Today things went from bad to worse. Silas's breathing took a major step backward over the past 48 hours, and his doctors are now talking about the possible need for a tracheostomy. Silas's condition is extremely rare to begin with (spina bifida occurs in only 1/1000 births and only 15/100 of them have symptomatic Chiari malformations), but to be in the even smaller portion of the population who not respond to decompression surgery was completely off our radar screen until today. The reality is only gradually beginning to set in.
Over the past few days Silas's breathing difficulty (stridor plus retraction) has increased instead of improving. His breathing problems stem from the fact that his little baby airway is made extremely narrow by his vocal cords, which are paralyzed. When he gets upset or excited he breathes in deeply, causing the vocal cords to vibrate, and the extreme pressure sometime causes them to close off his airway almost completely as he inhales. His body overcompensates trying to suck in air while the airway is closed, causing his chest to cave in or retract.
The doctors in the PICU had three possible theories about why this might be the case and ran a series of tests today to solve the mystery. The theories were: (1) he acquired a viral infection that is exacerbating an already tight airway with inflammation, (2) he had a shunt malfunction or infection that is adding to his compression, or (3) his cranial nerves have not yet responded to the decompression and have deteriorated along the same timetable as if he did not have the surgery. Until today, the last option seemed unlikely because we have already seen improvement in his apnea and his swallowing.
First today we had a head ultrasound, which did not show anything abnormal and appears to have ruled out a shunt issue, although we'll be following up with neurosurgery tomorrow. Next, the Ear-Nose-Throat doctors came and placed a hand-held scope into Silas's throat by way of his nose to view his vocal cords. They saw no movement of his vocal cords, which appear to still be completely paralyzed, and they found no evidence of inflammation or infection. At the end of the day, the consensus among the doctors we saw today was that unless there is a major change in his ability to move his vocal chords, he would need to tracheostomy to help him breathe. No one is comfortable sending us home with breathing problems this severe. Having the tube put into his throat really upset Silas and caused him to gasp and his heart to race (SVT) again and reminded us how little reserve he has. Amid all of these tests Silas continued to amaze us with his toughness. He even managed a few smiles between gasps.
The trach would bypass his vocal chords and allow him to breathe easier. It's possible that his vocal chords start working eventually, in which case the trach could be removed, but it may also be in there for a long time. There are no other surgical procedures that can be performed on infants to relieve his condition beside the tracheostomy. It would be wonderful to see his stridor end, which would clear the way for the more typical baby activities that he's missing because he's using so much energy just breathing, but a trach can also be very limiting (for Silas and for his parents). One of the reasons a trach seems so disappointing to us is that Silas was named after someone who sang in times of trial, and we want that to be true for him as well. We are praying that his vocal chords regain their full function before he has to go through the procedure. It's humbling to realize how something as essential as breathing can be dependent on something as small as a vocal cord or a nerve in the brain stem that controls it.
Even though the test results and what they mean were very difficult to hear, we had a brief few hours of peace this afternoon and evening when Silas was allowed to lay by us in the chair in his room that has become our bed this past week. Laying next to Mama, he drifted off to a peaceful and quiet sleep. Mama was so relieved she drifted off too. He even stayed calm and quiet as Dad took his shift laying next to him. It was enough to renew our spirits an give us all strength for whatever comes tomorrow. It also gave us hope that he might now be improving slightly. We feel so grateful for the encouragement from friends and family relayed to us through calls, text, email, facebook, etc., and we know that even though this is a really tough time for our small family, we are loved, and Silas will grow up surrounded by people who accept and treasure him.
