Friday, February 27, 2015

Mechanical Valve Issues

Please excuse the big ugly head IV. It may not look like it, but Evanna is looking/doing soo much better.

Evanna's transfer back to the Stollery Hospital here in Edmonton went very well. They just kept her very sedated, paralyzed, and bagged her the entire way.

Nick and I arrived around 9pm and they were rounding on Evanna when we walked in. They continued to keep her on a heparin infusion, and played around with the epinephrine, norepinephrine and milrinone levels (drugs that help the heart pump). Dr. Guerra stated that she had been paralyzed for the transfer, so he was surprised when I told him that Evanna had in fact been paralyzed for the past 48 hours. The goal was to keep Evanna as stable as possible during the night, but Dr. Rebeyka was aware that if things took a drastic change during the night, he would come in to do something about it. Nick and I left around 10:30pm and checked in at the Days Inn hotel. We're currently on the waiting list to get back into the Ronald McDonald House, but we're not sure how long that'll take.

This morning we found out that Evanna had a great night. They've restarted Evanna on dexmedetomidine and midazolam, as well as up her hydromorphine (these are all types of sedation medications). Though Evanna is on a LOT of sedative medications - she still wakes up, no longer has to be paralyzed and is much more content. She even smiled at Nick this morning, and it was just so good to get a glimpse of our baby girl.

They did initially book Evanna for another bronchoscopy, but a few things have come up, and those plans are now on hold. They took her to the cath lab for a fluoroscopy, and it revealed that BOTH leaflets of her mechanical valve are not moving/working. Originally we thought it was just one leaflet not working, but now we know it's both. They're stuck open, so blood is able to get through, but it's still a scary situation.
Dr. Rebeyka, the intentivists, and Kid Clot (hematology) decided that they would really like to avoid a THIRD open heart surgery. Instead there's a drug called TPA, and it's essentially a "clot buster", HOWEVER, there are also so major risks involved with this drug (brain bleeds, etc), so my nerves are at an all time high at the moment. They just began running it, and it will take about 6 hrs to run its course. Tomorrow they will take her back to the cath lab and do another fluoroscopy to see if the TPA worked. If it doesn't, then they may have to do another open heart surgery to replace the mechanical valve.

So why did the mechanical valve stop working? Well it appears that here heparin levels dropped significantly in Calgary and weren't within therapeutic range anymore. This caused a clot to form on the valve and the leaflets to stop moving. Thank goodness they stopped in a more open position, allowing blood to still go through the valve, but it's still been a scary few days.

Though it kind of sucks that Evanna had to be retransferred back up to Edmonton, Nick and I feel so much more comfortable here, and can relax a little knowing she's in good hands.
Thanks for continuing to follow along,

xo The Irvine Family

Thursday, February 26, 2015

STARS Transfer to Edmonton

(25.02.2015 - 7pm)

|| UPDATE SINCE BLOG POST || So Evanna's condition has continued to worsen over the last few hours. They're starting her on Epinephrine, putting her on nitric oxide and doing another ECHO. Even with her on 100% oxygen, her sats keep dropping below 70. Due to all of this, they're also putting another line in her so they have more access. I absolutely hate that we're not in Edmonton for safety reasons and I'm incredibly scared.

(02.25.2015 at 11:30pm)

Evanna has somewhat stabled out at the moment, but we're nowhere close to being out of the woods. Two members of our ward have dropped by this evening to give Evanna a blessing, and I know everyone is praying really hard for her. I'm finally heading home to get some sleep, and I'm beyond exhausted. In the meantime I've received many comments, messages and texts - please don't feel offended if I don't get back to you tonight, but know we're grateful for the support.
I'll update everyone on Evanna's progress tomorrow. Though I started to put laundry away last night, I am NOT unpacking my Edmonton suitcase just yet.

(02.26.2015 at 11am)

|| EVANNA UPDATE || So Evanna is doing slightly better, but she's still incredibly sick.  She's gotten bacterial infections in the past, but this completely different (she normally responds very well to antibiotics and tweaks on the ventilator).They're best guess is some sort of chest/lung viral infection.
They've been able to wean her epinephrine and oxygen down, and they're wondering if she'll respond to nitric oxide so they can wean her oxygen even more. They still have to keep her pretty sedated and paralyze her at times to help improve her blood gases, and words like "Edmonton transfer" and "ECMO" are being thrown around. For anyone who knows what ECMO is, knows how serious and critical the situation is, and we're definitely hoping to avoid that. The other problem we're running into, is due to Evanna's unique heart anatomy, Calgary can't hook her up to ECMO, and she has to be somewhat stable for Edmonton to do it either (they have to go through her chest, and you can't do that if she were to code and your doing chest compressions). Now, we're not at that point yet, and ECMO is only used as a last resort, but I'm trying to explain the gravity of the situation.

We're having a multidisciplinary meeting at 3pm, and so hopefully we'll know more of where we want to go from here after the meeting. Last night at midnight, I contacted our primary intentivist Dr. Cave in Edmonton, and he too has talked to Calgary and is aware of situation.
Thanks for the continued prayers and messages of support.


This morning during an ECHO, they had trouble viewing Evanna's valve, so they began to think she's had some sort of clot/blockage. They sent Evanna for a CT and fluoroscopy at 2pm and called the Stollery PCICU to arrange a transfer back up to Edmonton. The CT and fluoroscopy and did indeed confirm a blockage - but it is NEW, as an ECHO from a few days ago showed that the valve was working fine. They did a test, and it showed that her heparin levels were no longer within therapeutic range and were very low. They decided that not only did Evanna needed to be transferred back up to Edmonton, but that she needed to go by STARS, as regular air transport involves the airport, and Evanna needs to go directly to the Stollery.
At 4pm STARS arrived and they began to pack her up and give a report to the physician flying with her. The STARS Paramedic that is on this transfer also happens to be a coworker of Nick's, and he took our information and promised to keep us updated.

As of right now, Evanna is on her way back to Edmonton via helicopter (heavily sedated and paralyzed), and Nick and I are packing up and hitting the road to meet her at the Stollery Children's Hospital. The past 24 hours have been crazy, as they were only talking about the possibility of transferring her back to Edmonton, to now her going by helicopter. We feel a little relieved that she's going back to the Stollery - where they know her the best and have the resources to fix things.

xo The Irvine Family

Wednesday, February 25, 2015

PCICU Day 136 - 143

Photo of Evanna taken on Monday (23.02.2015) before she got really sick.
So Evanna's transfer went incredibly well, and she slept the entire way. It's been a crazy few days, and it just goes to show how quickly things change around here. We initially didn't think Evanna would be transferred until later this week, but a bed became available, so Calgary wanted Evanna transferred as soon as possible.

Evanna had a pretty good week last week, but it was clear that a medium paced wean off of the ventilator was not going to happen. So last Wednesday the intentivists met together, and it was decided that a very slow wean in Calgary was what was best for Evanna and our family. Nick and I were slightly disappointed, as we really love the PCICU at the Stollery, and were in no rush to transfer Evanna back to Calgary - especially because she was still intubated and we're in limbo between trying to extubate Evanna one last time and a trach. Nick will also joke that he now has to put up with me more often now that I'm back in Calgary - oh true love at it's finest!

We told the intentivists that while we'll go along with their plan to transfer Evanna back to Calgary, we want the transfer to be less painful then last time, and to transfer her any day BUT Saturday. I was in a wedding party for my friend's wedding back in Calgary, and it would really cause a huge inconvenience if they were to transfer Evanna that day. I understand the whole bed situation, and you take a bed when you get one, but these plans were made last year, and it if it meant that we had to pass up on a bed - so be it.

On Friday morning, Nick' mom came up to Edmonton so we could get ready to leave, however, Evanna was going through some withdrawals that morning (a combination of a decrease in her sedation medication, and the fact that they had to turn off her hydromorphine for 2 hours to give her another transfusion), so she wasn't very happy and we were worried about leaving her. But by the time Grandma Sandy arrived, Evanna started to come around and wasn't as agitated. We were suppose to leave around 2pm, but the Calgary transfer team was bringing a kid up to the Stollery, so Dr. Ryerson asked if we could stick around to have a mini meeting with everyone. Nick and I left immediately after we talked with Dr. Gilad (the Calgary intentivist), and drove back to Calgary. That evening, our ward was having a couple's Valentine's activity, and because I haven't seen some of my friends from church in a very long time, I asked Nick if we could go. It was a good evening filled with lots of fun and hilarious games, as well as hugs and well wishes for our family.

Saturday morning finally arrived, and it started really early. All the bridesmaids got ready at my mom's house, and we left at 9am for the Calgary Temple. I'm so glad that Nick and I got to attend the sealing, and it was definitely something I needed. Bev looked absolutely stunning, and she was just glowing. It was pretty much the first time that I had met Andrew (her fiancĂ©), and it was so sweet to see how emotional and happy he was to be there and get married. After we left for pictures, and boy - was that wind COLD! 
After the sealing, Nick and I had received a text from his mom stating that Calgary had a bed, and wanted to transfer Evanna that day. Thankfully Edmonton was able to hold them off for a day, and the PCICU asked that we give them a call back when we had a free moment. So while we headed to High River for the reception, I called the PCICU and was able to talk to Dr. Ryerson. She told us the plan, and though it was a tad inconvenient for me, a Sunday transfer was in the works. 
The reception was beautiful, and it was so great to see everyone. I didn't stay in too long once the dancing started, but Bev understood my situation and was fine that I ducked out a little early.

At 6am on Sunday morning, I hit the road back to Edmonton so I could be there for the transfer, and pack our stuff up at the Ronald McDonald House. Nick had to work that morning, but it ended up working to our benefit. Because Nick works in the area of the children's hospital, he ended up taking the Calgary transfer team to the airport, and was hoping to be the unit that would pick her up when Evanna arrived back in Calgary. The transfer teams got to the Stollery around 1pm and began to pack Evanna up. It always amazes me at how long it takes to do that, but they're obviously making sure that she is as safe as can be before she's put on a plane. Once the transfer team left the hospital with Evanna, Sandy and I rushed back to the Ronald McDonald to pack the rest of my belongings into my car, and do a quick cleanup of our room. By the time I had left city limits I had learned that Evanna was already 30 minutes away from touching down in Calgary, and Nick wasn't going to be able to pick Evanna up as he was held up at another call. However, one of Nick's friends and old partner Tia was assigned to pick Evanna up and she messaged me that she was on her way to the airport. It was definitely nice knowing that someone we knew was going to pick Evanna up and be there with her. I was informed that the transfer went very well, and Evanna slept the entire way. Nick was actually able to meet the transfer team at the hospital, and was able to walk with them into the PICU. At one point the charge nurse asked "Who's the overly concerned Paramedic?", too which someone responded that he was Evanna's dad. I guess it looked really confusing because he was in uniform and came in with the transfer team, but was stroking her head and holding her hand lol. 
Sandy and I arrived about an hour later and once things truly settled down I left and headed home to go to bed (7 hours on the road can take a toll in yeah).

Monday started out okay, but Evanna started to take a turn for the worse. She spiked a temperature, so they ran cultures and started her one some antibiotics. Thank goodness one of my favourite Calgary nurses was assigned to Evanna, and kept on top of everything. By the time I left that night, Evanna was doing very well and I was happy with how things were going.

When I came in on Tuesday, I came into a much different situation. Evanna was fighting the ventilator (meaning she wouldn't takes the breaths the machine was giving her), and no matter what they did, they couldn't calm her and fix her. She was puzzling all of the intentivists, and the only thing that would fix it was to paralyze Evanna. They decided to do an emergency CT to see if that would give them any answers. Unfortunately the CT didn't show them anything, and they were kind of at a loss as to why she was acting the way she was. They brought in Infectious Disease and they suggested a stronger antibiotic. Later that night they switched her over to NAVA like I had asked during rounds that morning, and she improved slightly. They also had to take out the arterial line from her right arm because it was starting to blanch and cause issues, however, they were unable to get another one in her that night and decided to wait until this morning to try again. It was an extremely long day and so once shift change happened I left the hospital and headed home.

Today has continued to be rough. Evanna has had infections before, but this one is a doozy. Nothing is coming back on the labs and it's been difficult to keep her oxygenation up. They did get a new arterial line in her left foot, but even on 90% oxygen (that's a lot!) she's been difficult to keep stable. They paralyzed her again around 4:30pm and she's been bagged and suctioned multiple times with nothing coming up (usually they'll get lots of secretions when they suction when she's sick). They are throwing the book at her in regards to try and bring her around and are at a loss to explain why she's acting the way she is. Due to the fact that we have to keep her very sedated, she can't be on NAVA (she has to be breathing on her own to trigger the machine), so she's currently just on the regular ventilator.

Moving forward we are hoping that whatever she is fighting will subside. All the disciplinaries (ENT, Respirology, Cardiology, PICU, etc) are all meeting tomorrow to discuss a plan (long term), and Nick and I are discussing what we would like to do (trach or try another slow wean and extubation). Until Evanna is over whatever it is she has, we won't make any concrete plans, and she will for sure be having another bronchoscopy (a request made by Dr. Rebeyka) either later this week or early next week.

I know I keep asking this over and over again, but please keep Evanna in your thoughts and prayers and that a solution will present itself. A decision to proceed with a tracheostomy weighs heavily on Nick and I, and we are just scared to make the wrong decision. Do we just trach so we can work towards bringing Evanna home, or do we try another extubation in hopes that Evanna will succeed and avoid a trach? Surgically and anatomically, Evanna should technically extubate, but no surgery can fix muscle and lung weakness, and only time NOT intubated can fix that.

xo The Irvine Family

Visitors! We (well I) absolutely love visitors, so if you are at all interested in coming to see Evanna and I (Nick isn't here as often), please feel free to do so. Before you do, here are a few tips: 
1. There are no set visiting hours. The PICU is a 24hr unit, so as long as I'm here (which I usually am from 9am-9pm, give or take), you are able to visit.
2. Message me first! Either by text or Facebook, let me know when you would like to come by. I try to space people out, so not everyone is coming at the same time, and make sure I have nothing planned (like errands) while you're here.
3. Feeling sick? Have a cold or runny nose? Then please stay away! Being in the hospital already exposes Evanna to her fair share of bugs, and the last thing she needs is another one. Also, I'd appreciate you be up-to-date on your vaccinations. I'm not talking about flu shots - just your good ol' fashion child vaccinations (in case you're wondering where we stand on the whole vaccination debate - now you know).
4.  Please know that visitors are the highlight of my day, so try your best to come when you say your going to come UNLESS you're not feeling well (see #3). We're going through a very emotional and exhausting journey. I've opened up and been very public about our situation, and invite you to come visit, hang out and see for yourself what we go through everyday. Though I don't take it personally when people cancel, it doesn't necessarily help either and can unfortunately add to my stress level. Again, I LOVE visitors and having someone here to talk to during the day. It makes my day and lightens the mood, but cancelling plans to come visit is just another thing I'd rather not deal with.

Sunday, February 22, 2015

Surprise! Calgary Transfer

This is going to be a very small post, but just quickly updating everyone to say that Evanna is currently being transferred back to Calgary right now. While Nick and I were at the wedding yesterday (in which I was a bridesmaid), we were notified that Calgary had a PICU bed, and they were going to go pick her up. Thankfully Edmonton was able to hold them off for a day, and they were able to come today instead. So on very little sleep, I drove back up to Edmonton this morning at 6am to help pack things up at the Ronald McDonald House and be there for when they transferred her.

As I post this (part of it was written while they were packing her up), Evanna is currently being flown back to Calgary, and is probably already halfway there. I'm racing back to Calgary to meet them, and overall it's been a really long couple of days. I'll do a bigger blog post/update tomorrow, as I just haven't had time to do a more detailed one right now with everything that's going on.

xo The Irvine Family

Monday, February 16, 2015

PCICU Day 130 - 134

It's been a relatively good week, and the intentivists are happy with Evanna's progress. She's still intubated, but they're basically conditioning her so she'll hopefully extubate successfully.

On Thursday they took out her two chest drainage tube's (the JP's for all my medical friends), and completely turned off the epinephrine (it's an adrenaline drug that helps your heart pump). They have continued to come down on her sedation, and she is almost back on the same levels she was on going into surgery. Evanna did get another fever, so they started her on some antibiotics again HOWEVER, they've been able to leave her ventilator settings alone. Usually when Evanna gets sick, they have to increase her ventilator settings and go up on everything, but they've still been able to make some decreases - small ones, but they're still decreases!

This morning they took out her pacing wires as they've never had to use them, so there was no point to keeping them in (these were put in during surgery and were there just as a precaution). They also took out her central line today, as she's not on that many IV medications anymore, and still have IV access through her PICC line. Yesterday Evanna was on just on a PEEP of 7 and pressure support of 6. They turned the rate completely off, however last night with both her fever and very little ventilator support, she started to get very tired (and her lactate started to climb), so they increased her ventilator settings to give her a break. This was completely fine with me, as the whole point of conditioning is to just do periods of decreasing, and then let her have a break (just like going to the gym!).

There is also something I wanted to share with everyone that is new to us, and will effect Evanna and her care, even after we bring her home. From my last post, I mentioned that Evanna had a mechanical valve placed in her conduit. At the time, I really had no idea what that entailed, and it wasn't really explained to me until it was already in place. When Evanna originally went into surgery, we had only talked about a pig valve being put in, however, a mechanical valve was put in instead. There are both pros and cons to this, but the cons just caught me a little off guard. The pros is that in the in the long run, mechanical valves are much better, as they don't break down and they last a very long time. However the downside is Evanna will now require daily heparin shots. At the moment Evanna gets two needles everyday, but there is another drug that only has to be given once a day, so we're hoping to switch to that as soon as possible (probably when we're closer to being discharged). Still, it wasn't something that was initially explained to me, so it caught me by surprised when I came into the PCICU the other day, and Evanna was being given a needle - and it wasn't your normal vaccination. Don't get me wrong, it's not like we would have said no to the mechanical valve - it's what's best for Evanna in the long run, but it's just a little stab to the heart when I start to picture what our life is going to look like when we bring her home. It's mind blowing that the only medication Evanna was on before surgery was a little bit of Omeprazole for some reflux, and I can't even begin to imagine what drugs she'll be on when we bring her home.
Dr. Cave did say there is another way to administer the heparin by inserting something under her skin once week, so we don't have to poke her everyday, but that's still a lot of pokes for one little girl. Eventually, when Evanna is older, she may be able to go on Warfarin (which can be taken orally), but they generally don't like to put small kids on it, as they're prone to fall down and hurt themselves more easily, and there's a higher risk for uncontrolled bleeding. Dr. Cave said he's extremely sorry that what's involved with a mechanical valve wasn't really initially explained to us, and that I was caught by surprise. I told him that it's not like we would have said no to the mechanical valve, as it IS better for Evanna, I just didn't think a mechanical valve equals daily needles for Evanna. Nick has already joked that I'm going to be the one that has to give her all her shots, as he wants me to be the mean parent lol.

The plan for the week is to continue to slowly decrease her PEEP and pressure support over the next few days, and hopefully try extubating later this week. While they're a tad more optimistic that she'll be successful, we're still not out of the woods and have to keep our fingers crossed. We are discussing when we'll be transferred back to Calgary, but no set plans have been made just yet.

I'm excited for this upcoming weekend, as I'm making a quick trip back to Calgary for about 40 hours, as I'm a bridesmaid in a friends wedding. I haven't slept in my bed for almost 80 days, so it'll be a nice and welcomed break from the hospital. Nick is coming with me to make a date out of it, and his mom is coming up to Edmonton to be with Evanna while we're away.

Thanks for the continued support and prayers. We've gotten a couple of generous and beautiful care packages over the last few weeks, and the kindness of both friends and strangers is unbelievable. We have been so incredibly blessed with such kind and caring people in our lives, and I hope to one day return the favour. I also need to give a little shout out to Nick's awesome supervisor (hi Wade!!), for being so understanding and taking care of our family. Nick still works as often as he can, but there have been times when I needed him here in Edmonton (Evanna's bigger surgeries and when she coded back in October) to be with me and Evanna. When I posted about Evanna's second open heart surgery about 2 weeks ago, Wade quickly sent Nick a text asking him if needed that day off before Nick got a chance to call him and ask Wade himself. Nick is surrounded by so many caring coworkers who have taken a great interest in our daughters well-being, and I will be forever grateful.

xo The Irvine Family

PS. For those interested, I tend to do more daily-ish updates on Instagram, so feel free to follow me at @kierrai

Saturday, February 14, 2015

Faces of CHD: Meet Ruby

February 7-14 is Congenital Heart Defect Awareness Week. To help raise awareness, spread knowledge and share the effects of this disease, Jayme of The Paper Deer Photography Blog, Christie of Lemon Squeezy Home and Kierra of The Irvine Home are each sharing one story of a child with CHD every day to showcase the trials, triumphs, journey and heartache of this devastating disease. We invite you to read and share this project to further our voices. Thank you. #Facesof CHD


Ruby Jane was born on June 5th, 2013 at IMC hospital in Murray, Utah. We were overjoyed to have our baby here! The baby we had prayed for and wanted so badly for two years before becoming pregnant with her. One of the first questions we asked the doctors was if she had craniosynostosis, like our Bailey had when she was born. The answer was no, and we were so relieved. We didn’t want to re-live the surgery that Bailey had as a tiny 2 month old. When Ruby was 2 days old, we were told that she needed an echocardiogram because the pediatrician in the hospital was hearing a significant heart murmur. It is normal for babies to have murmurs when born but he was worried about how loud it was. We were still in the hospital because I had her via c-section. She had her echo and that afternoon the pediatrician came to talk with us. He had sent the echo results to Primary Children's Hospital and they got back to him, telling him that Ruby had a rare heart defect called Tetralogy of Fallot, and she would require open heart surgery at some point. He was trying his best to explain it all and nothing made sense--he was no cardiologist and we were no “heart parents”. All I could think was how my precious baby was perfect. I was so extremely sad for Ruby. I called my mom and sobbed to her over the phone, and she cried with me. My mom told me not to get online and research it. Initially I didn't hop online, but Dillon did and landed on a blog with bereavement pictures--he didn't tell me this at the time (wise man). Shortly after, Dillon left to get some dinner. It was late and getting dark outside. I held Ruby, grabbed my laptop and typed in Tetralogy of Fallot. We had asked the pediatrician to spell it for us three times so I knew what to type. I was alone with Ruby for about an hour and it was one of the darkest hours I’ve had. I kind of just lost it and cried and cried. I held Ruby as tight as I could and prayed as hard as I could. Dillon got back and could see that I was not doing well. He took Ruby from me and sat on the bed and rocked her. I was just so worried that we would lose her. Dillon knew just what to say--he comforted me and said it was ok to be sad. And then he told me, “Christie, this is how God sent her to us. This is the way He wanted her to come.” And that’s all it took for me to feel an immediate peace. My sweet husband’s words came back to me again and again through the following months and really strengthened me. I still lost it, I still had moments of breaking down. Since then I’ve come to an even greater realization that God didn’t want her to have the heart defect–of course He would not want her to suffer. But through the trial we were all given, we would learn things that we couldn’t have otherwise–and He allowed for her to come with a broken heart so we could change for the better. The coming weeks were filled with doctors appointments to Primary Children’s Hospital, echocardiograms, EKG’s, chest x-rays, weekly weight checks and oxygen saturation checks. By the time Ruby was 26 days old, I had taken her to 15 doctors appointments in a 22 day time frame. We were told to expect 3 surgeries based on her first echocardiogram. At least. That was the average for Tetralogy of Fallot. 
2 months old copy

We finally seemed to get into the routine of things by the time she was 2 months old. We were told not to let her cry hard, and certainly not longer than 5 minutes or she could have a blue spell--meaning she could pass out due to oxygenated blood not being able to get through her body enough. The goal was to make it to 6 months for surgery for weight and size factor, but not to go much past 6 months or the heart wouldn’t be able to correct itself as well. Ruby was doing well, apart from her weight gain. She was happy, healthy, and her oxygen saturation was always in the 90’s, which was so great for a heart baby. She had so many people praying for her. I knew it could be so much worse--she could have had blue spells, she could have had her oxygen saturation dive down and needed surgery sooner, she could have needed oxygen or a feeding tube, she could have been born blue and life-flighted to many things that could have gone wrong and they didn’t. We were so very blessed. The older she got, the more signs of heart failure she had. Sweating while she ate, being extra tired, and some weeks she actually lost weight which was scary because they talked about moving surgery up if she stopped gaining. Every ounce was a celebration :). We had a sedated echo November 21st and they said the pressures in Ruby’s heart were really high. In her right ventricle they were in the 80’s (it should be zero. 80 is labeled as severe). But they also said that her pulmonary valve was looking really good--better than the previous two echocardiograms. We were called a couple weeks later and told her surgery would be December 17th with Dr. Eckhauser. Ruby’s surgery approached and we tried to be as prepared as possible. People continued to pray for us and even as life got insanely busy and more trials and tests came our way, we were blessed with continued peace. We went to Primary Children’s Hospital Tuesday morning and within an hour handed her over to the anesthesiologist. She looked back at us as they headed through the doors. We received calls about her during surgery: When the incision was made, when she was put on bypass, when she was taken off bypass (two hours later), then the surgeon came in to talk to us about all he had done. Waiting during surgery is very emotionally draining. The hole in her heart they knew was big, but he started patching it and realized there was a much larger hole behind the one he was patching. So he had to un-sew it and start over. Which is why she ended up on bypass for so long. The right side of her heart was significantly thick, so they had to shave some off the outside as well as cut into her heart and shave some off of the inside of the right ventricle. They tied off her PDA that was supposed to close shortly after birth and partially patched a hole in the upper chamber. They had to sew through her tricuspid valve because the hole was so large in the bottom chamber, but he was hoping as it scarred it would close that up. He did a lot of work-- more than they anticipated. We finally got to go back and see her at 12:50 pm. 
DSC_0001-001 1

Nothing can prepare you for what you see after such a major surgery. It is heartbreaking. She was intubated still and just hooked up to so many things. Unfortunately, she was in pain, indicated by her not sleeping like she should have been. She was very restless and just moaned and moaned in her sedated state. I asked the nurse if it was because she was in pain and she said she most definitely was. What a helpless feeling, not being able to comfort your baby or hold them. So very hard. That night was very awful. We were lucky enough to get a room even though everyone had told us to go home and sleep because patients are totally sedated and sleeping that first night, but I could not leave her there and Dillon felt the same way. Dillon went to sleep first and I stayed with her--she was so restless. The nurse was very frustrated because she only wanted her to be comfortable and she wasn’t. Ruby started to itch a lot too and her little face became really red and swollen. I am so glad we didn’t feel like we could go home. I cannot imagine her being awake so much and not having Dillon or I there. I slept for about 4 hours after Dillon woke up and when I came back Dillon reported the same as me, that she was just miserable. The second day was probably even harder. They kept trying to get on top of her pain and itching but she had little relief. She didn’t sleep peacefully hardly at all. On the second day they took out the Right Atrium line, an IV that went directly into her heart. But it bled. And bled and bled. It wasn’t supposed to. It bled for at least a couple hours. More and more people started to gather in her room and check the chest tube drainage and talk about what needed to be done. My brother-in-law Greg happened to show up just at this time. Next thing we knew, there were probably 7-8 people in there. Dillon was outside the room talking to Greg. The nurse practitioner turned to me and said, “The RA line tore her heart and it’s bleeding and she’s losing too much blood. The bleeding is not stopping so we will have to go back in and repair the tear.” I went out to tell Dillon, in tears. He was in tears too now, and came back in the room with me while Greg waited outside the room. The surgeon was called down and more people came into her room. They looked at the drainage and the amount she’d lost and Dr. Eckhauser looked at me and said, “If this bleeding doesn’t stop in the next 20-30 minutes, we’re taking her back.” I tried not to look too scared, but I think I failed because he tried to calm my fears and said, “It’s sounds worse than it is. I just have to unstitch her, take the wires out of her sternum and patch the tear.” She had already gone through so much. I was so worried for her poor little body and the setback this would be to her. I turned to Dillon and whispered, “Pray hard!” We were so scared. The bustle of people continued and we knew she needed a blessing. So Dillon grabbed Greg and they gave her a blessing. The nurse checked her chest tube drainage and there was a difference already--literally within 5 minutes of her blessing. The surgeon came back and saw that the bleeding was slowing down. They continued to monitor her but decided that the situation was no longer of concern. What a blessing. She continued to improve and we were able to bring her home in time for Christmas! Miracle after miracle. We are not positive this will be her only open heart surgery, but they are hopeful. There are many problems that can crop up in her future, which is why she will continue to have cardiology check-ups for her entire life. She also had some unclear genetic testing results, but there was not enough research done on them to know what they may mean for her. But we are thankful for how well she is doing. We are enjoying every second with her. She is such a blessing to us!
Ruby Jane is now 20 months old. This picture is from when she turned 1 :)
What does CHD Awareness mean to me? It's letting people know that it can happen to anyone, and it is much more common than people realize. We were so unsuspecting, so in the dark about the heart world. CHD Awareness is encouraging people to donate blood and to make sure to get newborn screening done using pulse oximetry. It's helping others to hopefully trust themselves and get their child checked if they feel that something may be wrong. It's encouraging others to become organ donors, and to make decisions now as to whether or not they would allow their own children to be donors if that unthinkable decision had to be made. It's pausing our busy lives to give of our time and efforts to help those around us who may be living a nightmare in this heart world. It means helping others see the faces behind CHD, touching lives and hopefully making a small difference through sharing Ruby's story, along with others' stories. CHD Awareness means that I will forever be passionate in helping Heart Families feel any sort of peace. There is no cure. And more research needs to be funded. 

I've recently set up a blog that encompasses a small portion of CHD Awareness, called Love From Ruby. You can also visit me at Lemon Squeezy Home and instagram @lemonsqueezyhome. 

Please continue to

Friday, February 13, 2015

Faces of CHD: Meet Logan

February 7-14 is Congenital Heart Defect Awareness Week. To help raise awareness, spread knowledge and share the effects of this disease, Jayme of The Paper Deer Photography Blog, Christie of Lemon Squeezy Home and Kierra of The Irvine Home are each sharing one story of a child with CHD every day to showcase the trials, triumphs, journey and heartache of this devastating disease. We invite you to read and share this project to further our voices. Thank you. #Facesof CHD

At Logan's two week check-up, I knew something wasn't right when my son's primary practitioner looked concerned as she listened to my baby boy's heart. She let me know she had heard a murmur. She was going to have another doctor take a listen and check his oxygen levels. She mentioned maybe making a three hour trip to The Children's Hospital in Minneapolis some time that weekend. 
Four pulse-oximeter machines came and went from our room. I cried when I read 76. Nobody could seem to understand as he wasn't retracting, he wasn't distressed and he wasn't blue. However, it quickly went from "you may have to drive this weekend, to your child is being flown out, and the flight crew is it's way now."
It happened so fast when they arrived. They put an IV in my baby's tiny hand, strapped him down inside big isolate and took off. We left immediately after, still having no idea what, if anything, was wrong. I watched the blinking lights of the helicopter for a good fifteen minutes and when it finally left my sight I remember thinking "he will be there in about 20 minutes and I still have a two and a half hour drive" It was the most agonizing drive of my life. When they called to update they said Logan had a really rough flight. His oxygen saturation's dropped in the 30's and he at one point quit breathing. It wrecked me not being able to be there for him.
Tetralogy of Fallot was the diagnosis shortly after he landed. He will need heart surgery in the next couple days the Cardiologist said. I couldn't fathom any of it. I cried so hard I couldn't reiterate anything I was just told to my husband and mom who were in the car with me. The rest of the weekend was spent in the hospital monitoring Logan and keeping his oxygen saturation's stable. 
Our four (and a HALF) year old, Emma, was able to visit most days in the hospital. If her playful and sweet personality can't make a person smile, I don't know what will. 
Logan underwent his first open heart surgery on Monday 10/13/14. The surgeons began at 9:28 and he was in recovery exactly two hours later at 11:28. A shunt was placed, which was a temporary repair. 
Nothing can quite prepare you for seeing your child after a surgery like that. I have never had such mixed emotions, happiness and relief he was out with no serious complications, and unexplainable heartache that he was even in this position to begin with. I hardly recognized my own baby with all of the swelling from fluid accumulation and tubes coming from his tiny body. With the help of the amazing Cardiac team at The Children's Hospital, Logan made a beautiful recovery and was home just seven days later.
A month later and we had almost forgotten about the DiGeorge syndrome testing. When we got the call they had great news, the 22q testing was negative. Then, we received some not so great news. We were told Logan is missing a piece of his 16th chromosome that no one has ever been documented missing before. This means they have no idea how it may (or may not) affect his health and development in the future. I took this especially hard for a couple of weeks, how could they not know ANYTHING. The unknown truly IS frightening. After a trip to the Geneticist I was feeling much better as she couldn't get over how wonderful Logan is doing now. There is still fear, but it gives our family hope. 
Our little miracle is now four months old and sweet as can be. We are enjoying every day with him as he grows at his own pace and hits new milestones. However, there is always concern as he outgrows his shunt that it may fail. 
Logan's saturations have been dropping quite low recently and has become a concern to his team of cardiologists and surgeons. He will be going in for his second open heart surgery on February 19th for the full repair of his heart defect (that's less than a week from now!).
We are so incredibly thankful for the outpouring love and support from our family, community, and even strangers. We are extremely humbled and our hearts, too, are forever changed. We will be so happy once Logan's heart is completely repaired and healed and we can put this chapter in our lives behind us.

CHD awareness is so very important. Early detection is critical in order for successful treatment. The more awareness spread the more possibilities for new and improved treatment. Encourage newborn pulse oximeter tests as this can be an early indicator. I also cannot stress enough the importance of well child check-up's. I hate to think what could have happened if I didn't bring my baby boy to his two week well child check and if it weren't for our incredible family practitioner. 

- Courtney (Logan's mom)

You are welcome to follow the Boysen family on Instagram: @courtneyboysen

Thursday, February 12, 2015

Faces of CHD: Meet Ava

February 7-14 is Congenital Heart Defect Awareness Week. To help raise awareness, spread knowledge and share the effects of this disease, Jayme of The Paper Deer Photography Blog, Christie of Lemon Squeezy Home and Kierra of The Irvine Home are each sharing one story of a child with CHD every day to showcase the trials, triumphs, journey and heartache of this devastating disease. We invite you to read and share this project to further our voices. Thank you. #Facesof CHD

Meet Ava Monroe!  Chandler and I (Kaila) have the privilege of raising this extraordinary little, now 6 month old, life together.  We live in Georgia, about 45 miles west of Atlanta, and have been married for just shy of 3 years.  After being diagnosed with PCOS several years ago, we did not see a baby in our near future at all.  Especially after being told we would need to seek fertility to conceive.  But just before Christmas 2013, we were shocked to find out that we would be welcoming our little miracle in the summer.

Ava’s journey in the CICU (Cardiac Intensive Care Unit) began at just 12 hours old.  Our brand new baby girl was transferred to Egleston very quickly.  And thanks to a very easy delivery, I was discharged at the same time.  This particular CICU was open-concept.  There were 26 bed-spaces, and every last one was full.  This was seemingly nice for the first… several hours.  We met a few parents, listened to their experiences and swapped pictures.  But then, roughly 6 hours into our stay, the baby in the bed space next to us lost his battle.  My heart still drops as I replay the mother’s screams in my head.  They echoed through the halls and into my soul.  I remember telling God, “do not let that be me”.
Other than surrounding chaos, our first five days were full of optimism.  The week consisted of meeting with doctors & surgeons.  They came up with her plan of care and informed us that we were looking at 10-14 days of recovery before we would be home-bound.  Ava was not allowed food that first week due to her specific CHD (Congenital Heart Defect) & required Prostaglandins to keep a special duct open until her surgery.  She had no oxygen requirements, just IV’s & central lines (which prevented us from holding her).  On day 6, we walked alongside our baby girl as they wheeled her back to the OR.  There was really no time to process any of it as we were immediately dumped into a waiting room FULL of people, including much of our large family.  After hours of waiting, the surgeon came and found us.  He greeted us with sweat dripping from his surgical cap.  Waiting for his words was the most nervous I had been up to that point.  He told us that he had repaired Ava’s aortic arch and tried to get to her VSD but couldn’t cover it all.  He went on to say that her pressures in her right ventricle were “a little funky” and that he would watch that closely but he hoped it would work itself out as her heart got accustomed to it’s new “flow”.  Thankfully, it did.

Nothing could have prepared for what we walked in to.  Just hours prior, we had handed off our seemingly perfect, 5 lb, 14 oz. child.  The sight that greeted us when we walked back in to the CICU was simply terrifying.  They had to leave Ava’s chest open to allow room for swelling and extra fluid that had been pumped into her during surgery.  All that was covering her tiny beating heart was a clear patch stitched between her chest bones.  The nurses were whispering to us, assuring us that she looked very good and letting us know we could touch her hands and feet.  They didn’t want us talking to her too much or stimulating her, being that her little body had just experienced such major trauma.  It was all we could do to text our families goodbyes.  Truly, we could not have faced anyone in those 4 days that she remained open.  They were excruciatingly long.  Once Ava was closed, they started lifting her sedations relatively quickly.  There was such hope in seeing her big round eyes open after so long.  I felt so disconnected from her, but so admirable of her at the same time.  I just remember feeling like, we finally made it!  Our new life is about to start.

Two days later, they were ready to extubate her (remove her breathing tube)!  We were anxiously awaiting this moment because this was a huge step toward going home.  As soon as they allowed us back in the unit, there were nearly 10 people scrambling around Ava’s bed-side.  They were already switching her from the oxygen she was on to a RAM Cannula (which gives additional pressure to help expand the lungs) and they assured us that it was very common for post-op babies to need a little extra respiratory help immediately following a surgery such as hers.  However, within the hour, it was clear that Ava’s respiratory status was declining and they emergently re-intubated her.  Little did we know, this was the beginning of a new journey for us. 

Ava’s first three extubations, were chalked up to her just needing to regain strength.  After all, time was not much to ask for after what our girl had endured.  So time we gave her.  After nearly a month since surgery, it was time to extubate for the fourth time, things were going surprisingly well.  So well in fact, that my mom came to sit with Ava while my husband and I made the hour+ drive home for the first time.  The second, and I literally mean the second, that we opened our front door.. I received a call from the CICU.  The doctor was rambling and rambling and I just remember screaming, “What Happened?!”.  Ava had been re-intubated.. but that was not all.  She had a line that had been in her right femoral artery for over 3 weeks.  They speculated that it must have formed a clot because after the nurse flushed her line.. Ava lost all profusion to the lower half of her body.  There was not adequate blood flow being delivered to her lower extremities.  We immediately got in the car and headed back to Atlanta.  My mom had been rushed out in the haste of things.  Ava was started on blood thinners and they were hoping that things would resolve quickly.  By the time we arrived, her left side looked seemingly normal, but her right leg was very alarming.  It was modeled, and there were spots of black and purple from her hip to her toes.  The lower you looked, the worse/darker it got.  The vascular surgeons sent their best ultrasound tech over to see what they could conclude.  Our doctors seemed to be shocked by what had happened, they said if this happened once a year that would be a lot.  The vascular team could not locate the clot, but informed us that it was not likely that our daughter would keep her right leg.  Devastated is too light a word.  When you are battling such a devil as CHD, and trying to make your mental list of things to prepare for, losing limbs is not on that list. 
The following days, despite what the vascular doctor had expected, her leg continued to improve.  And after a week, losing her leg had turned into, as the doctors put it, “JUST losing her toe”.  I remember dwelling on that word, “just”.  Many may have counted their blessings at that point, but I was just so exhausted and so bitter.  And after roughly a month and a half in the hospital, nothing had gone right, nothing.  Once we got over that hump, we were back to the old grind of trying to successfully extubate.  But again, the days were turning to weeks and every failed extubation turned into another.  Each time more devastating than the last.  Then, we had a terrible scare.  One morning, about a dozen doctors were gathered around Ava’s bedside as we walked in.  I had seen this before, around other babies, and it was never good.  They were all looking at her x-ray.  Some on their phones with other doctors, some with pharmacy, others checking labs and speculating amongst themselves.  We had seen her chest x-ray every single morning since we had been there.  That being said, we noticed it immediately:  Her heart was doubled in size.  To any heart parent, this is a nightmare, as it typically indicates heart failure.  She had several close calls with cardiac arrest and her heart began taking on the stress from all of her respiratory troubles.  Thankfully, with the help of cardiac medication and another intubation, the stress on her heart slowly retreated.

But Ava was just pitiful.  Her respiratory decline came quicker with every extubation.  I will never forget hearing the whistle of air as she fought for every unassisted breath.  Her violent retractions bore visibility to every centimeter of her ribs.  My heart was breaking, and my faith, I would have laughed at the very word.  The doctors did not think we would be bringing our daughter home, and although we never spoke it to each other, my husband and I felt that dream slipping away.

During these horrible months, Ava had gone to the OR several times for bronchoscopies to look at her airway.  It was the same each time.  She had suffered damage from the breathing tube.  But from what they could see, nothing significant enough to be causing her respiratory failure.  They then did an exploratory cath procedure.  But came back with nothing, her lungs and pulmonary arteries were working well. 
Every time we would check something off the list, the more time had been wasted.  All the while, this tiny person was fighting for every breath and we were no closer to offering her relief.  The days were so long, and the nights were longer.  Something as simple as gas pain was enough to send her over the edge, and cause her to need to be emergently re-intubated.  She had no energy left.  My baby was aging, but not growing.  I reached my limit of emotional, spiritual, physical exhaustion and pain.  I could not watch her go on like this any longer.  I felt defeated.  As I laid my head on my precious daughter that night, I whispered to God, with a lump in my throat, and a shattered heart, that he could have her back.  Nothing in this life will ever be so painful.  My husband and I sought nothing but each others arms, we cried until we were shells of human beings.  My husband told me that, as long as Ava fought, we would fight with her.  All it took was one look to see that there was not much left in her tiny, beautiful body.

That Wednesday, after a rough start to her final attempt at kicking the breathing tube, Chandler demanded her to be re-intubated.  We knew where it was headed and did not desire to see her struggle, that intubation was simply a relief.  From there, things moved quickly in the direction of Ava getting a Tracheostomy and G-tube.  It was the only way we would have a chance at taking her home, and to relieve her of her burden.  Within 48 hours, she had already had the surgeries.  And for the first time, in over 3 months, things were looking up.  Ava began to smile and stay awake for longer periods of time.  She had been on so many sedations for so long, and every wean just brought out more of her incredible personality.  Hope for her future flooded our hearts!  We were moved to the TICU (Technology Dependent ICU) and her recovery only expedited.  Within two weeks, she had weaned off the ventilator for 12 hours at a time!  The TICU team was blown away!  We had thought that we would be going home with nothing more than an empty car-seat, but our Ava fought for us, and I am so proud to say that she proved us wrong.  After 102 of the longest days of our lives, we were discharged with a positive prognosis in every way. The trach is temporary while she outgrows her airway trauma, for her lungs get to healthy and strong. We had fought through all of her drug withdrawal, we managed to keep all 10 fingers AND toes, & lastly, her heart was stable!  They told us that other than the need for the mandatory medications and monitoring, Ava would grow to have no limitations. 
From conception to survival, she is our miracle.

I have to say a thank you to the family and community that held us together when we had reached the end of ourselves.  When we had no faith, you were our intercessors.  When our emergency funds became depleted, we not once needed for anything.  We could not have made it without the support of these beautiful people.

It is hard to sum up what CHD means to me.. I suppose I have to say, it is a reckless love.  I have never known a more reckless, selfless, scary, or beautiful love since coming to know so many families in the CHD world.  The parents, the siblings, the families of donors have shown us/displayed such a variance of this.  Not one of them/us can control our fighter’s outcome.. and even so, the love is immeasurable.

- Kaila (Ava's mom)

You are welcome to follow the Biagini family on Instagram: @urbnmama @kailabiagini and @chandlerbiagini

Wednesday, February 11, 2015

PCICU Day 128 & 129

So while the past 24 have been a bit rougher with Evanna, I'm still going to say that overall, this recovery has gone a lot smoother compared to previous surgeries. For those who many not remember, Evanna's recovery after her last 2 major surgeries (I'm not counting the diaphragm plications), were much more of a rollercoaster, as she needed a lot more sedation, and even required being paralyzed multiple times.

Evanna had a pretty good day yesterday. She kept having higher than normal O2 levels (well high for her), and so they had to keep coming down some ventilator settings multiple times throughout the day. The doctors were able to find a good balance in regards to her sedation, as she seemed somewhat comfortable, and would even open her eyes from time to time. Though she was very groggy and "stoned", she still would look at Nick and I and knew that we were there.

Today has been a little rougher. Evanna threw up last night, and so they had to stop feeds and insert an NJ tube before they could restart feeds again. She's VERY puffy, and they also had to give her another blood transfusion as her hemoglobin levels were trending down again. Also - they had put a new arterial line in last night, as the one she had in, had been there since the beginning of January, and it wasn't drawing blood anymore among other issues. I'm a little annoyed because we (well the intentivists) had asked for a new arterial line when she went to the OR, but she came back from surgery with the exact same line. So basically, the whole arterial line situation could have been avoided if the OR had put a new one in like we had asked.

Anyways, I'm hoping Evanna's day gets better so we can continue to decrease her ventilator support. Dr. Cave mentioned yesterday that the bronchoscopy video from Monday is "floating around" among the medical team (he made it sound like it had gone viral lol), and everyone is a little more hopeful that she'll extubate (Nick and I are still on the fence about it, and we don't want to get our hopes up just yet). We've slowly begun to discuss a transfer back to Calgary, but until Evanna is much more recovered, nothing is set in stone.

Thanks for the continued prayers and support,

xo The Irvine Family