The next day I met a new doctor (Dr Marquardt) who assured me we would find out what was wrong. He would do a couple of tests and then we would do an angiogram and all of this would happen within a couple of hours. His confidence and reassurance gave me hope that a solution could be found. The nurses told me what was happening and what to expect every step of the way. This was very helpful and lessened my anxiety about being in a hospital.
The angiogram seemed to surprise the medical staff. They were pretty sure I had a blockage in my heart that was causing it to enlarge and to act unpredictably. The angiogram showed no blockage whatsoever. This was a relief on one level, but further puzzling on another. Dr Marquardt was not daunted, but even more determined to find the cause of the symptoms. “If it’s not your heart, then it must be your lungs.” he said, so he ordered a perfusion lung scan in order to get a good look at my lungs. According to the patient handbook –
a perfusion lung scan is the best screening study looking for obstructive pulmonary vascular disease … the most frequent cause being pulmonary emboli, acute or chronic.
By that afternoon he had found several blood clots in my lungs, five of which were quite large. He presumed they had traveled to my lungs from my leg – deep vein thrombosis. To make sure, he sent me off to have my legs x-rayed. Sure enough, they had. My right leg was blocked significantly. Some of the clots had migrated to my lungs from there. Within seconds I was put on anticoagulants to thin my blood to allow it to flow more easily. The thinners would also keep blood from sticking to the clots and enlarging them even further. In the meantime, my diagnosis, finally, was obstructive pulmonary vascular disease, or more specifically, CTEPH — chronic thromboembolic pulmonary hypertension. But what the heck is that? I’d never heard of it before. I looked it up once I got home and found this definition:
The blood vessels in your lungs, called pulmonary arteries, have their own pressures, similar but separate from your regular blood pressure. If this pressure is high it causes pulmonary hypertension (PH). PH makes the right side of your heart work harder. Over time, the right side of your heart becomes enlarged, making it harder to pump blood. CTEPH, or chronic thromboembolic pulmonary hypertension, is a rare form of PH. In CTEPH, a thrombus (clot-like mass) gets stuck to the lung’s blood vessel wall and blocks blood flow.
A diagnosis is just the first step in fixing the problem. This sounds self-evident but by now I was far enough along on this journey to know that to some treatment professionals the diagnosis was not just the beginning but also the ending. Once diagnosed it was, “Ok, that’s what wrong with you. Here are some pills. Good luck”. The patient handbook explains the importance of pursuing treatment:
Pulmonary hypertension, as a result of chronic thromboembolic disease (CTEPH), is potentially curable with pulmonary endarterectomy surgery. Consequently, correctly diagnosing patients with this type of pulmonary hypertension and evaluating these patients with the goal of establishing their candidacy for surgical intervention is of utmost importance. Since advanced NYHA functional class status is associated with increased mortality, early recognition is essential and referral to a specialized center familiar with the evaluation and management of chronic thromboembolic pulmonary hypertension is strongly encouraged.
I was released a day or two later. Upon my release, Dr. Marquardt told me about the UCSD hospital in San Diego that specialized in removing the clots, thrombo-endarterectomy. He told me how to look them up on the internet. Well, if you want to get grossed out, you can look this up for yourself. The videos are scary and messy. You’ll see doctors pulling out big red gooey blood clots from people’s lungs. Yikes! My first reactions were, “No thanks. I’ll get by without going through this. I will probably die a lot sooner but I’ll be spared from this gory experience.” After a few days passed I’d go back and look at the videos again. Each time I got closer to thinking, “I want these things out of me. They are hurting me”. I had flashbacks of scenes from the first Aliens movie where these awful creatures had invaded the body of Sigorney Weaver. OK. I’m in. Let’s get these things out of me. I naively thought it would be easy to move ahead with this decision, but of course it wasn’t.
I gave Dr. Marquardt the green light to send a referral to UCSD medical center where they specialized in thrombo-endarterectomy. I can’t pronounce this procedure, and I don’t know what some of the words mean in the definition below. I share it so you can try to figure it out for yourself.
A Pulmonary Thrombo-endarterectomy is a cardiothoracic surgical procedure, which removes organized thrombus from the pulmonary arteries using the techniques of median sternotomy, cardiopulmonary bypass, hypothermic arrest, cardioplegia, and special dissection techniques. The chronic thrombi are incorporated into the arterial wall, thus requiring meticulous dissection in a bloodless field.
Once Dr Marquardt completed the referral to UCSD I waited anxiously to hear from them. Weeks went by. I called and waded through their phone tree a few times and was assured that they had my records and would get back to me as soon as they could evaluate my case. When I still didn’t hear anything from them, I called more. I made sure ALL my medical records had been forwarded to them. Again, I was assured my case would be evaluated and I would be informed as to whether or not I would be accepted. Several weeks past. I assumed they had not accepted me so I asked my General Practitioner to refer me to Stanford. Stanford accepted me within a couple of weeks and over the next three months we began the long drives to Stanford for tests and consultations with doctors.
Throughout this process of chasing down medical records and begging various medical records clerks to forward my records, I learned the sad state of what I will loosely refer to as “no interoperability.” By this I mean that hospital electronic records often do not “talk” to each other. Getting one’s records transferred to other providers is not a slam dunk. It takes vigilance and patience. Finally, I got a complete set of all my medical records and carried them with me to every appointment. If a Dr. said he didn’t have my records, I’d whip out my copy and ask them to make a copy for themselves right there on the spot.
In the meantime, while driving back and forth to Stanford, I was also referred to a cardiologist and a pulmonologist in the UCD system where my General Practitioner was practicing. The cardiologist did another EKG and thumbed through my medical record. He couldn’t find some of the key elements in my chart, so I had to show him where things were. He final told me that I might as well accept this diagnosis. He said I could go to different doctors but they would all tell me the same thing. I would need to be on blood thinners the rest of my life. I was not resistant to the blood thinner suggestion, but I was holding on to the hope that the clots could be removed. This alternative seemed to be outside of his scope of practice and knowledge base as far as I could tell. He clearly had not read the patient handbook which states:
Pulmonary hypertension as a result of chronic thromboembolic disease (CTEPH) is potentially curable with pulmonary endarterectomy surgery. Consequently, correctly diagnosing patients with this type of pulmonary hypertension and evaluating these patients with the goal of establishing their candidacy for surgical intervention is of utmost importance.
The pulmonologist ordered a couple more tests and after reviewing them he told me nothing about my condition had changed. The clots were all still there. He said to come back in four months. Four months? Are you kidding me? I want to get well – not just sit around for four months. Since I was going to Stanford he may have thought I was taken care of. Not sure.
By February the doctors at Stanford agreed that the blood thinners had done as much as they could do. The clots had been there for a while and had attached themselves to the inner walls of my vessels. They were not going to shrink or be minimized in any way by the blood thinners. As you will read in the clip below from the patient handbook, my clots were now assumed to be permanent.
What is also known is that the appearance of lung perfusion scans change little beyond 8- 12 weeks even while on antithrombotic therapy, and as a result, the obstructions can be assumed to be permanent beyond that time.
The next step was to refer me to UCSD since I seemed to be a good candidate for surgery to have the clots removed. Within a week of this advice, and before Stanford could complete their refer, UCSD came across the referral that had been made by Dr. Marquardt the previous September. They do not know what happened to my referral since it never takes this long to evaluate a patient for candidacy. For some reasons, and we’ll talk more about this in the section on spirituality, my initial referral had been delayed by five months.
It was mid-February. I was napping with my dog – taking our early afternoon nap on the couch which now seemed to be a prerequisite for getting through a day. The phone rang and a cheery, non-apologetic voice from the UCSD pulmonary clinic told me that I had been accepted into the Pulmonary hypertension chronic thromboembolic disease (CTEPH) program. Furthermore, in a few days or a couple of weeks, someone would call me and give me a date for the surgery. OMG. The dog and I both leapt from the couch and then stood there looking at each other. Our lives were about to change. It took several days before the call came with the surgery date of April 24th, six weeks from the day of the call.
I went back on YouTube and watched several examples of clots being removed from lungs again. They were gruesome and frightening. Once I got over the petrification, I decided I wanted these things removed from my lungs, even though it was going to hurt and scare me and be a long recovery. I was going to go ahead with the surgery if I received the final approval from the medical team at the hospital.
The surgery was scheduled for April 24th, with the preceding week being filled with several tests that would let us know exactly what was going on in my lungs and heart. This meant I had two more months of waiting for something scary, painful, and life-threatening. I’m not good at waiting for anything. I can barely wait for the guy in front of me in the grocery store to pay for his stuff and move on. I’m even worse at the post office when the guy in front of me is telling his life story to the post man. Two more months of waiting was really going to test me on many levels. More about this is the section on spirituality.
While I wanted the clots out, I wasn’t sure I wanted to go through the process of having them removed. I was still sort of on the fence. What pushed me over the edge was a dinner date my husband and I had with our friends, Ken and Chris (both doctors). I called Ken to tell him we were going to our hotel room and would meet him and Chris in the lobby. He kept me on the phone for longer than necessary but I didn’t think much of it. We were halfway through dinner when Ken asked me about my intentions regarding the surgery. I perseverated. He and Chris looked hard at me and he said, “Lori, when I was on the phone with you, you could hardly breath. You need to go through with this.” Ken had read all my medical reports and all the info from the hospital. He is often quick to find fault with things that aren’t right. So when he said the UCSD folks know what they are doing and had their act together, I made the final decision to go ahead with the surgery.
The week before surgery finally arrived. This week was set side to do several tests to determine my fitness for surgery. Here’s how the handbook describes the events of this week.
You will have several tests to help us in our PTE evaluation. Some of the tests are routine and are ordered on every patient who is admitted to the hospital. These include a urinalysis, a chest x-ray, an electrocardiogram (EKG), and various blood studies. The tests listed below are part of your evaluation.
VENTELATION TEST. This portion of the lung scan allows us to see where the air exists in your lungs and the perfusion portion allows us to see where the blood flow exists and if pulmonary emboli are present. To perform the test, you will then be asked to inhale a small amount of radioactive gas. A special camera called a Gamma camera will take pictures of your lungs to examine the distribution of airflow. Then a small amount of radioactive solution will be injected into a vein 1n your arm. The Gamma camera will take pictures to examine the distribution of blood flow in your lungs.
ECHOCARDIOGRAM (ECHO). This test will measure heart size, detect communications between upper chambers of your heart and show the speed and direction of blood moving in your heart and in your pulmonary artery by using sound waves.
RIGHT HEART CATHERETIZATION PULMONARY ANGIOGRAPHY. You will see a lot of equipment and personnel in the catheterization room. They are needed because the procedures are complex. Cardiac catheterization will evaluate the performance of your heart. One or more narrow plastic tube (catheter) will be introduced into your heart through a vein in your neck. The catheter will be guided through the network of veins with the help of x-ray (fluoroscopy)until it reaches your heart. It will enter the right atrium, then the right ventricle, and then the pulmonary artery. Pressure recordings will be made and blood samples will be withdrawn for analysis in each area of the heart. An angiogram will also be performed. During this procedure, an iodine-containing dye will be injected into the pulmonary artery and pictures of the blood vessels will be taken in rapid succession.
lVC FILTER PLACEMENT. This filter is placed into the inferior vena cava (the main vein that returns blood from your legs). This device will potentially prevent any clots in the leg veins from reaching the lungs. You will be given a mild sedative and asked to lie on a fluoroscopy table. The filter will be inserted through a vein in your groin or your neck by a physician.
This is the only test where we hit a snag. I was prepped and the filter was ready to insert when the technician found that I had not one but two vena cava. This complicated things since they didn’t know where to put the ilter. So, we left it out.
COMPUTERIZED TOMOGRAPHY OF THE CHEST (CT CHEST SCAN). CT scanning provides a three-dimensional image of the chest. Such an image is far more capable of revealing information about the mediastinum (the tissue between the lungs which contains the heart and pulmonary arteries) and the lung tissue itself. Prior to the test, a small amount of contrast dye will be injected into a vein in your arm.
CAROTID DOPPLER. A carotid Doppler examination is a method of non-invasively evaluating blood flow through the carotid arteries, the major arteries which supply blood to the brain and which are located on both sides of the neck.
It was during this week that I learned more about my situation than I had in the past seven months. Each time a test was administered the doctors would explain everything to me. I saw x-rays of my lungs and clots for the first time. I understood what strain my heart was under as it tried to pump blood into my lungs.
By the time the surgery date arrived, I was confident that this was the best choice for me and I had confidence in the medical team. The surgeon, Dr Pretorius, and his team came into my room the night before surgery and went over everything with me again. They explained that no one had died from this surgery. I found this was very reassuring. They told me that this process was not going to be easy, but it would be worth it. They asked me if I understood what they were going to do during the surgery. “I think so. Let’s see, I think you are going to knock me out for at least 30 hours, lower my body temperature to 60 degrees, hook me us to a heart-lung machine which will keep my blood circulating and oxygen flowing. Then you will cut open my breastbone, pull the skin and bones back so you can access my lungs and heart. Then you will stop my heart and cut into my lungs where the clots are. Once you find them you will try to cut them away from the blood vessel lining where they have permanently attached themselves. You will do all of this within 30 minutes on each lung because if you go longer I could suffer brain damage. You will then sew everything back up. Do I have this right?” Yes, that was about the size of it. Do you want to go through with this? Yes.
They mentioned one more thing before leaving that night. Many patients who undergo a significant surgical procedure such as this will awaken with temporary psychosis. It will pass. This was at least a scary for me as the surgery itself. Having spent my entire career with folks who were seriously mentally ill and understanding their pain and some of my own, I wasn’t interested in having this experience. I wanted to wake right up and begin making sure all was well. I wanted to be in my right mind. So this was the last thing I had to give up to accept my healing. OK, let’s do this.
I remember being wheeled into the operating room the next morning and being met by a team of good natured professionals. They were happy and clearly liked working with each other. I felt like I was in good hands. Then it was “lights out” and what ever happened during the next 30 hours is not in my conscious memory and I am grateful for that. However, the patient handbook clearly spells out what went on during those 30 hours. Here’s a more medically accurate description of the surgery:
PTE (pulmonary thrombo-endarterectomy) surgery is the removal of old blood clots (now scar tissue) that are plugging up pulmonary arteries. lt falls into the general category of “open heart” surgery, although the surgery is not done on your heart. It is a complicated procedure because the clots in your arteries are stuck to the artery wall, thereby narrowing the center of the blood vessel. To remove the clots, the surgeon must open these arteries while no blood is flowing through them. In fact, your blood will go through the heart-lung machine (bypass pump – so named because it bypasses your heart and lungs) during the surgery. The heart-lung machine will oxygenate the blood and pump it through your body. The surgeon will enter your chest through an incision made in the sternum (breast bone). You will also be made very cold during the surgery. When your body is cold, your oxygen requirements are reduced. General anesthesia is required for PTE surgery. After you are asleep, the doctor will place an endotracheal tube (small plastic tube) through your mouth and into your trachea. This will be connected to a ventilator that will breathe for you while you are in a deep sleep. An infusion and monitoring catheter in your neck, a blood pressure monitoring line in your groin, and a catheter to drain urine will also be put into place.
Thirty hours later I had a glimmer of consciousness. I remember my husband holding my hand. At first, I thought it might be God holding my hand but then I recognized my husband’s fingers and rings. I couldn’t react or acknowledge anything. I just knew he was there. A little later the surgeon came in and ask me to wiggle my hands. I had no way of responding. I didn’t even know where my hands were. Then he said to wiggle my feet, and for some reason I did that very well.
A few hours later I regained consciousness in the intensive care unit. I had tubes coming from several places that monitored my body functions.
After the operation, you will be brought directly to the ICU. We will keep you asleep until the next morning. When you wake up, the endotracheal tube will still be in place to help you breathe. You will not be able to talk because the tube passes through your vocal cords. The ventilator will be breathing for you. You will have a small tube that goes into your stomach to empty the secretions in your stomach so you don ‘ t become nauseated. You will have several monitoring lines in place. The one in your wrist will be an arterial line. We will monitor your blood pressure with this and take blood samples to determine your oxygen level. Another monitoring line called a Swan-Ganz catheter will enter the chambers of your heart through a large vein in your neck. Through this we can measure the pressure in your pulmonary artery and the amount of blood being pumped through the heart (cardiac output). The Swan-Ganz catheter will also help us monitor fluids and pressures to maintain a stable fluid balance.
The incision in your sternum is closed by wiring the sternum together. The wires stay there permanently. Under the skin, there are sutures, which dissolve. And on the outside there are no sutures; there is a protective “bioglue” that is applied that simply peels off as your incision heals. After your surgery, when they close the sternum (breast bone), there is air and fluid that remains in the chest. Two to three tubes will need to be left in your chest to drain out the remainder of this air and fluid. They are called chest tubes and may be removed several days after your surgery.
You will also have temporary pacemaker wires coming from the top part of your incision. They are connected to a temporary pacemaker outside of your heart and are used after the surgery to regulate your heartbeat. You will have a Foley catheter (a small tube to drain urine from your bladder) so you won‘t have to use a bedpan or urinal.
Remember, all these tubes and the equipment you ‘ll see around you are temporary. Your main job in the ICU is to rest and allow us to care for you. The more relaxed you can be the better.
I couldn’t talk at first, just whisper. Food arrived and I was going to eat but I couldn’t get my hands and arms to work in a coordinated effort to get the spoon to move. So, my husband fed me a few easy to eat things. My sister and my husband took turns feeding me. My brother was there too and he told me stories. His girlfriend was there too and she brought me magazines and games. A couple of times we all walked around outside and then convened in the snack bar for treats, just like well people. I knew they were all looking out for me and helping me with judgment calls, since my judgement was not reliable. They also had an uncanny way of knowing when to let me take risks and keep moving out of the patient role. It helped a lot to have them there – to remind me who I was and that I was still me.
People had told me that the worst part of this procedure would be the pain. Yes, I had pain, but I also had some pretty good pain pills so I could control most of the pain. Coughing really hurt but everyone cheered when I coughed since it was good to cough up gunk that was trying to get out of my lungs. While the pain was challenging at times, the hardest part for me was the utter vulnerability, dependency and helplessness. This was very uncomfortable for me and motivated me to begin taking my life back. Over the next 6 or 7 days I took a little bit more of myself back as I was able to do so. I could walk around the floor, supervised at first and then on my own. Three days after the surgery, I was finally able to take a shower and wear my own pajamas. I could feed my self and order what I wanted to eat. All these things helped me regain a since of myself and helped me remember my strength and fortitude.
The staff and medical team were magnificent. They listened. They let me have my way as much as possible. They got me up and helped me walk. My family and friends came and walked with me. This walking was the part that helped me the most in terms of recovering myself from a state of dependency.
I was released from the hospital 8 days after surgery (at least six days before the shortest anticipated length of time). I have been home now for four weeks. I get a little better all the time. Some days I overdo it and pay for it the next couple of days with long naps and aches and pains. So, I am trying to find a balance between doing my best to be well and also respecting my body’s need for rest and recuperation. I miss the people at the hospital. I feel like we went through an intense and intimate time together and then I left and am isolated from anyone who even knows what happened (with the exception of my four family members). I have asked to be referred to a pulmonary rehab program so I can be with others who have similar situations.
I’m back with my original pulmonologist and he has set up tests to see how things are progressing. Today I had a “sniff test x-ray”. The test showed that my left lung still has “profound paralysis.” During the surgery my left phrenic nerve was damaged. This is the nerve that connects my brain to my diaphragm and is how my brain tells my diaphragm to breath. In 50% of people, this nerve regenerates and restores itself to full functioning. I am hoping there is a way to help heal this nerve so my diaphragm is back to normal. The pulmonologist says we will wait a year to see if this happens. He still gives me appointments four months out, but in the meantime, he sets up tests, so I think it’s all working out.
Each day I walk my dog as long as I can before getting tired. The times vary from day to day. I meditate and continue my prayer times. I am also driving short distances now, which is really a relief and release. I’m impatient about getting my energy back. I want to be well now, but I know that’s not entirely realistic. The patient handbook gives me some ideas on what to expect:
In the vast majority of patients, within several weeks to two months post-operatively, there is a dramatic improvement in their exertional dyspnea, and often abatement of 02 supplemental needs. As a rule, symptoms such as hemoptysis, exertional chest discomfort, lightheadedness, and fluid retention resolve. For most patients, the surgery is a “cure”, and they are able to return to work and recreational activities. Provided the patient adheres to chronic anticoagulation therapy, we have rarely seen a recurrence of embolic hypertension during long-term follow-up.
I am now two months past the surgery and all of the above conditions have abated, with the exception of shortness of breath. While my breathing is much better and continues to improve daily, I am still short of breath upon exertion. I am much better than I was prior to surgery, but not back to my normal breathing. I think this will continue to improve over time. I may have to wait for my diaphragm to catch up with the healing that has happened throughout my body. I can wait. This was a big thing to happen to my 73-year-old body, so I know I need to cut it some slack.
I am still on oxygen. It helps me feel much better. It is a complete pain in the butt – dragging it around, tripping over cords and tubes, having others trip over the tubes and cords, dog becoming entangled in it and having my face jerked off when I walk beyond the range of the cords. Ouch.
Approximately 70% of patients require supplemental oxygen either continuously or with exertion and sleep after leaving the hospital. Supplemental oxygen is usually required for 6-8 weeks because the pulmonary circulation needs time to re-establish itself.
I am still in the process of finding the right level of blood thinners, anticoagulant, as they call them. I go to the lab each week to have my blood tested and doses adjusted. This will become less frequent once I stabilize.
Final instructions are:
You should continue to take a walk on level ground 3-4 times daily at home using your oxygen (if it is prescribed). It is recommended that you take an anticoagulant for the rest of your life. If you are taking warfarin, your Prothrombin time/ INR needs to be checked regularly in order to prevent new blood clots from forming. Until the incision in your breast bone (sternum) heals, you should not lift anything that weighs over 5-l 0 pounds. We also recommend that you do not drive a car for at least one month. The sternum usually takes two full months to heal completely.
I have complied with all of these instructions except for the driving. I started driving at the end of the second week. I just couldn’t stand to comply with that one since I had done so well with everything else.
Finally, I am so grateful for the wonderful expertise of the doctors and staff at UCSD. They are an amazing group of people doing ground breaking work. I am so glad I had the opportunity to use their services.
