Thursday, December 15, 2016

One Week with a Cochlear Implant

The initial phases of having a cochlear implant requires a lot of programming to adjust amount of current going to each electrode. This process is called MAPping, and Nolan will be going back to NYU frequently in December to get new MAPs. We went back this Tuesday-Wednesday to fine tune his hearing.

He has adjusted very rapidly to the cochlear implant and prefers it to his hearing aid, though he definitely does better in noise when he has two ears to hear (and his hearing aid is required for school to verify he is getting the best signal possible). 

Within 48 hours of switch-on, he was playing the piano. He states the sound is a little bit different, with emphasis on the "little bit." This is spectacular, as we were concerned the piano might sound significantly different to our budding pianist. 

A week later, he declares the sound as "normal" and happily plays the piano. We are impressed and did not expect the adjustment to happen so quickly.

He was also able to hear in the water for the first time. He loves the Neptune for this purpose. The Neptune cannot do all of the things the Naida can (noise programs, etc.) but it is perfect for water use. It does require a slightly stronger MAP than his Naida to get the same sound quality for Nolan.

We drove back to NYU on Tuesday after working and going to school for half a day. Nolan had a rough start in the morning as he was tired and is not a morning person. MAPping was a little difficult because he would point to a 5 (out of 10) for sound volume - the target is a 6 - and then point to a 4 when his audiologist increased the sound. This is inconsistent so we did some MAPping with live voice and found a good program for him. Talking to him later, he wanted it "louder" so when she would increase the volume, he would point to a 4 to make her increase it even more. We told him that he should be consistent with the number he chooses and not make it relative - hopefully our next MAPping session will be a little easier.

His new MAP is fantastic and he was given two different MAPs to use - one at a stronger level than the other. He set himself to the second program by the time we went over for his speech assessment.

The AVT was thrilled with his progress and obtaining a cochlear implant was clearly the right decision for Nolan. His speech understanding has soared from 36% best aided to over 90% in just one week. This is not a formal assessment since he has not been tested in the booth yet, but informally he understands most single-syllable words without speech reading using his implant alone. He won't need to be seen by the speech/language department again at NYU until we're at a year post-implant. We will be going to University Hospitals in Cleveland to have an assessment and determine what we should work on.

We were going to pick up his new Link hearing aid on Tuesday, but decided to hold off until our next session. Since Nolan still has 2 MAPs loaded and the hearing aid can only hold one program, we will wait until the 27th when Nolan has just one MAP loaded.

He has loved his "noise" program, which allows him to hear whoever he is facing and blocks out background noise. We also added an "echo" program for use in the gym, where reverberation is an issue. So he has four programs - MAP 1 (which he is not using as it is too quiet already), MAP 2 (the program he is on most of the time), Program 3 (noise) and Program 4 (gym/reverberation).

We are glad we have a little break from traveling back and forth to New York City. We will go back on the 27th and get another MAP and his hearing aid.

Sunday, December 11, 2016

Cochlear Implant Activation Day

We drove into NYC a day early, because NYU was amazing and managed to squeeze in Nolan's post-op appointment on the Monday before his activation. We stayed in Secaucus this time, because hotel rates in the city at Christmastime are ridiculous. The hotel was great, and we drove to the mall near the Newport Station and took the PATH train in from Jersey City. There was some traffic, but it wasn't horrific and was definitely worth the savings in hotel fees.

Nolan's appointment wasn't until 3:00 pm, so we drove to Hoboken to check out the Cake Boss bakery. We were able to meet this really awesome family while we were in town. It was really cool to meet everyone in person, and my boys LOVED Cormac, Colin, and Ciaran.

We wanted to do something fun that all five boys would enjoy, and the Liberty Science Center was closed on Mondays. We ended up going to a place called HudsonPlay, which has an indoor Ninja Warrior style obstacle course. It was kid heaven.

Nolan's post-op was at 3:00 pm and we were on a tight schedule to make it, so we ate at McDonald's and caught the PATH train to 33rd street. We made it into the Otology department just in time. We were taken back and Nolan was seen by the surgeon, who said everything looks as it should. Nolan still can't swim for a little while and we have to use Ciprodex ear drops for another week, but this is all normal for a recovering tympanoplasty. You can't even see the scar behind his ear - the surgical technique is amazing.

The appointment was over quickly, and we left to explore the city. We wanted to see Rockefeller Center decked out for the holidays, so we walked over and enjoyed looking at the lights. The boys enjoyed going into the Lego store and the Nintendo store. We decided to go back to Jersey City for dinner, and ate at Orale - living in Western NY, we are always desperate for great Mexican food.

It was nearly 11:00 pm by the time we settled into bed that night. We had walked a lot and everyone was exhausted, so we had no issues with falling asleep. The activity of the day was fantastic to wear everyone out!

We were up early on Tuesday, since we needed to drive through traffic and get into the city during rush hour. We didn't have to wait long before we were called back for Nolan's activation. They did an initial check on his implant and electrode #11 wasn't working, but we were told losing one electrode wasn't a huge deal. Still, I was disappointed - I hated losing even one electrode. Fortunately, the issue was caused by an "air bubble" and disappeared when current was run through the array. Big sigh of relief! All 16 electrodes were up and running.

Volume levels were tested (we couldn't hear these tests - they were all inside Nolan's head) and once set, the microphone was turned on.

He was so nonchalant about it - and it exceeded our wildest expectations. We expected him to hear "whistles and beeps" on the first day, but he was able to understand open-set words within the first two minutes.

He was given four programs that day - the first three with increasing levels of volume and the fourth one was quieter, in case the volume was too much for him on that first day. Within 30 minutes of leaving the appointment, he was on the highest volume level.

We wandered around Union Square and the Christmas market and had lunch at Max Brenner (the chocolate is to die for). Nolan's battery wasn't fully charged from the factory settings and died in about 2 hours. We were all really tired, so we opted to head back to the hotel to re-charge the battery and relax.

Nolan has been able to describe the sounds he is hearing. All voices sound much lower-pitched to him, so I sound like a man with a deep voice. Dennis is unintelligible and has a voice too low to register. Crunching leaves sound like high-pitched dinging. Clapping and water running sounds normal. He has an echo of sound on some low-frequency consonants, especially the "D" sound (this should resolve with MAPping). He is also refusing his hearing aid, so we know it isn't from the acoustic hearing in his left ear (that ear is severe so there isn't a lot of sound coming in without a hearing aid).

The next day, we returned to the cochlear implant center and Nolan was given four new programs. The first three were of increasing volume levels again, and the fourth one was a noise program. Nolan can use that program to "tune out" background noise and will only hear the person he is facing. We also picked up Nolan's new Neptune processor, which is totally waterproof and can be used for swimming, snow activities, and in heavy rain.

Before heading out on the long drive home, we went to the National Museum of Mathematics. They have many hands-on activities and it was a perfect stop for a couple of hours.

I couldn't believe how well Nolan was hearing with his implant - just one day after activation. He could hear me on the car ride home without looking at me - despite the road noise.

He has been activated for 5 days now and his progress is absolutely astounding - but that is another blog post. We are over the moon with how well he has adjusted to the cochlear implant, though now our battle lies in getting him to continue to wear the hearing aid in his other ear. We insist for school hours, but when he has the option, he removes it and prefers the implant by far.

Monday, November 14, 2016

Surgery Day

Living far from the cochlear implant center means a significant amount of planning is involved for each appointment. For Nolan's surgery, the boys' grandma flew in to take care of Matt while we were in New York City. Surgery was planned for Friday (Veteran's Day), but we needed to leave on Thursday. The boys had a half day of school on Thursday, so the plan was to let Nolan go to school and leave directly once school was dismissed.

Unfortunately, that same day, a man shot his wife and went on the lam in our local area, causing all of the schools to go on lock-down. The schools are fantastic about keeping our children safe, but this was quite stressful as we worried about where the man could be and wondered how long the lock-down would last. In addition, Dennis is leaving for a business trip in China shortly and was trying to get Visa documentation through as the trip and the Visa deadline are in close proximity. I managed to get the boys and Dennis managed to fax the necessary paperwork through by 3:00 pm, which is when we left. We made it to our hotel in Secaucus, NJ at 10:00 pm - which was a pretty fast trip! The benefit of leaving later was the total lack of traffic late at night.

Nolan's check-in time was 8:00 am for a 10:00 surgery, and we knew we needed to get through the Lincoln Tunnel. We left early and hoped the traffic wouldn't be too horrible - it wasn't and we made it to the hospital early.

 Driving into NYC in the morning

Nolan is not a morning person

We checked in and they took Nolan back immediately - which was fantastic. It wasn't long before he was weighed, checked, and allowed to watch a little T.V.

Watching college football

The anesthesiologist stopped by and we went over his history, and we opted for the pre-surgical Versed to help ease any anxiety prior to surgery. Nolan can sometimes become very anxious prior to surgery due to his past experiences, so the Versed was a good choice. It did help him remain relaxed through the rest of the morning.

Dr. Roland stopped by to mark the ear undergoing implantation, and Nolan was dressed for surgery and ready to go.

 Ready to go

Marking the ear

I was allowed to go back with him and he was soon asleep. Dennis and I went down to the lobby (Child Life is fantastic at NYU and escorted us down to the lobby to show us where the cafe was located). We ate quickly and then went back to the waiting room, where we were updated frequently as to Nolan's status. The surgery took about an hour and a half, and Dr. Roland came out to tell us the tympanoplasty was done and the cochlear implant was in place and working perfectly. Everything went as smoothly as it could have gone. 

It took a little while to see our guy, as the recovery room was a little overcrowded that Friday. They moved him to recovery and we finally saw him at around noon. He was given a little extra fentanyl as he was in some pain at that point of the day.

Sweet buddy

For other parents who are going through the same thing, the first couple of hours in recovery were pretty rough. Nolan would wake up whimpering and was in considerable pain. He received a little more fentanyl and then returned to sleep. We had to do this twice, and it was hard to see him struggling those first couple of hours. We are very grateful to the nurses, who took fantastic care of him and also recommended the fentanyl, as when he finally woke up from the final dose, he was MUCH better.

By 3:00 pm (just four hours post-surgery), he was awake, talking, hungry, and thirsty. The turn-around was dramatic and he was doing amazingly well! He drank a good deal of ice water. He requested a popsicle. Unfortunately, there were no popsicles to be found. He did get a peanut butter and jelly sandwich, but continued to ask for a popsicle. Child Life stopped by to check on him (did I mention how wonderful they are?) and immediately went on a popsicle-hunting mission. A few minutes later, he was all set.

Popsicles make everything better

Child Life also gave him this adorable teddy bear - so cute!

A friendly bear is always good

Nolan was doing well and was released at 4:00 pm. Right into rush hour traffic in Manhattan. Oh, and the new security measures around Trump Tower. And also? Trump protesters. A perfect storm of awfulness for driving in a city that is known for its "normal" congestion. 

Manhattan traffic chaos

We needed to get to the Lincoln Tunnel. We wanted to get OFF THE ISLAND. So many streets were blocked and we could not get down many of the streets that would lead to the Lincoln Tunnel. In 1.5 hours, we had gone three miles.. in a circle. It was a nightmare! Nolan was a real trooper and was entertained by his Kindle in the backseat. He was really hungry, but we were stuck in traffic and there was nothing we could do.

We finally managed to make it to the Lincoln Tunnel. I was never so happy to see a tunnel in my life!

O, Lincoln Tunnel, I adore you

We made it to our hotel and Nolan was so hungry he was nearly in tears. We walked into the lobby, but it turns out the chef had called in sick that day so there was no food available at the hotel. With the traffic outside, we couldn't get to the restaurants on the other side of the freeway, so we went to the only restaurant within walking distance from the Hilton Garden Inn - a Dominican food place. The food was good, but Nolan was really tired and decided he wasn't very hungry after all. 

Not very hungry after all

After dinner, I carried him back to our hotel room and Dennis went to the car to get our supplies. Nolan was really wiped out, but was absolutely delighted to find a cookie bouquet waiting for him in the room from this amazing family. 

After a really long and stressful day, it was such a sweet and lovely thing to find!

I managed to get Nolan into his pajamas and he settled in to watch Shrek on the television. He seemed to be OK and fell asleep around 9 pm.

Resting after a long day

He was really unsteady and dizzy that evening, so I was grateful that he settled in and fell asleep. He did wake frequently through the night, but we finally just turned on the TV and allowed him to watch it as needed throughout the night. We don't allow much television at home, so this was a treat for him.

We were able to remove his bandage in the morning. I wasn't sure what to do about this, as we didn't have scissors with us. We tried using toenail clippers, but we were never going to get through it with clippers! We found scissors at the front desk and managed to snip it off. 

Ready to go home.

We were very grateful to head home. We stopped for breakfast somewhere in the Poconos and for lunch in Corning, NY. We made it home by 3:00 pm.

Nolan has been doing really well, though he is sore and still a little dizzy. Every day seems to be a little better. He is doing really well this evening and is a lot more talkative and energetic, so I think he will probably be close to "normal" tomorrow. 

He has been loving the special presents and treats. His aunt and uncle sent him a massive teddy bear, and he is pretty happy about the gift.

Giant bears are full of awesomeness

I am sure he will be back to school soon, though I am worried about roughhousing or Nolan falling, so we may keep him home this week. He has two half-days this week, anyway, so I'd rather he be fully recovered before sending him back to the germ-filled school (strep and everything else is going around at the moment).

We only have three weeks until activation day. I can't believe how quickly time is flying! We are very excited for him to get his new "ear!"

Wednesday, September 28, 2016

Surgery Date Set

Playing in Brooklyn

We returned to the Big Apple to see the surgeon with regard to Nolan's cochlear implant. The hearing tests already demonstrated he would be a good candidate for the implant, but this appointment was to determine if he was a good candidate medically. The staff at NYU is just amazing - truly, they are world-class. Dr. R. looked at Nolan's MRI and CT scan results from when he was a baby (normal anatomy). His anatomy is good, so there will be no issues with the device placement.

The one thing we were worried about is the gaping hole in Nolan's right eardrum. We have been told the hole is about 15% to 20% of his eardrum, and I had no idea what they were going to do about that. Would they need to do a second surgery to seal the eardrum? Would that preclude him from candidacy? I truly had no idea, and was hopeful it wouldn't be that big of a deal.

Fortunately, this is NYU and they are amazing, so there will just be one surgery and Nolan will have his tympanoplasty when the cochlear implant is placed. I am extremely grateful this will be a "one and done" surgery. This will also prevent more infections from happening to that ear, as most infections arise because he has a massive hole in his eardrum. We are very careful to keep that ear dry, but it will be a relief not to worry about water in the ear anymore.

Surgery is scheduled for November 11, which is fantastic as Nolan will miss less school (Veteran's Day Holiday). He will obviously miss some school the week after surgery, but we are trying to keep the missed days to a minimum.

NYU  Medical Center

He will be "switched on" December 6 and December 7. He will get his first MAP (program) on December 14.  Hearing with a cochlear implant is a process, and the programming has to be adjusted frequently in the initial stages. The sound will likely be strange for a while, but with time and therapy his brain will adjust and the sound will normalize.

We drove home immediately after the appointment and made it home by 1:00 am. Fortunately we won't return until November 10, so we have some downtime before the crazy travel schedule hits again!

Saturday, September 24, 2016

Goodbye, Hearing - Hello, NYU

Life is insanely busy and I don't often update Nolan's blog - but here is my yearly post (ha). Nolan is now 9 years old. We have had an insanely cool summer, visiting Quebec City, Tadoussac to see beluga whales in the wild, and hiking the via ferrata in Ausable Chasm.

Last December, we went to Cleveland Clinic to evaluate Nolan's hearing. At the time, there were some small changes in his left ear (his good ear), but the right ear was largely stable, ranging from 60-90 dB (moderate to severe). His aided thresholds were in the mild-moderate range.

His speech perception using live voice was 80% in the "bad" ear and 100% in the good ear. We knew we would need to upgrade his hearing aids from Bolero Q SP's if his hearing degenerated anymore, but also knew that if his right ear lost more, he might be in the range for a cochlear implant.

Over the summer, we were getting more "whats" and non-responses from Nolan. If he was looking at you, he could understand you. If he wasn't looking at you, he sometimes wouldn't detect that you had spoken. The left side still seemed to be getting sound, but the right ear appeared to have great difficulty. I did the Ling sounds for just the right ear, and the only sound he picked up was "ah."

I knew this meant he had lost additional hearing, and made the call to NYU's cochlear implant program. I wanted an evaluation to get him into the system just in case the right ear had dropped. If he didn't qualify, that would be fine - but I had suspicions that his right ear was losing residual hearing quickly.

They reviewed his audiogram from last December and said he wouldn't be a candidate with that audiogram, but considering his history we should bring him in for an evaluation. We had hotels booked and made the 7 hour drive to New York City (this drive takes longer than 7 hours with traffic, but is still well worth the trip).

NYU performed a full cochlear implant evaluation for Nolan. We first met with the LSLS (Listening and Spoken Language Specialist) and the audiologist who would be testing Nolan's hearing. We explained that we knew he was not a likely candidate, but preferred to err on the side of caution since he had been having difficulty lately and is in the fourth grade - we don't want his academics to suffer if the left ear decides to take a sudden dive and we lose his "good" ear.

The testing was broken up into three days. Each day, Nolan sat in the booth for 90 minutes and performed various listening tasks. Day 1 was the unaided audiogram. I don't have a copy of the audiogram yet, but my suspicions were confirmed. The left ear is largely where it was at Cleveland Clinic (though his speech reception has declined slightly), but the right ear is now profoundly deaf. His best threshold was at 90 dB in the low frequencies, dropping to over 100 dB in the highs (to no response at 8000 Hz). Nolan's speech reception was 48% in the good ear with speech at 85 dB, and was very poor in the right ear (24% at 95 dB).

Suddenly we were no longer "getting into the system," but entering true cochlear implant candidacy evaluations. Cochlear implant candidacy requires a lot more than unaided testing, so we would return the following day for testing in the aided condition.

Nolan really wanted to see the Statue of Liberty while we were in NYC, so we left the clinic to explore Manhattan.

 Taking a cruise to see Lady Liberty

At the World Trade Center on September 12 

We returned to the cochlear implant clinic the following day to repeat a lot of the previous testing, this time with Nolan's hearing aids on. The audiologist said words and sentences, but this time the volume was at 60 dB instead of 95 dB, since he had hearing aids on. His left (good) ear was fantastic - he was able to get a score of 84% in that ear. The right ear, unfortunately, only scored 36% for words and 27% for phonemes, even with a hearing aid on. His right ear, even in the perfect silence of the sound booth, just wasn't really hearing clear speech. His candidacy looked like a surer thing, but they wanted to try one more thing first: more  powerful hearing aids.

Nolan has super power aids which are supposed to accommodate a loss of up to 110 dB in the high frequencies, but he was at or beyond that level unaided. We needed to determine if the Naida UP (the most powerful hearing aid on the market) would bring his aided hearing into the desired range. 

We left the clinic to explore more of New York City, including the Natural History Museum. 


Checking out Times Square

The third day of testing would be the determining factor for whether or not Nolan would qualify for an implant. Nolan had language testing performed (his language skills are insanely high, due to very early intervention and a mother that never shuts up   extensive exposure in the home environment.

He was brought back into the booth, this time with the Naida UP's. The UP hearing aid brought his left ear into the mild range for hearing levels and he obtained 100% speech recognition with it. We were thrilled! The right ear, however, did not fare as well. He was aided to the mild/moderate range with the Naida UP in the right ear. He did improve his speech understanding with the Naida UP, but was still missing half of the speech signal, at 60 dB in quiet (live voice).

This is a tricky situation - as he might do well with the Naida UP hearing aids, but his hearing loss is progressive, the right ear is already profound, and if we continue to wait the left ear will likely drop - leaving him with very poor hearing while trying to navigate the fourth grade. It is also likely the right ear will continue to drop, so we would be spending a significant amount of money (near $8000) for a very brief period of time. There is no telling when the left ear will drop, but it always trails the right ear by a few months to a year. We decided that if he was a candidate, we would proceed with implantation of the right ear. This will give him a "stable" ear (once rehabilitated) in case the left ear decides to take a dive on him in the future.

It was decided that Nolan would be a candidate for a cochlear implant in the right ear, and we were able to look at the three major brands who manufacture the device. Each brand is slightly different, but all serve the same function and operate in the same manner. It is a bit like buying a car - all are reliable and will get you to your destination, but the features vary from model to model. We took home a ton of literature from that appointment and returned to our home in Western NY to figure out which brand we want our son to have - for the rest of his life. While we know they all work just fine (cochlear implant centers wouldn't use a brand that didn't work), we need to determine what the best brand would be for Nolan.

We are returning to NYU tomorrow to meet with Nolan's surgeon. We are bringing Nolan's CT scan and MRI from when he was a baby so we won't have to repeat those tests. The process is long and involved, and we have to get insurance approval, the surgery scheduled, turn on the device, program the device, and do a lot of auditory-verbal-therapy to help his brain learn to use the signal generated by the implant. While it will take some time for him to adjust to it, his hearing will be better with the cochlear implant than it will be with his hearing aid. 

Tuesday, November 3, 2015

It's Been a Long Time

I haven't updated Nolan's blog in a long time. Primarily because I now work outside the home (and have little time for writing), and also because things have been going "hunky-dory." There is some exciting news on the home front: Nolan is growing on his own and we will be able to remove his feeding tube later this month. This is a wonderful development and we are absolutely thrilled. He'll never be a giant, but he is growing and otherwise thriving.

He plays soccer and loves it. He plays the piano and is flying through his lesson books - he has a natural talent and his fingers fly over the keyboard. He does wonderfully in the academic arena and is excelling in his third grade class.

His hearing, however, continues to deteriorate. This past spring, we had his PE tubes (grommets) removed and paper patches applied to try to heal his eardrums. Unfortunately, the right ear is stubborn with its infections and he had an infection in the recovery period. The paper patch didn't "take" in that ear.

The ear infections continue in that ear. It is a catch-22: they won't do a tympanoplasty in that ear until it is "dry," but the infections are not likely to stop, particularly since he has such a large hole in that eardrum. The nurse practitioner estimated about 15%-20% of his eardrum is missing. We had a hearing test done in an infection-free time and found the following:

The right ear is now in the severe range from 1000 Hz-8000 Hz. He does hit moderate at 500 Hz in that ear, but all other frequencies are in the severe range. The left ear is doing better, at a moderate-moderately severe level across the board. The conductive component is still there. The ENT does not believe it is due to the hole in his eardrum, though we cannot be sure. Our current ENT has decided to ignore the right ear and simply focus on the better ear. His better ear (the left one) is aiding to 25-35 dB (the "As" on the audiogram above). Our current audiology practice does not test the aided ears separately, so the worse ear is not evaluated.

Unfortunately, we are seeing some signs of speech "slippage" from Nolan. His high frequency sounds are less clear. Words like "kits" are becoming "kiss." He currently has (yet another) ear infection in the right ear. Buffalo cultures it each time, delaying the treatment time and causing that ear to continue to degenerate.

Since the audiology practices locally don't test the aided ears individually, it is hard to tell what, if anything, the right ear is perceiving. When we do the Ling 6 test with the right ear aided (taking out the left hearing aid), he only detects the "AH" sound.

I am concerned about the right ear "dropping off the map," so I contacted Cleveland Clinic for a second opinion. The audiology practices in Buffalo are not practicing according to the current best practice guidelines for pediatric audiology, and Nolan is not aided as well as he should be.

Cleveland Clinic is amazingly responsive and have a plan in place. We have an appointment for December 21 with audiology and the ENT surgeon there: we will see if there is anything that can be done for the right ear (since there is a perforation in the eardrum) and we will see what can be done with his aided hearing results in the sound booth. Cleveland does real-ear testing (Nolan has never had real-ear testing) and will test each aided ear individually. There are several options available to us, and the ultimate goal is to allow Nolan to hear better. It is possible his hearing aids can be cranked up to allow him to access more sound, or we may need a technology change.

In any case, there is our update (hey, it's only been nearly two years)!

Sunday, January 19, 2014

New Glasses

The optometrist's shop must have rushed Nolan's new eyeglasses frames after they ordered the wrong ones. Within 24 hours, we received a call stating they were ready to be picked up!

He is so excited to have his new glasses. He picked out an orange case and was thrilled to wear his new specs to school. These are definitely the right frames, and Nolan is pleased that the arms match his ski helmet (both are green).

We will pick up his new hearing aids on Wednesday. I am looking forward to it, because every other word is "what?" at the moment. I think his old aids are on their last legs!