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.