Hemoptysis, or coughing up blood, is one of the scariest complications of cystic fibrosis, although it doesn't have to be. “Hemoptysis” is also the name of a heavy metal band, and that is truly scary! Hemoptysis, the medical condition, is relatively common in adults with CF and rare in young children.
As many as 50 percent of adults with CF will occasionally see some streaks of blood in the mucus they spit out, and a much smaller percentage of patients (3-5 percent of those older than 15 years) will cough considerably more blood up. Although hemoptysis can be fatal, it rarely is, even among people who cough up a lot of blood, which is defined as more than one cup (8 ounces) in about 20 minutes.
Most often, hemoptysis is evidence of increased infection.
Like most infections, infections that cause hemoptysis are nearly always treatable. The increased infection in one small area of the lung irritates a small blood vessel, causing it to bleed. The difference between coughing more frequently and spitting out blood is that it can be very frightening to see the blood. To put this in context, even though it might look like your whole lung is bleeding, it's not. If you were to spread out someone's lungs (Don't do it! This is just a teaching point.), they would just about cover the surface of a tennis court. Well, if someone has hemoptysis, the area that's bleeding is likely to be the size of a little pebble on that tennis court.
Treating minor hemoptysis (streaks of blood in mucus) is just like treating any lung infection: increase or change antibiotics (oral, inhaled, or perhaps intravenous [IV]) and improve airway clearance. Some doctors suggest taking extra vitamin K, as that can help blood clot.
If you're bringing up a lot (more than one cup) of pure blood, you need to let your CF care team know, and you may need to be hospitalized. You will definitely need antibiotics, likely by IV. Depending on how much blood you have lost, you might even need a blood transfusion.
CF experts disagree on the role of airway clearance for someone who's bringing up large amounts of blood. Without question, gentle airway clearance is good (Clear those airways! Get rid of blood, which is a hospitable environment for germs! Open up areas of the lung for breathing!). But more vigorous methods, such as the vest, chest physical therapy, and positive expiratory pressure, may or may not be useful.
If brisk bleeding continues, some CF experts recommend pulmonary artery embolization, a specialized procedure where a small catheter is threaded through blood vessels to the lung, and a small plug is injected to block off the bleeding vessel. This often works, but the result may be temporary, and like so many medical procedures, this one carries some risk. Your CF care team will recommend treatment that is appropriate for you.
People with CF have asked if there are factors or medicines that make hemoptysis more likely. The simple answer is no, but each person is different. Individuals and their lungs don't always follow what the textbooks say. So, if you notice that whenever you do “x,” or take medicine “y,” you seem to have more hemoptysis, let your care team know, and they'll likely say, “Well, let's avoid x or y.”
Not all people with CF experience hemoptysis. For those who do, it's likely the result of a lung infection that has gotten worse. If it happens to you, let your CF care team know. They will almost always be able to treat this sometimes scary problem successfully.