding the multisystem pathophysiology of the disease greatly helps nurses provide holistic care for these patients when pulmonary exacerbations occur. Clues to assist in detecting and managing patients with cystic fibrosis mutations are discussed. Critical care nurses will always manage the inherent ethical, social, and legal issues that confront patients with cystic fibrosis even when genetic testing and interventions are more fully implemented. Studies of the clinical, psychosocial, quality-of-life, and genetic issues associated with cystic fibrosis are needed. Knowledge of the information in this article can assist critical care nurses in managing patients with cystic fibrosis who have these challenging clinical problems. Because patients with cystic fibrosis are living longer than before, more of them experience signs and symptoms of multiple organ dysfunction. Critical care nurses need to advocate for these patients during the patients' acute exacerbations.


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