As many of our friends know, we are expecting our second sweet baby in November this year.

Yesterday, our tiny baby girl was diagnosed with hypoplastic left heart syndrome.  We spent the afternoon down at CHOP having a fetal echo cardiogram and talking about treatments with the providers there.

Here is what we know so far:
Around four to six weeks gestation, our baby’s heart valves developed incorrectly, causing the right ventricle (which pumps blood to the lungs) to be very large & the left ventricle (which pumps blood to the rest of the body including the brain & other vital organs) to be almost non-existent. They have no idea what causes this to happen, but it is very rare & up until 30 years ago, was always a lethal defect.

Our baby’s heart is not going to get better on its own & the doctors cannot fix it so that she has a normal heart. Fetal circulation is different that circulation of a newborn a couple of ways - baby is getting oxygen from mama, so it doesn’t need to go to the lungs to get oxygenated. Because of this, there are several shunts in place - the foramen ovale (between the two upper heart chambers) and the ductus arteriosus (between the pulmonary system and the aorta). Usually the ductus arteriosus closes a few days after birth, but since our baby’s left ventricle can’t pump blood, they will give her a drug called Prostoglandin to keep this shunt open, which will allow her brain and body to get the oxygen it needs for a few days until surgery can be performed.  She will need a series of three surgeries (one within a few days of birth, one at 4-6 months, and one around two years). The right ventricle will have to do double duty for a while and transport oxygen to both her lungs and heart until the final surgery, when they will somehow reroute the vessels so that the lungs get blood without any pumping. If she makes it through the first surgery, the survival rate is something like 85%.

These procedures are only 30 years old, so the prognosis for adults greater than 20-30 years old is unknown, but the doctor suggested she may have some complications and require additional therapies down the line. Some data that has been collected suggested that these babies might have some developmental delays, but nothing too severe.

We don’t know if the baby has any other anomalies. The ultrasound looked ok besides the heart, but they would need to do an amniocentesis to find out if there are any other problems. If there are other problems, her survival rate will be significantly decreased. They suggested that we have the amniocentesis if it would cause us to electively terminate the pregnancy (this is NOT on the table) or so that we can prepare for other things to come, but it didn’t sound like there was any real benefit to the baby (& there are some risks including induction of preterm labor), so we are weighing the risks & benefits of that.

What we do know is that the baby needs to be born at CHOP or transported to CHOP as soon as possible after birth. We would like to have the baby at CHOP so that I’m not stuck in recovery at Lehigh Valley while our little one is being treated elsewhere. She will have her first surgery within a few days and will remain in the cardiac ICU for a few weeks & then hopefully come home with us.

We are mourning of the idea of having a normal birth and brining a sweet, healthy baby home right away this time, but we are thankful that there are treatment options and that CHOP is only an hour and a half away.

I am overwhelmed and quite honestly, heartbroken -I may spontaneously burst into tears at any given time over the next couple of months. We know we are in God’s hands, though, and are trying to be strong.

We appreciate all of your prayers and messages of love and support.

If you can, please pray specifically for a full term pregnancy with no other complications, that baby girl does not have other anomalies,  that she does well with her surgeries, that my insurance will cover these things, & that I can manage a little more maternity leave this time so that we can adjust to having a sick little one and a toddler. (If anyone at LVHN feels compelled to donate even a tiny amount of vacation to us, we would be extremely grateful - and I know that’s asking a lot, so believe me, it isn’t expected). Please pray for peace for us, too.

Here is the video that CHOP suggested we share with friends and family.

I will share updates here as we have them (but they will be sparse for the first few months).