Yesterday evening, Silas was struggling to breathe, had very severe stridor, weakness in his arms, and several spells of apnea in which he stopped breathing for 5-10 seconds. We took him to our local emergency room, where they coordinated his transfer to UCSF by medical transport around 3am.
Over the past few weeks Silas has been showing signs that his Chiari malformation may be becoming symptomatic (see explanation below). His doctors increased the flow of his shunt the week before last and ordered a series of tests to begin to evaluate whether he may need further brain surgery. Last week, he was up at UCSF for a sleep study and a bronchoscopy on Wednesday and Thursday. The sleep study showed that he was having spells of both obstructive and central apnea at night, and the bronchoscopy showed that his stridor is now associated with bilateral vocal cord paralysis. Both of these conditions are serious and are consistent with symptomatic Chiari.
We learned the results of these studies yesterday and were told that Silas might need to undergo Chiari decompression surgery in the near future, but would need another apnea test on Monday which would evaluate his oxygen and CO2 levels while he is receiving oxygen. In the meantime, we were to watch him for a list of warning signs. Last night he started demonstrating pretty much all of the warning signs all at once, so we brought him in.
Silas is now in good hands and being evaluated by the doctors at UCSF. He had his oxygen study today and responded well, which is a good sign. He is in good spirits here, although he is pretty uncomfortable with all of the tubes and monitors attached to his body. He has an oxygen tube under his nose, which he doesn't like very much, and several wires attached to nodes on his chest. He spends a good part of his waking hours trying to remove the wires and tubes from his body and put them in his mouth (a good sign developmentally but a little bit distracting for his parents who have to play goalie).
The scary thing about the Chiari malformation is that its symptoms can emerge quickly and lead to rapid deterioration of brain stem function. It is the leading cause of death in children with spina bifida, and is particularly dangerous in infants. Although all children with spina bifida have the Chiari II malformation, the vast majority do not develop serious symptoms. But for those who do it can lead to serious complications with feeding and breathing. Thankfully, Silas has not had any serious problems feeding, so he is a very good weight and size. The doctor monitoring him today thinks this puts him at a real advantage. Until recently, he has also not had serious breathing issues, which is encouraging, because there is reason to trust that these new problems are due to compression on his brain stem rather than 'wiring' issues. Decompression surgery could theoretically relieve this pressure on his cranial nerves and restore normal function.
Many of the symptoms for Chiari look like symptoms for hydrocephalus, and the consensus among neurosurgeons today is that the hydrocephalus must be ruled out first before undertaking the very serious Chiari decompression surgery. Hydrocephalus often exacerbates problems with the Chiari and, since decompression surgery is so invasive, neurosurgeons need to be confident that the symptoms will not go away when the hydrocephalus (ventriculomegaly, or big lateral ventricles) is reduced through shunting. If the symptoms persist, decompression surgery is used as a last resort, and it sometimes has to be performed on an emergency basis. Vocal cord paralysis and apnea are dangerous symptoms, but they can often be reversed through the decompression surgery. Sadly, the surgery is not always successful, and there are serious risks both with the surgery itself and in delaying. We are very worried about him, and would really appreciate your prayers.
Thankfully, we are confident that Silas is in good hands here. He will continue to be monitored in the hospital tomorrow and will have a full neurological evaluation and an MRI on Monday followed by a consultation with his neurosurgeon (the same doctor who placed his shunt back in January, who we really like), who will make a recommendation about the next steps and possible surgery.
We do not know what is coming next, but we packed our bags for a week and will be here in the hospital as long as we need to be. Thanks for remembering us in your thoughts and prayers. We'll continue to post updates here as often as we can.
