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coughing up blood

is it serious to be coughing up blood in cystic fibrosis
Thank you for your question,

Coughing up blood (known as haemoptysis) is usually caused by a ruptured small blood vessel. It is a common complication in people with CF.
Each CF centre may have local guidelines in place to manage that bleed, which will vary depending on the findings of a clinical assessment.

Additionally there are some guidelines available from the UK CF Trust and some American guidelines (see the references below) which help categorise bleeding as mild, moderate or severe. These guidelines also make some recommendations regarding airway clearance during an episode of bleeding as well as other possible therapeutic options.

Mild: Streaks of blood are seen in the sputum and this is a common finding in patients with CF and requires no specific treatment. Persistent streaking however, may indicate a pulmonary exacerbation requiring appropriate therapy. The UK CF Trust guidelines state this can be common in CF and there is no need to change your airway clearance.
Moderate: Coughing up blood up to half a pint (less than 250mls). The UK CF Trust guidelines state that your normal airway clearance may need changed and your CF physiotherapist will be able to advice you on appropriate airway clearance techniques.
Severe: Coughing up blood to more than half a pint (more than 250mls). The UK CF Trust guidelines state your normal airway clearance should stop until the bleeding settles.
The American guidelines only distinguish between "minor" (which corresponds to mild haemoptysis) and "major" haemoptysis.

Major haemoptysis is defined as acute bleeding of a large amount of blood (often defined as 140ml in 24 hrs) which could be life threatening. Recurrent bleeding of substantial volume (e.g. more than 100ml/day) over a short period of time (e.g. 3-7 days) is also termed major haemoptysis. Treatment options in case of major haemoptysis: 1. discontinue drugs with influence on coagulation, such as aspirin, NSAID or penicillin. 2. Inhaled drugs which may be pulmonary irritants should be discontinued such as N-acetylcysteine and aerolized antibiotics. 3. Search for and correct coagulation defects. 4. most major bleeding episodes appear to be associated with pulmonary exacerbations, therefore effective treatment should be initiated probably not penecillins should be used. 5. no evidence that discontinuing physiotherapy is either beneficial or harmless: decision is left to physician and patient, please also take the UK CF Trust guidelines mentioned above for this into account. 6. Arterial closure or operative lobektomie in severe cases.
Apart from those guidelines, some new trials which are used by some centres are the inhalation of epinephrine (however, there is no proof of the effectiveness by scientific data) and tranexamic acid (based on some case reports).
You should discuss all episodes of coughing up blood with your own CF team; they will be particularly interested in whether this was fresh and the volume.
Best wishes,

Cystic Fibrosis Trust Clinical Guidelines for the physiotherapy management of cystic fibrosis. Recommendations of a working group January 2002. (Page 23).

Schidlow et al. 1993 "Cystic Fibrosis Foundation Consensus Conference Report on Pulmonary Complications of Cystic Fibrosis" in Pediatric Pulmonology 15: 187-198.

Kind regards
The answer is edited by: Prof Stuart Elborn