Jabulani Jonny

Hey all,
There hasn’t been much going on in the last week, but we did have another doctor’s appointment in Atlanta today with the fetal cardiologist.  She was looking again at Baby Payne’s heart to get a better idea of if there is truly an obstructed atrial septum.  Again, the upper two chambers of the heart have setpum (wall) between them, but normally there’s a little hole in the septum to allow blood to mix back and forth.  This helps to regulate pressure in the lungs and helps the lung vessels to develop appropriately.  Well with HLHS (Hypoplastic Left Heart Syndrome) babies, they really need that hole to be there because the lungs have to be near perfect for the 3 repairative operations needed for the HLHS diagnosis to work. 

See, with HLHS, the left side of the heart does not function, or really even form, correctly.  As far as I know, the left side of the heart is tasked with getting blood to the lungs.  If it doesn’t form correctly then no blood gets to the lungs, so the baby doesn’t get oxygen.  Basically the three procedures for HLHS creates a way for the blood to bypass the heart and drain into the lungs using only the difference in pressure.  Follow?  Normally the blood pressure of the lungs is lower than the rest of the body, so the blood will flow to the area of lower pressure, get oxidized and filter to the body.  That’s how the three surgeries create a workable solution to HLHS.  Well, in order for it to work, the lung vessels have to be fully formed so that it creates that low pressure environment that will allow the blood to drain to the lungs.

Enter the Obstructed Atrial Septum diagnosis.  Remember when I said it was normal for babies to have a hole in the atrial septum to allow blood to mix back and forth?  Well that is extremely important for babies with HLHS.  What that hole, or perforation, helps to do is to regulate the pressure in the lungs while the baby is in the womb.  If that blood flow doesn’t mix back and forth, the lungs build up pressure which means the lung vessels get thickened walls, kind of a muscular build up of the vessels.  What that does is increase the pressure within the lungs, which means that the procedures needed to repair the HLHS may not work if the vessels are not able to fully develop and lower the pressure in the lungs. 

So as we were looking at the obstructed atrial septum and the HLHS issues, we had a long talk with the fetal cardiologist.  For babies with the obstructed atrial septum to have a chance at all, you pretty much have to deliver in one room and do an immediate heart cath to open the hole in the septum to allow the pressure to stabilize in the lungs.  Even with doing that though, some babies’ lung vessels don’t fully recover and they won’t be considered good candidates for the three surgeries needed for the HLHS.  Children’s Hospital of Philadelphia is one of the leading children’s hospitals in the nation at these types of procedures.  Mind you, this procedure in particular is still fairly new, relatively speaking, and babies with HLHS and an obstructed atrial septum have about a 50% chance of survival, or less. 

We will probably have to go to Philadelphia when Sunette is 36 weeks along.  She’s currently at 34.  So we have two weeks to get ready to go to Philadelphia.  Did I mention that we were in the middle of trying to get our house sold and move to Columbus?  Not to mention all the other "life" stuff that we have.  We will probably be in Philadelphia for several weeks after birth, for the baby to recover from the first of the HLHS procedures. 

So that’s where we are right now.  We covet your thoughts and prayers.  We continue to thank God for blessing us with this child.  We consider a miracle that we got pregnant in the first place.  I just don’t see us getting this far and not coming home from Philly with our baby.