Let's start with a bit of physiology. There are several tissue types that make up the lung. The alveoli are air sacks that look like little clusters of grapes. These fill with air and are responsible for the gas exchange of oxygen and carbon dioxide into and out of the blood. The bronchi and bronchioles are the tubes that funnel air from your mouth, through the trachea, down into the alveoli. The pleura is a double-lined sack that encases the alveoli, expanding and contracting as your diaphragm moves up and down. There is an inner pleura (the visceral pleura) and the outer pleura (parietal pleura), and between them is the "pleural space" filled with a thin fluid that allows the two linings to slide against each other smoothly as you breath. A healthy set of lungs is illustrated in the diagram below.
In general, a pneumothorax is an emergency. However, if you live long enough with a disease like this, you'll come to find that there are different grades of emergency. I've had two big pneumos in the last month or so. The first one I knew was there but I didn't go to the hospital for about three days. I waited because sometimes I get little ones that get better on their own without any intervention; these probably feel a lot like pleurisy. But this one didn't get better - it only got worse. The second one this time around was bigger and I knew that it was serious right away, so I called my doc and went to the ER that day.
That said, a CF pneumothorax is a bit different than other types of pneumos (yes, we are so very special). Because CFers with advanced lung disease have a very significant amount of scarring, we rarely get complete pneumos like regular people (that is, where the lung completely collapses) because the scarring pins the inner pleura to the outer pleura, kind of like tacking up a tapestry to the ceiling of your room. I guess that's kinda good, right? Might as well look on the bright side of this whole situation. So when I've had my pneumos, they've always been either "small" or "moderate" by radiological standards. Each time my docs felt safe enough to have me wait a few days before making a decision on what intervention I needed (with daily x-rays to make sure it wasn't getting bitter). Often times people with CF get apical pneumos, meaning that the pleura at the tops of the lungs (the apexes) collapse because infection/inflammation causes the most damage to the topmost lobes first.
In CF, pneumos are caused generally by severe infection or inflammation that degrades the integrity of the inner pleural lining so that it tears and allows air to leak out of the lung space. Often times there can be a vulnerable spot, then a big coughing fit can cause the tear. The pleura are highly enervated, meaning that they have a lot of nerves, and when something is wrong, you will feel it! It can be very painful. I don't think I've ever had pleurisy, which is painful inflammation of the pleura, but pleurisy and pneumos are likely connected. Some people get pleurisy and never get a pneumo, and others (like me) get pneumos but no pleurisy. Some CFers get neither. You tend to be "the type" or not. But if you've had one, you're probably gonna get more.
I got my first pneumo in July of 2014. Spring and summer is usually when my biggest lung problems happen. I think it has to do with the warm, humid air and potentially the pollens, although I don't have any allergies. My lungs really like cold, dry air, so they're very happy in the Vermont winters. This first time, there was a bit of pain, enough for me to not be able to sleep well, and I had this weird "floppy" sensation in my left lung when I bent over. With my typical "it's not big deal" attitude I waited a day, then biked to the grocery store and called my doc. They asked me to get an x-ray, so I took the bus to town (about an hour) and they told me I had one. I was admitted to the hospital right there. I think I brought some clothes and stuff, but I can't remember. This was all very new for me. I didn't know how serious a pneumo was. We started IV antibiotics and waited a few days to make a decision. They convinced me that a pleurodesis was the best option.
Now normally, for non-CFers, if you have a pneumo they'd put in a chest tube immediately to vent the air that is trapped between the pleura. But for CFers, since our pneumos aren't as emergent as complete collapses, sometimes we skip this step. Sometimes it's small enough that we don't need to do anything and the body just reabsorbs the air on its own, and the two pleura going back to normal. For a while. But if you have CF, once you've had one pneumo, you're pretty much guaranteed to get another one. So the "permanent" solution to the problem of recurring pneumos is a pleurodesis. I'll describe that procedure in a little bit.
So I got the pleurodesis surgery and it was "successful". It was the most painful thing that's ever happened to me, but, "successful". The next July, one year later, I was hiking with my herbal classmates on our annual camp out, on a very hot day, and in the middle of the trail I felt it. It was undeniable this time, and the pain was worse than before. And it was on the same side. I was pretty angry. Not only was this at the most inconvenient time possible, but the docs had all but guaranteed me that the surgery would prevent this from ever happening again on that side. The x-ray showed a small pneumo on the left side and I was admitted to do IV antibiotics. The docs said it was unusual for someone to get a pneumo on the side that was pleuradesed, but obviously, it's possible. However, because I had done a pleurodesis on that side, they said all we had to do was wait for the body to reabsorb the air in that little spot. The procedure I had done the previous year would prevent the collapse from getting any bigger. And it was true. We waited and it healed on its own. But I missed the field trip.
Since then I've had that characteristic pleural pain several times on both sides, mainly at the tops of my lungs, but I just waited it out and it resolved on its own in a couple days. I haven't had another significant pnemo on the left side since the one in 2015.
This year, at the end of April, I had pleural pain on the right side that started on a Saturday night (after about 5 hours of very intense drumming for a dance class that day, which caused a lot of inflammation, apparently) and it got worse over the weekend until I finally went in for an x-ray on Tuesday and got a diagnosis of a pneumo on the right side. It was small, and so I was admitted to the hospital to get two weeks of IV antibiotics (one week at home). I was put on continuous oxygen because oxygen has an easier time being reabsorbed by the body than other room-air gases like nitrogen, so if you fill the lung space with oxygen the air in the pleural space can get reabsorbed faster, theoretically. I decided to wait and do no intervention other than that. I knew what a pleurodesis felt like and I was desperate to avoid it if I could. A week later the x-ray showed it was completely resolved, so I went home and rested until the pain went away, then I got back to normal life about a week and a half later. But it was too early. After a long weekend of work and hosting a guest in my house for three days, I had pleural pain all night and then that morning I sat up and felt the pain suddenly intensify. I knew immediately that I had made a terrible mistake. I had not allowed myself enough time to heal before becoming active again. I went in for an x-ray and it was confirmed: the pneumo was there and it was bigger than ever.
I was admitted to the hospital on Monday and we just waited it out and watched the pneumo. It didn't get any bigger, but my worst fears were confirmed: we'd have to do an intervention or this would just keep happening, even if it did heal again. On Tuesday I decided to schedule the pleurodesis. On Wednesday they put in a chest tube with interventional radiology (guided under a CT machine) to vent some of the air, since it was getting harder for me to breath and painful to walk around. Although that hurt a little it was really not a big deal. On Thursday I got the pleurodesis.
I've had a lot of surgeries in my life. A lot. But I will say that the pleurodeses were the most intense that I've had. I'm not saying this to scare you off or make you decide against them, because basically when it comes down to it there's not much choice. But it's important to know what you're getting into. I didn't know the first time, but I was really glad that I was prepared for the second one. Here's what it is.
A pleurodesis is a procedure done with laparoscopy where the two pleura are fused together, "permanently" preventing a big pneumo from ever happening again. This is done by abrading the space between the two pleura and spraying it with talc so that the tissue becomes so inflamed that it scarifies and fuses together, removing the pleural space. If this sounds painful, that's because it is. In fact, they'll tell you that if it's painful that means it's working.
The procedure is short, only takes about an hour. You generally wake up with a chest tube and two or three other incisions. I decided to get an epidural both times, which was a REALLY good choice. The epidural and the chest tube stay in for two to four days. The chest tube drains any excess air and fluid that has been left over in the pleural space after surgery. Once the tube stops draining and the pneumo seems to be resolved, they'll take the tube out, and once the tube is gone you generally don't need the epidural anymore.
The epidural is essentially a method of injecting an anesthetic or pain killer directly into the space surrounding the spinal cord so that you numb a finite area in the chest without getting the negative side effects of systemic opiate painkillers such as dizziness, nausea, and constipation. Getting it in is not that bad. It's pretty quick and doesn't hurt more than getting an IV put in. The first time I had a hard time accepting the concept of a needle going into my spine - that freaked me out a bit. But once it's in they just put a little catheter in just like a PICC line, and you don't feel it at all (it's numb, that's the point). Without the epidural I don't know how I would have gotten through those first few days post-op. I highly recommend getting one. They often don't work perfectly and their placement can get dislodged pretty easily, but something is better than nothing. Otherwise you'll have to rely on oral/IV opiates and I hate those. They make me really nauseous and dizzy and woozy. I don't personally feel susceptible to opiate addiction, but that is also a risk that I'd rather minimize. If they place the epidural high enough then you won't need a urinary catheter or anything and can go to the bathroom on your own (though for the first couple days I was bed-ridden so I had to go in a commode that they brought to my bed). If they put the epidural too low, that will numb the pelvic nerves and make you incontinent. But that's not usually a problem with pleurodesis because the surgery is done up high.
This most recent time I got the epidural, once I was transported from the recovery room to my hospital room the tube must have moved around a bit and it was numbing my left side but not my right, exactly the opposite of what I needed. The pain was getting extreme, so I asked the anesthesiologist to come and fiddle with the line until it finally got to a spot where the right side was numbed a little, maybe like 30% (though the left side was 100% numb). Better than nothing! Way better! I also took ibuprofen and tylenol. Yeah, loading up on painkillers is not my favorite thing, but with pain that extreme you need everything you can just to be able to breath.
Sleep was difficult, but every day the pain diminished a little bit so that I could lower my epidural dose (the opiates in it were making me nauseous). Hey! The anesthesiologist taught me a little trick that I'd like to share. If you're feeling a wave of nausea, put an alcohol pad right under your nose and inhale that for a few minutes. It really works! I think smelling salts or essential oils would also do that trick, but alcohol pads were an easy option at the time. By Sunday the chest tube was no longer producing fluid and the epidural had stopped working altogether. So both were taken out that day, which was very liberating! Every day thereafter the pain improved, and by Tuesday I relied solely on ibuprofen. Ibuprofen works WAY better than tylenol in this situation, likely because of the extent of the inflammation (tylenol is not an anti-inflammatory, only a painkiller). But I didn't take ibuprofen for the first few days after surgery because I thought that if the point of the surgery is to cause inflammation, then why would I take an anti-inflammatory? I'm not sure it would have made much of a difference. For about a week post-op I had a low grade fever, which is normal (caused by surgical trauma). For about four days after the surgery I had a bit of edema in my feet and ankles, which is caused by laying in bed and not moving around. It improved with taking a cleavers tincture and by walking around, even just in my room or down the hall and back. When I got the chest tube out and I could walk around the hospital again, the edema went away.
The pain is worst when inhaling deeply, coughing, and any time that I take those involuntary sharp inhales that we're constantly doing and don't notice. Like when you burp, yawn, sneeze, or just need to inhale deeply once in a while for no apparent reason. You'll notice every single one of those for a few weeks. My back muscles also got all locked up and sore from tensing up as a result of the pain. That's improved a bit with better chair support and sleeping in my own bed at home.
I was desperate to get home so they let me out on Wednesday, 6 days after the surgery, with home IV antibiotics. I have been trying not to exert myself at all since getting home. I've learned my lesson! I'm just gonna sit around and write and read and watch movies for a while, and maybe work in my garden a little, but not too much. No lifting or twisting or biking, though I have been going on walks again. It still hurts to cough and inhale deeply. I really don't like taking so much tylenol, which is my only choice in terms of strong pain killers that don't mess with my head (I really hate the feeling of not being able to think clearly). But if I don't take the tylenol then I can't breath deeply and coughing is excruciating, and I have to do both those things in order to heal the lung properly. So I take my herbs alongside it to keep my liver healthy. I'll have to wait a very long time before getting back to climbing, which makes me sad.
I feel that I am healing much faster than I did last time I had the surgery four years ago. There are a lot of factors to explain that, but the two most important ones are that I am physically stronger and healthier due to climbing, good nutrition, and regular use of medicinal herbal tonics. Plus, I have been using several herbs that are specifically indicated for tissue healing.
I am/was taking herbs during my recovery for five specific reasons: to reduce inflammation (pre-op), to protect my liver and help it metabolize the intense drugs and anesthesia, to protect my kidneys from the drugs (particularly vancomycin), to speed tissue healing, and to reduce pain.
When I got the second lung collapse this time around, it was clear to me that I was experiencing a very high inflammatory load at the time. When I feel inflamed I usually get a low-grade fever, I feel very tired, and my lungs feel characteristically "inflamed". I had not been able to do chest PT or use the Vest for several weeks after my April pneumo in order to let the lung heal up on its own. This let mucus build up on the bottom of the right lung and that CT I got later showed I had a small pneumonia down there. That's why we started back up on IV antibiotics. Anyway, I felt inflamed and I know that the inflammation is a cause of my lung collapses. I take a daily tincture of baikal skullcap (Scutellaria baicalensis) normally, but I took an extra amount in those days before I got the surgery. I discuss baikal skullcap in this article. I also stopped eating inflammatory foods such as things with sugar in them and refined carbohydrates, and ate a lot more leafy greens and simple foods like broths and soups. I increased my dose of fish oil and continued to take Indeptra, which contains turmeric curcuminoids. However, I stopped taking turmeric and fish oil 24 hours before surgery because both of those things can reduce clotting and thus increase the risk of surgical bleeding. Also, after the CT scan I began drinking tulsi tea (a.k.a. holy basil, Ocimum sanctum) daily because tulsi has been studied as an effective tool for protecting healthy cells from DNA damage due to radiation exposure (DNA mutation from radiation exposure can lead to cancer, by the way). Throughout the month of May I probably had about 30 x-rays, but the CT equals A LOT more radiation than that.
To protect my liver from the drugs, particularly tylenol (acetominophen) which depletes hepatic gluathione, I took milk thistle capsules (80% standardized silymarin, about 1000-2000mg daily). Milk thistle seed stimulates the liver to produce its own endogenous glutathione, our body's most important self-made antioxidant. I started taking larger doses of this right after surgery to help my body clear the anesthesia as well. Just like turmeric, it has to be taken with fat to be absorbed.
To protect my kidneys from vancomycin I use a tincture formula that contains goldenrod leaf and flower, astragalus root, and dong quai root (Angelica sinensis). My vanco level and creatinine were getting too high, probably as a result of surgical trauma plus co-administration of high doses of ibuprofen (also filtered partially through the kidneys), so I took higher doses of this tincture than usual.
To speed tissue healing I took two herbs: solomon's seal root and comfrey root. Solomon's seal is great for tissue healing, especially in joints, muscles, tendons, and ligaments. Comfrey is good for all types of healing (one of its common names is "knit-bone"), and is preferably used topically, though can be used orally for internal tissue healing. Both herbs contain allantoin, a constituent that speeds cell proliferation. Comfrey root has the most allantoin and it is the most powerful herb for wound healing. However, comfrey has two drawbacks and needs to be used carefully. 1) it works so well at speeding tissue healing that if applied topically over a wound that is infected, it can cause the skin to grow too fast over wound, sealing in the infection, causing an abscess (essentially causing healing from the outside in, which is the opposite of what you want). So we always recommend that comfrey be used topically several days after the initial wounding, when the wound is growing granulation tissue and is no longer at risk for infection. Taken internally you don't have the same risk for sealing in infection, but you have a higher risk for the second drawback: 2) comfrey root contains pyrrolizidine alkaloids (PAs) which are toxic to the liver. If comfrey is taken internally for long enough and at high enough doses, there can be liver damage. So the solution is to take it for a short amount of time (about two weeks max at high doses), or to only use it topically. People with underlying liver issues, like CF-related liver disease, should not use comfrey internally. Solomon's seal is a better choice in that situation. I needed to take comfrey internally because you can't apply comfrey topically to a lung! Taking milk thistle alongside it made me feel better about my liver though. I took comfrey for this year's first lung collapse, which healed on its own within a week and a half (though not permanently). I think the speed at which the healing happened had something to do with how much comfrey tincture I was taking. Then, after the pleurodesis, I took comfrey tincture for about a week afterwards. Again, my speedy recovery may have something to do with it. My x-ray one week and two weeks after the surgery showed the pneumo is completely gone and the lung is re-inflated and back to normal (though the pain is not gone).
Though not an herb, another wound aid I use is honey. Honey has been used for thousands of years to help skin wounds heal faster and to kill infection. Honey is strongly antimicrobial, soothing, and anti-inflammatory to help heal skin quickly and cleanly with minimal scarring. Unlike salves or oils, honey can be applied to a wound immediately. I use medical grade manuka honey that I bought years ago, but any local REAL honey will do (but do not buy "honey" from a supermarket unless it's local and organic because some of the cheap stuff on the shelf is not honey at all, it's corn syrup). The best place to get real honey is at a farmer's market or directly from a bee keeper. The nurse told me that my hospital is now using honey in dressings, especially for drug-resistant infections. I love how some traditions are so effective that they're making a come back.
In terms of pain management with herbs, I didn't have that much luck. For chronic pain, herbs like Jamaican dogwood, cannabis, corydalis, and others can be really helpful. But this was acute pain, and it's harder to manage with herbs, especially without getting all woozy (which I hate). This is something I will have to do more experimenting with. There are some topical preps that I haven't tried yet, like lobelia oil, and datura or belladonna salve (DO NOT try these last two without an herbalist's supervision).
I wanted to write this post for those of you who are going to have this happen to you and want to know more about it. Just like I expressed in my post on hemoptysis, I believe that when we're faced with a situation that's potentially life threatening and scary, if we know as much as we can about the situation and our choices, we can feel more in control and less afraid. Meditation can also been helpful for its calming effects but also for encouraging is to take deeper breaths.
I also want to say that in the past, having a pleurodesis was a factor that could disqualify you from getting a lung transplant. That is not the case anymore. Operating on lungs without pleurodesis is easier, but it's no longer prohibitory. At least that's what my docs told me with regards to the transplant center in Boston that they refer to.
Ok, last thing. You should also know that once you have recurrent pneumos, it's a bad idea to fly in planes. The change in air pressure in the cabin as the plane ascends can cause a potentially small and unnoticeable pneumo to expand and get really serious. But if you have a habit of flying with a doc or EMT and a chest tube kit, then it's no problem. Joking. I've rationalized this in a really positive way: no one should be flying in planes anyway due to the unacceptable level of greenhouse gases and air pollution that they produce, so now I just don't have the option at all. No more temptation. Hurray! Actually, my dream is to one day take a freight ship across the sea to Ireland. That would be an adventure!