Francisco J. Soto, MD, Kathryn A. Hale, MD, Diana R. Quintero, MD--Baylor College of Medicine, Houston, Texas, USA
Introduction: We report a young patient with cystic fibrosis (CF) and recurrent episodes of hemoptysis with only transient relief after several bronchial artery embolizations (BAE) and with progressive deterioration of pulmonary status. She was started on tranexamic acid after BAE failed, with excellent control of hemoptysis and with minor side effects.
Case Presentation: 21 year old woman with cystic fibrosis, advanced lung disease, and oxygen dependant, with first episode of hemoptysis in 1996 which was treated conservatively, next one in March 1999 (two cups of blood, approx. 240 cc each cup) requiring hospitalization and embolization, and subsequently in July 1999, October 17 1999, November 9, 1999 and November 16, 1999. During each episode she expectorated at least one cup of blood, requiring hospitalization, bronchial artery embolization (BAE) and admission to the Intensive Care Unit (ICU).
Her past medical history includes pancreatic insufficiency, diabetes (on insulin pump) and sinusitis. She uses 2-3 liters of oxygen x nasal canulla at rest, has a gastric tube and a port-a-cath placed in April 1999. Sputum cultures have grown Pseudomonas aureginosa, and she has been on intravenous (IV) antibiotics at home since November 1999. Her baseline spirometry shows FEV1 1.05, FVC 1.27 from July 1999.
On December 1, 1999, two weeks after the last hospitalization for hemopty,sis, it was decided to start the patient on tranexamic acid based on isolated case reports showing decrease in hemoptysis and prevention of recurrence while the patients remained on this therapy, after they had failed BAE. The starting dose was 250 mg four times a day, which was increased to 500 mg four times a day 10 days later for persistence of streaking. Since this date, and during a 5 month follow up period, the patient had only 3-4 minor episodes of blood streaking in sputum, which were managed with occasional extra doses of Tranexamic acid, and didn't require any additional interventions or ICU admissions. Her main complaint had been a sluggish blood return from her port-a-cath, which eventually required replacement of the catheter. On April 4, 2000, she finally underwent a bilateral lung transplant.
Discussion: The importance of treating major hemoptysis in CF is not only related, to the risk of immediate mortality which can be as high as 11%, but also for the preservation of pulmonary function, life style and psychological well being of the patient. Medical treatment with IV antibiotics and aggressive therapy like BAE is recommended in major bleeding. In specialized hands, BAE has been shown to be a safe and effective treatment of hemoptysis in CF. However, it is a procedure that involves, serious risks, and additional therapies for control of hemoptysis, in difficult patients are well appreciated. Tranexamic acid has been used as an adjuvant therapy for blood dyscrasias, prevention of rebleeding in intracranial aneurysms, oral surgery procedures in patients, with hemophilia, etc. Its use in CF patients with hemoptysis has only been recently described, with optimistic results when other therapies have failed to prevent rebleeding, and with only minor side effects reported. Since the mode of action is related to its antifibrinolytic activity by the inhibition of plasmin and plasminogen activation, the main side effects are mainly thrombotic, and the major contraindications are any increased coagulopathic state, microscopic hematuria and retinopathy. Its use in the presence of indwelling catheters such as Portacaths has to be done carefully balancing risks and benefits.
Conclusion: Blood streaking in sputum is a common event in CF patients, which frequently resolves on its own or with antibiotic therapy. Major hemoptysis however, is a serious condition, which can lead to progressive deterioration of the pulmonary function or even death. In difficult cases where BAE doesn't offer complete resolution of symptoms, other therapies like Tranexamic acid have to be entertained. In this patient, we consider that the use of Tranexamic acid served as a bridge to keep her stable until the lung transplant was performed.
COPYRIGHT 2000 American College of Chest Physicians
COPYRIGHT 2001 Gale Group