Cystic Fibrosis/Hemoptysis

Overview

Cystic fibrosis (CF) is a genetic disease which predominately effects the lungs.  As the disease progresses complications such as hemoptysis (coughing up blood) become more common. Life threatening bleeding is rare, however bleeding can effect normal daily living and is often associated with acute worsening of lung function. Massive bleeding occurs in about 1 in every 115 cystic fibrosis patients every year.


Symptoms and History

Blood in sputum is the definition of hemoptysis. Bleeding may range from blood tinged sputum to grossly bloody material. Coughing up blood may be an indicator of CF exacerbation.


Diagnosis

Hemoptysis is defined as scant (< 5 ml), mild (>5 ml), moderate, and massive. Massive hemoptysis is defined* as coughing up more than 240 ml over a 24 hour period or 150 ml of blood over a 1 hour period.


Treatment

Scant hemoptysis may require no treatment. Mild and moderate hemoptysis may require treatment for CF exacerbation. Most cases of hemoptysis resolve spontaneously. CF patients with massive hemoptysis should be treated with bronchial artery embolization. At times bronchial artery embolization will be performed in CF patients with less than massive hemoptysis if it is not resolving and is effecting treatment.


* There are various definitions as to what qualifies as massive hemoptysis.

Flume, Patrick A., Peter J Mogayzel Jr, Karen A Robinson, Randall L Rosenblatt, Lynne Quttell, Bruce C Marshall. Cystic Fibrosis Pulmonary Guidelines Pulmonary Complications: Hemoptysis and Pneumothorax. American Journal of Respiratory and Critical Care Medicine. Vol 182, No. 3 Aug 01, 2010.

Brinson GM, Noone PG, Maur MA, Knowles MR, Yankaskas JR, Snadhu JS, Jaques PF. Bronchial Artery Embolization for the Treatment of Hemoptysis in Patients with Cystic Fibrosis. American Journal of Respiratory and Critical Care Medicine. Vol 157, No. 6, Jun 01, 1998