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.