Starting Soccer

Soccer is awesome.

We missed Nolan's first soccer practice because we returned late from his ophthalmologist appointment. He was rather upset about this, but cheered up considerably on Saturday: he would get to play soccer at last!

We was even more ecstatic when he received a jersey. The Under-4 league has jerseys already, and the Under-6 league does not - this meant that he constantly told Matt: "look at my shirt, Matt. I have a soccer shirt! You don't have a soccer shirt!" This began to wear on Matthew after a while (he should get his uniform by the end of this month).

I brought the FM system to the field with Nolan. I hadn't indicated any "special needs" on his soccer enrollment form - so it was probably a bit of a surprise to his coach. To be honest, I had simply forgotten to write it down. We're so used to his hearing aids, that we don't think about it much. I stuck the mic on her shirt with a brief "don't worry, he does just fine" and walked away.

And Nolan did great. He ran with the ball, he listened to the instructions, and he played the games. He was slower than a lot of the other kids (he's never going to catch the other preschoolers in duck-duck-goose), but that is mostly because he is smaller than most of the other kids. Those short little legs have to work harder to keep up!

Nolan: the little guy on the left.

He is extremely excited to play the rest of the season. He wouldn't take his shirt off when we got home, and was very proud of being "Number Seven." Soccer makes him happy, and that makes me happy.

The Letter is Being Faxed

Our pediatrician's office is in the process of drafting a letter to our insurance company, in the hopes that they will authorize our trip to the Cleveland Clinic. They asked me for a general synopsis of Nolan's medical history and our reasons for wanting to go to the Cleveland Clinic, so I sent them the sheet of paper I take to each new doctor (his medical history is now long enough that I can't spit it all out in a few seconds). Here is what that paper looks like:

Nolan's Medical History

Gastrointestinal: Severe GERD unresponsive to medical therapy, mild delayed gastric emptying, slow bolus transit time noted during first impedance probe.

Urology: Posterior urethral valves, ablated in June 2009.

ENT: Obstructive sleep apnea unresponsive to surgical measures (tonsillectomy and supraglottoplasty), chronic otitis media (four sets of PE tubes by age 3) congenital bilateral hearing loss (moderately severe mixed hearing loss), severe laryngomalacia. Wears 2 hearing aids.

Neurology: History of severe central sleep apnea (resolved), mild hypotonia.

Vision: Anisometropic amblyopia (myopic in the right eye); astigmatism in both eyes. Wears glasses.

CURRENT MEDICATIONS:

Nexium, 20mg, 2x per day. Zantac 30mg, 2x per day.

PRIOR HOSPITALIZATIONS and tests:

October 24, 2007: ABR diagnosing mild/moderate bilateral hearing loss.

February 2008: Genetics evaluation: Negative for Connexin 26, Connexin 30, and mitochondrial A1555G mutations (non-syndromic hearing loss panel).

March 18, 2008: sedated CT scan of the inner ear (normal).

March 27, 2008: PE tubes placed (set #1).

August 11, 2008: Sedated MRI of the inner ear (normal).

December 2008: Second set of PE tubes placed.

May 2009: Gastric emptying scintiscan (normal for reflux/mild delayed gastric emptying)

June 1, 2009: Cystoscopy and ablation of posterior urethral valves.

July 9, 2009: Upper GI barium (normal).

August 2009: Endoscopy, 24 hour pH/impedence probe (inpatient: severe GERD detected).

January 20, 2010: Sleep study #1: severe central apnea and severe obstructive apnea.

February 10, 2010: Sleep-deprived EEG (20 minute EEG) for staring spells (normal).

March 23, 2010: Sedated MRI for central sleep apnea (normal).

April 13, 2010: Tonsillectomy, Adenoidectomy, PE Tube Set #3 (inpatient PICU monitoring).

August 2010: Sleep study #2: Severe obstructive sleep apnea, mild central sleep apnea.

October 12, 2010: PE tube set #4 (long term “T” tubes placed), Bronchoscopy: Severe Laryngomalacia diagnosed.

November 10, 2010: 24 hour Pharyngeal pH probe: reflux persistent despite PPI treatment. Zantac added to medication regime to stop reflux.

December 3, 2010: Echocardiogram and EKG (normal).

December 2010: 24 hour Pharyngeal pH probe: some reflux still noted with PPI and H2 blocker treatment.

December 14, 2010: Supraglottoplasty and lingual tonsillectomy (inpatient, PICU).

May 3, 2011: Sleep study #3. Central sleep apnea resolved. Moderate obstructive sleep apnea, persistent despite tonsillectomy/adenoidectomy and supraglottoplasty. Fundoplication recommended by ENT. Bi-Pap or C-Pap evaluation recommended by sleep neurologist. Upper GI barium scheduled for 06/27/11; Sleep neuro exam scheduled for 06/21/11; trying to get into Cleveland Clinic for a second opinion from a pediatric geneticist.

Our reasoning for choosing the Cleveland Clinic for the genetics evaluation site was written as follows:

"Due to the repeated surgical procedures, sleep apnea, chronic ear infections, chronic sinusitis, severe GERD (unresponsive to medical therapy), delayed gastric emptying, hypotonia, urology, vision and congenital hearing issues, we are concerned that there may be a single cause underlying the various symptoms that Nolan has experienced. Prior to consenting to additional surgical measures or procedures (recommended fundoplication; C-Pap), we would like to ensure that no genetic syndrome underlies his various issues. The Cleveland Clinic has a renowned genetics program, including a hearing-loss genetics subunit, which is unrivaled in the area. If there is a syndrome or other “issue” at play, we feel confident the Cleveland Clinic will be able to discover the cause – if there is no underlying cause, then we will feel confident with their assessment that his medical history is caused by separate (unrelated) incidents."

Hopefully the insurance company's doctor will look at his history and approve another genetics consultation without giving us a lot of hassle. Our pediatrician's office has been wonderful and is supportive of us getting a second opinion.

The letter should be faxed to the insurance company today, and we should have an authorization approval within 30 days: once the authorization comes through, the Cleveland Clinic will schedule our appointment with the geneticist. This will likely occur in August or September, as it takes a while to get into the Clinic.

In the meantime, our biggest worry is Nolan's fatigue and general "droopiness." This is due to his hypotonia (we think) - the hypotonia affects his upper body and arms the worst; his feet and legs seem generally unaffected. He still has the head lag, though his development hasn't been delayed by it. Still, the effect can be striking in the afternoons. He fatigues while walking, and gets very droopy while sitting or standing.

Nolan, a little droopy in the afternoon

There is a part of me that wonders if his muscle tone issues underlie most of his other problems. The GI tract is made of smooth muscle, so the slower motility and the reflux would be affected by a muscle tone problem. His obstructive apnea could be due to low tone, in addition to reflux and the persistent laryngomalacia. For kids with late-onset laryngomalacia and muscle tone issues, the airway collapse is due to an intrinsic weakness in the system.

We're extremely hopeful that the geneticist at the Cleveland Clinic will be able to piece this together - and if she can't, that she will be able to reassure us that these issues are not connected and that nothing new will pop up to surprise us in the future.

I Want My Sister!

Nolan, his FM system, and the Tilt-O-Whirl.

I want was driving to Dennis's softball game, when Nolan suddenly shouted from the backseat:

"I want my SISTER!"

Considering that Nolan doesn't have a sister, this was a rather unusual request.

After some considerable questioning, we determined that his "sister" was not a girl, was not a friend, and was not a human child.

He finally clarified,

"I want my sister so I can hear!"

Oh. He wanted his system. His FM system. Wow - that's a first!

The new settings on his FM system are fabulous. I do have to listen to the aids to verify they're set to the right program, but once it is set, he loves it. I didn't realize how often I was tapping him on the shoulder to get him to face me, or shouting over the front seat of the car to the back seat. He used to repeat the same questions endlessly in the car, because he couldn't hear the answer until we screamed it to him. With the FM system, that issue has disappeared.

He wore it to the zoo, he wore it to his last day of preschool, and he wore it to the amusement park yesterday morning. It is simply brilliant. He can hear everyone else, but he can hear me at a slightly higher volume than everyone else. He can also hear me over distance, which is amazing. Normally he can't hear when he gets about 10 feet away in an outdoor environment, even with aids on. With the FM system, distance is not an issue. It was awesome to call to him as he was running the wrong direction in the crowded amusement park, and have him hear me calling to him to stop running.

We plan on using it at his first soccer practice this morning. Hopefully the rain will hold off long enough to let the kids play soccer!

Newest Audiogram

I received the report from Nolan's most recent hearing test in the mail today. The results look remarkably similar to the test results from Buffalo Children's last November, but they are still a change from the last hearing test at our normal audiologist's office (particularly for the left ear). The low frequencies are still a mixed loss, but everything above 1000Hz is sensorineural. His bone conduction scores have dropped by about the same amount that the air conduction scores have dropped, which indicates that the small new loss is sensorineural (inner ear loss) in nature.

For those who aren't familiar with audiograms, Nolan can't hear anything above the plotted lines when his hearing aids are off. Basically, he can't hear any speech at normal conversational levels with his aids off, though he can hear things like rock music, lawn mowers, and other loud things. With his hearing aids on, he gets most of what lies in the middle frequencies, but still misses out on the high and low frequencies (hearing aid adjustment issues).

Thankfully, the worst hearing is at 65dB (I thought I had seen a 70dB mark on the audiogram in the office, but he is still just moderately severe in the low frequencies).

His speech reception thresholds changed from 40dB in each ear to 45dB (right ear) and 50dB (left ear). His hearing aids are still set for a mild loss in the high frequencies, which explains why John Tracy was showing under-amplification in those frequencies. He has lost a bit in the low frequencies, too - he seems to lose a bit at one frequency (or in one ear), and the rest follow suit shortly thereafter. Fortunately, it is a very slow loss - it has taken almost four years to go from mild/moderate to moderately severe.

We will return on July 5 to confirm the level of loss and (finally) adjust his hearing aids to match the loss.

An Eye Appointment, In Pictures

Eating lunch in the car.

Heat makes Nolan wilt.

Looking over downtown Erie, PA

Matt plays with Legos during Nolan's appointment.

Dr. S's Office is like Disneyland.

Playing in the clubhouse.

Waiting to see the orthoptist.

Can you see the one that pops out? No, I can't!

All done - we got lollipops and we're on our way!

Nolan's right eye is still amblyopic, but the vision has improved a bit. He now corrects to 20/40 with glasses, which is pretty good for a kid with amblyopia! We are going to give the glasses another three months and re-check his vision in September. On the plus side, it gives his eye a little more time to improve. On the negative side, if we have to patch in September, he might have to wear a patch to school. Hopefully his eye will improve enough to avoid patching altogether!

Audiology: Check (Sort Of)

Hanging out at the Buffalo Zoo

The drive to Buffalo is long, but fortunately we have invested in hand-held gaming systems reared travel-hardy children. Nolan was a little recalcitrant about going into the audiology booth, swinging his legs and beginning to throw a fit. I used blatant bribery (a trip to the zoo) to get him to cooperate. I'm not above zombifying my kids with electronic entertainment or offering bribes anymore. I think I have "arrived" as a mother.

He cooperated nicely. I think the last full hearing test done at his regular audiologist was over a year ago. Their last record still shows a rising configuration in his left ear, which made his loss moderately severe rising to mild. Our testing at Buffalo Children's last November showed a flat moderately-severe loss, so I wasn't surprised when our current audiologist showed the same thing. I did see one point hit the 70dB mark, which makes me a little anxious. That's a little close to the "S" word (severe) for my liking. Thankfully that was only one frequency.

Before increasing the amplification in his hearing aids, they want to confirm with another hearing test in a month.

Oh, goody.

The strange thing is that his air-bone gap has largely disappeared. This means that his loss is purely sensorineural again - either he's been throwing false air-bone gaps (it happens), or he has a truly fluctuating/progressive hearing loss that proceeds in a very weird fashion (the drops appear conductive at first, but solidify into a sensorineural loss). Weird.

I suppose time will tell. Hopefully he'll stabilize where he's at.

Tomorrow we are going to Erie (a slightly shorter drive, but still an hour each way) to see the ophthalmologist. To patch or not to patch, that is the question!

This is otterly amazing!

A Busy Week Ahead

Nolan's preschool field trip to the Fire Station

This week is a little busier than most:

Monday: Matt's preschool, Nolan's speech therapy, and Matt's second preschool.

Tuesday: Drive to Buffalo for a hearing test, and to adjust Nolan's FM system. Matt's first soccer practice.

Wednesday: Matt's preschool, then drive to Erie for an ophthalmologist appointment (we get to find out if Nolan gets to rock the fashion world by sporting a patch and going pirate-style). Then Nolan has a soccer practice, and Matt has swimming lessons.

Thursday: Nolan's last day of preschool, and his "birthday" during class. I have a mothers of preschoolers steering meeting, and Matt has preschool.

Friday: Matt has an end-of-year picnic for pre-K at a local amusement park, so we will be spending the day having a ridiculous amount of fun in the sun!

I love the summer months, but June does get a little insane with the end-of-school parties/events, and then we have a bazillion doctor appointments for Nolan. He sees urology on the 16th (this is routine, and we are hopeful he will be discharged from this specialist). The sleep neurologist is scheduled for the 21st, and the upper GI is scheduled for the 27th. At least one trip a week to Buffalo Children's is on tap for the remainder of June: if only the drive were shorter!

The good news is that preschool ends on the 24th of June (for Matt), so July should ease up a bit with the schedule. I plan on spending a lot of time at the local parks, municipal swimming pools, and on "field trips" to the beach!