Summer!!

Today marks the official beginning of summer for us!  I know the actual season doesn't begin until tomorrow or the next day, but it's summer in this house full of teachers!  We celebrated tonight by grilling steaks and eating dinner in the living room so we could watch a movie and stay up a little later than usual.  Dani has figured out with my part time schedule, she can ask me at night before bath and bed, "Are you working tomorrow?" and if I say yes, she resigns to her regular bathtime schedule.  But if I say no, she says, "Can I just take a shower with you in the morning?"  So we have around 2 months of easy nights and showers in the morning.

Ryan had some visitors from the local school district today.  The deaf/hard of hearing teacher and district coordinator came by to meet him and see if they could provide any services for him.  We are really lucky to live in a district that has these services available.  I really like that she is pro-ASL and will incorporate sign language into her sessions if the parents request it.  We talked about where we are in the process for CIs for Ryan, and they were really glad that we are going to try out the hearing aids first.  It's hard to have any hope for them helping him since he is profoundly deaf in both ears, but if there's a chance they can work, you definitely want to know that before you pursue cochlear implants.  We also discussed the neurological portion of it, and they basically said we are doing everything right by seeking the opinion of a neurologist while still moving forward with all of his hearing issues.  It's really unsettling for me not to know what's going on.  I know we may never know what CAUSED his hearing loss specifically if it is sensorineural, and I'm fine with that.  I just want to be sure it IS sensorineural and not auditory neuropathy. 

I actually called the neurologist last week to get us on a list to be seen sooner if there's a cancellation.  Otherwise, we will be visiting her on July 9th.  After a well-visit a week ago where our pediatrician gave me a copy of the MRI report of his brain, I spent a lot of time googling words like brachycephaly (this one just means he has a flat head.  Wish they could've just written that!), ventriculomegaly (this is excess fluid in the ventricles, which the ENT had told me over the phone, and which we already know about because it corresponds with his mild hydrocephalus), thinning of the corpus callosum (not good, do not google), and hypomyelination (also not good, again, do not google).  Now you can see why I am a bit anxious for his neurology appointment.  Obviously I have lots of questions.  More prayers in this direction would be greatly appreciated.

Despite all of these new and slightly terrifying findings, Ryan has been interacting with us more and more!  He is finally starting to look at our faces, he's smiling more readily and laughing heartily.  He's getting better at holding his weight up and not arching every second.  He reaches his arms and legs out excitedly when he knows he's about to get picked up, and when I pick him up and lift him to my shoulder, he reaches both arms around my neck and squeezes me.  He will then trace down my arms until he grabs hold of my elbows and then he'll just hold tight for a while.  It's the sweetest thing. 

Tomorrow he has an echo to check his heart for pulmonary hypertension, and then we'll have a visit with his cardiologist and pulmonologist on Monday to go over everything.  At our last appointment, they had mentioned possibly starting to wean him of oxygen by this appointment.  I'm not quite sure he's ready yet because he still drops his sats if he gets his cannula prongs worked out of his nose while he's sleeping.  They typically don't go lower than 89/90, but it's still low enough to show he needs that extra support still.  Hopefully the echo will at least show that his pulmonary hypertension is improving if not resolved, and we can wean down on some of his meds.  Any other great news on top of that would just be icing on the cake, but of course we'll take it! 

Thank you for continuing to keep Ryan in your prayers.  His constant drive to fight is such a blessing and I know all of your prayers are holding him up and pushing him to improve.

Big Sister Update

Dani had a pretty big milestone today, and Dave and I were just talking about how cute it was, so I wanted to get it down in writing so we can remind her of this day someday when she's older.  She remembered her first dream!  If you've seen this girl's bed head, you know she must have lots of dreams when she sleeps, but whenever I asked her about them, she didn't seem to really understand the concept of dream vs. reality.

This morning she woke up in a particularly cheerful mood.  When I walked into her room, we had our normal good mornings and discussed what she'd like for breakfast.  As she was climbing out of her bed she said, "Mommy, do you want to see my special dollhouse?!"  She was so excited, so of course I joined in her enthusiasm and said, "Sure!" thinking she was going to take me out and show me one of her regular dollhouses in the living room.  Instead, she brought me to our sliding door that leads to the backyard and said, "It's outside!  It has lots of slides.  Hey, where is it?"  She put her face to the glass and searched the yard, bewildered.  I asked her if it was just a dream and she said, "Oh.  Yeah." and walked into the kitchen for breakfast.



I don't know why this struck me as so adorable, probably her face when it lit up as she remembered the special dollhouse she had dreamed up.  In a way, I was glad she didn't get upset or hurt when she realized it WAS just a dream.  She just went along with it and carried on with her day.  Hopefully her happy dreams and special dollhouses will visit her again tonight. :)

House Institute

Today we had our first visit with Dr. Luxford at the House Institute.  I was really pleased with the outcome, and I'll try to summarize as best I can.  Sometimes I feel like I should bring a tape recorder to these big appointments so I can remember everything!

We went to House because they came highly recommended, and when we made the appointment we asked for the doctor with the most experience with children.  They recommended Dr. Luxford, and so far he seems great.  Today was just the initial consultation in their Orange County clinic.  Everything else will have to be done up at their main office in LA.  They have a children's center up there, and our next appointment will be there.  In the meantime, all of Ryan's testing up until this point will be sent to him there for review.

Here's what I gathered from our meeting.  They highly recommend trying out hearing aids first to see if they help at all.  Once you do a cochlear implant, there's no going back.  Any chance of hearing will be destroyed by the surgery, and there's always the chance that the ABR that showed him to be profoundly deaf could've had a false read.  With older patients, they'll do behavioral tests to see if they respond to any sound at all, but at Ryan's age, he wouldn't be able to do them.  So, from what I understand, we'll likely get him fitted for some hearing aids to use on a trial basis and see if they make any difference.  If they don't work, then we will move on to cochlear implants.  We will start with one ear, and monitor for up to a year to see if it's helping.  He was one of the doctors who likes to give you the worst possible outcome and then if something better happens, we can celebrate instead of giving us hope and then having to disappoint us if it doesn't work out.  I'm used to that from the NICU days, so I'm still very hopeful that the implants will help, but I understand why he doesn't want to get our hope up yet.  We will just have to wait and see and continue to pray our hearts out that Ryan can have some hearing.

Dr. Luxford asked us a lot of questions about Ryan's history, and was very good about acknowledging the need to hold off and make sure his overall health is stable enough before such a long surgery.  He would need to be sedated for over 2 hours, so he would want to make sure everything was cleared by cardiology and pulmonary.  I will definitely ask about it next week when we have our Pulmonary Hypertension Clinic appointment.  He made it sound like as long as everything checked out, we could be looking at possible surgery within the next 6 months, so we'll see.

I know this shouldn't be the most exciting part...but, it sort of is pretty darn exciting.  We were under the impression that since anything related to hearing was part of an exclusion on our insurance, we would have to pay for everything out of pocket.  This was pretty crazy considering a cochlear implant can cost upwards of $30K from my understanding.  Well, we found out today that insurance typically DOES cover CI surgery!!  I was very clear in questioning that because I really thought it was excluded because it's related to hearing, and he said, "No, because it's a surgical procedure, it should be covered.  And if they denied you for some reason, it would be a very easy appeal.  The only thing they don't cover typically is hearing aids."  So, the hearing aids will be loaned to us (I think) on a trial basis so we can see if they even work before we invest in those.  What an amazing blessing and stress-reliever it was to hear that news!  Of course, part of me is still worried that the bill will just show up in the mail because we've dealt with our share of insurance snafus, but he was pretty convincing that it should be covered!

Thank you all for the kind words, happy thoughts, and prayers you sent Ryan today.  We will continue to update as we learn more about all of this.  In the meantime, we have his 15 month well visit tomorrow, and we're hoping he has gained some weight so we can cut back a bit on the formula.  Prayers in that direction would be greatly appreciated!

California Deaf-Blind Services

So last week we had a lovely visit from two very nice lady's from the California Deaf Blind Services.  They met Ryan and stayed to watch and learn about him, in addition to talking with a few of the therapists who work with Ryan.  They try to come up with recommendations for everyone to help Ryan understand his world and adapt to living with fewer senses available to him.  They stayed for 2 hours and gave us some really great ideas that I'll try to summarize here in case anyone else is looking for ideas for their own kids:

1. Introduce toys or objects made from different materials so he has a more diverse tactile experience, for example, wood, metal (e.g., mixing metal bowl), or a soft brush.

2. Include activities that encourage face-to-face interaction in every work session. Following is an article, “Early Interactions with Children Who are Deaf-Blind,” that expands on this recommendation. It also includes other strategies that may be useful for Ryan: http://www.nationaldb.org/NCDBProducts.php?prodID=34
3. Include “infant massage” in Ryan’s routine. This may help with his need for proprioceptive stimulation and may encourage a deeper connection between his parents and him. CDBS published an article on this topic, “Infant Massage for Babies with Sensory Impairments,” which can be found at http://www.cadbs.org/newsletter/resources-winter-2011/

4. Redirect Ryan’s attention to another activity when he is softly hitting his chin or his head. If this action increases when he needs to sit for a long time, such as at feeding time, have an exercise session with him beforehand so he can release any extra energy and be a little more relaxed when he is sitting down.

There were a few other suggestions that were more specific to Ryan, but I felt like these 4 could be helpful to anyone else out there looking for more information on working with deaf/blind children.  I will proudly add that they were very impressed with how well he was reaching out for objects, grabbing things with both hand, and bringing them to mid-line.  They also commented on how active he is, always rolling around, grabbing his feet, even doing his bridges (he loves to arch his back and get up on his head and tippy toes in the bridge position).  The best part was when I picked him up and put him in my lap, he turned right away and looked up at me like he knew exactly who I was.  They noticed that right away and said, "Oh look, he recognizes his mommy!"  I know that seems strange to be excited about.  With a full term baby, that sort of behavior would be a given by now.  But with Ryan, it was a huge milestone.  I really feel like the past week or so he's been started to look more at my face and realize that I'm someone different than everyone else.  He is also getting really good at snuggling and cuddling.  He'll reach his hands out when he knows he's about to get picked up, and then when I get him up on my shoulder he will put both his arms around me and rub my arm.  Such a huge improvement from when he used to just arch his back and try to wriggle away from anyone who tried to hold him. 

We discussed how hard it was for the first few months after he was home.  We really felt like we were walking on eggshells with Ryan.  He spent so much time in his chair getting his feeds, and his reflux was so bad we had to leave him sitting upright for another 30 minutes after the feed was over.  Then it seemed like any time we moved him he would throw up from the reflux.  So, unfortunately, he spent a lot of time in his various seats or on his back.  I know looking back, we really couldn't have done it any differently because he was so medically fragile back then.  Now that flu season is over and his reflux has improved, we are able to do so much more with him.  We've been taking him out every day of every weekend and are really looking forward to summer when we can do it daily.  You can tell that getting out of the house and being more active has helped him improve immensely.  We still have a long road of development to go down, but we are very hopeful for our little man. 

We also have the House Institute appointment set for this week on Wednesday.  This will be the initial consultation with the doctor who will hopefully do the procedure for the Cochlear Implant.  I had a great conversation over the phone with a really kind and helpful friend of a friend who also has a son who wears CIs.  She gave me some really great advice and helped me create a list of questions to bring in to the first appointment.  I'm really looking forward to finding out more, and I'll update as soon as I can.  Any prayers for a good appointment would be greatly appreciated!

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!

The Latest Edition of "The Results are in..."

So, after another ridiculous hullabaloo with the ENT's office, we finally got the MRI results today.  I'll spare you all the details because, well, they really don't matter.  What matters is that his ear anatomy looked fine.  I actually spoke to the doctor on the phone, who read me the radiologist report, and told me that his ears' anatomy looks normal.  Yay?  <----- That question mark is intentional.  I really want to be excited about this news.  And I am, seriously, I am thrilled it looks normal, but the bottom line to me is, what does that mean then?  I asked him a ton of questions, and I'm not sure I'm completely satisfied with all the answers, but at least we have an insight as to what his ears look like to start us in a direction.  Here is a loosely translated "transcript" from what I remember of my Q&A session with the ENT during recess this morning:

Me: OK, so if his ear anatomy looks normal, what's causing his deafness?
Dr:  It could be the nerves inside his cochlea.
Me: So, you can't see that on the MRI?
Dr: No, we were just looking at the auditory nerves.
Me: OK, so as long as the auditory nerves look normal, he'll be a candidate for the cochlear implant?
Dr:  Well, there's still a long list of tests and hoops we need to jump through, but yes, his anatomy looks like he'll be a candidate.  You and your family will need to meet with psychologists for screenings.  It will be a big deal for him to go from not hearing anything to hearing some sounds, so we have to make sure everyone is psychologically ready.
Me:  You know he's one, right?
Dr: Yes, but you all will need to be screened as well.  You'll meet with social workers, etc, etc.
Me:  So, if the nerves inside the cochlea were damaged, is there any way to see that or do you know what might have caused that?
Dr:  No, there's no way of knowing.  It could've been congenital.
Me: You mean like he was born deaf?  Could it have been because he was so premature those nerves just didn't develop properly? Or could it have been ototoxic medicines?
Dr:  Maybe, but there's no way to know.  It could've just been random congenital deafness.
Me:  But there's no history of hearing loss in our family at all.
Dr:  It can be random.
Me:  Could it be neurological?  Did the radiologist mention anything in his notes about his brain in the images?
Dr:  He just mentioned that he does have an enlarged ventricle.
Me:  Well, he was diagnosed with mild hydrocephalus, but the neurosurgeon said it didn't need to be followed, and he could just have a large head.

And so on...

So, now I'm wondering.  What the heck???  What does this all mean?  The great news is that it seems like he will most likely be a candidate for a cochlear implant, but I just don't know what to think.  If his anatomy looks normal in both the CT scan and the MRI, shouldn't we start seeing a neurologist?  Of course this is just me thinking out loud (well, out loud with my keyboard) because I know I'm going to be asking for another neurology referral as soon as I see our pediatrician next week.

I wish I had more answers for everyone, believe me!  But we will work to get to the bottom of all this and see what we can find out.  Dave and I are thinking that in addition to the neurologist, it's probably time to start seeing one of the audiology centers we've heard of in LA (House Institute) because they seem to be the best, we're just going to have to face the music with the insurance not covering any of the hearing stuff. 

Thank you all for continuing to pray with us for Ryan's hearing as we get this all figured out.  What a road we'd never imagine we'd have to walk down!  But your love and support has certainly guided the way, and I know that MRI was a miracle.  To hear the words, "The anatomy looks normal..." was such a blessing and I knew it was all in God's hands.  I'm praying now that He can continue to unravel this mystery and point us down the right path to getting our little man hearing whatever he can as soon as possible.  I'm going to share this blog post all over the place in the hopes that by some miracle, someone who knows something about audiology can maybe send me a message or leave a comment and let me know their insight. :)

Still Waiting.

I'm sending out a quick update because I know all the Ryan supporters out there are waiting on pins and needles right along with us for these MRI results.  After the MRI on Thursday they told me the radiologist who reads it should have the full read out by Monday at the latest.  So, Friday morning I called the ENT's office to see if I could set an appointment to go over the results.  They said they needed to request the results first, and then it will take a few days to get them, and then we can set the appointment once we have them.  Seems sort of backwards to me, but whatever.  All I can say is, it's a good thing I called first thing in the morning to get the request for the MRI started!  I told her, "OK, then I guess I can just call you guys on Monday to set up the appointment since CHOC said you should get the results by then?" Lol.  Hey, if it were her kid, she'd be anxious to know, too, right?!  Hopefully when I call on Monday, Ryan's doctor will be in the office and can just go over the results with me on the phone like he did for the CT scan, but we'll see.  Keep those prayers coming, it's going to be a LONG weekend!