Everly Lynne Smalling

The morning of Wednesday January 8^th, we woke up at 3 AM, anxiety and excitement preventing us from ‘sleeping in’ until our 4 AM alarm clocks.  The day we would get to meet our daughter, had finally arrived!  After months of classes, stocking her closet, preparing the nursery, and preparing ourselves mentally, we were terrified yet excited for the arrival of our first child.

The c-section was a breeze.  A few pokes here, a little pressure there, and then bam, suddenly we heard the anesthesiologist look over the curtain and exclaim “wow that’s a lot of hair!”, followed by the blissful sound of our baby’s cries.  Brad brought our girl to me wrapped in blanket, and I fell in love with him all over again seeing how quickly and deeply he fell in love with our daughter.  They placed her on a scale by me and to everyone’s shock, our ultrasound-estimated 11 lb baby, was a very average 8 lbs 3 oz and 20 inches long.  To keep me distracted as my OB stitched me up, the anesthesiologist, nurses, and assistants filed around and asked us what her name was.  After we listed the names we were still considering, the staff along with Brad and I decided that Everly Lynne just seemed to fit our little dark haired beauty.   Someone then handed the baby to me and let me hold her as they rolled me back into our hospital room.  In my memory it seems like just a joyful blur that would then be followed by yet another whirlwind of activity.

Back in our room, a nurse placed Everly under the lighted examining table and remarked that her recessed chin could make breastfeeding difficult. After she did the initial exam, she called in three other nurses, all whom confirmed that her lungs and rib cage seemed to be working much too hard to breathe. They then called in a nurse practitioner, who, within five minutes had a neonatalogist join in the room.  In a fog, we heard them explain that she had cleft palate, an underdeveloped jaw, and a short tongue.  As her monitors kept showing her inability to get enough oxygen, the neonatologist advised they would need to move her to NICU.   After we spent such a short time with her, Everly was whisked off to NICU where she is predicted to be spending at minimum the next two to three months.

It’s been confirmed our Evie girl was born with a rare condition known as Pierre Robin Sequence.  Undetectable in utero and affecting less than 1 in 15,000 births, PRS is believed to occur between weeks 7-12 in gestation and is a fluke unrelated to medication, genetics, etc.   It is thought that when the skeletal system is developing, the mandible gets stuck in a downward position toward the collarbone as though someone was pushing the head down.   As the face is pushed into the collarbone, the jaw is unable to fully develop, resulting in a short recessed chin. Then, when the tongue develops, there is not enough space for it to go forward so it instead develops too short and pointing straight up.  This short upward tongue then results in a u-shaped cleft palate in the roof of the mouth that is larger than normal cleft palate, thus completing what is known as the Pierre Robin Sequence.    The main challenges for PRS babies are breathing and eating, both obviously essential to survival.  Breathing is a challenge, because the tongue is so far back and upward over the throat that babies struggle to have a clear airway and can choke if laid on their back.  Feeding, because: 1. the baby cannot create suction without a palate, 2. the recessed jaw and chin make it anatomically impossible for a baby to feed from a breast, and 3.the shortened tongue so far back in the mouth prevent a baby from being able to pull fluid back into the throat even from a bottle.  We have begun attempting to feed her using a unique special needs Haberman nipple, which is extra long and extends near the back of her throat.  The person feeding her squeezes the nipple to force fluid into her throat and then we rely on her being able to swallow the milk from there.  It’s a tricky maneuver to avoid making her feel like we’re drowning her, and will likely take some time for her to learn to trust and adapt.   Until she can take an entire bottle at a time this way, the remaining milk that she cannot swallow, must be given via her feeding tube.

 

Just like any new mom, I am often overwhelmed and trying to learn as I go, and I am grateful for the wonderful NICU staff that helps me during this learning curve.  Bath time and diaper changes are as you can imagine, just a little extra tricky since we try to avoid putting her on her back. Additionally, Evie has needed to be suctioned out several times when fluids go up through her palate into her nose and down her throat at the same time…learning how to suction her in an emergency will be our next topic to address. The next year in particular is certainly going to be filled with some major challenges and God-willing, some major milestones for Everly.  Despite the tears and the heartache, the arrival of Everly has brought more joy into our hearts than we knew was possible.  When Brad or I rock her bare skin on skin as she falls asleep, the sense of love is simply overwhelming. When she whimpers and when we hear her congested breathe, it just makes us love and hurt for our little fighter all the more.  She is quite simply, the greatest gift we’ve ever received. From the first day we learned I was pregnant, we attributed her life to God’s Will, since medically it had been unlikely I’d be able to become and stay pregnant.  We prayed during pregnancy that her life would be a light in the world, and that prayer now remains.

Prayers:

We’re praying for Evie to learn to adapt and eat her entire bottle from the Haberman nipple.  Our hope and prayer is that she learns to swallow and adapt to her challenges, so that we can remove the feeding tube.  Additionally, the neonatologist has warned that as her tongue and jaw begin to relax, we should not be surprised if she starts having sleep apnea and choking episodes, so we ask for prayer that she proves him wrong!

 

Praises:
So far, Everly’s airways have remained open as long as she’s on her stomache. The fact that she can breathe and didn’t need a tracheotomy at birth means that she’s so far avoided one of the two major challenges of Pierre Robin Sequence!  We are also very cognizant of the fact that in a different day in age or even just in a different part of the world, PRS would be a death sentence.  That we have such excellent medical care and still have Evie here with us, leaves us beyond grateful.