Tuesday, June 9, 2015

My Introduction

When it comes to primary spontaneous pneumothoraces, I do not fit the textbook profile. According to the Society of American Gastrointestinal and Endoscopic Surgeons, PSP is up to 10 times more common in males than in females, with an annual incidence of about 20/100000 patients per year. Risk factors are tall stature and low body mass index, and most often occur during adolescence or soon after. Smoking is also a risk factor. I, on the other hand, am a small 5'2", 120ish pounds female. My first pneumothorax occurred the week before I turned 35. I have never smoked. I have always been relatively healthy, citing an excellent diet and a busy lifestyle that I like to pass off as exercise. I live Salt Lake City, Utah -- a beautiful place, but one with horrific air quality -- and after owning a successful floral design firm for 10 years, I am now a stay-at-home mom to three kids ages 2-11. As far as lung issues in my family history go, I had a younger sister who suffered from Cystic Fibrosis and passed away at age 12, and a grandmother who battled lung cancer. But that's it. I've only known one other person whose lung collapsed spontaneously, which has made this journey a rather lonely one. Its easy to find medical information about the hows and whys of PSP, but despite the abundance of patient message boards floating around on the internet, I have struggled to find specific, detailed, first-person accounts to help me know what to expect as my condition progressed. One can only provide so much information in a paragraph, and so everything I did read blurred together with redundancy and left me with little comfort.

As I laid in bed three weeks post-op expressing this vein of frustration, my husband suggested I relate my story in the detail I myself wished to read, in the hope that I might fill someone else's void. And so what follows are the chronicles of my own journey with chronic PSP.

The Beginning

September 2014. We live a mile away from my youngest son's elementary school. On Thursday afternoon, my 2 year old and I set out with the stroller to walk to a group of 6 neighborhood kids home from school. Half way there, I started to feel an ache in my upper back, at the bottom of my left shoulder blade. It had been a busy day of errands and constantly getting a toddler in and out of an SUV in hot weather, so I figured I had pulled something earlier in the day and was just now feeling it. Not one to be deterred by something like a pulled muscle, I continued on to school. By the time I pushed my daughter's mammoth jogging stroller up to the elementary school's main doors (requiring a climb up a small hill, of course), the ache had increased substantially. The entire left side of my chest and back was in pretty serious pain, from the bottom of my rib cage, radiating up my shoulder and neck, to the bottom of my jaw. I stood in the shade waiting for the kids, trying to stretch out those muscles, thinking this must be what it feels like to throw out your back. In doing so, I realized that the shortness of breath I figured was a result of pushing a stroller in the summer heat had not subsided, but was getting worse. By the time all the kids were out, I was also experiencing dizziness, nausea, heart palpitations, and try as I might, I could not seem to regulate my breathing. I didn't want the kids to worry, though, so we set off for home. It was all I could do not to pass out. It felt like someone was sitting on my chest and I couldn't take a deep breath no matter how hard I tried, but we walked the entire mile back to our neighborhood. When I got home, I laid down on the couch, but that seem to cause the pain to intensify, so much so that I couldn't get my body back into a sitting position.

Now I have to interject here and let the reader know that I am tough. Like, REALLY tough. My tolerance for pain is quite astounding. I've given birth with failed epidurals and little more than a wince and an "ouch." For my kids to see me in so much pain was concerning, and my oldest son called my husband, Tyler, who promptly came home. After quickly Googling my symptoms, he wanted me to at the very least go to InstaCare (because Google said I was having a heart attack), but I refused and asked him to get me some ibuprofen and let me rest for a bit. A couple hours of rest later, I got up and pulled together the dessert I had been assigned to take to a baby shower that evening. I decided against attending the baby shower, but I did go about my evening as normal -- helping with homework, putting kids to bed, watching TV with my husband, etc. I promised Tyler I'd make a doctor's appointment in the morning if I didn't feel better when I woke up. I didn't sleep at all that night because I just couldn't get comfortable and the pain seemed to get worse the moment I laid down. By morning, not only was I still having chest pain and shortness of breath, but I could feel something -- literally feel something -- when I bent over. My husband could feel it, too, simply by placing his hand on my back. It felt like a muscle or something was catching on itself or my ribs when I bent up and  down. This was especially puzzling, so I reluctantly made a doctor's appointment.

I got the kids off to school and Tyler went to work, then I dropped my daughter at the sitter's and drove myself to the doctor. They looked at me a little cross-eyed but performed an EKG to make sure I hadn't had a heart attack. It showed I was tachycardic, but no heart attack. The doctor came in and asked a few questions. I was so short of breath I had a hard time talking, but I'm a pretty perky patient so I was joking and smiling though the whole appointment. The doctor said she was concerned I might have a blood clot in my lung and suggested I head to the ER. So I got back in the car and took myself to the hospital. I had a full day of mom-stuff scheduled. Both of my boys had half-days at school and I was supposed to be watching someone else's kid that afternoon, so I called Tyler and asked if he could take care of all that for me as I would likely be a few hours in the ER. My doctor had called ahead, so upon arrival to the ER, they rushed me right in -- prime-time television style -- and in a flurry of activity, did another EKG, took a bunch of blood and gave me a chest x-ray. After a good, long wait, the ER physician eventually came in and said, "Well, you don't have a blood clot. However, the x-ray shows that you have a significant pneumothorax." "A what?" "Your left lung is collapsed by at least 50%." I figured that was better than being on blood thinners for the next 10 years, so I was relieved. He explained that while unusual, lungs can collapse spontaneously, and marveled at the fact I had waited almost 24 hours to bring myself to the ER. Apparently most people with that size of a collapse would have called 9-1-1. He gave me the run down: I would need to have a chest tube inserted in order to get the air out and allow my lung to re-inflate. "How long will that take?" I asked, "Because I have a babysitter scheduled for tomorrow and dinner reservations to celebrate my birthday." He laughed and told me that most people require a hospital stay anywhere from 3 days to a week in order for the pneumo to be resolved. I was pretty peeved.

Two delightful guys from radiology came and got me. In between the jokes and chit chat, they explained that having a chest tube placed is a torturous process -- way worse than child birth. It has to be done while the patient is awake, strapped to a table, underneath an x-ray, and while they do their best to numb the insertion site, that does nothing to help the pain that occurs inside the body. I asked for (and was given) morphine before they began. One of the guys held my hand and both of them kept talking to me as the radiologist was doing the procedure: An incision is made between the ribs and the tube is pushed into the chest until it rests along the lung. The chest tube is connected to a plastic box called a pleurovac, which collects and measures any fluid that drains from the lungs. The tube is also connected to suction to help drain the fluid and get the air out so the lung can re-inflate and any assumed hole in the lung can heal itself. While it definitely didn't feel good, I was able to keep my composure, most likely thanks to the morphine. One of the guys marveled, "You didn't even flinch!" He probably said that to everyone, though.

When it was over and they were getting ready to take me back to the ER while I waited to be admitted, they asked, "Do you have someone here waiting that we can go update?" "No, I'm here by myself." "You brought yourself to the ER with a pneumothorax?" They got a kick out of that. I eventually made it to my room and had to call home to get supplies for my unanticipated stay. While I was alone (because my poor husband was trying to find someone to take our kids so he could visit his poor wife), I quickly became somewhat of a celebrity among the nursing staff. -- not only because I'm young and so healthy and don't fit the profile of a spontaneous pneumothorax, but because of the circumstance surrounding it. I told my story at least 10 times during my stay.

They started off by giving me hydrocodone for pain relief. Yeah, right. There was a tube inside my chest. Every movement I made was painful. And the worst part about having such a high tolerance for pain is the fact that I consequently do not respond to pain medications. I require the heavy stuff to make a dent. Luckily, nurses stepped in and provided me with morphine so that my muscles could relax and I could sleep as much as possible, because even sleeping was painful. I've read other accounts where patients were allowed self-administered morphine...lucky dogs.

I was given a chest x-ray every 4 hours or so to check on my progress. Luckily, I am a ridiculous over-achiever, so 24 hours after being admitted, my chest x-rays showed I was improving at a fast enough rate not only to go home, but to have my chest tube removed before I was discharged. When it was time, I was told to sit on the edge of the bed, exhale, and then inhale, hold my breath, and bear down, at which point the guy yanked the tube out. I can't even describe that feeling -- chest tubes don't slide out, they are literally yanked followed by resistance. I swear I saw stars when it happened, but boy did it feel good to have that thing out of my chest! They put a water seal dressing on it and sent me on my way with no other follow-up instructions that basic wound care. The doctor said there could not have been a better, more textbook outcome in any way. My nurse said that in 12 years, she'd never had a patient with my diagnosis go home sooner than 3 days.

I still had about a 10% collapse when they sent me home, so I was told to take it easy for at least a week. When I got home, I went straight to bed and slept for 18 delicious hours. It felt wonderful. I had some residual pain from the chest tube that didn't last incredibly long, though the incision site was too tender for me to wear a bra for a couple weeks. I felt like it took a solid three weeks before I was no longer easily winded and could go about my day without any issues.

As we were leaving the hospital, the doctor warned us that once someone had one spontaneous pneumothorax, the chance of recurrence was very high, especially within the first year. For a short while after returning home, I worried about that. Should I change my lifestyle? Was I a ticking time bomb? It didn't take too long for reality to take the wheel, though, and I plunged head-first back into my chaotic life. A month later, I had a tiny little scar that was hardly noticeable, and I was functioning at 100%.

Three more make Four

For a few months, everything was great. Because I was never referred to a pulmonologist or even my Primary Care Physician for follow-up, I continued on with my life as if that was just an odd bump in the road. It was a great topic of conversation at dinner parties, but overall, I felt fine. We got through the Christmas season and greeted the New Year with the expectation that 2015 would be better for us.

A week into January, I was sitting in church when I felt almost a pop inside my chest. It was tiny, but I felt something. This was quickly followed by familiar chest pain (once you've experienced one collapse, you know what it feels like without a doubt). I looked at my husband frantically and mouthed to him that I thought my lung had collapsed again. We went home and I immediately went to bed to rest. Not only am I tough, but I am also stubborn, and while I am usually not the type to self-diagnose, I am super sensitive about seeking medical care when I think it could be unnecessary (and I'm painfully cheap and ER visits are expensive, so there's that). As I was evaluating my symptoms, I determined that because my shortness of breath was minimal, it must be a rather small collapse. My internet searches all told me that small collapses are usually treated with observation only, so I decided to wait it out on my own. I spent the following week resting and the pneumothorax eventually resolved on its own.

We figured that was the recurrence the doctor had mentioned and maybe I was in the clear, until a month later -- boom -- another small one. And the month after that? Yet another small one. At this point, I was frustrated. I didn't have a week out of every month to lay in bed. I didn't see them as life threatening (because I hadn't been told otherwise) but these pneumos were quickly affecting my quality of life. And yet each time, I assured myself that that had to be the last one. Having no specialist to turn to, I simply didn't know what to do.

Monday, June 8, 2015

The Frustrating Fifth

On April 24, I woke up with pressure behind my left shoulder blade. I rolled my eyes and got out of bed so I could get my kids off to school. An hour later as I was blow-drying my hair, I noticed the pain was increasing and my breathing was becoming labored. It was more intense than my previous three, but not nearly as painful as my first collapse. A nagging feeling told me I should call someone to come watch my toddler so I could go to the hospital, but I kept pushing it aside and went on with my day. That evening, I let my husband know that we may want to think about making another trip to the ER, but I never made time for it. The next morning I felt quite a bit better. Tyler still wanted to take me in, but I continued to drag my feet. I have a lovely friend who is a physician, and I finally decided I'd ask her opinion. If she told me to go the hospital, I would. This is the text I sent her:

"Need your advice.
Since my pneumothorax in September, I've had 3 other occasions where I've felt pain that presented itself identical to my first pneumo but with significantly less intensity and almost no compromise in my breathing aside from having some difficulty taking deep breaths. In each of those situations, a few days to a week's rest was enough to get me back to normal. Yesterday I woke up with chest pain. Didn't say anything to Tyler about it and hoped it would subside. It was pretty intense all morning--steady pressure on my left side, some SOB, dizziness, pain when I breathed deeply, pain radiating up my shoulder and neck, and the odd sensation that I could feel something bubbling or rubbing at a specific spot in my chest when I bent over (this happened the first time, too). I told myself the smart thing to do would be to go to the ER, but I'm stubborn and stupid and didn't. The pain eventually subsided, though the pressure remained, and I kept a low profile for the rest of the day. Had a hard time falling asleep, but when I woke up this morning, I felt markedly better. If I'm resting (and not laying down), I'm pretty much fine except for a dull ache in my back at the base of my left shoulder blade and some pressure in the front under my left breast. I don't feel like my breathing is all that compromised. Just a little resistance when I breathe deeply. I'm fatigued but not dizzy. I can still feel the strange bubbling when I bend over, but even that is not as pronounced as yesterday. I do not feel like my life is in jeopardy. (In fact, I just did the dishes and changed a diaper) I hate the thought of going to the ER when I'm not "that bad," but am I being irresponsible by not? I highly doubt my current condition warrants a chest tube, but I obviously am not a doctor, nor do I have the chest X-rays to prove that. If I feel like I have measurably improved since yesterday and am not getting worse, do you think I'm okay to wait it out, or should I bite the bullet and go? "
She called immediately. She let me know the "bubbling" I was feeling was subcutaneous air in my chest cavity. She was concerned about the frequency of the collapses and pointed out that I couldn't really know how severe the collapse was because my body could be learning to adapt to side-effects of the collapses. She acknowledged my desire to not overuse medical resources, but told me she was going to contact the on-call pulmonologist at our University Hospital to see what he said. When she called back, she told me she had explained my history to him and that he recommended I go to the ER for a chest x-ray. In their opinion, five pneumothoraces definitely warranted some follow-up care, most likely surgical, and I needed to have a paper history in order to receive that referral. My friend said she was calling the ER to let them know I was coming and then heading over to pick up my kids so Tyler could drive me to the hospital.
An hour later the ER physician came in and let us know that the x-ray showed another spontaneous pneumothorax. He said it likely had been larger the day before (and that I should never wait to seek medical care for something like that because it is potentially life threatening, etc., etc.). At the time of the x-ray, it was not large enough to require a tube, rather I'd be sent home with oxygen therapy and more rest. They paged the cardio-thoracic surgeon on-call, and I was instructed to make an appointment to see him that Wednesday to discuss options.
The oxygen machine arrived that night, and from then on, I was stuck at home with a 50-foot plastic leash.

Meeting the Surgeon

A few days later, Tyler and I met with Dr. David Affleck, cardiothoracic surgeon at St. Mark's Hospital. His nurse practitioner was excited to be meeting with someone so young -- I guess their average patient is quite a bit older. My first impression of the surgeon was mixed. He seemed a little abrupt, perhaps a little cocky, and his answers to my questions were a little too cut and dry. Tyler liked him, though. Thought his confidence was reassuring. He explained that the culprit behind these collapses were likely something called blebs -- blisters of air on the surface of my lungs. I may have been born with them, they may have grown at some point in time, but when they pop, air is released into my chest cavity which creates pressure and causes my lung to collapse. The fear is that as this continues happening and my lung becomes weaker, one of these spontaneous pneumothoraces could quickly turn into a tension pneumothorax which is very dangerous and often quickly lethal. His suggestion was to perform a surgery called a bleb resection where he would go in and remove all the blebs he could find on that lung and any compromised tissue and then staple everything shut. After that, he would do a procedure called chemical pleurodesis. He would spray medical grade talc all over the inside of my chest cavity. This would essentially burn the inside of my chest, causing significant inflammation. As it healed, my lung would scab to and eventually adhere to my chest wall so that if my lung were to collapse again, it could not do so catastrophically. He said they would do this via VATS: video-assisted thoracic surgery, but that I needed to be prepared that it could potentially require a thoracotomy. He warned me that it would be extremely painful but that I should be back on my feet in a couple weeks. I had done a little research prior to our appointment and read other patients complain about chronic or long-term pain, but he quickly dismissed this as extremely rare.

They wanted to do the surgery the following day. I dug my heels in and said "no way." Not only would preparing for such a surgery take a little time -- I had three kids to worry about here -- but I had a massive school fundraiser that I had devoted a significant amount of time to, and I was determined to see it through. Two weeks. I would have the surgery on May 13.

I was nervous after leaving the office. I was putting the fate of my respiratory system in the hands of a guy I had just met and didn't even particularly like. I wondered if I should get a second opinion, but at 35, its not like I have a lot of friends that have the name of a good cardiothoracic surgeon in their phone contact list. I happened to have an appointment with my OB/GYN that week -- a very well connected doctor whom I trust implicitly. When I asked her about Dr. Affleck, she gave a glowing recommendation, saying she had known him since she was an intern and that he was a brilliant surgeon and she wouldn't hesitate to let him operate on her. I decided to take her word for it and stick with him.

The following week was the school fundraiser. I had to try to squeeze surgery prep into any spare moment. It was such a chaotic time that by the Friday before my surgery, I was sick with a head cold, terrified that the illness would derail my plans. My sister was flying in from Denver to watch my kids, so I simply could not move the procedure date. My husband sent me to bed to rest all weekend.


My pre-ops were scheduled for Tuesday the 12th. I was registered, received my hospital bracelets, had blood tests, an EKG, and a chest x-ray. I met with the Admit Nurse who provided me with my surgical instructions, the anti-bacterial soap I'd need to wash with, and the paperwork and brochures they're required to send home. She also told me what to expect of my surgery day and encouraged me to warn my husband so he would be prepared as well. She said the surgery itself would take approximately 2 hours. I would not be sent to recovery, rather I would be taken immediately from surgery to the ICU where I would be for the entire day and overnight. While in the ICU, I would be intubated and restrained (in case I woke up and started pulling out tubes) and would remain asleep until they were able to successfully wean me off the ventilator. My husband would be allowed in the room for 30 minutes every two hours, but no one else could visit until I was moved. When I was stable enough, I would be moved to the heart/lung unit and could expect to stay there until at least Sunday, but likely longer.

I left the hospital with bracelets and a masked smile. In reality, I was terrified. No one said anything about intubation or restraints! The surgeon had mentioned the ICU, but only as a matter or getting more individualized care. I had to keep both hands on the steering wheel driving home to keep them from shaking. I spent the rest of the day with a lump in my throat until I picked up my sister at the airport that evening.

Surgery Day

We showed up at the hospital right on time. 9:00 AM. I was taken to the room we were to wait in and given a gown to change into. My room shared a bathroom with the adjoining room and I could hear patients coming and going while I continued to wait. Someone finally let me know there was an open heart surgery before me that was taking much longer than expected and to sit tight. My surgeon came in around noon, quickly signed my left upper chest, and let us know I'd be skipping the ICU and he'd be sending me to recovery instead. He didn't bother to explain why, but at that point, I was already being wheeled to the surgical staging area where I received my IV, etc. Not much time passed before I had to say goodbye to my husband and I was wheeled into the surgical room. Luckily, my surgical nurses were very friendly and put me at ease. I was given a spinal block, hooked up to oxygen, and then told I was going sleep.

When you're waking up from anesthesia, its not a gradual thing -- you snap awake. You're not "with it" by any means, but you're awake. At the point I woke up, I could immediately feel that I was struggling to breathe. Every breath I took was accompanied by searing pain, so each rapid inhale came out as a squeak. I don't know if my eyes were closed or if the intensity of the pain was so severe that I couldn't focus on anything, but I could hear people scurrying around me. A woman's voice exclaimed, "She can't breathe. She's in so much pain she's crying. We need to get her something fast." I remember thinking, "Oh, that poor girl! I wonder why she's crying? I hope they can help her!" not realizing they were talking about me. I was able to focus on the face of a kind woman, a nurse, who leaned in and said, "We're getting you something to help with the pain right now, and I'm also going to give you something so you don't remember any of this." And from that point on, I'm blank. I don't remember being transferred to my room, or even getting settled in. I have to rely on my husband's memories for that.

Tyler says the surgeon met with him after the surgery and told him that he didn't find an impressive amount of blebs (he had expected a full-blown cauliflower of blisters to be covering my lung), but he did find a lot of scar tissue that had to be removed in addition to the blebs present. He said he sprayed every possible surface with the talc and that my lung would be "rock solid" from that point on.

Once I started coming out of my drug-induced haze, I remember feeling the nagging presence of the chest tube, as well as the foley catheter that wouldn't be removed until the following day. In addition to the chest tube that was hooked up to suction, I had two incisions on my back that had been cauterized and were tender, and I was on oxygen. Things hurt, but the spinal block was doing its job and the pain was "manageable" for a time. By the time the night nurse came in, though, I was ready for morphine. She was hesitant to give it to me because my blood pressure is naturally so low, but the oxycodone they were giving me was doing nothing for the pain. I knew at the very least, I needed morphine so my muscles could relax. Every breath hurt, and unless I could relax, I would never be able to sleep. Once she relented, I drifted off and had a very restless sleep.