So I started my job (same law firm, new location) in St. Louis on Monday. Wednesday I had a fetal MRI done and yesterday we met with a whole bunch of doctors...neonatologist, uroligist, pediatric neurosurgeon, OB (MFM specialist), ultrasound, nurse, NICU coordinator, took a tour of the NICU, genetic counselor, family counselor, footprints coordinator....literally all of those people we met with! We were at the Fetal Care Center at Cardinal Glennon from 8AM until 3:30PM. (My work is beyond understanding!) So here is a summary (yes summary) of what we found out:
Evie's Spina Bifida (SB) starts at L4 and goes somewhere between S2-S3/4 (it is really hard to tell in this region since the Sacral area is 5 vertebrae all fused together to form one big bone, they won't be able to tell EXACTLY where it starts closing again until she is born). The SB is completely open. When we did our research on the internet (just FYI if you ever get a scary diagnosis try as best you can to stay off the internet!) and saw diagrams of what SB was and it will show a little baby with a spina cord that kind of goes and then comes out, loops up and then goes back in. Hers doesn't do that. It never loops up. It is in place all the way down but there is NO covering. No cyst, no skin layer (that they can see) no anything. It is quite literally a gaping hole in her back, exposing all of her nerves.
This is the biggest problem at the moment. Since there is nothing over the spine her nerves are being damaged by the amniotic fluid and her spinal fluid isn't able to be contained. Also for concern, the area in which the opening is, is right where all the nerve endings come together. So there are multiple nerves being damaged, not just 1. We anticipate with this location Evie will be able to have good function of her hips and thigs but the bottom of her legs will more than likely be pretty weak and she may need leg braces to help support her to walk. This isn't to say she won't need a walker or crutches to learn to walk and most of her motor skills will probably be delayed.
The hardest part for me emotionally about the back issue of this diagnosis is knowing that there is no covering. Her nerves are nicely tucked away and protected. They kind of go in and out of the back and float around. I think of like seaweed, it floats and moves around like that (in my head) -- the doctor agreed that is sort of what they are doing. I haven't met or talked to anyone who has a child with SB that hasn't had a cyst or skin covering the nerves so I am really unsure what to expect at this point.
Also -- to make matters MORE confusing...what Evie has going on isn't technically, technically SB. SB is when the cord either comes out and has a cyst or covering or doesn't come out and has a cyst or covering. So while it is classified as Spina Bifida for a generality, it is really just what they call an open neural tube defect. Again, don't look it up on the internet, the internet is far scarier than what I think we are dealing with.
Our doctor did reassure us that this case is what he would consider to be a "small to medium" sized cased. He has seen much, much worse and everything else with Evie (will explain later), is looking good to this point.
So, those are the back issues. With SB there are other issues with the brain and legs to deal with, though.
So let's do the legs. Evie's legs are normal right now. Her feet are not clubbed and are pointing in the direction they should. Her bones look nice and straight at this point. While this is FABULOUS news, that doesn't necesarily mean it will stay that way. Since there are so many nerves being damaged from floating around there is a possibility that the nerves could get damaged down the road and cause club feet or something else. At this point we are just believing that God is taking care of her and will protect her and heal her. We are at peace with this.
Now, the brain. Most of the time when people talk about the brain that is what they are most worried abut. With SB that isn't always the case. There are minimal learning difficulties with kids with SB in the region Evie has it. It is mostly just mis-shaped parts in her brain. For starters, her head, when looking down, so what would be the top of our heads, is shaped like a lemon right now. It isn't oval and kind of comes more to a point at the front. This will correct itself before she is born and when she is born you shouldn't be able to tell she ever had "the lemon sign". She also has the "banana sign". (I know, we have an entire basket of fruit up there!) -- Your cerebellum is supposed to be shaped like if you cup your two hands into the letter "C" and then moved your hands close together so that your thumbs touch -- that is what it should look like. But since her spine is pulling down where her opening is hers is more shaped like a banana, so start moving your thumbs down and you can see how it starts to look like a banana instead of the 2 "Cs". This doesn't cause any harm, it is just another thing that shows that her spine is tugging a little bit. Hers isn't completely rounded out in the middle, you can still see where it goes in a bit like it should, just not all the way.
Now, onto the ventricles. The ventricles are the spaces in your brain that drain fluid away from the brain. They should be under 1mm to be considered "normal". Hers are at .9 and right at 1mm. While this is on the top range of normal, they could be a lot worse already at this point. We don't want them getting too large. We are completely prepared for her needing a shunt after she is born which will help drain the fluid from her brain and deposit it in her stomach where it will be absorbed. She will be monitored closely after birth to see if she needs to have the surgery to place the shunt.
Her head is not considered large at this point. While that isn't an indicator of much since Joe has a small head to begin with, it is a sign that the fluid that is already around her brain isn't considered severe to this point. For obvious reasons, the increased fluid and swelling of the head can cause major issues, and I would LOVE to avoid them!
Now onto what will happen once she is born -- I will deliver baby girl via C-Section sometime between 36-38 weeks depending on her development. While it seems logical for her to come earlier than that to avoid any more possible nerve damage to her spine, there is no proof that delivering early will prevent further damage. So, it makes sense to not cause any more problems than we already have and let her cook as much as possible without harming her or letting me go into labor. I will deliver her at St. Mary's Hospital in St. Louis. We wil have her for no more than 2 hours before she is taken. This could potentially be the only time Gavin gets to meet his baby sister until she is released from the NICU. We will hold her (only on her tummy to prevent pressure on her back) and take as many pictures and get as many kisses as possible. She will then be transported via ambulance in her mini NICU unit with Joe to Cardinal Glennon Children's Hospital which is about 5 miles up the road. She will get placed in the NICU there and undergo surgery to close her spine and skin up before she is even 24 hours old. As long as the surgery goes ok and a plastic surgeon isn't needed to close her skin, (if there isn't enough skin, slits have to be made in her back to get it to stretch and a plastic surgeon is needed to do that), she will be able to come home in 2-3 weeks. If the plastic surgeon is needed, it could be up to 3 months. Depending on the flu season and any sicknesses that could be going around, Gavin may or may not be able to be in the NICU. We have to be prepared that he won't be able to go in, but we are optimistic he will be able to.
Each room at the NICU is private with a pull out bed and rocking chair. They have the little incubator looking beds when she is being weaned off the heat and little mini cribs for her. We can decorate her room and make it like home. I am excited about that part. The visitation is a little interesting there. There aren't many rules, except NO children under the age of 16 are allowed back unless they are a direct sibling of the child in the NICU. Grandparents and parents are allowed 24/7 and all other visitors must leave by 8pm. You have to be on our pre-approved list to get back there and have to show ID at 2 different checkpoints in the hospital. Each NICU nurse has a maximum of 3 babies at any one time (depending on the conditions of each of her kiddos). I get to eat there for all my meals for free since I will be nursing and their cafeteria food is actually really good!
I am pretty upset about being so far away from my little baby and everyone else gets to see her except me and then who does the family go see, her or me? I know that it is what is best for both of us, I just hate that we are so far away and I won't get to see my baby and she won't get to hear her mommy.
After she is released we will have numerous follow up appointments the first 3 months. If everything is stable she will come back at 6 months and then at a year. I am not even looking past the NICU at this point but at least we kind of know what we are expecting. The hospital has "clinics" every month where the entire team of SB specialists will be in the same place at one time to do all of our appointments on the same day to prevent us from running around, so that is great.
Now to my family that is reading this -- my sister, Joe's sisters and all our nieces and nephews have a 1-2% increased risk of having a baby with SB. It is highly recommended that all of you take increased doses of folic acid at least 1 month prior to attempting to conceive.
We have confirmed that Evie does not have the Cystic Fibrosis gene (my sister is a carrier so I was tested -- Evie's bowels were also showing "bright" on the first ultrasound which could be a sign of CF -- but she is all good!) AND -- we just found out Evie's chromosome count is all good! So no Downs Syndrome or any other disorder. The only thing on our plate is the SB and that's enough for us!
I do believe that is what we know for right now. Yesterday was complete information overload and I am SO thankful my mom was there to take notes and ask the questions we were too worn out to ask. I got home and read all her notes and sometimes it seemed as though I was hearing some things for the first time!
We are now going to fight for our baby girl, be her biggest advocate and do everything in our power to do whatever we can do for her. When we first got this diagnosis we kept asking "why us" now we are at peace with all of this and are thankful to have been chosen to be her parents.
I have been reminded again and again that sometimes everything can't go perfect because if it did people wouldn't need Jesus. When things like this happen it can be easy to be mad and turn away but it is in times like these that we need to RUN to Jesus and let him be in control. I am working on the "being still" part and letting him be God, because afterall, HE has this all planned out for us! He is showing us his handy work and that he is still in the business of miracles and we are just so thankful, overjoyed and humbled by it all.
Thank you to all of our friends and family for your continuous love, support and prayers. Thank you DC for all of your prayers over the past weeks and for praying for us last Sunday. We miss you all!