Tuesday, June 4, 2013

Reintroducing Ryan

First of all, I have to say that the internet is an amazing thing.  And it really is a small world after all.  Thank you so much to all of our friends and family who shared our blog post yesterday with their friends and family.  The response was incredible, and I'm feeling a bit better about everything.  I just want to give a quick recap/brief medical history for our little guy because in sharing that post, I was forgetting that all of these new people reading up on Ryan wouldn't know any more other than what I wrote about in the MRI post.  Hopefully this will give even more insight into what could be going on.

Ryan was a micropreemie born at 25 weeks.  He was in the NICU for 7 months before we were finally able to bring him home on oxygen and with a g-tube for feeds.  He is still on low flow oxygen due to his chronic lung disease, and he also has pulmonary hypertension, which will hopefully resolve as his lungs grow and improve.  He is currently 14.5 months old (actual)/11 months adjusted.  He receives services from the Braille Institute because he had ROP, which led to laser surgery in both eyes, which led to some scarring that needed to be removed, which led to a surgery (vitrectomy) in his right eye, which led to a cataract, which meant that the lens in his right eye needed to be surgically removed.  So now he wears contact lenses to replace that lens, and while we can tell that he sees some things, we aren't sure how much he sees.

Now for his hearing.  In October, as we were getting closer to discharge from the NICU, they performed the regular newborn hearing screening test and he failed in both ears.  So they referred us to the local hearing center 6 weeks later to take the test again.  He failed again in both ears.  We also noticed in this time that he really wasn't reacting to sound, even really loud sounds like a tantruming 2 year old in the same room. :) So, in December we went in for our first attempt at an ABR.  We really didn't want to sedate him if we could avoid it, so we tried to get him to sleep through it.  He did sleep for 45 minutes, but they needed around 2 hours, so we weren't able to finish the test.  The audiologist told me at that point that, even though the testing was incomplete, they could already tell that he definitely had severe or profound hearing loss in both ears.  We were going to have to have another ABR under sedation.

We had his Mic-key button procedure scheduled for January, and this was supposed to be a quick and easy 5 minute procedure that required sedation.  So, I arranged for the audiologists to come over to CHOC to complete the ABR at the same time so he wouldn't have to be sedated twice.  The director of audiology actually came and did the test herself, and the news was distressing.  She found that even with pitch sounds ranging all the way up to 95 decibels (the equivalent of standing by the speakers at a rock concert were her words) he had zero brain response during the test.  She said he had bilateral profound deafness, meaning he was "stone deaf" (as some people call it) in both ears.  As much as we hated the news, we were just glad we knew and could try to move forward.  She told us the next step would be to see an ENT and examine the anatomy of his inner ear to see if hearing aids or Cochlear Implants (CI) would help him.  She said the chances of a hearing aid helping him with his level of deafness was pretty slim, but there was a chance the CI could help.

In March we had a CT scan done, and his cochlear bones look completely normal.  Then we had to wait for the MRI because we needed clearance from his cardiologist to get sedated again.  We finally had it done last week with a special cardiac anesthesiologist so we could get the ball rolling on getting him hearing.  From what I have been hearing from the many wonderful people who have been commenting and sending me messages, it really is GREAT news that his anatomy looks normal.  I knew it had to be good, I was just confused as to why he was profoundly deaf if everything looked normal.  As I was saying yesterday, the ENT said over the phone that it could be random congenital deafness, and that the nerves inside the cochlea could be damaged or incompletely developed.  The MRI was looking at the auditory nerves which are outside of the cochlea and carry sounds from the ear to the brain.  From my understanding, if those nerves are damaged, then the CI might not be able to help.  But since those nerves are normal in Ryan, he should be a candidate for CI.  The reason I was so confused about him being congenitally deaf was because it just seems like with Ryan's extreme prematurity, exposure to so many ototoxic medicines in the NICU (lasix drip and many antibiotics), etc, it just seems like those trains of thought would lead to a more logical conclusion that the nerves didn't develop correctly since he wasn't developing inside the womb.  But, I'm not a doctor :)

One other thing that confuses me is that I swear he COULD hear at one point.  I have two memories that serve as evidence.  First, there was a day I was visiting in the NICU and they were testing out the fire alarm system.  Every few minutes the alarms would flare for an hour straight.  All the babies had special plastic covers to protect their ears, plus we wouldn't open their isolettes unless absolutely necessary.  So they were completely covered up and enclosed with baby "earplugs".  Every time the alarms started, Ryan would start crying.  I honestly don't know how he would've have known those alarms were going off unless he could hear them, and it sure seemed like he heard them.  Then, in August we were transported up to CHLA for an eye surgery (this was still part of his 7 month NICU stay before he ever came home).  While we were there, before the surgery, a therapist came to assess Ryan's development.  She had a musical toy that she held at either side of his head, and he definitely turned his head in the direction of the sound on both sides.  We never had any reason to believe that Ryan couldn't hear until he failed that first hearing screening in October.  So maybe somebody out there knows this...if it is congenital, would he have been completely deaf from birth, or could he gradually lose his hearing over time?  My gut tells me that after he had the eye surgery at CHLA in August, he got a very bad infection (steno) in his trachea that put him on isolation and required strong antibiotics.  After that, he wasn't assessed for his hearing until the newborn screening which he failed.

I hope this clears up some of the questions that have been asked about Ryan.  I wish I had time to reply individually to each person who contacted me, and over the summer if you are still willing to speak with me, I'll probably be sending out lots of individual emails.  In the meantime, I'll be sharing this new blog post will all of those who have had questions because I think I covered everything that was asked.  If you do have other questions or new insights based on this new information, please do not hesitate to contact me or comment here.

We contacted the House Institute today and set up an initial consultation with Dr. Luxford for next week.  Thanks to Darci for letting us know they have a satellite campus in Orange County, because we'll be able to meet him here first before we head to LA for more testing.  I will definitely keep you all updated.  Thank you again for all the support!

PS- I forgot to mention Ryan gets 4 hours of therapy a week (infant stim/speech, feeding therapy, and 2 hours of physical therapy).  He is also seen by a really nice lady from the Braille Institute one hour every 2 weeks to work on visual stimulation.  Tomorrow we will have our first ever visit from California Deaf Blind Children's Services, which I'm really excited about because I've heard great things.  I have been in contact with our regional center and called numerous resources for deaf/hearing impaired children.  Our local school district said I was the first parent to call them up randomly on my own, lol.  They are going to participate in his next IFSP in August so he can start getting services through them, too.  Dave and I have been learning ASL online for the past few weeks, and we use signs with Ryan throughout the day.  Now I think I've covered everything, but there's a chance I could come back on here to edit more!

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