Pregnant and in heart failure part one

I've had a few people ask how being pregnant with EA is. This, for me, is a long, complicated, emotional story, so I'm breaking this post into parts. Part one gives a little background and the first part of my pregnancy. This is a dark post. If you bear with me to the end of this series of posts it gets better, I promise!  This isn't typical, even for someone with Ebstein's. It certainly never occurred to me that this is how my pregnancy would play out.

Going back a few years - 12 to be exact - before I got pregnant. I had my first heart surgery. They had to replace my tricuspid valve, and it was decided that a mechanical valve was my best option. I was put onto warfarin. A month after this (a new record, I was told) I got a blood clot. Long story short, they managed to get most of it dissolved and decided that my INR levels should be higher than the recommended for someone with a mechanical heart valve (3.5-4 for the first two years and then they figured I'd be okay at 3-3.5). At some point in the future I may go into this story more, but for now that's the important part.

After my first surgery I was in amazing shape. I actually went to the gym five days a week - and enjoyed it. Then I got into university, met my husband, and life moved on. I had all my yearly check ups, and they were all great. I got a job teaching high school. The first year I taught, I got pneumonia for the first time in my life. I'll admit, I whined. A lot. I took a whole day off work (which was really unusual for me to do). Then life went on. The next year I got pneumonia again. Just the one time.  I knew other teachers who had the same thing happen - teaching is stressful. You work 12 hour days on average, plus you get stuck a small room with germ factories who, even at 15, can't remember to cover their mouths when they sneeze. In high school you get four or five different groups of kids. That's about 120 chances every day to catch something. To be honest, I was amazed I wasn't sicker. The following year (2012) rolls around. Between January and June I got pneumonia not once, but FOUR times. The last time I should have been hospitalized, but my doctor took five days to look at my X-rays, so by the time he realized, I was already over the worst of it.  That August I went and got myself the pneumonia shot. Problem solved, I thought.

At this point, my husband and I had been married for two years and together for 9. We decided we wanted a baby. My cardiologist okayed this, as did my family doctor. We started trying in August 2012, and I tested positive on Sept. 7 - my husbands birthday. 

The happiest my pee ever made me!

That day I went to my doctor to change blood thinners. I had been taking warfarin, but it causes birth defects. He hadn't had any pregnant patients on blood thinners before, but luckily I always ask a million questions and knew that I had to be on heparin. He looked up the dosage, wrote me a prescription, and sent me home. I also had to make an appointment with my cardiologist, which was for Sept. 25.

That September there was a heat wave, and my classroom was in a portable. This means that it averaged about 32 degrees Celsius. This, along with morning sickness, made me feel awful. I actually had students asking me if I was ok (and the occasional one asking if I was hung over - I looked pretty bad I guess). I took to lecturing leaning against a table in the front because I was dizzy and hot and generally feeling awful. 

Then I passed out. Or rather, blacked out after getting out of the shower and walked face first into my bathroom door so hard I knocked myself out (it was not one of my finer moments). I managed to get the bathroom door open and yell to my husband that I was ok. It took a lot of effort. So I was understandably furious when he asked me why I was telling him this. Apparently he hadn't heard anything over the volume of his video game.

My doctor said that some women do pass out when pregnant. It has something to do with a rapid change in blood pressure and/or blood sugar, and not to worry. So I didn't. Until it happened again when I was away for the weekend. Only this time I turned blue. And I stayed pretty blue. When I got back I went to see my doctor again. Again, he told me not to worry. This time I was worried though. I asked him to run tests. I got him to listen to my heart to make sure my valve was still ticking (a sure sign that a mechanical valve is still working). So he listened, sent me for blood work and an ECG, and told me that more than likely I was dehydrated and had low blood pressure. Drink more water and eat food high in salt and I'd be fine.

When I went for the test, the lab tech took a look at my ECG and told me she was flagging my results and if I hadn't heard from my doctor in 45 minutes to call him. When I called I got an incredibly rude receptionist who said that yes, the doctor had seen the results and I was fine. At this point I couldn't do anything without turning blue. The twenty feet from my living room to bathroom was the longest twenty feet ever. And I was exhausted. I was pretty sure I wasn't okay. Luckily the next day I had an appointment with my cardiologist.

After three hours of tests I was told that I had a blood clot on my valve. Clearly this is not good news at the best of times, let alone when you're six weeks pregnant. They admitted me immediately, and started me on IV heparin, hoping to break up the clot. It was the best chance for my baby to survive. 

After four days or so I started to feel better. Everyone was optimistic. They decided to send me for a TEE, where they put an ultrasound camera down my throat to get a good look at my heart. If this went well I could go home. 

It didn't go well. The test revealed that I had either a large clot or tissue growth over my valve. I had two options: clot busting drugs or open heart surgery. And I needed to decide ASAP. My valve was stuck closed. This is far more dangerous than if it was stuck open. My cardiologist came in to discuss options with me. Either way, she explained, I would likely lose the baby. The clot busters may or may not work. If they didn't work and I had to have the surgery, there was no doubt I'd lose the baby. If I lost the baby because of the clot busters, they would have to wait up to six weeks to operate. I likely wouldn't last six weeks. She called her mentor in Toronto, who called his or her colleges around the world. There was only one case that was even remotely similar. No one knew what to advise me to do. So it was up to me.

Let's take a step back for a moment. This is pretty bleak. People were telling me that I needed to make a decision now. Everyone is focused on me, and trying to keep me alive. My baby was already considered a loss. My baby, who I knew was a girl, who I knew I was going to call Elliott, was being written off. This was not okay. Everyone, including my family, kept saying I could have another, that it was so early in it didn't really matter. Looking back, my family was concerned I was going to give up. But at the time, I felt betrayed. I could have another, but I wanted this one. It was not their intention, but I have never felt more alone than I did in those few days.

In the end, after weighing all my options, I decided to have surgery. It was scary, but I felt it gave my baby the best chance. They fit me in as quickly as they could. I decided on Wednesday morning, and they scheduled my surgery for Thursday morning. Wednesday night the hospital ended up performing a heart transplant so I was bumped. They didn't know when they could get me in. Eventually they decided they would bring a team in and operate on Saturday October 6 - thanksgiving weekend. My surgeon told me in our pre op meeting that he had never had a pregnant woman keep her baby after this surgery - ever. 

After my surgery I was taken into the CICU (cardiac intensive care unit). I was still unconscious when they sent in someone from maternity to check on the baby. My husband, mother and sister were all there, gathered around a tiny, portable ultrasound machine. No one had any hope they would see a heart beat. Yet there was one, going strong. I heard about it later from the nurses - not telling me, but talking amongst each other. Everyone was shocked. My family was thrilled, especially when I woke up, because she was the first thing I asked about. My baby had survived so far.

What's in a name?

There has been a lot of discussion around labels and how helpful they are, and the CHD community is not exempt. I have always been aware that I have a label, and it has affected my life.

As I said earlier, I was diagnosed at birth in 1983. Many doctors today are still rather uninformed about Ebstein's Anomaly, and I can only imagine what it was like then. I hear horror stories about doctors recommending termination of a pregnancy if the baby has EA, and new mothers are still on occasion told that their babies probably won't make it into adulthood, based purely on the EA diagnosis. Although this can be true, the medical literature that I've seen indicates that until the baby is born they can't actually determine exactly how severe it is. When I was pregnant I spent hours researching, as I was told that I had a slightly higher chance of my baby having EA. I had a number of people - including friends - who were shocked when I said that I wouldn't terminate my pregnancy if my baby had EA, which I found offensive on so many levels. It is a very personal decision, but I feel that in my case it would be the wrong one. I felt as though they were telling me that my life wasn't worth living - after all, I have the same defect. It was frustrating to explain that I was researching so I was prepared if my baby did have EA. Not so I could make a tough decision. But I digress.

I was really lucky with my label. I needed it for certain aspects of my life, and not just the medical side. It helped me understand why I couldn't keep up with the other kids in school sometimes. It also helped me see doctors and get test results ASAP. But the cardiologist who labelled me also gave my mother very clear instructions - I would know my own limits.  Let me decide when enough is enough. This is where the real issue with labels comes in, in my opinion. 

In telling my mother that I would know my own limits, my cardiologist ensured I would live a normal life. Looking back I'm not sure how my mother dealt so calmly with everything. We lived in rural Nova Scotia until I was 10 - about a half hour away from the hospital in good weather.  Never once was I told I couldn't do something because of my EA (although I have a sneaking suspicion that I couldn't take swimming lessons because of it and not because it was in the ocean like my mother always said). I played outside for hours on end. I ran as much as I could. When we moved to BC, I played badminton, swam, rollerbladed, and did all the other things that kids do. When I got tired, I'd stop. I always knew my limit, and thanks to my cardiologist, I was the one who got to decide what was enough.  My mother does readily admit that it was hard. A few days ago she told me that when I was playing outside there was always someone actively watching me - just in case. This came as a surprise to me. I never felt as though they were watching me. Never once did I feel as though I was being either monitored or restrained in anything (reasonable) I was doing. Because of this, I got to live a normal life, and feel like a normal kid for the most part.

I've never been one to sit around and have pity parties. I've never really babied myself in physical activities (granted, I have on the rare occasion during gym class pled heart problem to get out of something I really hated). I knew I was labeled, but I tend to think of my EA the same way I think of my height: it is what it is; at times inconvient, and occasionally limiting what I can do (especially when I was younger and wanted to go on the fun rides at the fair), but generally I don't wander around thinking about it.

The problem with labels is all too often people use the label as an excuse to become what is expected of them. Labels become limiting. It wasn't until I needed my first surgery I let my label limit me - and really, it wasn't the label that limited me, but my body.

So yes, I feel labels are important. It helps other people understand you or your child in many cases. People with EA can live a relatively normal life, in most cases. But the times when they can't - when they can't keep up with others, when they are stuck having heart surgery rather than enjoying life, when they have to take medications that most people don't deal with until their later years - this is when you need the label. But a label is not an excuse to not live a full life. It is not an excuse to live in a bubble and take no risks. A label is an explaination. It is not what makes a person who he or she is.

 I am not a label, or a statistic. I am a wife, mother, sister and friend who also happens to be the 1 in 200,000 who has EA. I love reading and knitting, but I also love camping, boating, hiking and swimming. So go ahead and label me. Just remember that although I have EA, I am so much more than my label.

Becky with the heart thing

Growing up I was always the kid with the heart thing. Gym teachers feared me. Kids thought I was weird. I didn't understand what all the fuss was about.

I was born with a congenital heart defect (CHD)called Ebstein's Anomaly. The long and short of it is one of my heart valves was misplaced and didn't meet up when I was born, resulting in a heart murmur, monitoring and eventually surgery. One in 200,000 people or so is born with this. I've been told it's the third rarest heart defect in the world. Seeing this now I can see why I was known as Becky with the heart thing. But it's only recently I've understood what all the fuss is about.

See, overall I'm a fairly normal person (unless you ask my family - they will laugh rather hysterically and point out that I'm weird in so many ways. Not because of my heart, but just because I'm me).  Unless I'm in imminent need of surgery, you can't tell I have anything wrong with me. I don't look like your classic heart patient. I'm not blue. I'm usually full of energy, unless my daughter has pulled an all nighter like she likes to do on occasion. I guzzle coffee like there's no tomorrow. I'm not over or underweight. If you put me in a line up you wouldn't pick me out as a heart patient, let alone one who has had open heart surgery twice.

I'm hoping that by sharing my experiences and insights I can spread a little awareness that 1) people with CHDs are more often than not regular people and 2) no matter how big and scary life can get at times, things will get better. I may sneak in a few other bits of some of my passions too - knitting, babies, humour, reading and probably whatever is striking my fancy when I'm writing,

Just so we're clear, I may be Becky with the heart thing, but I am not what most people think of as someone with a heart problem.