Pulmonary Complications of Cystic Fibrosis

Abstract

Earlier diagnosis, treatment of exacerbations, and the use of long-term therapies have all improved the lifespan of patients with cystic fibrosis (CF). However, the natural history of CF airways disease remains one of worsening bronchiectasis and obstructive airways impairment. The progression of airways disease leads to eventual respiratory failure, but some will suffer other acute respiratory complications that require intervention, including pneumothorax, massive hemoptysis, and respiratory failure. Here I discuss the pathophysiology of these complications and the patient-related and treatment-related factors associated with their occurrence. Knowledge of these associations may play great importance in treatment decisions regarding the care of the patient and the respiratory therapist should be aware of the implications. Since disease severity is associated with all 3 conditions, aggressive treatment of the underlying condition is imperative, which includes the performance of airway-clearance therapies. Though some might argue that airway-clearance therapies might aggravate or even precipitate complications such as hemoptysis and pneumothorax, others will defend that there are airway-clearance therapies that might be safely performed. Aerosolized medications such as dornase alfa and tobramycin have been associated with a lower incidence of massive hemoptysis and are recommended therapies for patients with advanced airways disease, yet they are also associated with a higher incidence of pneumothorax, which suggests careful assessment of their potential bronchospastic effect in patients with advanced airways disease. The respiratory therapist also plays a key role in the care of the patient with respiratory failure. Here is also discussed the role of ventilatory support and airway-clearance therapies in the patient with advanced stage disease. Now, more than ever, the patient needs caregivers with the knowledge and sensitivity to provide appropriate palliative care.