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.