Go-Time

We want to thank everyone for their love and prayers.  We breathed a huge sigh of relief last night when the ENT surgeon performed the laryngoscopy in front of us and showed us on the screen that her airway was open below her throat, so she does not need a tracheostomy.  Knowing her actual airway below the tongue is clear confirms that what is obstructing her airway is the combination of the cleft palate, recessed jaw, and upward-pointing tongue.  So now we need to move forward to address those three issues to clear the airway. Her surgeon, PRS expert Dr. Matthews, developed an internationally-used scale by which physicians can measure the severity of Pierre Robin Sequence.   10 mm between the upper and the lower gum line is considered severe and dangerous; Evie’s are 12 mm apart.   

 

While Brad and I were preparing for her to have to have jaw distraction, we were shocked to learn from the surgeon today that he thinks we need to move quickly and therefore she’ll be having the surgery tomorrow. As in Thursday, January 30^th at 4:30 PM.  In a way, it’s somewhat of a relief to be moving so quickly because it doesn’t allow us much time to build up fear and dread.  Everly will be intubated and anesthetized for about two hours as the surgeon breaks her jaw and then places the distraction device into her mandible, which long-term should result in four small scars on her face from the implants.   Additionally, Dr. Matthews will surgically release the tongue, cutting tissue to allow the tongue to come forward and lay more flatly, to lessen the obstruction of her airway.  She will then receive a picc-tube, which will serve as a more long-term IV port, in which she’ll receive all her medications. Some of these medications include antibiotics, narcotics, and paralytics to prevent her from moving for several days while her face heals.  She will be fed through her ng-feeding tube for the next week and her breathing will be done solely by a respirator, which will silent her cries should she ever be awake enough to do so.   

Adding a whole new level of heartache is the fact that we will be unable to hold or move her at all for the next week following the surgery.    After this next gruesome week is done and they believe the initial operation has healed, the respirator will be removed and she will have the large jaw distraction device begin moving her jaw forward day by day, then holding the jaw in place for about two weeks once the distraction has reached the proper distance.  In roughly four to six weeks, the distraction should hopefully be removed, and then the challenge of teaching how to eat all over again will begin.  As one kind nurse said to us as she moved us to a more quiet corner of the NICU with a window, “it’s going to be a long haul.”

As you can imagine, we are feeling overwhelmed to the point of nausea and are simply terrified for not just what we know will happen to our sweet girl, but also scared for what could happen.  There are significant risks involved with anesthetizing, intubating, and drugging a baby this young.  However, to protect her airway and life, we are left with no choice.  We pray that this surgery does indeed successfully bring her lower jaw forward and allow new bone to extend the jaw out, while also bringing the tongue forward and out of her throat.  We hope this surgery and procedure to be the worst thing we’ll endeavor, that she’ll be spared complications or infection, and that her body responds favorably to the hell we’re about to put her through. 

Crushed

Day twenty-one in the NICU and things have gone from bad to worse.  Much, much worse.

About a week ago, Brad and I became increasingly concerned and uncomfortable with Novant’s neonatologists’ approach to Everly.  Three different neonatologists (all who are part of the same group doing rounds at all the Novant hospitals) , after looking at her, had assured us she seemed to have a mild case of Pierre Robin Sequence, and thus that our goal was simply to get her eating revved up.   However we became doubtful of their guidance when we saw no progress being made after a couple weeks.  Also noticing her increasing respiratory struggle, we began pushing for answers and alternative options.  After exploring a transfer option to Novant Main, Levine Children’s, and to Duke Children’s, we inquired at Novant Matthews repeatedly about possibly transferring and were advised to stay put and be patient with Everly’s feedings.  Still disconcerted, I reached out to several parents around the country, whose children also had a ‘mild’ case of Pierre Robin Sequence. Many of these kind and helpful parents told us that a gastrostomy tube was their saving grace to get their child home and to allow the child to learn how to eat on their own in the comfort of their home.  We then inquired about the tube, and since the neonatologists seemed to have no better alternatives, they finally agreed that we could transfer to Novant’s Main Hospital, where pediatric surgeons could evaluate her for a g-tube.  Another reason we were anxious to move to Novant Main was that Everly would finally be seen in person by a world-renowned Pierre Robin plastic surgeon that we’d previously consulted with in his office while Evie had still be in the NICU.  Dr. David Matthews had impressed us by his conservative approach and extensive knowledge of PRS, so we hoped that his evaluation would match that of the neonatologists and we could simply proceed with trying to get her to eat.  And so, Everly’s first car ride was in an ambulance on January 27^th, from Novant Matthews to Novant Main in uptown Charlotte.  A fourth neonatologist helped arrange for a pediatric surgeon to look at her, and told us that we could likely have the g-tube surgery by the 29^th and potentially take her home within a week where we’d feed her through the tube.  We were nervous about the idea of anesthetizing and intubating our newborn to then implant a device in her, but little did we know at the time that her simply needing a g-tube would have been something we’d only dream of 24 hours later.

Our entire world was shattered that Tuesday night, when Dr. Matthews came to see her in person.  He immediately identified her Pierre Robin Sequence case as ‘extremely severe’ and raised several concerns about respiratory and feeding obstruction due to her tongue, and concern that she may additionally have soft tracheal tissue and be at risk for tracheal collapse.  As Brad and I began shaking at her bedside, we were given two possibilities for what she would next need: one, a tracheostomy or two, jaw distraction and tongue surgery.  So, the tentative plan is now:

1. She has had a 3d CT scan to get a better picture of her oral anatomy including the severity of her recessed lower jaw, cleft, and airway. We are awaiting Dr. Matthews’ review of that information.  This evening an ENT physician will scope her throat via a laryngoscopy  to determine her level of larynx and tracheal obstruction. If severe, she would require a tracheotsomy for at least the next two years simply to ensure she can breathe.   As you can imagine, we are presently beyond terrified for this consultation.

 

2. If she does not need a tracheostomy, we would instead have to have jaw distraction done. This is a barbaric procedure and is a last-resort type procedure used only when an infant’s airway and therefore life, is at risk:
1. Her jaw would be broken and they would insert a distraction device between the upper and lower mandible.  This exposes her to anesthesia and then narcotics, as she’d have a morphine drip for the intense pain
2. She would be on a respirator during the entire one to two week distraction process, which would disallow her to even be able to audibly cry
3. Over the course of one to two weeks, they would crank the device to bring the lower mandible forward. This is a painful procedure for the infant and requires a rather inhumane-looking device.
4. Once the jaw is brought forward, the distraction device would be left in for 2-3 weeks
5. The distraction device would then be removed via surgery, again exposing her to anesthesia.Image of Jaw Distraction
   
   
3. Tongue surgery will be performed at the same time as the initial jaw distraction surgery, to relax the tongue and bring it forward so that she could hopefully eat and not be at great of a risk for the tongue to obstruct the airway.

5. Around one year of age, she will also need major cleft repair. Because she was born with no hard palate and a cleft in her soft palate that is about twice the size of a traditional cleft palate, it will be major surgery to bring together her soft palate.  She would then just never have a hard palate.

It is beyond heart-breaking for us to think of the procedures, pain, and 2-3 month additional NICU stay Everly will now require.  She is an absolute cuddle bug, who usually cries only because she wants to be held. With her big beautiful eyes, head full of soft dark hair, and seeming aesthetic perfection to us, it is still hard to wrap our minds around the severity of her condition.   The thought of what we have to put her through is nauseating, and when we add to that the risks associated with infantile anesthesia, narcotics, and radiation, we find ourselves in a horrible nightmare that we cannot wake up from.

Prayers:

We are struggling with anger and bitterness with the entire group of  neonatologists whom misdiagnosed, mistreated, and gambled with Everly’s life for the last three weeks, all while also delaying any form of effective treatment for three weeks.   We’re now living in a constant state of panic and fear for the challenges ahead.  We find ourselves simply begging God to minimize her pain and discomfort, and right now and to help give us strength should we learn tonight that she needs a tracheostomy.

Day 14

Today is day fourteen for Everly in the hospital and I think she, Brad and I are all beyond exhausted.  Everly’s progress over the past week has been stagnant, and the ‘treatment plan’ if you will, has become experimental.  The neonatologists agreed to try letting me room-in here at the hospital in a labor and delivery room across from the NICU to test whether me providing consistency for her 24/7 will help her.   And because after I was discharged and we found the six nights we spent at home with her still in the NICU to be emotionally nauseating, I was all too happy to move back into the hospital.  Unfortunately, we’re now on day three of this test and don’t seem to be seeing a difference in her eating.  Yesterday they began yet another test, removing her feeding tube to see if without artificially filling her stomach, her hunger levels would increase and motivate her to eat more.  We’ve not seen success of that test either and she instead has lost more weight. According to the physicians’ caloric calculations, Evie needs to be eating 70 CC’s eight times a day from her bottle to begin gaining weight.   Right now she’s averaging about 35 and hasn’t been able to increase her intake in the past week. 

The crux of the problem seems to be that Everly has to work SO much harder than a normal baby just to eat and breathe therefore tiring very easily, a catch-22 since that extra effort means she’s burning more calories and therefore needs more calories than an average baby. She tries to hard to suck on the special needs Haberman bottle, but cannot create any suction due the lack of any hard palate and the massive cleft in her soft palate.  Everly also tries to swallow all the milk we push into her mouth, but easily becomes overwhelmed and panics as her recessed tongue far back in her throat likely makes her feel like she’s choking. It kills us to watch her struggling to eat, trying to clear fluids from her cleft and often becoming so overwhelmed that her sternum starts shaking and her lips take on a shade of blue.  Seeing how hard this little girl works just to do normal things breaks my heart while at the same time making me already so proud of the fight she has within. 

We're extremely thankful for the sweet friends and family that have gone out of their way to support us with love via phone calls, texts, food, and prayer. Though we've been slow to respond, please know how much we value your encouragement.

 

Praises:
Everly has continued to maintain her airway and the physicians think she’s most likely in the clear now for major respiratory issues since she’s made it this far without obstruction.   We also are grateful for the encouraging meeting we had with a pediatric plastic surgeon, who gave us hope for her long-term outlook once we make it through this first year or two.

Prayer:
We’re feeling overwhelmingly discouraged but know we need to live patient and trusting in this circumstance.   Too, we ask for prayer for wisdom for the neonatologists, nurses, and ourselves for how to best help Everly.   Most importantly though, we are simply praying incessantly for Everly to learn to eat, grow and thrive.

Evie is so tuckered out after trying to eat!

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.