The problem with blogging, or more accurately NOT blogging, is you get used to not blogging and then you have so much time to make up.

The last I blogged James was born. Today he is in his swing in our den at home sleeping. How did we get from birth with a breathing tube to the swing? Lots of blanks for me to fill. And I will. I hope you can bear with me. I want everyone to know his journey.

Once James was born all the doctors did their assessments and tests and determined him to be in good health, minus a small little life threatening heart defect ;).

Jason and I were thrust into a whole new world that we thought we were prepared for but you realize quickly how unprepared you really are. We learned about oxygen saturation percentages and heart rates and flow vs. liters and versed (and I'm not sure if that's how you really spell it).

During this initial assessment an echocardiogram was done and found James to NOT have Hypoplastic Left Heart if you can believe that! His left side was small but it was pumping! He was downgraded to critical aortic stenosis with a hypoplastic aortic arch. What does that mean exactly? Well, I don't know. Not exactly. But I got the jest of it. Aortic stenosis and mitral valve stenosis are the beginning factors of HLHS. Since James's mitral valve was not as bad as they originally thought. He still had many issues. When I say he was downgraded this isn't to mean he was ok by any stretch of the imagination. But now we had a few different options. We could continue on with the Norwood or we could try for a catheterization procedure to balloon open the aortic arch which would hopefully allow more blood flow through the arch and therefore allow the left ventricle to grow and keep him a two ventricle baby. Any time you can keep a child a two ventricle baby this is obviously the preferred method. But would Baby Brother be a good candidate for this procedure? The doctors would discuss it at Cath Conference on the next Monday morning.

I spent the weekend being wheeled between my room and James' room. Jason slept at James' bedside the entire time except for one night when my sister slept in the room with him. We got a room at the Ronald McDonald House and when I was released on Sunday I tried to settle in.

All of our family left by Sunday except for Jason's mom. She stayed with me. The plan was for her to stay with me the first week and my father in law to go back with Jason which would help Jason get the kids to and from school and just generally help out. They would come back the next weekend at which point they would all go back leaving me alone but only for a day or two when my in laws would be back for another couple of days before they had to leave to go back to England. They would leave on Thursday and Jason and the kids would come back on Friday and then my mom would come and spend week 3 with me. This would get me through the first surgery and some recovery time before having to be alone.

Monday morning at Cath Conference they decided James was a good candidate for the valvuloplasty of the aortic valve.

So James would go to the Cath Lab on Tuesday, January 7th.

Option 1:

Intervention ~ HEORIC MEASURES

(In other words not fully expected to work)

Planned C-Section in the Wolfson's Heart Cath Lab.

They are expecting Baby Brother's condition to be so deteriorated that they can't risk the time it would take to get him from the regular surgical room to the cath lab so delivery in the cath lab is ideal. There are two rooms in the cath lab so one would be set up as my delivery room and the other set up to immediately perform the atrial septostomy.

On hand will be the adult surgical team for me to include anesthesiology and my OB. Then there will be the Cardiologist team, NICU team and Echmo team for him. Lots of scheduling to do so delivery will most likely be moved up to December 27th or 30th. For those who may not know, like myself, the Echmo team is the bypass team. This is the bypass machine that keeps people alive when having open heart surgery or some other procedure that requires bypass.

As soon as Baby Brother is pulled from my belly they will rush him to his side of the cath lab to perform the surgery. There was a bit of debate between Jason and I as to what to call this procedure. I keep saying catheterization because they way they get in to do the atrial septostomy is through the groin in the same way a heart cath is done. Jason says it's surgery because, well, it is. I'm not denying it is a surgical procedure, just trying to differentiate the difference between going through the groin as opposed to actually opening his chest. (if that make sense to you)

They would try to perform the atrial septostomy immediately and this would hopefully work to open his atrial septum. The struggle is they think Baby Brother will be so sick that he will need to go on bypass. Why is this a problem? (Besides the obvious!) This is a problem because in a normal catheterization you go in through the groin, perform the procedure, come back out, put lots of pressure on the incision area until bleeding can cauterize. If he has to go on bypass then the problem comes with bleeding. A bypass machine is constantly pushing blood through it. The mechanisms are plastic. So the blood flows through plastic tubes, essentially. Because of this they have to use blood thinners in the blood to prevent the blood from cauterizing in the tubes. If they are using blood thinners in the blood then thinned blood is what is going into Baby Brother. Once they go to pull the needle (or whatever it is) out of his groin there will be no way to stop the bleeding and he will bleed out. If they can get the needle out and stop the bleeding then Baby Brother has to start fighting to get off that machine. They will only keep him on it for 2 weeks before we have to make some very hard decisions. The other problem with the bypass and thinned blood is the risk of bleeding on the brain.

I asked if it was possible, in a perfect world of course, for them to get the procedure done before Baby Brother would have to go on the bypass machine. She said Yes! Absolutely. But that would be a perfect scenario and so far your baby hasn't wanted to do anything perfect.

She said the first 48 hours are going to be crucial. That's when they would expect him to "crash and burn".

Once he comes off bypass (if he even goes on, I'm still praying!) and is making strides, then and only then will they determine if he is a good candidate for the Norwood procedure. That is the first procedure to mend the Hypoplastic Left Heart.

If we choose this option Dr. Lacey wants us to reconsider an amniocentesis to check for chromosomal abnormalities. She wants us to make as an informed decision as possible. To a layman she said let's see if there is anything else going on with this little guy because you may not want to fix a heart that's attached to a down syndrome baby. This is the part that has me MAD! She never specifically used a specific abnormality but I can read between the lines. Don't get me wrong she is a very nice lady and a wonderful cardiologist BUT I didn't have an amnio for a reason and the reason is I DON'T CARE! Jason and I agreed a long long time ago the risk of the amnio is too great. Especially since the results would not change a single thing. If this baby had Downs or any other something, you know like Hypoplastic Left Heart, we would still treat him like the gift from God that he is. He is a precious spirit that Heavenly Father thought Jason and I capable of handling and I will not play God! If HLHS doesn't scare me (even though it kinda does) then some other abnormality never will! I'm sorry, I'll step off my soap box. Bottom line is we have declined, yet again, an amnio. This is also the reason for determining if he will be a candidate for the Norwood. I suppose if he has something else wrong they will decline to operate on him. I'm not sure what makes one baby a good candidate and another not but they will operate on my son! If he can make it through the atrial septostomy under all that pressure then the Norwood will be a piece of cake.

***WARNING*** THIS IS A LONG POST WITH SOME VERY UNCOMFORTABLE CONTENT

Thursday started out a normal Thursday. I went to class and after school I received a phone call. It was Samantha in Dr. Lacey's office. She wanted to remind me of my appointment at 1 (the appointment has always been scheduled through Wolfson's and therefore the automated system calls me a couple of days before and I have to press whatever number to confirm. Dr. Lacey's office has NEVER called to confirm the appointment.) and to make sure my husband will be with me. The last two appointments my mother  and mother in law had attended with me and my father was planning on attending Thursday's appointment. So I said "well he wasn't planning on it" and Samantha said Dr. Lacey was specifically requesting he be there because they had quite a bit information to go over with the both of us. I said I would call my husband and try to get him there.

I called Jason at work and of all the days this was the one day he had tons of work to do. We were both hoping this was more of a "hey, we're getting close to delivery so we wanted to kinda go over things" but at the same time we have Fetal Clinic scheduled for December 6th to go over those things. He said he would make the time because if she's asking for him to be there it was obviously important. I called back just because I was curious and asked if the information was about delivery or about the diagnosis. I was informed it was about both. Great ~ Now I've got the next several hours to worry about what on earth the doctor could tell us. What could have changed between two weeks ago and now? and how would Dr. Lacey even know since she hasn't seen me in those two weeks?

I got to the appointment first. The agreement was I would text Jason once they were about 20 minutes from being done with the scan. They can take upwards of an hour so what was the point in making him sit through that? He was about 15 minutes away so that would give him time to get there before the doctor came in. When I got to Wolfson's to check in the woman said "oh, they just called asking if you were here yet". Really? I got there 5 minutes BEFORE my appointment. This anxiety to see me was making me even more anxious! Needless to say I wasn't sitting for very long with the Cable lady came and got me. (her last name is cable...) She did the scan, I texted Jason, Dr. Lacey came in for a couple more views, Jason got there and we sat down to hear the news.

Forgive me for not having all the information from that appointment but there was a point where I couldn't breathe, much less write.

Dr. Lacey starts by telling us she has been in several meetings the last two weeks. (remember, we famous. Now we're about to become infamous!) She has met with her Cardiologist team, the surgical team, the high risk team, the this team, the that team.. they all have the same opinion. Baby Brother is not well. They have been watching his atrial septum deteriorate over the last couple of scans and just as she expected, it is even worse with this scan. I've said before his atrial septum must be open and floppy. His has thickened and become restricted. At the last appointment she explained the need for an atrial septostomy. This hasn't changed. The difference is now it's even more dangerous. There is a number grading system for the seriousness of the restriction of his atrial septum. They take his scans and the doppler from those scans and they measure length between one output and the other and come up with this grade. 5 or greater is a mild restriction. 5 or less is moderate to severe restriction. (I'm still curious what 5 actually means...) So Baby Brother's grade was 4. This indicates moderate to severe but Dr. Lacey is confident his restriction is not moderate. At the last appointment he presented with a mild case but the appointment two weeks before that he was still open and floppy so you can see the deterioration in his condition. {Side note ~ I'm sweating just typing all this out. Takes me back to being at the appointment where my face went flush, there was ringing in my hears and it sounded like the Charlie Brown teacher trying to talk to us} This situation is putting increased pressure on the lungs and pulmonary veins in the heart.

Extraordinarily high risk. That is the description she used over and over again. She has downgraded his survival chances. He was given 80% originally. Now she said it's "50/50 and it may not be that good".

We now have two options

Option 1:

Planned C-Section to be performed in the Wolfson's Heart Cath Lab. This has NEVER been done before at Wolfson's. Ever. Jason asked if they would name the procedure after me. Dr. Lacey laughed and said "maybe the room".

Option 2:

Do nothing and enjoy what little time we may have with him holding him, loving him, kissing him, taking pictures with him and then letting him go.

{sorry ~ will go into greater detail of these options in different posts as this is causing me too much pain right now. And there is so much information.}

My mom went with me to my pediatric cardiologist appointment this time. It was nice to have her there. I'm glad she got to go and talk to the doctor and try to understand a little bit more what is going on. It also turned out to be wonderful to have her there for support.

And it has nothing to do with Baby Brother....

But we'll take it!!

Actually, we don't really have good news. We just haven't had any bad news and that, in and of itself, is good news.

This has been a good week. Blogging and sharing our story with our loved ones and friends has been very cathartic for me. It has allowed me to talk about this in a way I couldn't before with the people I want to lean on.

My emotions are running high. So very very high. I just never know when it's going to hit me. In the beginning I couldn't start this story without the tears coming on full force. Now I can get through 2/3 of the story before they let loose. This is progress! I'm finding great comfort in knowledge. You know the old adage Knowledge Is Power, well that's exactly how I feel. Interestingly I have found for Jason it isn't exactly the same. He wants to know about the procedure and about length of stay and those sorts of things but feels he doesn't need to know about the things that don't matter. Now, don't get him wrong, everything matters, but what he means by that is I got some advice to ask about a particular part of Baby Brother's heart and it's condition because that *should* tell us if this will be a very serious case or a normal serious case. So I want to know that information. Jason doesn't. Because knowing the answer won't change the fact he will need surgery or that there may or may not be complications. When I sit back and really think about it he's right. The answer to that question doesn't really matter. BUT - I still want to know the answer. Now that I know about HLHS I want to become an expert. I know there are many of us like that out there. There is a point where you know too much so I'm toeing the line. Making sure I learn enough but not too much.

I have had so much support from the heart community. They all say the same thing - not really the club they want to welcome people into but one of the best communities there are. I feel very fortunate to have the friends and family I have who then connect me with even more people in this community. I have had some really wonderful advice. One woman even wants to help me put together a health binder. Said it really helped her going through the process. I have a shopping list! Binder and 2 packs of dividers. It makes me feel like I'm making progress and really preparing myself for what's to come.

As a final note I want to address a very sweet friend's concern. She was brave enough and loved me enough to admit she felt conflicted reporting her good news about her baby girl due in November while I am going through such a hard time. I can't tell you how this touched my heart. Here is my friend who has struggled for 10 years to have a baby and she's concerned about hurting my feelings because of my situation. So ~ to all our friends and family ~ if you stop sharing your good news I will be hurt! I NEED to hear the good news. I WANT to laugh and love and live your journey with you. This is so important to me. When I can revel in your good news my bad news doesn't seem *so* bad.

Sorry if this post seems to go all over the place. Once I start typing I can't control where my thoughts might lead. (I should probably use this as a warning on all my posts from here on out)

PS ~ Spellcheck really hates HLHS and Hypoplastic.

Hearing this diagnosis was the first time I had ever heard of such a thing. It is a Congenital Heart Defect (CHD). There is no immediate family history in my family or Jason's of Hypoplastic Left Heart Syndrome but there doesn't need to be. There are so many unexplained diagnosis(es)? Diagnosi? What is the plural of diagnosis???

Here is an interesting CHD fact: 1 in 100 children will be born with a CHD.

HLHS? 1 in 5000 children will be born with HLHS. Two-thirds will be boys.

Scarier even? There are SOOOOOO MANY CHD's.

Interesting Points: My cousin's son (on my dad's side) was born with HLHS and is today a thriving little boy. This gives me hope! I am now "friends" with another "heart mom" (through facebook and email, that's why the quotes around friends...haha) whose son had HLHS and some other issues and he is now a thriving little boy. This gives me even more hope!

I never envisioned myself as a "heart mom" but this is our new normal. I'm a heart mom! Jason is a heart dad! I'm not sure there is a better man suited for the job.

There is still the stages of grief I am going through. I have accepted the diagnosis but I'm grieving over what could have been. It's very selfish of me and I'm trying so hard to let go of this. I've been reassured this is extremely normal.

I found a great and informative animation on youtube. It's a little long but well worth the 7 minutes if you are interested in HLHS. It also goes through the procedures Baby Brother will endure.




We continue to ask for prayers for our family. We need Baby Brother to be big and strong! All of him! Especially his little heart.