Sorry we have not given a super lengthy update lately. We just arrived in Arizona yesterday! Rachel got out of the hospital on Sunday and has been doing really well since getting out. She still has some fluids from the surgery in her heart and around her chest cavity, but since she obviously doesn’t have the chest tubes in anymore she is on some medication to keep flushing those out. She is still having trouble turning her head left and right (due to surgery), but that has been improving the past couple days. She has been eating lots of solid foods over the last few days. The solid foods appetite isn't quite back to where is used to be and the doctors say it probably won't be for about 4-6 weeks or so. But since Rachel has super human powers, she will be back to normal in a week. Anyway, once out of the hospital she has been trying to walk around a lot more. Yesterday was a long day for her. We drove a lot and then had a 3.5 hour flight to Phoenix. A lot of sitting for her. The biggest problem is that her legs are sore from the first procedure on the 19th (and a little from a wire in her leg from the surgery), so it is difficult to sit upright for very long. All those catheters (seven to be exact) really did a number on her legs. So just some soreness if her legs are bent too much. But that's what the recline features are for in cars and airplanes right? So quick story. We got to the airline to board and of course we had a wheelchair for Rachel. When we got to the gate, there were 11 wheelchair people. Yes, 11. Not to be confused with 12 or 10. So since she is so pretty and sweet, she was, in fact, boarded first. Some of the old people weren't too happy about it. Suckas! So when Rachel got on the plane the flight attendant just loved Rachel and her braided pig tails. They were so nice and gave us extra snacks and on the plane. At one point in the flight, Rachel needed to use the extremely spacious bathroom. The flight attendant stopped her and put toilet paper down on the seat for Rachel and told her she would guard the door for Rachel in case she needed help. Then she gave Rachel her drink with a lid and straw. Super awesome people! Rachel was fine. When it came time to get off the plane, again all the older people weren't very happy they all had to wait to get off the plane (there were so many wheelchair people that the rest of the plane got off first), but when it came their turn, Rachel was the second person off. The flight attendant even started barking orders at these older people trying to get off all at once and said,"Everyone will have to wait, this young lady just had open heart surgery and I'm going to get her off first. When she is off, then I will get to the rest of you." Haha it was awesome! Of course Rachel was super embarrassed, but I thought it was cool. Anywho, she is doing much better and has been walking more. She is especially enjoying sleeping in her Mom’s TempurPedic bed that has all the reclining features (Rachel’s dream bed), so it’s just like the hospital bed but huge and much more comfortable! She will start using Facebook again soon, but she has still been having a hard time focusing with all the drugs, so she still doesn’t like getting on the computer. All in all, she's doing much better and we love to see her improve! Thanks for all your prayers and help!  Pictures to come.

9:37 PM she was moved out of the ICU into normal care or whatever it's called? She is now able to sleep without being woken up every hour to have blood taken for blood sugar count or an x-ray, etc. They also took the 6 inch tube that was in one of her veins in her neck out. It's true. I was there it was pretty cool. Rachel said it felt like a fishing line. It was stitched to her neck to hold the it up because of the four tubes going into the one weighed so much. Anyway, she was given one ambien and two tylenol and is now sound asleep. She looks so much more normal and healthy now. The three tubes still draining her chest cavity are still in but they should come out tomorrow. And then she should feel so much better. All she could eat for dinner was some sips of cream of chicken soup, strawberry ice cream and some sips of milk. Other than that she was so tired sitting up in her chair. She has some nausea on and off today, but she was mostly super tired.

She got out about 2-3 hours ago and is in the Cardiac ICU for at least 24 hours. She keeps waking up with a super concerned look on her face. Poor Rach. They may try taking her breathing tube out soon. She looks great with her braided pig tails!

Just met with the surgeon. They repaired her valve! He said that her (tricuspid )valve looked ablsolutely perfect to repair, so it was repaired! There was nothing heroic done to be able to repair the valve, it just looked perfect to repair. So on a severity level of leakage from 0-4 her valve was leaking about a 4-5. Now that it has been repaired leakage levels are down to a 0-1. They said they also found a tiny hole (apparently most people have them but is more pronounced in Ebsteins patients) in the heart from one chamber to the next that they patched up so it no longer leaks now. They also made the right ventricle and atrium smaller (I call it a heart tuck; like a tummy tuck) sense they have been enlarged over the years because of back flow. So no replacing of the valve was even attempted. He said that by repairing her valve like they did, that it would last way longer and better than any new valve could do for her. Woo Woo! Thanks for the prayers everyone! Now another couple hours to finish up and bring her to the Cardiac ICU.

10:19 - Rachel is on the bypass machine and they have begun the heart surgery!

9:38 is the official start time of the surgery. This is when the first incision was made. They will now start hooking here up to the heart and lung bypass machine.

Ok so my last post apparently didn't stick. But they just took her to the prep room (that was about 8:30). She will spend about an hour in there and then off to the surgery room. They say she should be done around 1:30PM. They gave her the equivilant to Valium before rolling her off, so she was all smiles and giggly before leaving. They will bring us a new update afternoon a little bit. Thanks for the prayers!

Just got to the hospital and we are now in pre-op. She is all gowned up and ready to go. Lisa, I'll definitely keep posting.

It's me, Rachel! Eric and my Mom made a Facebook group to quickly update people who wanted to be notified about how things are going, and it was getting a bit redundant for Eric to keep coming over here to post some updates as well. But I know a couple people who follow our blog aren't on Facebook, so I wanted to put a few updates from the last couple days. Eric's updates are nice because they are just the quick to-the-point essentials, but I'm going to write in more detail about some things that occurred over the past few days especially for members of our family who had more questions. 

If you aren't in the Facebook group and would like to be, just leave a comment here and Eric will add you. He wasn't sure the other day when he was going through my Facebook account who all in my friends list would want to be added, so don't be offended if you weren't :)  Lastly, if you are visiting for the first time and have no idea what the heck is gong on and why I am having surgery, you can do a bit of light reading on a post I put up a little while ago here. Alright, so it's really not light reading at all, so you may need to skip down through much of that post to get to the nitty gritty. I was going to put up a straight forward and brief outline with the main facts about my conditions and what the surgery is for, but I that didn't happen yet, so for now you get the long-ish version :) 

I already mentioned this in the FB group, but for those who do not check that I also wanted to mention this here - Before I was able to start doing more things in the hospital the other day, my Mom and Eric read me some comments our friends and family have left, and I just wanted to express how appreciative I really am of everyone's kindness and thoughtfulness. I am incredibly thankful and blessed to have so many people praying for me, and I know that the power of prayer is real. Thank you all so much! I am very overwhelmed with gratitude. 

Ok, here's the rundown of the last few days:

 

Eric and I flew into Minnesota on January 17th and met my Mom at the airport who was flying from Arizona. She has of course acclimated to Arizona weather over the past 6 years and is freezing here. The other morning it was about -24 with the wind chill - my Mom was so cold! It's a good thing she kept all of her Colorado outfits from long ago. 

January 18th - pre surgery appointments

We started appointments at the hospital early the next morning on the 18th. I had some tests and also met with my cardiologist again along with some other doctors in training with her. She went over the importance again of why we need to act now with this surgery, and I am really glad she went over that again with my Mom there. It is much easier to understand things when the doctor explains them rather than me trying to explain with my limited knowledge compared to the docs. She re-emphasized the importance of operating on my heart now to capture what ventricular function I still have to lead to a much better recovery and activity level for the rest of my life. She said there are several patients who refuse to have surgery since they are not experiencing very many symptoms (if you read my other post, symptoms do not always correlate with level of severity for Ebstein's Anomaly strangely enough), and then they waited too long and lost a lot of their heart function before surgery. Then after surgery they were not able to be nearly as active as they could have been and they probably won't do very well the remainder of their life. My Ebstein's is at the highest severity level, so she wants to make sure we get things taken care of before it starts declining any more.  

Right now my tricuspid valve is regurgitating at least 40% or more of the blood pumping through and it is working at least 50% harder than the left side of my heart, so it is important not to let that get worse before taking action. If we wait much longer to perform the surgery to repair or replace my valve, my heart may expand too much to the point of losing its ability to recoil, and a significant amount of ventricular function will be lost. Even surgery at that point will not be able to help turn things around much. If you read my previous post, you'll remember that the right side of my heart is now 4 times larger than the left side, so at the rate things are going, it will be hard to reverse much in a surgery if time keeps ticking without operating.  

My cardiologist also specializes in women with congenital heart defects and pregnancy, which is super awesome in my case for what we want in the future. She again let us know that depending on how well my heart functions after the surgery, pregnancy should still be possible. It may take at least 6-12 months before we really can tell how well my heart will be functioning before we can determine if pregnancy is safe, but she is very optomistic with things since we are capturing fairly reasonable ventricular function right now. A pregnancy would still be considered high risk and I would need to be followed very closely, and (I hope this is not too personal to share) she predicts that if my heart is functioning well after surgery that I may be able to have 1-2 children safely, although if my heart is functioning incredibly well after that I may be able to have a third. It is so difficult to predict how things will go because every Ebstein's patient is completely different so she says we obviously won't know until those times come. She said that in general, most patients in their studies were only able to have 1 or 2, but again it's so up in the air. If we aren't able to have any or as many as we feel we would like in our family, of course adoption is always an option! 

I then got a Holter monitor on, and then we met with the electrophysiologist doctor who was going to perform the EP Study and ablation the next day. We really like him. I have had most of my accessory pathways that cause arrhythmias and tachycardias taken care of in my previous ablations. I have always had some occasional palpitations and weird beats since those, but they are nothing close to the severity level that they were before my last ablation. However, over the past year and a half or so, I have noticed an increase in palpitations and also have have noticed more strange heart rhythms during exercise. The doctor gave an example from studies that fibrillation will never happen in a mouse but always happens in elephants - the larger the heart, the more likely you will have rhythm problems. As my heart has continued to increase in size over the years since my last ablation, the rhythm problems have been increasing again as well. He wanted to find out if they were flutters associated just with my Ebstein's problem or if I also had some accessory pathways come back from my Wolff Parkinson White Syndrome I was born with that were already ablated. 

January 19th - EP (Electrophysiology) Study & Ablation

The next day (the 19th) we went to the hospital at 5:30 am to report for the EP study (SO TIRED). What he was going to do was map out my heart with catheters placed in veins in my legs and one in my neck (the mapping is the "study" - if any accessory pathways are found that need to be burned, that would be the "ablation" part). The catheters have electrodes on the tips that can conduct electricity to stimulate arrhythmias so they can find any accessory pathways present that need to be ablated. Ablated is just another word for burned. Only one of the catheters has a special probe on the end that will burn, or ablate. The burning is meant to create scar tissue. Electrical signals in your heart cannot move through scar tissue, so the scar tissue is what helps prevent arrhythmias where any accessory pathways are located. 

Here are a few pics before being wheeled back to the op room: 

During the EP study, the doctor did find one of the main accessory pathways that I was born with that looks like it has started to come back since my last ablation. He ablated it, and he explained to us afterwards that it was a really good thing we found that before my valve surgery since the pathway was right in the location they will be working on my valve. If we didn't ablate the pathway before then it would have been extremely difficult to move around the valve later to ablate it. He also found some other areas in my heart with fibrillation, but they are in locations that will be easier for the surgeon to take care of during my surgery on Monday by doing what is called a Maze procedure. He said the accessory pathway he found actually conducted pretty strongly, so he was able to find it pretty quickly. 

I got out of the procedure that afternoon, and when I woke up I had to lay with my legs pretty flat for a couple hours to prevent blood clotting (which happened after my last ablation due to bleeding not stopping after the catheters were removed leading to an abdominal surgery, so they were being extra careful this time!). I was able to sit up and move my legs a little after a couple hours, and then after doing that for about an hour I was finally able to get up and take a short walk. The sites in my legs and neck were really sore and tender, especially in my neck. They said that they had to stretch my head quite a bit to the side for a couple hours during the procedure, so that combined with having a catheter down my vein there made it pretty sore. I have quite the awesome bruises on my legs too! I iced my neck most of the night in the hospital to help the tenderness there, because I could barely move my head around. 

January 19th - last bit in the hospital and final pre-surgery appointments

The next morning in the hospital I was able to start walking a lot better. The doctors met with us late morning and explained some things a little more. One of the fellows of the doctor who performed the ablation was telling us that after reading the reports from my previous two ablations how amazing it was that in my first one they were able to catch so quickly that a catheter had punctured a hole in my heart. She mentioned if that happens and they don't catch it within exactly 10-20 minutes and operate asap, there isn't any chance that you will live. We already knew how serious the situation was when it happened, but I never really had it emphasized (to me at least) how the doctor really was quite skilled in being able to notice so quickly that it had been done so that my life was able to be saved with the open heart surgery right after. The size of that perforation was about the size of the tip of a sharp pencil, so I was very blessed to have it found and be sent to surgery so quickly to repair it. 

I was able to be released from the hospital in the afternoon, and then we went to meet with the surgeon who will be doing the surgery on Monday along with his assistants. We kept hearing from my nurses at the hospital how lucky I am to have this surgeon. We knew he was the top in the field for this type of operation, but it is still extra comforting to keep hearing such amazing things about him from people. The surgeon also also told us how lucky we were to have the electrophysiologist we had who performed the ablation and that he is one of the top doctors in the nation for that type of procedure too. He put it this way: "he could do that whole ablation blind folded...literally." We just keep feeling so blessed that we have access to such incredible medical care!

Getting released from the hospital! 

The surgeon explained what he'll be doing in the surgery. He said since I am still interested in planning for a family that it changes their approach a little bit. He wants to do everything possible to make sure my valve will be able to function well enough to last through a pregnancy. He said that my valve is misplaced quite a lot and it is more likely that we will need to completely replace it, although he will still try repairing it and determine in the surgery if it looks like it will hold up for very long. I was born with some of the valve leaflets being tethered to the walls of my heart which of course prevents them from closing, in addition to the whole valve being misplaced very low in my heart. Even if a repair is possible, it may not be strong enough to last through a pregnancy, but a replacement will very likely be strong enough for that. If a replacement is needed, a tissue valve will be used (mechanical ones are not an option for me because they do not last as long and also you have to be on blood thinners for life, which then would prevent any pregnancy possibility). They use a pig valve tissue, but it is not live tissue which means that you don't have to worry about yoru body rejecting it. The "shelf life" of a replaced valve varies in everyone, but he said typically for my age bracket, I can expect about 8-12 years before needing a new one. He did say that they are working on new techniques with newer technology that may be much more developed in 10 years or so by the time I may need a new one so that I possibly may not need complete open heart surgeries each time. It will be interesting to see! Aren't all these technological advances miraculous? 

Here's a real model of the valve they would use if I need it replaced - the white stuff around it is cloth so they can sew it all in!

In the surgery he will also be operating on some other areas of my heart to try and shrink the size of my heart a little. It won't ever go back down to the size of an average person's heart, but he can try making it a little smaller. After he explained how he is going to do that, Eric said it is like I am getting a tummy tuck but it is a heart tuck instead :) 

Most of the heart stuff is at St. Mary's Hospital, which is just a block or two away from the main Mayo Clinic campus (it's still a Mayo facility). Here's a shot of the crisp winter scene outside of St. Mary's: 

 

Overall I am feeling much better the past couple days. I even went to Sacrament meeting today! I am still pretty tender in my neck and legs, so I kind of walk like a gimp, but I am doing pretty great! I was just instructed to avoid germs like the plague before surgery, so I have been taking it easy. We haven't ever experienced a normal recovery from an ablation, so it's nice to see what that's like for a couple days at least before the surgery tomorrow! My Dad also flew into town on Friday night, so it is nice to have him here now as well. 

Thanks again to everyone for your support and concern. I was kind of embarrassed at first to have so many people outside of our family know everything about what's going on, but I also have truly felt the power of all your love and prayers, and I know that I need that despite my initial shyness about people knowing. So thank you very much for your prayers, and I hope that I might be able to bless your life in some way as well throughout my life. 

So they just came in for Rach to take her to the surgical room/EP study room. She looked so pretty in her little hospital gown. They will come out with the first update around 10:30AM CDT. So for now Jerolyn and I are eating breakfast down in the cafeteria. Bananas in oatmeal for me... Oh and a small chocolate milk. Mostly because Jerolyn and I brought some not so healthy snacks like chocolate covered Ning cherries, salt and vinegar chips, white cheddar popcorn, oatmeal chocolate chip cookies.... So eating a little healthier today is probably best.