We're In For a Long and Wild Ride or, What I Know So Far

We've pretty well established at this point that I like to research and plan.  These are the things that calm me when I'm freaked out.  Plus, I'd rather learn this stuff while I have the time and energy to process it, rather than when I am recovering physically and emotionally from birth, let alone the wringer of open-heart surgery on my child.  So, it should come as no surprise that I've spent a lot of time this last week researching Jellybean's condition.  Obviously, any of the information I've found is subject to change based on Jellybean's specific situation, official diagnosis, and the protocols of CHM, but it's helpful to have some sense of the breadth of the challenge we're facing.  That said, here's what I've found so far.

The CDC reports that, each year, only 960 babies in the US are born with hypoplastic left heart syndrome (HLHS), or 1 in 4,344.  According to Boston Children's Hospital, HLHS "is one of the most challenging congenital heart defects to care for."  Everything I have found certainly supports this statement.

The first surgery--the one done at 7-10 days of life--lasts approximately 6 hours.  After it's completed, they wait several days before closing the chest to prevent the compression of the heart by the rib cage until the heart has had a chance to adjust to the new circulation.  After surgery, kids remain in the hospital about a month.  The good news is that the success rate of this surgery is high, with 90% or more of the kids making it home.  However, of those that make it home, another 10-15% are lost before the second surgery.

The care at home between the two surgeries looks pretty intense.  In addition to the weekly check-ups with the cardiologist, and likely lots of meds, Jellybean will probably be on oxygen and an apnea monitor.  Weight must be checked daily to make sure he's eating enough, and a pulse-ox monitor has to be used twice a day to check his oxygen saturation.  He may be on a feeding tube, depending on whether he's strong enough to feed himself; the low oxygenination caused by the mixed blood flowing through the body can make children suffering from HLHS too tired to feed well, so they often need supplements to make sure they get more calories per swallow.  Sometimes, because they had an intubation tube in so early, they associate having something in their mouth with that sensation, resulting in difficulty taking a bottle or breastfeeding.

Although I've found descriptions of the second and third surgeries, I haven't found much on recovery or mortality stats or care afterwards.  But, quite honestly, that's about a year away, and based on what I've already learned, I will have so much on my plate between now and then, I'm not sure I need to know more just yet.  The goal, for now, is to be ready, rested, and as prepared as possible for whenever Jellybean arrives, because once that happens, the roller coaster is gonna take a sudden, turn down a high, steep curve that will take my breath away, and I don't want to fall out.

Just Another Manic Monday

So what do we do?
Nothing. Strangely enough, it all turns out well.
How?
I don't know. It's a mystery.
-Shakespeare In Love

Today was our third fetal echocardiogram and, although we still don't have a firm diagnosis, in terms of what needs to happen, the official diagnosis doesn't much matter at this point.  What we know:  Jellybean's left ventricle is still significantly smaller than the right.  In fact, it is no longer growing like they had hoped, as it no longer reaches the apex.  In addition, the mitral valve which serves the left ventricle is also small.  This means that Jellybean has hypoplastic left heart syndrome.  In addition, he only has one great artery.  There should be 2--the aorta and the pulmonary artery.  He appears to have an aorta, given its shape, but it looks like it's attached to the right ventricle, which is where a pulmonary artery would be.  Thus, they aren't quite certain which one he has.  It could also still be truncus arteriosus, but that diagnosis requires certain features they aren't certain he has.

Ultimately, the result is the same for either diagnosis.  He needs a series of three surgeries, the first of which occurs at 7 to 10 days.  The second surgery generally occurs at 6-9 months, although it can be done as early as 3 months if necessary, or as late as 12 months if everything is going well.  The third surgery is done anywhere from 18 months to 3 years depending on how the child is doing.

Mortality is highest between the first and second surgery.  75 percent of children who get through all three surgeries survive.  15 percent of those who survive go on to have serious complications.  They have only been doing this for roughly 30 years, so they have no idea about long-term survival.  They can say that they have lots of patients in their 30s, which is good news.  The cardiologist indicated that she has some patients who have had these procedures done who are competing in college-level athletics, so there can be some degree of normalcy.  There's no way to say up front which category Jellybean will fall into.  We'll just have to wait and see.

So, what does all of this mean for us?  Well, Jellybean will need to have an ultrasound and be placed on prostaglandins and an IV shortly after birth, so they want me to deliver in Detroit.  We are in the process of scheduling an appointment with an OB recommended by the cardiologist.  However, the more problematic issue is that they want me to have a spontaneous, vaginal delivery as close to 40 weeks as possible.  Because the surgery will require him to be on by-pass, they need his lungs in the best condition they can be, which means letting him cook as long as possible.  In addition, because vaginal delivery helps squeeze fluid from the lungs better, they don't want a c-section.  At the same time, they don't want him in there too long, so induction may be necessary if we go too far past 40 weeks.  Ideally, I would be somewhere closer than 2 hours away from Detroit when I went into labor.  As of yet, we haven't quite figured out how to make this happen.

Assuming delivery goes as needed, he will be in the NICU at the women's hospital for roughly 4 days before being transferred to the NICU at the children's hospital where they will then ultimately do surgery #1.  We don't yet know how long to expect him to be in the hospital post-surgery, but upon discharge, we can expect to go to the doctor weekly until surgery #2.  We are hoping we will be able to see the local cardiologist for these visits and not have weekly trips to Detroit, but we don't yet know about this.  We also don't know if/when Jellybean is allowed to go to daycare, which makes my ability to return to work a huge issue.

We are continuing down what appears to be an extremely long and stressful road, with no real end in sight.  We certainly have our work cut out for us figuring out the logistics of how to make all of this work in the best way possible.  Sadly, this diagnosis means we will not be going anywhere for Thanksgiving, Christmas, or New Year's and it means we can't have the celebration come to us because too many visitors would place Jellybean at increased risk of infection.  Given the likely timing of the second surgery, we also likely won't be able to attend family reunions and our favorite gaming convention next summer.

When we moved forward with having a second child, it never occurred to me that he might require more medical care than Lil' Bit.  But, it is what it is.  All we can do now is research, plan, and prepare as best we are able and know that things will work out, even if it seems like a huge mystery at this point.