What it is
Hemoptysis (coughing up blood) is a result of bronchiectasis, which is the scarification and weakening of the walls of the bronchioles in the lungs. When the little airways in our lungs become scarred and lose their elasticity, any kind of trauma (i.e. coughing) or inflammation can cause them to tear or collapse. When this happens, blood from an adjacent bronchial artery can flood into the airway and into the lungs, causing us to cough up that blood. Most of the time a very tiny bit of blood gets into the airway before the body quickly repairs the tear with a clot or by clamping down on itself to staunch the bleeding. This is when we see streaking in our sputum or when we get a small (a teaspoon) and quick bout of pure red blood. When larger bronchioles tear it becomes a little harder to create a clot quickly, so a larger amount of blood can get into the lungs. This is when you get several tablespoons to a cup of blood out. The body is very good at finding ways to stop the bleeding, and even in a situation where a cup of blood is coughed up (I've had this happen, definitely scary) the body will usually find a way to clot the tear or staunch the blood within a few minutes. For more info on hemoptysis, visit my page CF 201.
A teaspoon or a couple of tablespoons of blood is nothing to get scared about, however it is a signal that something down there needs some attention. It is a sign that you are battling an infection or have some acute inflammation in the lungs that is exacerbating your bronchiectasis. For me, this is usually a sign that I need to focus my attention on reducing inflammation (either through antibiotics or paying closer attention to my diet and supplements). I can also sometimes get it in times of acute stress, excitement, or exposure to an environmental pollutant, like hanging out around a campfire. In addition, if I have an acute infection and I do some kind of strenuous aerobic activity that causes me to huff and puff and cough heavily, that can also trigger hemoptysis. When I'm sick, I'm obviously going to continue exercising and doing aerobic activity, however if I see streaks in my sputum that day, I'm gonna tone it down a little to allow the body to heal that tear so it won't get any worse.
I usually get hemoptysis right when I lay down in my bed to sleep. I think that the sudden change in airway pressure from standing to laying down can cause the little bronchioles to collapse or tear more easily. When I know that I am at risk for some hemoptysis that day, I ease into the horizontal position by first reading in bed for a few minutes in a reclined position propped up on a pillow. This usually works as long as I first lay with the non-bleeding side down (if you can tell which side it's coming from).
Everyone's hemoptysis is going to be a little different, and so the triggers will be a little different. I've learned to notice the subtle changes, feelings, and sounds in my chest that may alert me to the risk of hemoptysis. The key is to pay close attention to your actions and what could be causing the hemoptysis so you can reduce your risk. A lot of it is out of our conscious control, however we can always do something, big or small, to help or hurt the situation.
What to do
If there are infrequent streaks of blood in the sputum, it's not that big a deal so there isn't really much you need to do other than keep an eye on it and think about how you could take further action to reduce your inflammation.
If you cough up a small amount of pure blood, somewhere between a teaspoon and several tablespoons, I've been advised to stop using pulmozyme for 24 hours. Pulmozyme is designed to break up mucus, but it can also break up a fresh blood clot. I continue to do the rest of my treatments normally. Also, try not to do any overly-strenuous aerobic activity that's gonna make you cough hard. It's very important to continue exercising, but maybe choose something more mild, like walking, biking, or yoga. Rarely if ever does yoga cause me hemoptysis, even though there are inversions and a lot of laying down and getting up. But again, you're gonna have to see how your unique body responds. After coughing up pure blood, reflect back to see if there were any particular triggers. Did you eat something inflammatory? Where you exposed to any pollution? Did you get really stressed out today? Were you exercising too hard? Do you feel an infection coming on? And make sure to eat as many anti-inflammatory foods and supplements as you can, including vegetables high in antioxidants, healthy fats, fish oil, and anti-inflammatory herbs.
If you cough up a lot of blood, like half a cup or more, you need to call your doc. They can advise you on what to do. Sometimes people can cough up a cup of blood once in their life and never do that again. Sometimes keeping on top of your health with good diet, exercise, and diligent adherence to your treatments can prevent big episodes and resolve the issue. In these situations, no further action needs to be taken. I've coughed up more than a 1/2 cup of blood twice, and each time it was a sign that I needed an admission. The second time I got more serious about considering a pulmonary embolism. Since I was doing pretty much everything I could with my diet, exercise, and treatments and still the hemoptysis was happening, it was time for me to consider a more serious intervention.
If you cough up blood and can't stop, call 911 and get to the hospital! I have been told that although hemoptysis is very common is CF, this kind of emergency where someone can't stop bleeding is very rare. Almost always the body is able to find a way to stop the bleeding. But it is good to at least be mentally prepared for the worst and understand how to manage it if it happens. If the blood doesn't stop, the risk is that you might drown. Yeah, that's a frickin' scary-ass idea, huh? It doesn't happen right away though, maybe within an hour or so. By then an EMT will have intubated you and you'll be fine, on your way to the hospital for an emergency embolism. I've also been told that laying on the side that is bleeding is a good idea so that the other lung will be prevented from getting any blood in it. Again, I've been told by multiple doctors that this pretty much never happens because people usually get an embolism way before things get this bad. So don't lose sleep over it. It is a scary thing, but fear does us no favors. I've prepared myself for this very rare possibility by educating myself as to what could happen and how I should act if it does. Knowledge is power, and so it helps to allay my fears when I know more about the situation. That's why I'm writing this post, to help others be unafraid in the event that the worst happens.
Bronchial Artery Embolism
If you've had several big episodes of hemoptysis, or your hemoptysis is getting worse and more frequent, it may be time to ask your docs about a bronchial artery embolism (sometimes called a pulmonary embolism). It is a procedure where an interventional radiologist attempts to pinpoint the spots that your lungs are bleeding and to embolize them. The word embolism means to create a blockage in a blood vessel so that blood can no longer flow through it. If you are no longer actively bleeding, before you get the embolism your pulmonologist will most likely do a bronchoscopy to try to find any evidence of the spot that is bleeding, ideally by finding the clot or evidence of leftover blood. If they can find the spot, that's really great because it will help the radiologist find that spot from the arterial side and embolize it more quickly. If they cannot find the spot, it is still possible for the radiologist to do the embolism because they can do a contrast x-ray (where they inject you with radioactive dye and map your arteries under an x-ray machine) of the bronchial arteries, mapping your branches to see where are possible spots of bleeding. This is what happened to me - they couldn't find the spot with a bronchoscopy, but they found plenty of spots with a contrast x-ray. So we did the procedure anyway and I'm glad we did.
Small bronchial arteries run alongside our smallest airways (bronchioles), which provide oxygenated blood and nutrients to our lungs. When an airway collapses it allows the adjacent bronchial artery to bleed into the lung. When an airway is having trouble fully healing and is breaking open over and over again, one solution is to embolize the adjacent artery so that it can no longer bleed into the lungs. This is done by shooting tiny plastic pebbles into the artery to block it up. If you know your anatomy, you may be thinking: Wait, won't that kill the lung tissue because it will be denied access to blood? The answer is yes, to some extent. But embolizing usually does not cause a significant damage to the lung tissue because our lungs create redundant connections to a fresh blood supply. But an embolism will cause at least a little bit of tissue death. This is why it's a last resort procedure that you don't want to do unless you really have no other choice. There's also no guarantee that the radiologist will embolize the "right" spot that is causing the bleeding - it's really hard to see what you're doing down there. Some people have to do it a few times to get relief.
If you've got a significant amount of bronchiectasis like me, you've probably got plenty of spots down there that have bled in the past or have the potential to bleed in the future. So even if a bronchoscopy can't find "the spot", a radiologist will most likely be able to find several places that are at high risk for bleeding and will embolize them as a preventative measure. This was the case for me. My bleeding is not completely gone, but it's significantly reduced and now I only bleed when I'm very inflamed, maybe once a month (as opposed to every week or so before the embolism).
I've had one embolism, and sometimes people have them done several times. But what does it feel like? Well, I can tell you the brochoscopy was the worst part of it for me. The actual embolism is super easy because you're gonna be sedated, and with versed (the nice stuff) not general anesthesia. The procedure itself doesn't hurt, but there are three possible issues that are not fun to deal with: 1) they have to access the bronchial artery through an incision near the groin. If you're a prude like me, this is not very appealing (but I did it anyway and it was fine). 2) After the procedure you have to lay down straight and not move for 4 hours because the arterial incision has to heal - no hemorrhages please! If you're unlucky, you'll get to experience what it's like to have to use a bedpan. Trust me, it will provide great material for jokes later on. 3) A day or so after the procedure you may feel weird pains in your chest that feel like intense muscle aches or cramps in the ribs. I'm pretty sure it's not the muscles that hurt me but the pain that I felt from the lung tissue death and the resulting inflammation that is needed for the body to clean it up. This pain may last a couple of days, and may even come with a fever. Although it's kinda painful, it does go away and it is just a sign that your body is cleaning things up and healing.
So that's a bit about hemoptysis and what to do about it. Ideally with a healthy diet and lifestyle we can reduce our bronchiectasis and prevent hemoptysis from happening. But in case it does happen, it's good to be educated on what choices you have. This is a pretty scary topic for most people, and some people would rather stick their heads in the sand and not think about it. But it is my opinion that the more informed we are, the more prepared we can be in these situations and the less scary they become. I've had a lot of fear about coughing up blood in the past, but I faced that fear head on and got over it. It doesn't scare me anymore, and I am much less anxious about it when it does happen. I feel a little bit more in control and prepared for the possibilities. Again, being informed is being empowered. I hope this helps.
Update: May 2018
A few years ago a new application for an old drug came across my radar: tranexamic acid. It is a drug that has been used in Japan since the 1960's to treat post-partum hemorrhaging in people who just gave birth. When I read that article I immediately thought: woah, we CFers could use something like that! So I asked around and it turns out that a couple of clinics had been experimenting with it for use in hemoptysis with good results. I asked my CF docs about it, and they had never even heard of it. There is precedent for its use in CF hemoptysis, and several CFers that I've spoken with have found it to be very helpful in acute bleeds. So if you think you might benefit from it, please ask your doc about it. I want more people in the CF community to know about this potentially life-saving therapy.