The Heart of the Matter

I am sharing this post because many of you have asked what caused me to have open heart surgery at such a young age. I also want to journal this for future reference.There is a lot of information here but I learned a lot of fascinating things during this process. I hope you find it interesting as well.

Since about the age of 20, every time I saw a new doctor I was asked if I had been tested for Marfan's Syndrome. I would answer yes but inevitably the doctor would check me for the various features associated with Marfan's anyway.

• Long arms, legs and fingers ~ Yes to all, (my arm span is longer than I am tall by an inch)
• Tall and thin body type ~ Yes, until I had children :)
• Curved spine ~ Yes, I had sever scoliosis as a child, an S in my spine
• Chest sinks in or sticks out ~ No
• Flexible joints ~ Yes, especially my fingers and hands
• Hyper extending joints ~ Yes, especially my elbows
• Flat feet ~ No
• Crowded teeth ~ No
• Stretch marks on the skin that are not related to weight gain or loss ~ No 
• High-arched palate ~ No
• Sudden lung collapse ~No
• Emphysema ~ No
• Asthma ~ No
• Sleep apnea ~ No

They would always conclude that I didn't have this syndrome because I didn't exhibit enough of the signs.

When I was newly married, I was diagnosed with mitral valve prolapse with slight regurgitation. This meant that my valve didn't close all of the way when my heart pumped blood and some blood came back through the mital valve during each heart beat. It wasn't a big deal, I just had to take antibiotics before going to the dentist and they wanted to keep an eye on it to insure it didn't get worse.

In November of 2010, I went in for a follow-up echo cardiogram to see how my valve was doing. To my surprise, my valve was no longer functioning improperly, however my aorta was enlarged. My doctor sent me to a cardiologist here in town who told us that I was tall and it was normal for me to have a large aorta because of that. My family doctor thought that was ridiculous and sent me to Oregon Health and Sciences University to meet with the cardiologists up there. The cardiologist (we will call him Dr. P.) thought it was laughable. Not only would my organs not be larger because I was tall, but my aorta wouldn't have shown an increased size in one echo when the ones over the previous five years showed it normal.

I met with the a geneticist as well as the cardiologist. Again they checked me for Marfan's Syndrome. After seeing that I only had a few of the physical attributes of Marfan's but also having one of the major components, an enlarged aorta, they decided to have a DNA test done. The results came back in 6 weeks saying I had an alteration in one copy of my  fibrillin-1 gene. It is a unique defect that has never been seen before. But because it is acting similar to the Marfan's defect, the doctors are calling it Marfan's and recommend I have my daughters tested because they each have a 50% chance of having this same genetic defect.

In March of 2011, I met with a genetic heart surgeon who referred me to a cardiothorasic surgeon whom does surgery for the exact problem I was facing. He felt my aortic root was too large and there was potential for an aortic dissection which would kill me before I could make it to the hospital. He suggested I get a cardiac MRI to get a more precise measurement of my aortic root and go from there.

In April 2011, I had the cardiac MRI. They put me into a narrow and long tube for two hours. I was told to take a deep breath and exhale, holding it for 30 seconds to one minute at a time. I had to do this every few minutes for the entire two hours. At one point they pulled me out to put dye in my IV and put me back in. It was very hard, especially since I am claustrophobic. The results were that my aorta was at 44 millimeters, up from the 42 millimeters a year before.

In November 2011, I had another echo which showed my aorta hadn't grown in 6 months and I was told to come back in another 6 months for a check up. I also took the girls in to meet with the geneticist and to get echo cardiograms. K's was fine but L's showed an enlarged aorta, 30 millimeters. The geneticist looked the girls over and said both girls had hyper extensive joints, L much more than K. We went ahead and tried to get them both tested for the same mutation I have. The insurance would only cover L's DNA testing, which came back positive for the same mutation I have. I was told to bring them back in a year for another echo.

The time for me to go back in for my checkup came and went. I kept putting off going but after a little over a year I decided to go back in again. Mainly because I had started having weird pains in my chest I had never had before. I scheduled an appointment for another echo in February 2013. The results showed my aorta had grown dangerously large but they wanted me to have a cardiac MRI to get a better image of just how large.

In March I went in for two hours of being stuck inside the MRI tube. It was even worse this time, I didn't think I was going to make it the end, but I did. The MRI showed my aorta had grown to 50 millimeters. My cardiologist said I had to meet with the cardiothorasic surgeon to discuss when to have surgery but I would have to do it definitely in the next six months, no more.

I met with Dr. S right after spring break and was told that it was serious and I must have surgery soon. He told us that if my aortic root was measuring at 60 mm they would schedule the surgery for a week out, if it was 70mm I would be in surgery that day. But since it was at 50mm I had some time to plan for it. Although he had just had a patient whose aortic root was 44mm and hers had ruptured causing her death. We talked about how long recovery would be and how it would effect day to day living with my young girls. M and I decided we would do it at the end of summer. M and I wanted to have a fun summer with the girls so that if I didn't make it through the surgery the family would be left with some good memories. We tried to do it the week before Labor Day weekend but Dr. S was going to be out of town. We decided to do it a few weeks after school had started so the girls could get into a routine and have something to keep them occupied instead of worrying about mom.

Goofing around with my favorite drink the night before my surgery.

It was a very hard six months for us. We only told our family and a few of our closest friends what was going on until a few weeks before the surgery. I didn't want to deal with the consent inquiries of how I was doing or the horror stories people always seem to come up with to tell you. I think M had a harder time than I did. At one point I became very emotional and spiritually depleted. Luckily I had my mom and close friends to help pull me out of it. M never really talked about it though.

Hanging out in the pre-op room between visits from doctors.

We didn't tell the girls until the week before I went in for surgery, we didn't want them to worry. We were pretty vague about the process and they took it well. My parents flew in to be with M and me during my surgery. M's mom came down to our house to take care of the girls. I went into OHSU at 6am on September 23rd. They took me back to a room to change into a gown and put in an IV. I was then visited by the anesthesiologist, various members of the surgical team, as well as Dr. S, my surgeon. We were told the surgery would take 7-8 hours if everything went well. They would start by making an 8in incision right below my collar bone and done my sternum. They would then use a saw to cut my sternum in half lengthwise and open my ribcage up with a rib spreader. I would be put on a heart/lung machine at some point. They then would cut out the enlarged aorta as close to my valve as they could get and replace it with some synthetic tubing which they would sew onto my aorta. Then they would look at my aortic valve to see if the leaflets were laying flat when it pumped the blood and not letting anything back through. If  there was a problem they would replace my valve with a mechanical one, if not they would put me back together. That consisted of drilling holes in my sternum an weaving wire throughout my sternum to pull it together. Then they put in three layers of stitches to close me back up. The bones would knit together around the wire after eight weeks. I will always have wire in me that will be seen in any x-ray machine, should be fun at the airport. :)

After coming out of surgery, still on breathing tube.

I went into surgery at 8:30. I remember being wheeled down the hallway towards the surgery wing but I don't remember ever making it there. The next thing I remember is waking up in a dimly lit room with a tube down my throat. I wanted it out but couldn't get it out. I didn't realize it at the time, but they had tied my arms down at my sides so I wouldn't try to pull any of the tubes out of myself in my semi-conscious state.

There was someone in the room with me and at some point they took the breathing tube out, that was around 11pm on Monday. M and my parents had been there until about 6pm when they were told I probably wouldn't wake up that night and they should go get some rest. They went back to their hotel room for the night. I was in and out of consciousness for the next day, mostly out. By Wednesday I was more coherent and able to move from the bed to the chair mostly on my own. Nurses helped me out of bed multiple times a day to help get my energy back and my lungs working better.

The doctors came in to take some wires out of my incision mid morning. Apparently it is normal for them to have wires attached to the heart coming out of the incision of the chest after surgery. They just hang there in case of a problem in which case it is easier to stimulate the heart instead of using paddles. I never had to have that done and they felt I was in the safe zone to have them removed. There were two doctors who came in and cut the few stitches attaching them to my chest and then pulled them out of my incision. It was SO painful! I am pretty sure I yelled. They also took out the huge catheter that was in my neck which they used to give me fluids and medication. I think someone said it was 3 millimeters in diameter. They also removed two of the four other IV's I had in my arms and the actual catheter I had so I didn't have to make trips to the bathroom. In the afternoon they moved me out of ICU and up to the step down unit.

In my room on the step down unit floor using my incentive spirometer.

I had my own room which was like a small hotel room. It was there that I learned the surgery went well and they only had to replace the aorta; my valve was in perfect condition. Dr. S did said it was definitely time to get it out of there; it was close to rupturing. I was told I lost a lot of blood but not quite enough to have a blood transfusion. They don't like doing that if it isn't absolutely necessary because it can cause issues during recovery. Because I had such low blood volume, I was very weak and light headed. It will take six months for my blood to get back to the proper volume.

Once I was in the step down unit I was told I needed to get up and sit in the chair regularly, go for short walks three times a day and use my incentive spirometer. That is a devise I sucked air into my lungs through to see how well I could breath. It helped to re-inflate my lungs and remove the liquid in my lungs after having been on the breathing machine. I was supposed to use it ten times an hour and I hated it. I usually only used it five times an hour but I did get up and move around a lot.

One of three bottles connected to drainage tubes in my stomach.

On Friday I had a busy day. It started out with a doctor coming in to say he was going to remove my drainage tubes. I had three 18 inch tubes coming out from under my ribcage that attached to plastic bottles. All the goo in my body drained into these bottles through the tubes. They were no longer draining which meant it was time for them come out. The doctor told me that I would feel a burning sensation for just a moment and then it would be all over. M was watching as he did it and this is what he said happened. The doctor started pulling, I started moaning/yelling and he said I'm done, I'm done even though he was still pulling the tubing out. He tried to do it in on swift motion but when he brought his arm all the way back, they weren't out yet so he had to move his hand up by my chest again and pull once more. If you think the wires being pulled out of my chest was bad, this was so much worse! There were three little incisions left but he said they would heal on their own without stitches. I don't know how but they healed before I left the hospital. So weird.

Kristan, Kristine and Kristy.

Anyway, later that day I had a lot of visitors come see me, which was so nice. Sue brought me chocolates. The 'K' girls brought me a Sunshine Basket full of goodies and cards. After everyone left I went for a walk in the hall. On the way back to my room I got light headed and started seeing spots in my vision. By the time I got to my bed I was having tunnel vision and could hardly see anything. It went away after 30 minutes but it was very scary. The nurses thought I had just over done it and told me to take it easy for a while.

Flowers from my best friend Emy, from Cali.

We had been thinking I would be able to go home on Sunday but I had a bad night Friday and Saturday I awoke weaker and unable to breath well in my incentive spirometer. On Friday I was able to breath at 2000 ml (they wanted me to be able to breath 2500 ml consistently) but by Saturday morning I could barely breath 750 ml. They did an x-ray to see how my lungs were doing but no one ever got back to us about  how it looked. On Sunday Dr. S said he wanted me to stay until Monday just to make sure I was doing better.

Pretty roses from my cousin.

I kept using my spirometer every 7 minutes like I was supposed to and taking walks five times a day. Not only did I want to go home, but I wanted them to quit giving me shots in my stomach. Several times a day they gave me shots, which kept me from developing blood clots, until I was moving around enough. But the medicine burned once it was in my belly. It went away after a minute but it wasn't pleasant. I was so happy when the nurse finally told me I was moving around enough to discontinue them.

Finally looking and feeling better.

On Sunday my girls came up to see me. It was so nice seeing them, I had missed them. I also think it was good for them to see how serious this was. My youngest is a lover but aggressive. She loves to run and jump on you when she gives hugs. I definitely couldn't have her doing that when I got home so we had to explain to her that she had to be very fragile with mommy. She understood and has been pretty gentle with me since.

My 8 inch incision from surgery along with the three holes from the tubes.

On Monday I finally got to go home. I was told I couldn't drive for 4 weeks. I couldn't lift, push, or pull with my arms or twist my torso. I also couldn't lift my arms above my head or my elbows above my shoulders for 8 weeks. After 4 weeks I could start lifting up to two pounds. After 8 weeks I could slowly start lifting more. I can never lift more than 20 pounds for the rest of my life. I can not do push-ups or lift weights. I can do modified sit-ups and I can only use 3 pound dumb bells.

I was bummed because my favorite workout is the power class at the gym. You spend an hour doing cardio and weight lifting with a barbell and weights. It involves lots of squats, lunges, bench presses, clean and press, tricep/bicep curls and push ups. It's the one class I lost weight quickly after having L and kept off the weight. I tried to argue with the surgeon but he said that I am still at risk for developing an aneurysm in my descending and abdominal aorta. I didn't even know you had those. After thinking about it, I have decided my health is more important so I will have to find some other way to stay fit and exercise.

Pretty flowers from my sweet Rachel.

I have to have yearly echos to make sure I don't develop any more aneurysms and that my valve doesn't stop working correctly. Looks like L and I will have many years of going to OHSU together. On the bright side, K was finally approved to have her DNA testing to see if she has this defect. I am hoping she doesn't, I don't think we can afford all of us going to get echos every year, especially with this new healthcare plan Obama's got us all on.

From our closest friends, the V's. And no, Randy didn't steal them from a funeral, I asked:)

My dad stayed a couple more days after I went home and my mom stayed with me for three weeks. It was nice to have her here to take care of my girls and me. The first few weeks home were much harder than I expected them to be. You don't realize how much energy children have until you are very sick and they are running all over the place. It was exhausting for me and I slept a lot. It was also hard for me to be home, seeing all the things that needed to be done and not being able to do them. My mom was here to do them but I hate asking for help and it bugged me that I wasn't able to do anything. I felt lazy and useless. Now it is frustrating because I am feeling much better but still I am unable to do much. You don't realize how much you use your arms until you are told you can't. Most days I feel like I understand how the T-Rex must have felt with his itty bitty arms.

About a week after I got home from the hospital my mom took me to the grocery store. I rode around in one of those electric cart things. I felt kind of stupid at times because I looked perfectly fine but had no energy. My dad said I should have worn a sign that said "I know I look fine, but I just had my heart hacked out!" And just so you know, those things drive pretty slow and aren't as cool as one might think. Although it might be fun to have a race with someone sometime, just saying.

I want to thank all our friends and family that brought us food, flowers, took our girls to and from school, and those few of you who took our girls for play dates so I could get some rest. We really appreciate all you have done for us. I should also give a shout out to the amazing nurses and doctors at OHSU. Everyone was so friendly and helpful. I had one nurse, Randy for the majority of the time I was their, and he was awesome! Funny, helpful, intuitive and always willing to explain things to us. I really can't say enough good things about that hospital.

I know it has been a while since I updated our blog but I plan on posting our fun-filled summer vacation over the next week. I just wanted to get the story of Getting My Heart Hacked Out written down while it was still fresh in our minds. Hope it was as informative for you as it was for us.