The Journey Begins

by Jordan and Brad 5:50 PM 10 comments

The Background

After our struggle to conceive and keep our first daughter Liliana, we never thought we would be able to have more children.  We have a son Jack and a daughter Liliana, and then God chose to gift us with being Juliet’s parents. 

We had a very easy pregnancy and delivery with our 8 year old son, Jack.  We thought all pregnancies would be the same.  We unfortunately found out after two miscarriages that this was not the case.  Heartbroken, and a couple of years later, we rounded up the courage to try again.  We were told we would have to seek fertility drugs to conceive again.  We chose to go down this path, and shortly after we found out we were pregnant again! 

Terrified to lose another child, we still remained hopeful this baby would be our new addition.  Almost immediately, I started having symptoms of another miscarriage.   After holding our breath during an ultrasound, our OBGYN had to tell us, our baby was not viable and we needed to either have a D&C or a natural miscarriage.  I have had both, and both options were really hard on my body.  I hemorrhaged previously with a natural miscarriage, and I had a rough recovery after a D&C.  I chose to go home and weigh the risks associated with both.  I have two blood clotting disorders, prothrombin gene mutation and MTHFR.  I am on low-dose aspirin for life, and lovenox blood thinner injections while pregnant.  The combination of all of these make either option undesirable, and the decision was a tough one.   After coming home I realized no one had checked my HCG levels.  I asked for them to be checked right away.  They were going up!  We were sent back to the OBGYN for another ultrasound.  Our baby Liliana had a heartbeat and was actually a twin!  

When the doctor thought her sac was collapsing and preparing for miscarriage, she was actually just forming.  She ended up being the rare mono-mono type twins, and we ended up losing her twin in utero.  I was on bedrest for the majority of this pregnancy with frequent trips to the OBGYN and Maternal Fetal Medicine office.   We were told several things during her pregnancy.  She had calcium deposits in her heart that they chose to watch, and we were even told she was a boy at one point!  The rest of the pregnancy was fairly normal, besides still having to take it easy until delivery.  We had a scheduled induction to make sure my blood thinner injections had enough time to circulate out of my system before her birth.  

The Big Sister

On May 24, 2014 we set out at 4:15 am to head to the hospital to deliver our baby girl.  At around 4:30 am my husband and I were rear ended by a drunk driver going over 90 mph in a 45 mph zone.  We were only minutes away from our house and still 30 minutes away from our hospital.  The excessive speed of the drunk driver caused our car to roll several times, throwing our vehicle into the tree line. We stopped rolling as the driver’s side crashed into the pine trees.  My door was the only one that was still able to be opened.  I crawled out of the vehicle, begging and pleading with someone to call 911 as I was starting to go into labor, and my husband was non-responsive in the driver’s seat. There was a man in the street that I was calling to, pleading with.  This man turned out to be the 21 year old drunk driver that hit us.  He proceeded to ignore me, and run to the woods to hide his liquor.  After what felt like an eternity, and going back and forth to try to wake my husband Brad, and to scream for help, the ambulances arrived.  They ended up taking me and my husband to the hospital in separate ambulances.  

Once we arrived at the hospital, we were taken to different parts to be assessed.  He had seven broken ribs that were broken in multiple places, a broken clavicle, a large cut on his leg, and a collapsed lung.  His prognosis was pretty grim, but he was a strong man in great shape, so after an almost 2 week stay in ICU/PCU he pulled through and was able to come home.

Once I arrived at the hospital, they took me right to labor and delivery.  They sent me away, and back down to the ER to be assessed.  I was hooked to fetal heart monitors, and the ER staff took care of stitching up my cut feet, addressing my face lacerations, trying to asses my broken ribs and mangled wrist,  looking at my bruised and cut up back and stomach.  That’s when they realized I had a seat belt mark across my entire stomach.   They still planned to go through with my induction even after all of this.  When my OB came to the ER and looked at the baby’s heartrate and a scan they did of the baby, they realized my placenta had abrupted during the wreck, and the baby was in distress.  They decided to take her right away via an emergency c-section.   

Brad, Jordan, and Baby Liliana all made it through that day, and survived.  Liliana is almost 2 and thankfully healthy and strong!  We thought after everything we went through with her, and two previous losses, we would never be able to conceive again without the help of fertility drugs.  God had other plans.

The Surprise

Two days after Liliana turned 1, I found out I was pregnant with a wonderful surprise! We thought for sure this pregnancy would be different, and surely we could relax and enjoy every minute of this pregnancy.  We even called the baby, our lagniappe, our little something extra. 

I started having miscarriage symptoms again. 

Our OBGYN had us come in for an early ultrasound given our history.   I was diagnosed with a subchorionic hematoma.  Basically, I had a blood clot or bruise in my uterus, and it was causing my uterus to cramp and I was bleeding periodically.  Once again I was on bedrest and trying to take it easy.  Everything seemed to go well after taking this time to let my body heal.  We made it to 12 weeks pregnant, the hematoma resolved itself, and we finally felt comfortable telling friends and family our exciting and surprising news.  We were going to be a family of 5!!  Our son Jack was beyond excited to be a big brother again, and our one year old daughter Liliana had no idea she was about to become the middle child.   

The Heartbreaking News

Everything during the pregnancy seemed to be normal, and we were reassured, we deserved a break and this would surely be an easy pregnancy, right?!

We headed to our 18 week anatomy scan nervous and very excited.  We decided to wait to find out the sex of the baby until delivery day.  What a fun way to welcome our newest child into the Boykin family.  We knew this would be our last child, and we wanted to make the delivery extra special.  Little did we know at 7 months pregnant, a new nurse, with a new doctor’s office would spill the beans and let it slip, our Lagniappe baby is a GIRL!!

Our anatomy scan seemed to be going normally, until the tech asked us again how far along we were while she was looking at the baby’s brain.  My heart sunk, I knew something was wrong.  We finished up and were sent back to wait for the doctor.  She confirmed our greatest fears with just a look.  Something was wrong with our surprise-a-little-something-extra-baby.  She diagnosed our baby with hydrocephalus, having a hole in its heart, and a single vessel umbilical cord.  

Hydrocephalus is a condition in which your body produces cerebral spinal fluid, and it has nowhere to drain.   It continues to build up in your ventricles in the brain, causing brain damage when the fluid build-up does not allow for the brain matter to grow.  This condition also often causes the baby’s head to grow too large and be disproportionate to its body.  We were sent to the maternal fetal specialist again, only to have our fears confirmed.  Our baby has several birth defects.  We were sent for an MRI, genetic testing and infections studies.  They confirmed the hydrocephalus caused by aqueductal stenosis, and our genetic testing and infection tests all came out normal.  

This left us dumbfounded.  What caused this?  I am so careful when I am pregnant.  I give up all the foods that could cause problems, I avoid cleaning the house with chemicals, I don’t drink, I don’t smoke, I take all my vitamins and I eat well.  We might never know what caused the baby’s anomalies.  While at Shands, the genetics team will test her further and do more extensive tests to see if there is a genetic component to all of her anomalies. 

We were scheduled to see the MFM/high risk doctor often, and we would eventually be sent to see a new OBGYN in Pensacola, to be delivered at the bigger hospital with greater medical capabilities.  With every appointment (and they were often) brought more heartache.  We found out the baby had more and more hurdles to overcome.  Over the course of her gestation, we found out her hydrocephalus was considered “severe”, she does have a hole in heart called a VSD, ventricular septal defect, a single vessel umbilical cord, she had fluid around her heart and in her abdomen, they originally thought she had two kidneys, then it seemed one was missing, and now it’s confirmed she has a horseshoe shaped kidney, she had an enlarged colon, and then we found out another life altering diagnosis.  

The Disguised Blessing

As we were nearing the home stretch, at 32 weeks we were told that our baby was suspected to have a CDH, congenital diaphragmatic hernia.  We were once again devastated.  This is an extremely severe condition with a long list of complications associated.  Because of the severity, we were sent to a specialist in Gainesville, FL at University of Florida Shands Children’s hospital.  They couldn’t confirm or deny she had the CDH.  It was suspected it was a sliding CDH, which is hard to pinpoint on an ultrasound.  If she did indeed have the CDH, she would need to be delivered at Shands.  They have doctors familiar with the care and repairs associated with a CDH, and they have ECMO machines, that would be able to sustain her life until the surgery.  We were so pleased with the team of surgeons that could take care of our baby at Shands, we decided this was the best place to deliver her.  If I delivered in Pensacola, she would have had to be life flighted to Gainesville, and if any other problems popped up after birth, they likely had a surgeon that would be able to fix it.  

Also, the neurosurgeon we originally met with in Pensacola, asked us during our first meeting if we had considered termination…I was 28 weeks pregnant.  I told him I thought that was illegal, and he said he didn’t really know, and I would have to ask my OB.  We were dumbfounded, heartbroken and shocked.  Termination was never an option for us, and to hear this at 28 weeks along, my world was shaken once again.  We chose another neurosurgeon. One that would value her life as much as we do.

After birth, she would need neurosurgery to place a shunt in her brain to drain the cerebrospinal fluid, possible surgery to fix the herniated diaphragm, and possible surgery to fix the hole in her heart.

In this way, the CDH diagnosis was a tremendous blessing in disguise, as it was God's way of directing us to the exact right hospital and perfect team of doctors to give our little fighter her best chance.

The Big Day

We set out to deliver Juliet at 37 weeks.  They wanted to once again control the blood thinners in my blood before a c-section, while allowing her to reach as full term as possible.  We thought we were prepared for everything that was to come.  We were wrong.

January 27, 2016 we were scheduled for a c-section, and our little darling would be born.
 

We were in the recovery/pre-op room getting ready for the moment we had been waiting for.  No one could really tell us what to expect of our little Juliet until she was actually born, had been scanned and had a full assessment by what seems like a million doctors, fellows, residents, nurses etc. 

The team of doctors that would be taking care of her in the NICU, were going over her history and issues. He was running through the list of anomalies, and threw in a term we had not heard of, a sacral defect.  I asked him to clarify.  He told us (minutes before I was taken into surgery) that Juliet had spina bifida.  Cue the room spinning, I wanted to faint, but I couldn’t give into my anxiety.  We were previously told she did NOT have spina bifida.  The doctors rushed to review notes, Brad ran to the car to get the a CD of Juliet’s original MRI, and after what felt like a lifetime, they didn’t think she would have SB, but we would have to wait to be sure at birth. 

Well, I was wheeled away, Brad got dressed in surgical scrubs, and we would soon meet our third living child, our second daughter, and our fifth baby.
   

The Cesarean was not routine. The doctor had to cut my uterus horizontally and vertically to get her head out due to the enlargement from the hydrocephalus.  Juliet’s head was 50 cm when she was born.   This is way over the average size.  I didn’t hear her crying, and I immediately asked why she was quiet.  My incredible husband was terrified, but never let me see his fear.  Juliet came into this world blue, and was surrounded by a team of doctors trying to get her to turn pink.  Brad would tell me later that hearing the young doctor say, "we have a pink baby!" was one of the most joyous moments of his life. 

Once she had color, they intubated her, and brought her close to me while I was finishing up my surgery.  I got to see her through hazy tear filled eyes, and really just from the side.  I knew her head was large, but I couldn’t really get a good view of my beautiful, perfect, God given gift.  We didn’t know we had so much love to give until all three of our children were in this world together.  She may have only been minutes old, but we were madly in love with this “little something extra”. 

The Initial Assessment

I was taken to recovery, Brad stayed with me and the doctors worked on Juliet and took her for imaging to see what was really going on in her tiny body.  She would indeed get neurosurgery to get a shunt placed in her brain at almost exactly 24 hours old.  The hole in her heart looked as though it was minor and would probably not need intervention, the enlarged colon didn’t seem to be causing trouble, and she did NOT have a congenital diaphragmatic hernia!! One major complication had been taken out of the equation! 

After further review, they found devastating news.  Even after getting MRI’s, an echocardiogram with a cardiologist, extensive ultrasounds and really an ultrasound every week for months at several different doctor’s offices, they missed something.  Something big.  Juliet has a coarctation of the aorta.  Part of her aorta is too narrow, and she will have to have open heart surgery to fix it.  They will likely fix the hole in her hear at the same time.  Juliet has to be stronger before her body can handle heart surgery.

The First Surgery  

On Thursday morning, January 28, 2016 at less than 24 hours old, our little princess had her first surgery - brain surgery to place the shunt.  We were nervous wrecks, but had faith in God and confidence in our wonderful surgeon, Dr. Pincus.  We followed our baby down to the OR where they made us stop outside the room, told her we would see her soon, and went back to the hospital room to agonize.  Less than an hour later, Dr. Pincus showed up at our door.  He told us that the shunt surgery was completely successful and Juliet had no complications.

The shunt is functioning properly as of now, and her fluid and swelling is going down.  Her head has been decreasing in circumference by about a centimeter everyday.  We were told once the built up fluid eventually goes down, she may have an abnormally shaped head, but we will deal with that down the line.  Her head will probably always be larger than an average child, but we hope that she will grow into it somewhat.  For now, it’s just more room for mama to put bows on that big beautiful noggin.

 

The Ongoing Challenges

Juliet had an MRI done after birth to see how extensive the brain damage is.  Right now her head is mostly fluid and very little brain matter.  As the fluid drains, the existing brain matter might essentially spring back and continue to develop somewhat normally, or the damage may have already been done.  She might be missing significant parts of her brain.  The neurologists can’t tell us one way or another, we will just have to wait and see. 

Juliet is also on PGE, a drug to help her aorta remain open until her open heart surgery.  This medicine is causing her lungs to produce extra secretions, and she struggles keeping her oxygen levels up sometimes.  She is still intubated, but she can breathe on her own now!  The ventilator is providing her with extra concentrated oxygen, and a little push now and then, but she is doing most of the work on her own.   The plan is this week to take her off of the ventilator, to see if she can fully breathe on her own.   They aren’t sure if she’s ready for this just yet.  Her lungs when suctioned, are producing more secretions, and they are often pink or red tinged from blood.  They think her lungs are irritated from all the suctioning, but it’s necessary and it’s a vicious cycle.  Also, she might not possess the gag reflex she needs to protect her airway.  

This is a major concern.  She can’t live on the ventilator forever, so the doctor’s feel they have to try to extubate her.  If the extubation goes poorly, they will have to try to reinsert the breathing tube.  This poses risks, as her throat might swell up, not allowing the tube to go back in and it might cause damage to her throat and or vocal chords.  Being on a ventilator comes with its own risks.  We need prayers for this to be successful. Brad and I will also be present when they remove her tube, so please pray for our strength to be there for her.  The thought of this procedure is keeping me up at night. 

Juliet was assessed by a neurologist today, and her basic reflexes are intact, woo hoo!  It does look like the pressure from the hydrocephalus is either pressing on her optic nerve, or she has some brain damage already associated with her eyes.  One eye is hard for her to control, and it kind of wanders.  Only time will tell.  She does look straight into her mama’s eyes with the eye she can control.  She melts my heart several times a day.

 

Juliet also seems to have a clefted nose.  It’s not terribly noticeable, but it’s there.  Her nair didn’t fully form in utero it looks like.  Thankfully, she does not have a cleft palate or lip.  She also has a few fused vertebrae at the end of her spine, and they will do scans to look at that in the future.  This is where someone might have gotten confused and thrown out spina bifida. Once again, how was this missed on our numerous scans?!? 

The neuro and cardio teams will be meeting this week to discuss what the next step will be in Juliet’s plan.  Please keep praying for the doctors to make the right choices for her, and for their wisdom.  Also, please continue to pray for Juliet’s strength.  She is such a trooper.  Her little body is riddled with tubes, monitors and poke marks. She has a PICC line, and it took 3 hours to get that line in.  A PICC line is a direct line to her heart to allow for medicine to be administered quickly.  She has tiny scabs all over her hands, feet, legs and arms where the medical team tried to get IV lines started and failed.  Poor little Juliet has had a rough first few days on this Earth. 

The Bonding

We have had an amazing staff of nurses in the NICU.  We spend as much time as we can just visiting Juliet.  I sing to her, Brad tells her stories and we constantly remind her how loved, wanted, and adored she is.  We tell her we love her so many times, as we want her to know she is our little something extra, our lagniappe, and she made our family whole.  One fantastic nurse allowed me to hold Juliet for the first time the other day!  I sat in a recliner in the NICU and she placed my baby with all her tubes, sensors etc right into my arms. 

Juliet almost melted into my body.  She is so at peace with me, as I am with her.  We need each other.  Her vitals are stable while she’s on me, he heartrate stays steady, my body regulates her body temperature, and I promise, she just looks happy.  We need to be with each other.  I am having a really hard time physically recovering from my c-section.  I am not sure if it’s because it’s my second one, or because of the trauma my body endured when they had to get her large head out of my small frame and uterus.  When I am holding her, I literally feel no pain.  My horrible searing pain is just gone.  Her baby hormone magic helps me, and it seems as though just my smell, my touch and my voice allows her to relax and work on healing.  

When she is in her bassinet in the NICU, she trembles, and looks so uncomfortable in her own skin.  I am having trouble struggling with spending all day by her bedside and then having to go home in the evenings.   Ideally, I would be with her 24 hours a day, and even sleep next to her.  When I overdo it and don’t take the time to rest, eat properly or recover, I end up shaking in pain and sobbing because the pain is too intense.  

Please pray for me to recover from the surgery quickly so I can be with her more often.  10 hours during the day is just not enough!  I spend most the night up worrying about her and calling the NICU to check in.  

The Ronald McDonald House

We have been lucky enough to be staying in the Ronald McDonald House here in Gainesville, less than a half a mile from the hospital.   It has been such a blessing to be close to the hospital, be saving money on hotels, and we have been fed really well.  Ronald McDonald House just asks for a $15 a day donation for housing, and we have already been here almost three weeks, with no end in sight.  Families, churches and organizations come in and bring meals to the families staying in the house often.  After a long day in the hospital, it is really nice to sit down to a hot meal and spend time with the other RMH families.

If you are looking for a place to volunteer or a charity to donate to, please consider this wonderful organization.  They are helping so many families to be close to their precious children who all have diseases, illnesses, and other challenges to overcome.  

The State of Things

All this to say, we weren’t expecting this beautiful baby, and now she’s here, we can’t and don’t want to imagine a life without her.  We are head over heels, bonkers in love with this little fighter, and we just beg, plead and pray for her to continue to fight.  

She has some major changes coming this week, and we know she’s strong enough to endure them all.  She has started drinking my breastmilk through her feeding tube, so we can only hope this starts to provide her with what she needs to start growing into the strong little girl I know she will be. 

Please pray specifically for her gag reflex and the extubation, that this will go well and will not damage any of her throat structures.  Pray that she will do well with respiration afterward, that her little lungs will be able to function fully in complete absence of the ventilator and that the secretions will clear up.  Pray again for wisdom and guidance for her cardiac and neurology teams, that they will be able to plan the optimal course for her and carry her through it with their expertise.

Pray for us.  Pray for strength and faith.  Pray for healing.  Pray for peace and rest.  Give thanks that you, as we do, have a wonderful God who hears our prayers and blesses us every day.