Sunday, April 30, 2017

We prepare for our Warrior Princess's 5th OHS

So we finally met with Dr.R for the official results of Lily's cath. After doing all the research from the information I was given, I knew what was coming, but it was nice to be given the rest of the technical terms and names. So let me catch you up. 
 Lily's coarch repair that was done at 5 days old, hasn't grown. When they attached her vein, they had to suture it all the way around, in a circle, which in turn has created scar tissue, and it is strangulating her arch. blood goes up into it, but all of a sudden runs into a traffic jam, because it is such a narrowing (stenosis). They will try to balloon that, it is in such an area, that a stent isn't an option. We haven't talked about if that doesn't work, it is something that I will bring up at the surgery consult. 

That is one repair. The second repair is the Ao-Rv Fistula. First, a fistula means, 
  1. an abnormal or surgically made passage between a hollow or tubular organ and the body surface, or between two hollow or tubular organs.
Ao= Aorta   Rv= Right ventricle  

So with that being said, Lily has a passage going from the bottom of her aortic valve to her right ventricle. What I was I told, a patch will be put in place again. So, this is where her original VSD was. The patch was disturbed when we had issues with the Left ventricular outflow tract obstruction (LVOTO). According to Dr.R, the original patch has completely corroded, leaving very little, if any of the original intact. So they will replace that. I am interested to learn what type of material they will be using this time and if they feel we will have a better chance of it lasting, or if we will end up having to replace it again down the line. I know some things can't be foreseen, like this, who would have thought this patch would have done this? But with Lily, we can't close her VSD in the cath lab, it requires open heart surgery, so it is a very valid question for me to ask.

The last repair that needs to be made, is replacing her St.Jude's valve. I have included a picture of it below, so you can get an understanding of what it looks like
The valve is made of metal and synthetic material. The leaflets are metal, so when they open and close, if you were to send a cath through there, they could snap the cath off, and it could kill her. I just wanted to help those understand, who don't know the heart as well as some. 

Lily has a 19mm valve put in when she was 2. The way it was explained to me then, that was the size of a petite adult female. She should never of needed it replaced unless, the valve failed, there was and infection and we had to replace it, so on and so forth. So after all my research I did and Dr.R confirmed the research I did, she has to have it replaced because her valve is "failing" at the top of mechanical valve. Meaning it is coming detached. Their solution to this problem is to put in a 21mm valve instead.

Now I have questions about that too for the surgeon. Trust me, they are written down, he's gonna love me. I have never met him, he has never met Lily, doesn't know that she throws curve balls in the middle or surgeries. So my anxiety is through the roof, thankfully my doctor has given me something to help with that. I spend a lot of time listening to music, clearing my head, and praying.

I finally started talking with Lily about her upcoming surgery, she seems to be taking it well, her biggest worry is, that her heels are going to hurt. She remember waking up from her cath and her heels hurt so bad, that we had to prop her feet up with pillows. I told her she wouldn't even notice her her heels hurting.

Then she turned to me, her eyes wide, and says, "Mom, you'll be there right? You'll stay at the hospital the whole time, you won't leave me, because you never left me when I was a baby. You won't leave me now, will you?"   

I scooped her into my arms snuggled her close and told her I won't leave the hospital until she leaves to go home. I heard a huge sigh of relief, I heard her heart slow down, she kissed my cheek, hugged me, and then ran off. 
I have been blessed with this amazing Warrior Princess, who's heart still beat strong, even when it is tired. She loves her life, she lives her life, and these last 9 years have been the best because I have had this sweet girl in my life.

Friday, March 24, 2017

Cath Results and Misplaced Anger

We went in with a hole in her heart and a leaky valve and walked out with so much more.

My processing has taken time, tears, cursing everyone and no one at all, and anger. Oh the anger has consumed me and it took me a while to place who and what I was angry at. So let me start with the  medical side of everything before we delve into my emotional side, because those aren't going anywhere.

So when Lily was in the Cath lab, we found out she now has pulmonary hypertension, which is a new curve ball for me. Now this little present is from the leak in the mechanical valve. So first Dr.G tried to put coils in, which back fired, then he added a plug. It is exactly as it sounds. The picture below shows you what it looks like. I outlined it in orange for you so you can see it a bit easier.

Once the plug was put into place, the blood started flowing correctly again. She is no longer coughing at home, which most likely means her pulmonary pressures are going down, but we won't be able to tell that in an echo, only another cath will let us know for sure. 

The VSD was non-repairable in cath, because in order to repair it, you go through the aortic valve. With Lily that is a no go because of her mechanical valve, you send a cath through there, the metal leaflets would break the cath and would kill her. The only way to repair the hole is to open her up, we are not opening her up for such a small hole.

But wait there is more. When Lil was a baby it was mentioned that she had mitral valve prolapse, but that it was so minor that it was nothing to concern ourselves with. So I filed it away, never truly forgetting about it, but doing my homework knowing, it isn't truly a huge defect, and she can live her life normally with it. Yet it was brought to attention again, and this time it was mentioned that it might be time we started talking a mechanical valve replacement there. All I could think is, "My child is going to have a bionic heart."

Why is this just now being brought up? Why hasn't it been seen on echos? Is it because her heart is backwards and really hard to see certain things? I have a million questions floating through my head and I have a month to wait until I can fire them off at Dr.R.

No here comes the doozy, the dino whooper, gut puncher, I cried, I screamed, I cussed, I've been so angry I haven't been able to talk with anyone about this.
The picture below is Lily's coarctation of the aortic arch, we fixed it during her first surgery with a vein from her wrist. The only time we worried about it was after her fourth surgery, right after we put the mechanical valve in. 

What you need to understand is, they don't make mechanical valves for children, they only make them for adult hearts. So here is a 2 year old little girl, getting a 19mm valve (the size of a grown petite female) put into her heart. So when the blood was being pushed up through the aortic valve into the CoArch, it started to balloon. It put a massive strain on it, also know as gradient levels, and we worried the work done on the arch wouldn't hold. But to our amazement, things leveled out and that was the end of it, or so we thought.
So what you are looking at in the picture above , the marked areas in orange, is now the stenosis, aka narrowing. So the ballooning was done on the front end of the valve but everything comes to a crashing halt on the back end. We talked about if a stint was a possibility but it most likely wouldn't hold because it is at an opening. In normal patients they would worry about clotting, but in Lily, being on blood thinners, she would just throw the stint. So we would have to think of a different course of action.

So you take all of these things and you put them together, you have to ask yourself is it time for an open heart surgery, or do we watch and wait. 

My anger comes into play with her arch, because that was a man made mistake. Please don't get me wrong though. I don't blame her doctors, I am not even blaming myself, even though God knows I did in the beginning, which is irrational. My anger resides in the fact that there is not enough research in pediatric congenital heart research. 

My daughter is a very small percentage of children that have a mechanical valve. Are you telling me this has never happened before? Are you telling me there was no studies done, telling use this was a possibility we took, when we put it in. 1 penny of every dollar  donated to the American Heart Association goes to Pediatric Research. 1 PENNY!!! How is Gods name is that fair? 

We destroyed a fixed heart defect in a child, by trying to fix another heart defect that doesn't have enough research behind it. And I am angry because of it.

This valve has caused this domino effect of problems. Like the pulmonary issues, do you want to know what took us so long to get back to see Lily after cath? I didn't find this out until the next day. Her lung partially collapsed and no one told us, NO ONE!!

I continually pray, to help ease this anger, to let go of this anger, because I detest it, but I know it won't go away until I get answers. 1 month, I have to wait 1 month. I better figure out an inner zen.

Peace and Love
J

Tuesday, March 21, 2017

Hazy lungs- Fevers- Home bound?

 I know I know, still no post about the findings from yesterday's cath, I am sorry, It was a long night, busy morning, and I am exhausted. We had an echo this morning, I have no idea the findings on that one, because Dr.B that did the cath yesterday is in Gilbert, so he is looking at the images over there, and Dr.Rhee is looking at the images 7 floors down, so I didn't actually get a say in if it looked better or not.
She also had an x-ray and it is showing her left lung is hazy. They told me that it should get better with lots of sitting up and coughing. Yet what worries me is the fever we are going home with along with the hazy lung but they aren't concerned. So after our third round of antibiotics, we are packing up our room and getting ready to break out of here and head home. She is going to relax and watch movies, while mommy gets to log in remotely, and work for the rest of the day from home. At least I am blessed and am able to work from home. My company was very understandable when it came to my daughter. Anyway, I promise I will post tonight about her Cath findings. 

Much Love,
Jenna

Monday, March 20, 2017

Processing & Low's


I know I said I would update this evening but after the day that we've had today, I am going to settle in for the evening with this lady.  She woke up long enough to smile for and no sooner did I take it, did she fall back asleep. 


We had a bit of an episode shortly after being brought upstairs. She started in on a coughing fit, she had just had a drink of water, and her lung pressures aren't helping. We were on 2 liters of oxygen from the nose canulas at that point, when all of a sudden, she just couldn't catch her breath. I could see the look of fear in her eyes, knowing she couldn't move and not being able to breathe. Her lips turned blue, she turned grey, alarms went off, her sats dropped 69, 68, 67, and I stood helpless. This happened in under 3 minutes, and in those 3 minutes it seems like 30. Her nurse came rushing in, we bumped her back up to 5 liters of oxygen and she was hanging out there until about 5 minutes ago, we are giving it a test run to see if she will tolerate us going down to 2.5 liters. So far her sats are hanging in there at 98*, so we are happy. 

I just got the ok to feed her chicken broth, a tear ran down her cheek and when I asked her why she was crying, she said it was because she was so happy she finally gets to eat. She has been a trooper. It is going to be a long night in here for me, every time I sit down and get comfortable, she asks me for something. But you know what, I am OK with that, 100% OK with that. 

As for the news we got earlier today, I am still trying to make heads or tails of it and see it objectively. My brain feels like mush right now, it really has been a long time since it has had so much thrown at it at once.

Thank you to all for the texts, phone calls, and follow ups on facebook too. I haven't been the greatest at getting back to everyone. I do apologize, just know I have seen them all.

Much love.
J

Update 3

She is getting her last few x-rays in cath lab right now, the doctors just came out to see us and fill us in on things. I will post in a bit, in depth the findings. We are staying over night.

Things are a little worse than we thought but that doesn't mean we have to rush right into fixing things right away. Overview so you know what to look forward to.


  • Coarctation of the aorta-her repair
  • Pulmonary pressures*
  • Mitral valve issues*
  • Coil's and Plugs
The bullet points with ** next to it are new issues. So stay tuned, I promise I will update soon, first I need to see my girl.

Thank you again to everyone for your continued thoughts and prayers. You have carried us through this day. So much love and thanks.

Jenna

Update 1 & 2

 Lily was in good spirits when we got here, she had her friend Tiptop and all was well. We watched the end of Cinderella and then part of Angry Bird, and then we started to panic. But first, look at this beautiful girl.

I could stare at her little face all day long. 

So back to panicking, one minute she was fine, the next she had tears rolling down her bright red face, she started sweating really bad, her heart rate jumped quickly, so it was time for the pink drink. VERSED. If you haven't had it, well, lets just say, it makes you feel sleepy intoxicated or giggly intoxicated, depending on who you are. It takes the edge off and helps you relax, or in Lily's case, sleep. I think the fact that she didn't sleep to well last night, contributed to her falling asleep too. 

I got to go back to Cath Lab with her, you would think she wouldn't have fought at all but man did she fight!! She hated the mask and even half strength fought to take it off. Then all of a sudden it was like a light switch, and she was out.


So now the actual medical side of it. Lily's mechanical valve was detaching right next to the coronary artery, The blood was back flowing around that area, they said it is something they see commonly in adults with mechanical valves. Because truth is, you don't see many kids with these valves, Lily is a small percentage, and an even smaller percentage to have it put in at 2. 

So the fixing solution....Yes you read it right, a fixing solution,...In cath!! As excited as I am, I have hesitance. 

They put a plug in it. I'm not sure what this plug looked like, I am going to have to go do some research, because I have no idea what exactly is happening, because even though this was talked about before she went into Cath, I had no time to do look into this. I'd like to know how long this is going to hold because a plug doesn't sound to promising when it comes to a growing child.

They won't fix the VSD while in there, they said where it is located would cause more harm than good. I am not sure how I feel about this answer either. Needless to say, 2 weeks won't go by quick enough, so I can see Dr.Rhee and pick his brain and ask him my 10 million questions.

For now, I sit here waiting for her to get out of Cath, because she's still not done, she's tolerating everything beautifully, so keep up the prayers, they are helping her so much. I have only cried once so far today, and by cry, a single tear ran down my cheek after I left the Cath lab, and got into the elevator with my husband. He held me in his arms an I was safe. To have my safe haven right now is helping my sanity, I just wish it helped my anxiety.

I will update again soon

Bold and Brave

The dawn of this day has already approached and started before I was ready for it. Yet here are the things I am ready for.
  •  I am ready for answers. 
  • I am ready for my girl to start feeling better.
  • I am ready for my anxiety to stop.

We are bold and brave!

Her heart cath is at noon today, so we have to be there at 10. We were told to expect to stay the night because she is the second case of the day. I will post when they start, finish, and the findings they tell me today. They may not tell me all, but I will learn something.

Please keep Lilyana in your prayers today. This beautiful Warrior Princess is about to go and battle. I know it may be just a cath, but even a simple procedure is a lot to handle for her body.

Thursday, March 16, 2017

I shall believe

Even if it's a lie, say it will be alright.
In just 4 short days, we will know the extent of what is going on in my little girls heart.

In 4 days, I might be able to breathe again, even if it's just for a second, while it is to inhale to sob.

In 4 days, I will be sitting back in a hospital room, listening to my daughters heart on a monition.

And I am not ready. 

How can I not be ready for a simple procedure? Yet a heart buddy just had a heart transplant?

It makes my fears seem so small in comparison.

Lily is not in heart failure but her heart is sick. Her cardiologist told me not to let cath lab stop her procedure on Monday because of her cough. You see Lily has this horrible cough, if you heard her, you would think she was sick, but you see, she's not sick with a cold, it is her heart. It is the VSD, the hole is getting bigger and bigger and the blood is mixing, and causing her cough. Which I knew before seeing him, it was her father who was uneducated. I am the one who stays ahead of medical. 

I've done so much research on her mechanical valve failing and the chances of it being the valve or her heart rejecting it per-say. The things you learn when you read medical documents, I could bore you with percentage rates and such. You see, this is how I calm myself, I research. 

But one can only do so much research and then I am back to,

Even if it's a lie, say it will be alright.

My chest keeps tightening, I lay in her bed, I hold her baby dolls, and I just smell her, because I'm so damn afraid of what is to come and what we are to learn. 

Research is good but research is bad....

Even if it's a lie, say it will be alright, and I shall believe.

Come Monday I will be a pillar of strength, but right now, when the house sleeps, I sit here, with my head in my hands, and cry, because she was supposed to be ok, she wasn't supposed to have anymore surgeries, and I'm scared

Friday, March 3, 2017

Hand prints on the wall

I wish I could say I was as strong as I once was, that I do not falter when I step but those would be lies. For six years I tucked away 2 years of chaos, 2 years of fears, 2 years of tears. We've lived in the moment, we've lived in the laughs, the fears, the tears. Tucking away the first 2 years of her life, never forgetting but not needing to worry about it anymore. The chances of another surgery were unlikely,so why focus on something with such a minute chance of happening? So I have watched my beautiful daughter grow into a beautiful young lady, listening to her dreams of being a baby nurse 3-4 days a week and an artist the rest of the week. I told her if she wants it, she can have it, all she has to do is reach for it and it is hers.

So now here I am, wondering if I started hoping to soon, something I never allowed myself to do when she was a baby. Things were to uncertain back then to dream to far in the future. But here we are, things were certain, as certain as what was you or I,or so it seemed. The hope I once felt is slipping away from me, and I am stuck in a perpetual sense of anxiety and fear, with the occasional bouts of anger mixed in. Never in front of the kids mind you, but there are times I sink down to the floor clutching my chest, counting my breaths, humming a soothing lullaby, anything that will snap myself out of state of panic. Unfortunately it usually ends in tears, big fat ugly, sobbing tears, that wrack your body, that leave you feeling raw and vulnerable. Once the tears have passed, I pick myself up, ashamed I have let myself become over emotional, when she is still alive and well.

 Yet that is the thing, the sense of foreboding is I don't have much time left with her. To many, you will think me saying something like that is a curse, to those of you who have lost will tell me not to say such things. Yet I am telling you this is just a feeling that sits with me, it is a long story so I will keep it short. I have had dreams for years about Lily's life and how it would be. I even dreamed her father and I would divorce and that there would come a time I would yell at him in a hospital room, telling him he was the reason she was there, while my new husband was there with me.

Lily's hand print from my stairway wall
That actually came to pass, it was when she was hospitalized for her kidney infection. I can give you many instances when this has happened, dreams becoming reality.It would just take to long. There is a point to this story, hang in there. To the left you will see Lily's hand print, we did this at a Hopekids event, I have one for each of my children and it goes up my stairs, I love it. Well, the other night I was walking down the stairs and it fell off, only to flutter to my feet in front of me. It left a really dark feeling in me, which I ignored, turning around, hung it back up, and continued on. Well, when I went to go back upstairs, sure enough, it had fallen off again.

Now, 6 years ago, something like this happening, would it have disturbed me, yes, a bit, but I would have let it be. 6 years later, I don't remember how to pull myself up by my bootstraps, and something as trivial as my daughters hand print falling off the wall, sent me into a tailspin of emotions.
Lily's hand missing from the wall line up.

I was left looking at the line up of my children's hands, feeling as if my daughters just jumped out of the line up. Now I know that sounds irrational, that is the crazy PTSD talking, but that was exactly how I felt, and oh God, bile rose in my throat. I wish I could say I was stronger.... I wish I could say I didn't falter in my steps.....but falter I did and strong I was not. I had my moment...I broke down, fell apart, but then pulled myself back together.

So today at therapy I brought this up and my therapist didn't brush me off, She listened to me and said she understands what I am saying of premonitions and such, because she's experienced moments of them too. But she put a spin on it and this is why I love her, and why you will too.

She said, "Jenna, do you think maybe it could have been a sign in, here is a friendly slap from Lily to remind you it has been almost a month since her appointment and you haven't heard anything from cardiology?' Maybe it was just Lily saying, "Mom, please advocate for me, do what you do best."

So I did call cardiology today and left messages and text cardiology to find out Dr.R was in procedures all day. I know they don't see her as a high priority case right now, but Lily's heart was fine back in August 2016. 6 months was all that it took for us to flip so suddenly, and as Dr.R knows, she can backslid further in a blink of an eye. I have to trust my instincts, which are very rusty, add to it she's not with me all the time either, all I can do is stay on top of clinic to get her to cath.

So still, no date until cath, but I am hoping we will have something by the end of next week. I don't think my anxiety can handle much more of this.

Friday, February 24, 2017

We're Back!!!

We're back!! I thought I had retired this blog. That is why I stopped posting on here, very unceremoniously, which I apologize for by the way, I figured Lily was so healthy, there was no reports. We went to the cardiologist every 6 months, got the all clear for another 6 months, wash rinse and repeat.

Now don't get me wrong, we have had some ups and downs medically, such as a kidney infection that hospitalized her. Her INR goes up and down so frequently, and sometimes it shoots so high she becomes a fall risk, that we have to weigh our options of safety. But compared to what used to be, that is nothing, speed bumps. Well, about 2 years ago now, maybe a year and a half, we noticed a hole (VSD) in her heart. At first Dr.R started wondering if she had an infection of the heart, there were so many things thrown around because you don't just get a "new" hole in your heart out of no where. So I went home, stressed, for the first time in years. Cried for the first time in years over her cardiac conditions, then I started thinking about things because it just didn't make sense to me. Well that night I had a dream that took me back to her 4th open heart surgery. Dr.C was telling me how when he was putting in her mechanical valve he noticed a very small hole, but not big enough for him to close. He said it would have done more damage at that time do it, because of how she develops scar tissue. He told her father and I it should close on its own.

So in the morning when I awoke, I dug out the journal I kept from her hospital days, found the entry from her 4th surgery, and sure as the sky is blue, I had it written down. I emailed her cardiologist immediately. (Her surgeon retired, and the hospital she had all her surgeries at had merged all its patients with the children's hospital, so they didn't have all those notes.) So he requested everything, I received and email later that same day telling me I was Clara Voyant and thanked me for keeping that journal as it helped.

So now, fast forward to today. Up until recently, Lily, even though there was that hole, everything was fine, it was there but it wasn't bugging anything. She does have what is called Sick Sinus Syndrome or SSS, but it isn't in a constant state.SSS is pretty a uncommon heart rhythm disorder. It is not specific to a disease, but rather to a group of symptoms that indicate the sinus node, the hearts natural pacemaker is not functioning properly. How did this happen? Well, Lily had an ablation after her 3rd open heart surgery because of her heart arrhythmia, and it weakened that node. Now, when her heart is at a rest rate it dips to a dangerously low rate at some points, it is more common when she sleeps. But it isn't a constant. So we watch, we wait, and one day she will need a pacemaker to keep her heart beating, but no yet.

The hole in her heart started out at .5mm (Think mechanical pencil lead). That's not a big hole, that is why we watched and waited. 6 months ago we were fine, but something shifted, and we think it has to do with her St.Judes mechanical valve. Dr.R thinks it is either 1: to small; which is very unlikely because the valve is a 19mm valve, which is the size of an adult female. OR 2: the valve itself is failing. She has leaking around the valve, which is screwing up the pressures in her heart. We have to watch those carefully because of the repair she has. The hole has gotten bigger and now when the heart is constricting, the blood not only goes through the hole, it gets pulled back, causing a back flow, mixing blood. The hole is still relatively small, I wasn't given a size this last time though.

Our course of action is a cath and a transesophageal echocardiogram. This is to get better views of what is going on in there. At which point they will take all that information to their surgery conferences and start formulating our next steps. If the valve needs to be replaced like he thinks it needs to be, she will have to have another OHS, she will go on bypass, and that scares me. Lily's heart has a really, really hard time restarting. Remember that SSS I was talking about, that comes into play here. I don't want her having a OHS being the reason she has to get a pacemaker.

I am a nervous wreck, as I always am, I will be until it comes time to walk through the fire. On the day of procedures and surgeries, I am so calm, and I know it is God's grace. He'll see me through this, he'll see her through this. And when we come out on the other side, we will be stronger for it, is just the passing storm and learning to dance in the rain again. It's been so long, I guess I've forgotten how.

I will update as soon as we get news of a date, or anything happens with Lily.