Bittersweet - The beginning of a long, arduous road

Sleepy girl

Most of you probably wonder where we got the name Mireya.  It is a Spanish name that means "miracle".  In picking a name, we knew we had to pick a name with meaning.  I knew we couldn't name our oldest Hope and give our second child a meaningless name, but at the same time, we didn't want to get all cliche and name her Faith, Grace or Charity.  We struggled with this choice.  Hope came naturally to us.  With all we had gone through to get to her, and all that we knew we had ahead, it was obvious.  For months, we looked up names with so many meanings - miracle, strength, warrior, love, etc.  Mireya was different and I liked that it was Spanish.  I have always liked the French derivative of it, but was afraid of how people would pronounce it.  Mireya is a little easier.  She will be Reya for short.  Given what we've learned about her since her birth, it seems to be the perfect fit.    

Today I am being discharged.  Without my baby.  I've done this before - it might be one of the worst feelings as a mother.  I knew this day would happen, again, but it doesn't make it any easier.  The birth of a child is supposed to be a deliriously happy time. The way we do it seems to always be filled with worry, stress, anxiety, sadness.   I see the other families on the postpartum floor gathering in their rooms, which honestly are more like hotel rooms than hospital rooms.  They are joyous and happy.  Guests come in and out.  Babies cry.  But when I am here, I am sitting here alone, my only company the whir or the yellow, hospital grade breast pump.  Nurses come in, and it feels awkward.  They are used to being cheerful and happy, because most of their patients are experiencing the happiest time in their life.  They all know where my baby is and what the current plan is - its in my records.  They let me come and go, and accept that I won't be in my room most of the time.    They are supportive, but reserved.

Mireya has been pretty sleepy since her fast and fierce arrival.  I guess it was exhausting.  We have been busy since then trying to figure her out.  She has definitely thrown us a few curve balls in addition to her quick entry into the world.  

Thursday night, when they went down to the NICU, Cardiology came over from Children's to check her out.  The news is not as good as we could have hoped, which is why it has taken me a little bit to post about her condition, as we were waiting to talk to our own cardiologist and see what he thought the plan would be.  Cardiology ended up doing two echos on Thursday night because they couldn't find the pulmonary artery in the first echo.  Then they couldn't find it in the second echo either.  They spent about two hours looking for it, without finding it.   They agreed upon an official diagnosis - Tetralogy of Fallot with Pulmonary Atresia and MAPCAs.  When we learned that Mireya also had a CHD, there were three possible diagnoses - Truncus Arteriosus, TOF / PA (no MAPCAs), TOF / PA / MAPCAs.  The first two were the more favorable diagnoses.  TOF / PA / MAPCAs is on the spectrum of worst case scenario in this situation and Mireya is a moderate case.  When Hope was diagnosed, the MAPCAs was the uncertain part and we were so relieved when we learned that she only had two collaterals, essentially taking the MAPCAs out of her diagnosis.  The MAPCAs make things a lot more challenging.  Our cardiologist, Dr Friedman, came by yesterday afternoon to help make some decisions and a final diagnosis.  He did another echo and was able to locate the pulmonary arteries, which is definitely good.  It makes things a little easier.  

So what is MAPCAs?  "MAPCAs" stands for "multiple aortopulmonary collateral arteries."  In TOF with PA, the pulmonary valve is closed, making blood flow through the pulmonary arteries to the lungs impossible.  The body is then either dependent upon a Patent Ductus Arteriosus (PDA) or these MAPCAs to deliver blood to the lungs for oxygenation.   The problem with them is that it is not an easy fix.  With Hope, she only had two collaterals, so the were easily replumbed into her anatomy, a conduit was placed between the heart and lungs, and the VSD (the hole between her chambers) was patched.  In Mireya's case, she has at least 3 and maybe up to 15 collaterals, but probably on the higher end of that spectrum, so it's not as easy to just plug them in. It will be a multistep process.  Her pulmonary arteries are small, so first she will need to get a little bit bigger.  Once she is bigger, in a few months, they will place a conduit, which will allow blood flow to go in the appropriate direction.  This will require open heart surgery.  Then they will start the process of figuring out what to do with the MAPCAs.  Some will be small and unnecessary and will be gotten rid of.  Others will be bigger and important and need to be kept.  Others will be small, but important, so they will need to be dilated by catheterization.  Once the collaterals are in position, the VSD will be closed via open heart surgery.  Up to this point, it has to stay open to help control the pressure in the right ventricle, otherwise you risk heart failure.  TOF / PA / MAPCAs is a multi-organ (heart-lungs) illness and if things were to get really bad, transplant is not really an option.  This is the simplest explanation I can give.  MAPCAs is a dreaded diagnosis to many.  It is rare, complicated, and no two cases are alike, making it harder to fix.  In summary, over the next few years, Mireya will undergo 2 or more open heart surgeries and many trips to the cath lab.  We will be in and out of the hospital - 2 days here, a week there, etc.   When we're not in the hospital we'll be going to check ups. Because of all the hospital stays, she's at higher risk for developmental delays.  Due to her anatomy, she may be more physically restricted because she may not tolerate heavy exercise.  

On a positive note, she will be coming home sooner rather than later.  This wasn't the desired plan.  I guess you could say its a concession prize for the long road we have ahead.  We were hoping to get the surgery over and done with and get on with life.  We had been warned during our fetal echo that if Mireya were to have MAPCAs, this is what would happen, but we felt pretty confident that we had ruled it out.  So we were sort of blindsided by this new turn.  Its funny how you are affected by what you are familiar with.  After Dr Friedman made his final diagnosis, the NICU team was all celebratory.  They were all saying, well that's great news!  In their world, going home is the end result and means success.  In our world, going home is not what we wanted right now and is not necessarily a happy thing.  It means we have a lot ahead of us.  I am trying to be happy about it - I know there are so many parents that spend time in the NICU that never get to take their babies home, so I am keeping those parents close to my heart, but at the same time, I am tired and want this to be over already.  I will try my hardest to enjoy bonding with my daughter and helping her grow big and strong, so that she can kick CHD's butt.  We will get to experience things with her that we did not experience with Hope.  When we brought Hope home, she was 6 weeks old.  She had been sleeping on her own in a crib, so she was already a fantastic sleeper.  She was already on a solid eating schedule.  While she was helpless, sick, and small, she was not as much a floppy newborn, but much more on her way to being an infant.  Mireya will come home as a newborn.  Hopefully she will learn to nurse and will be able to continue that post-op, relieving me from full-time pumping.  We will be able to bond with her earlier, so perhaps she will be more affectionate.  In the long run, this may be better for Hope, because it won't take us away from her for an extended period of time, but instead a little bit here and a little bit there.    

So please forgive me if we start to fall off the face of the earth in the coming months / years.  Know that we still care about you, but are busy trying to help our newest warrior conquer CHD.  We still love each and every one of you and are so grateful for the support so many of you have offered us as we fought it with Hope.  We are touched by the well-wishes and congratulations we've received as Mireya entered the world.  We appreciate and are humbled by all that our friends and family have done for us over the past few years.  It has not been easy and the end is no longer in sight.  There is a long road, arduous road ahead.           

We will get through this. God has a plan, and he chose us.  We have been lucky enough to be given another miracle and we will make the most of it.    

Squinting at the bright lights...where am I?

Happy Dad