Bryce's Cochlear Implant

December 8th was the big day. We were so blessed to have friends watch the kids for us while we were downtown. Traffic is always crazy and it was a little crazy but we made it. (Surprisingly I even beat Matt after I dropped Brenlee off with a friend.)

Bryce didn't have a clue what was going to happen and how could we really explain. Matt went back with him while they sedated him and then we waited and waited and waited. While we were waiting, we went to do our blood draw for genetic testing. There were 3 rounds of tests and the second round, the one for wolfram syndrome, was flagged with a gene that has a variance on it. We're not sure if this is just something they came across or if the gene is actually manifesting itself with Bryce. They've never seen a variance like it, so, they wanted to test us for more information.

Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness), is a rare genetic disorder, causing diabetes mellitus, optic atrophy, and deafness as well as various other possible disorders.

They've thought it could be mitochondrial and it's interesting that it can mimic some mitochondrial symptoms. Wolfram syndrome was initially thought to be caused by mitochondrial dysfunction due to its symptoms and several reports of mitochondrial mutations. However, it has now been established that Wolfram syndrome is caused by endoplasmic reticulum dysfunction. ( http://en.wikipedia.org/wiki/Wolfram_syndrome)

The doctor came out and let us know that the procedure went well. There's always the risk of hitting a facial nerve and that went fine. He turned on the device to make sure it works appropriately and it did but his response to it wasn't the 'response we hoped for.' We felt disappointed when we heard this but we knew and know that it's a shot but there might not be a change. 

He said they typically don't keep older kids over night but since Bryce has a balance issue, they wanted to keep him. It turned out to be a pretty crazy ordeal b/c he didn't care for the iv and had a breath holding spell.