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!

Wednesday, January 15, 2014

New Glasses and New Hearing Aids: FAIL

On Tuesday, we were supposed to drive up to Williamsville to get Nolan's new hearing aids. The audiology center called and confirmed the appointment, I took the day off work, and sent Nolan to school on Tuesday morning in a state of glee - he was very excited about his new "ears."

The phone rang an hour before I was supposed to leave. The audiologist called, and said that the hearing aids didn't have the correct FM receivers, so there was no point in driving up to Williamsville, since we'd just have to return a few days later to have the FM receivers installed.

Apparently, the audiologist had noticed the wrong FM receivers were shipped from Phonak when they arrived in December, and had her staff member place an order for the correct ones. The proper receivers never arrived, and she checked on the status the day we were to drive up to collect the hearing aids. Phonak had no record of Nolan's name and no record of the order. The new FM receivers have been ordered and are on a "rush" status, but we won't be able to collect them until Wednesday the 22nd. Nolan was beyond disappointed, but what can you do?

On the bright side, at least they managed to call BEFORE we had driven for 2 hours to the hearing center.

Today, we were supposed to pick up Nolan's new glasses. I took another day off work, and Nolan missed his morning classwork. We arrived at Spectrum and the technician pulled out a set of frames. Blue and brown ones.

Nolan said, "They're supposed to be green!"

Nolan with his old glasses at the optometrist. This was supposed to be the "before" picture.

There was some argument - the tech insisted these were the correct frames, until she checked the serial number we had actually ordered - but we had ordered the frames with the green arms to match Nolan's ski helmet. Once the records were checked, she admitted the wrong frames had been ordered.

So Nolan doesn't have new glasses, either.

Unfreakingbelievable.

Now I get to take 2 more days off work - though we're not sure when the new glasses will be in. They have to order new lenses and get the proper frame sent to the store.

Hopefully sometime by the end of January, Nolan will have new hearing aids and glasses. Heavens knows that we pay enough for these necessary items - if only the medical staff were competent enough to provide them!


Friday, January 10, 2014

Happy 6th Hearing Birthday

Nolan's sixth hearing birthday was yesterday. I would have posted a blog then.. but, truth be told, I completely forgot. I didn't remember until the audiologist called to confirm his appointment on the 14th, when I suddenly realized - hey, it is January 10th! Yesterday was the anniversary of when he first heard sound!


He has grown a lot since those first moments with his hearing aids. I'll never forget the day before he received his first hearing aids. It was a sunny day in January, and I was so anxious about the future. How would we communicate? Would he lose more hearing? Was anything else wrong with our precious baby? Would he talk? What would his life be like?


The next day, his ears were born. He could hear something without hearing aids at this point in his life. He had a moderate hearing loss, but all speech sounded like Charlie Brown's teachers. Hearing aids would bring those blurry sounds into focus - allowing him to hear all the sounds of speech.


My bobble-headed baby boy loved sound, and was a mere four months of age when we turned on his ears. Over time, answers came to my questions.

Would he lose more hearing? Yes. Nolan now has a moderately severe to severe hearing loss. He is likely to continue to lose hearing (albeit slowly). With technology, however, this isn't as much of a worry - he will always have access to sound, even if we have to change the technology down the road.

As for how we would communicate - well, we often have to tell Nolan to take a five minute break from talking. He is the most verbal person in our family. His receptive and expressive language is off the charts. He talks. He sings. He plays the piano. And sometimes the pipe organ.



He is one of the top readers in his class. I used to worry that he would need help with his reading - as it turns out, he is pulled out for a special reading group. This reading group, however, is for the advanced readers in the first grade.

Was anything else wrong with him? That question unfolded over the years. For several terrifying years, we agonized over what was going on with our little boy. The inability to grow, breathing difficulties, constant ear and sinus infections, amblyopia... every new symptom caused a wave of panic. Now that we have some answers, we are able to help him and avoid the vomiting attacks and other problems that come with dysautonomia (one of the unfortunate conditions that often comes along with Ehlers Danlos Syndrome). He is growing. His vision is now correcting to 20/30, and we have stopped the sinus and ear infections. Some things aren't "fixable," but he is stable and we know how to help him. I finally feel like we have a handle on things.

And as for what his life would be like.. well, it is simply regular. He goes to a regular first grade class. He plays on a soccer team in the summer. He is a bit of a daredevil and rides roller coasters that I will never, ever dare to ride. He calls himself the "green bullet" when he darts down the ski hill. He is a confident, articulate, rambunctious six year old boy.

Life is good.

Wednesday, January 8, 2014

New Hearing Aids and Glasses

We haven't picked them up yet, but Nolan's new hearing aids are on order. We will drive up to Buffalo on January 14th to pick them up - we chose the Bolero Q70 SP model. These hearing aids have 16 channels (as opposed to the eight channels Nolan currently has) and are water-resistant. They also have an integrated FM receiver.


The integrated FM is a huge plus. Nolan's personal FM system has not been working properly since he started school in September. He has a Phonak Inspiro, and it has had multiple issues. First, the transmitter was sent to Phonak and they did some basic maintenance... but the transmitter would not work once it was sent back to the school. Phonak replaced the transmitter when it was sent in for the second time, as there was a faulty circuit board. Then it was sent in for a third time because it wouldn't work. Now, one hearing aid won't register the FM signal - but this time, we think it is the hearing aid and not the FM system. Hopefully getting Phonak aids (instead of the Unitron aids he currently has) will help solve our FM problems. While his teachers are so happy to state that he "compensates well," lip reading and filling-in-the-blanks is not the same as actual hearing. He misses a lot.

He is also getting new glasses, which will be picked up the day after getting his new hearing aids. I am excited for his soon-to-be improved vision and hearing!

In other news, the windchill has caused our school to cancel for two days in a row (-30 Fahrenheit with the windchill, -12F as the "regular" temperature). It is warming up tonight, and will actually rain on Saturday. The kids are getting a bit antsy, as the weather has been too cold to play outside and there is a lot of pent-up energy here!

Monday, January 6, 2014

Matt's pH Probe Results


In November, we received a call from our ENT's office with Matthew's pH probe results. The probe shows he has severe nighttime reflux - over 109 episodes while he was asleep. This explains his frequent apneas and sleep arousals, which results in very little REM sleep.

Coincidentally, we had noticed a lot of behavior problems starting in October - I didn't make the connection at the time, but we took him off his anti-reflux medication prior to the pH probe. In hindsight, his emotional meltdowns and ADD type behavior was directly caused by the increase in reflux when the medication was removed.

The probe was extremely valuable for a number of reasons, including:

1. We know he has acid reflux affecting his airway, as opposed to other conditions that can mimic reflux.
2. We know he doesn't reflux during the day at all, but has severe reflux when sleeping.

The second point is particularly important. Originally, Matthew was on a low-dose (10 mg) of Prilosec, given once per day in the morning.  The information gained from the probe gave us the ability to medicate his GERD more appropriately: he is now on 40 mg of Nexium, given in the evening.

Once we started the Nexium, the turn-around in his behavior was phenomenal. He is no longer melting down on a regular basis, is able to focus, and is able to reason again.

We return to the ENT's office on February 5 to discuss his progress and determine what the next step should be (likely another sleep study to keep tabs on his apnea).

I have been absent from this blog - I am now working outside the home part-time and life is insanely busy! It is a whirlwind of work/school/tennis/piano/ski club: all positive, wonderful things, but writing has been placed on the back burner!

I will update on Nolan in another post - Happy 2014, everyone!

Friday, October 25, 2013

pH Probe for Matthew


We drove up to Buffalo again this morning to have Matt's pH probe placed. His supraglottoplasty failed and he still has sleep apnea, so we need to work out the reasons why he still has poor sleep and respiratory issues during sleep.

During his last laryngoscopy, his airway was very red and inflamed. Since he has the laryngomalacia, a presumptive diagnosis of reflux was made and he was placed on Prilosec. A very conservative supraglottoplasty was done (just trimming one floppy side instead of both).

Since he still has apnea, we need to accurately determine if he has airway reflux before considering any further steps. Since he has never been formally tested for reflux, we were told to take him off his anti-reflux meds a month ago. This morning, a probe was placed to detect reflux in aerosol form in his upper airway.

Placing the probe was, of course, an awful ordeal. No child wants to have a tube placed up their nose and down their throat. I won't go into detail, but let's just say that four nurses were involved, along with a papoose board and a lot of tears. Fortunately, the probe was placed quickly and in one try. Once it was in, Matt was fine.

We went home and he's been building a big Lego airplane we bought for him. Thank goodness for Legos.


We return in the morning to have it removed. I haven't actually seen any reflux events, so it will be good to determine if he actually has reflux or not. If he doesn't, then he doesn't need to take medication for it. In addition, revision surgery for his airway would be much less risky.

We'll see what the data shows.. we will get the results on December 4.

Hearing Tests

We took the boys up to Buffalo for hearing evaluations on Tuesday. Nolan didn't have a hearing test, but we did do another aided audiogram (new audiologist) and selected new hearing aids. We are still waiting on a price quote, but Nolan will be getting Phonak Bolero Q hearing aids. We're quite excited about this, as we feel it will let him hear better. Technology has improved over the past 6 years, so new hearing aids are a good thing!


Nolan did get new impressions, as he will receive new earmolds with the new hearing aids. He chose a new color, too - he loves changing his earmold colors!

Matthew had testing for central auditory processing disorder, which is called CAPD, CAP, or APD, depending on where you live. He does NOT have APD, which is good to rule out, as it has a similar symptom set as ADD and as sleep apnea. We already know Matt has sleep apnea, so the inattention/moodiness/zoning out are due to the apnea and nothing else.

We did, however, find that Matthew has developed some very minor hearing loss. It is nothing that can or should be aided at this point, but his audiogram looks something like this:

He used to test as 15dB across the board (normal for children). While the main area of the speech banana is not affected, he is now on a yearly monitoring schedule so that we can aid him if and when the loss enters the speech range.


I am waiting to receive his audiogram in the mail, as this might be slightly "off" from the actual one. The right ear is slightly better than the left ear. Basically, he drops to 50dB at 8,000 Hz (ultra high frequencies) and touches mild in the low frequencies. He is "normal" for an adult hearing level, but has a slight hearing loss for the pediatric age range (better than 15dB hearing level). This does explain why he mishears the S vs. SH sounds. He can 'hear,' but might have difficulty in noise and might have some trouble with ultra high frequency sounds.

This does explain some of the "ADD" type behaviors.. calling his name when he's watching TV and in a busy location - it is not the busy nature of the situation that is distracting him.. he is having difficulty hearing with clarity in noisy situations. In any case, this is something to keep a close eye on.