WHAT IS HLHS?

Between visits with various doctors and reading our 'homework' (material from said docs), we have a lot of information on hand regarding our sweet baby's heart.  Depending on how you look at it, there's some good news and some bad, but overall we have hope.  I've tried to break it down to the nitty gritty so as to avoid a final exam type of explanation.  Here's to hoping that I haven't butchered it too much.  Feel free to ask any questions, as I know we've had a million and one so far.

Hypoplastic Left Heart Syndrome is a congenital heart defect.  Most of the structures on the left side of the heart are small and underdeveloped.  This is a problem because this side of the heart pumps blood to the body which means the body can't get the appropriate amount of blood needed to support life.  A baby left untreated will become very sick after birth and if left untreated will not survive.

Essentially the 3-step surgical process will take a patient with a single functioning ventricle and create a heart that is able to support her long term.  The first surgery is performed in the first week of life, depending on the baby's specific anatomy.  The goal is to create a way to increase blood flow to the lungs via a "shunt."  This is often the most stressful surgery for a number of reasons.  Technically it is challenging because of the small size of the baby and her heart.  Too, newborns are physiologically more fragile than older children.  Some babies may require surgery on birth day while others are stable enough to wait until day 4 or 5.

The second surgery (a bidirectional Glenn shunt) is performed at around 6 months.  Again, timing is based around baby's specific condition, ie oxygen saturation levels, etc.  After her first surgery, her body will be at around 80-85% but by the time of her second surgery, she will be at around 70ish%.  Healthy heart people operate at 100%.  This operation allows for the passive return of blue (deoxygenated) blood, coming back from the upper part of the body, directly into the lungs. This is the first step at in lessening the workload on the single ventricle (right).  It significantly reduces the amount of blue blood that the single ventricle has to pump.

The third surgery (Fontan procedure) occurs when the baby is around 3 or 4 years of age.  During this operation, the heart achieves a normal workload.  In addition, the heart is appropriately septated.  In other words, the red (oxygenated) and blue sides of the heart are separated from each other and there isn't further mixing of blood.  Most patients have normal or near-normal oxygen saturation.  It also seals off any open communication between the blue and red sides of circulation.  In this way it significantly lessens the long-term potential complication of blood clots passing from either side.

She will also have at least 2 other surgeries - heart catheterizations - which are performed before each major operation to 'check out' the heart so there are no surprises during surgery.

The long-term results of this surgical process are still unknown as the operation has only been done commonly for the last 15 years or so.  The oldest surviving patients are in their mid 20s.  Whether this form of heart circulation will last for a lifetime is yet to be determined.  However even in this short time, enhancements have been made to the actual surgeries.  We are very blessed to be in the year 2011 and having options like this surgical process versus being sent home to wait.

Most children will reach adulthood in good health.  Some children will need other procedures such as a pacemaker and some will need a heart transplant at some point in the future.  Children are restricted from competitive sports and demanding physical activities but can otherwise do all the things that children enjoy doing, as well as enjoying a good quality of life.  So we don't have an Olympian on our hands, but that's okay...Mommy's not either.

MATTERS OF THE HEART

We met with our pediatric cardi and got to see our sweet baby girl's heart. She is looking great! The right side and all of its important parts look healthy. PRAISE. And as expected, the left side structures look the same. There is still some blood flow which is a good thing. We pray specifically that the blood flow is able to continue through the left ventricle and that it will GROW. According to medicine, it is unlikely, but God has healed the blind, so the impossible can certainly happen.

As usual, our little bit was a busy body, so it was hard to capture the images of her heart at times. We watched her pull both knees up to her chest only to then kick with all of her might. It was great to watch it on the screen as well as to feel it. And later, we got to see her PERFECTLY PRECIOUS baby feet. Warm my heart.

Things seem to be picking up now but our appointments are still about 3-4 weeks apart. Not much changes during pregnancy so we will take this 'lull' in developments. Specifically, we can pray for the following things:

-for the left ventricle of her heart to grow.

-for the right side of her heart to continue to be healthy so it can support the left side. it will become the real hero of her body post birth.

-for the rest of her organs to continue to be healthy, because compounding her condition with additional problems would be life-threatening.

-for her lungs to continue to develop until 39 weeks when she is delivered.

As always, thank you for praying for our baby...and our family. We pray for peace beyond understanding and know that through the power of prayer, miracles do happen.

NO NEW NEWS

In terms of Baby's development, there is no new news!!  PRAISE THE LORD.  We had a sonogram today at 21 weeks and she is looking good, according to Dr. Weiss, our maternal fetal medicine specialist.  Her measurements were normal as was her beautiful-sounding heartbeat and organ development (besides her heart.)  She is the size of a carrot this week and I feel her poking around inside all the time.  Perhaps from habit, I find myself looking down as if to see the faint imprint of a foot.  Soon enough, I'm sure.  

Her heart looked the same.  We'll get a better, more detailed look on Monday when we have an echo-cardiogram done.  We'll also hopefully get to understand a bit more about her anatomy specifically.  I look forward to this so that we can pray specifically for small miracles in its development.  

We truly feel carried by your prayers and have since we've shared our news.  It's by God's grace that we are so at peace.  God's plan is a good one, despite it being a little different than what we may have "planned" for ourselves.  Trusting in Him gives me peace knowing that I don't have to play event planner, because He already is.  In retrospect, I realize that although I truly believed God was in control of my life, that I had "let go and let Him" take the reigns, that I hadn't quite.  It was when I walked through darkness and truly surrendered, that I had finally let go.  And what a liberating feeling it really is.  Liberating in the sense that no matter how much pain I may suffer, there is always hope.  There is always peace.  There is always God.