Stop-Gap Measures

Nolan has been off of his Nexium for about four or five days. After two days on the phone and an hour spent at Rite-Aid, I managed to get his former prescription restored. He was originally approved for 10mg, twice daily Nexium through the end of December, and the new prescription shouldn't have voided that approval.

He is still denied the dosage he needs (20mg, twice daily), but the 10mg dosage is better than nothing. I gave him a dose of medication this morning, so he'll be back on the Nexium for four full days prior to the sleep study.

I have heard some stridor at night, and he still wakes frequently (even prior to the insurance company denial of the Nexium), so I am anxious about this sleep study. If he fails it, then he'll be scoped to determine if the laryngomalacia has returned. If it has returned, the next step is a revision supraglottoplasty. Revision supraglottoplasties usually come with a fundoplication to curtail the reflux - obviously not a path we want to go down. I'd really rather the Nexium and Zantac do their job to control the crazy level of reflux than go through (more) major surgery.

Denied (Again)

Nolan's ENT increased his Nexium dosage a month ago, because his weight has been increasing and he requires a higher dose to maintain effective suppression of his severe GERD. Nolan was on 10mg (twice daily) Nexium, which was originally approved through December 2011. Of course, the increase in dosage gave the insurance company yet another opportunity to deny drug coverage - we got the letter in the mail today denying Nolan's medication.

This means, of course, that Nolan is currently not on ANY proton pump inhibitor (PPI) medication. For a child with reflux so severe that it causes failure to thrive and his airway to collapse, this is probably not a good idea. Of course, the insurance company must know more about Nolan's condition than his various specialists (that sentence needs a sarcasm font).

As a recap:

1. Nolan was diagnosed with off-the-chart severe acid reflux. He was failure to thrive, had dysphagia, and was prescribed Nexium at a 10mg, once per day dosage.
2. Some improvement was noted, but Nolan began having trouble eating again. Obstructive apnea and central apnea was noted during a sleep study. The Nexium was increased to 10mg, twice daily.
   
3. Nolan developed laryngomalacia and severe obstructive sleep apnea. A follow-up pharyngeal pH probe demonstrated his 10mg Nexium was insufficient to control the reflux. 30mg of Zantac (2x per day) was added to his medication regiment. A follow-up pH probe demonstrated that his reflux was barely controlled (he still had at least one break-through reflux episode on the Nexium and Zantac).
4. A supraglottoplasty was performed to correct the severe laryngomalacia (caused by GERD). At Nolan's follow-up appointment to the surgery, his ENT increased his Nexium dosage to 20mg (twice daily) to completely suppress the acid reflux.
   

Our insurance company denied the claim, and he is not on his Nexium at the present time. This means that his pharynx is currently being assaulted by acid reflux, greatly increasing the likelihood of a recurrence of his laryngomalacia and obstructive apnea.

The Zantac is not sufficient to control his reflux (it is an H2 blocker, a "lesser" drug than the PPI's). The insurance company states that we have not:

1. Tried an H2 blocker with his Nexium.
2. Tried Nexium once daily.
3. Tried Prilosec (omeprazole, 20mg) twice daily.

Numbers 1 and 2 are clearly wrong. Nolan cannot take Prilosec because it comes in a pill form. He is three years old. Also, Nexium is very slightly stronger than Prilosec, and even the Nexium can't control his reflux without an additional H2 blocker.

And so, my little boy will continue to suffer from severe reflux, and cannot get the medication prescribed to him by his physician, because an insurance company determined it is too expensive.

The odd thing is, the insurance company is going to end up paying more when Nolan ends up in the PICU again with a revision supraglottoplasty.

Nolan has a sleep study on May 3, and is not on his medication. I don't expect the results to be pretty.

Happy Easter!

The eggs are dyed.

The eggs are hunted.

The rain is pouring.

We are getting ready to settle down to a dinner of ham and Pioneer Woman mashed potatoes. In the meantime, we wish everyone a very Happy Easter!

Swimming Lessons

Nolan loves the water. A lot. This scares me a little, since he can't swim, and has absolutely no fear around the water.

I tried the toddler/parent swimming lessons with him, but we always had to pull out because of the chronic, never-ending ear infections. With his long term T-tubes in place, he's been free of ear infections for an incredible SIX months.

I logged on to the YMCA website and signed both boys up for swimming lessons, in the Pike program. There is also a "Pike with Parent" class option, which I did not choose. I learned long ago that if I am in the water with Matthew, he will cling to my neck and drown us both not learn anything.

I am a little worried about Nolan's hearing ability on the side of the pool. Nolan fares pretty well without his hearing aids in a quiet environment, but the pool is noisy and has very poor acoustics. Being hard-of-hearing is deceptive: he appears to hear decently, but then little things show that he really isn't catching it all. He was sitting right in front of the TV, watching Disney's Three Caballeros the other day. It was after bath, so his hearing aids were out. He declared, "I can hear the music, but I can't hear the talking!" In other words, he was able to hear the background score, but couldn't hear the characters talking. He can "hear," but he can't pick out the words.

We'll see how it goes. He does use some sign language for receptive situations, and we might need it in this situation. Then again, he might surprise us all and compensate with observation and lip reading. He's a pretty stellar lip reader.

He will be in the same class as his big brother, so that might help. Until, of course, Matthew does his classic freak-out routine at the sight of the pool-swim instructor combination. Lessons start on Wednesday. It should be interesting!



Also, as an update, several people have informed me that Ear Gear does offer a hearing aid clip which attaches to glasses. If anyone is in need of such protection, the clip can be found here.

Spiderman Plays Soccer

We actually had spring on Friday. Honest-to-goodness, sunny, bird-singing spring. I am ignoring the smattering of white stuff on the deck this morning, because winter is on its way out. Hopefully for good. With the temporarily good weather, Nolan has enjoyed many days of playing superhero in the great outdoors. He's pretty insistent that he will either be Spiderman or Batman when he grows up.

In the meantime, he wears his Spiderman mask much of the time - this is annoying because every time the mask goes on, the glasses and hearing aids come off. Which means that Spiderman's Mommy gets frustrated and sends Spiderman on a hearing aid/glasses finding mission. I need to invent a mask that can go on OVER glasses and hearing aids, without causing their removal. Maybe I'll just sew the kid a cape and hide the mask - capes don't interfere with his whole equipment set up.

A couple of weeks ago, we went down to the elementary school to sign both boys up for summer soccer. Matt will play in the under-6 league, and Spiderman Nolan will play in the under-4 league. Normally, I wouldn't sign a not-yet-four year old up for soccer, but Nolan is insistent that he play sports like Matthew. Actually, he grabbed the pen and started to fill out his own application (i.e. scribbled all over one of the application pages) and shouted "I pway SOCCER!" So we signed him up.

From what I understand, the under-4 league (composed of three year olds - the minimum age to play) does not really play full-on games like the bigger kids. From talking to the parent volunteers, I think every kid gets a ball, and they run around like nuts on a field for an hour. Nolan's division doesn't wear jerseys - they get T-shirts and the parents supply a pair of black shorts. This is a good thing, because Nolan wears a size 2T-3T shorts, and the soccer league's "small" size is probably a size 5 or 6. Matthew's division will play actual games, and they get jerseys. I predict a lot of time spent on the sides of local soccer fields this summer!

I finally got Nolan's birth certificate in the mail, to prove that although he is the size of your average two year old (36.5 inches), he will be nearly four years old in June. He should be assigned to a team pretty soon. I hadn't thought about it, but Julia's mom brought up a good point about hearing aids getting wet on the soccer field... perhaps we'll don the Ear Gear when we're going to soccer practice. Too bad Ear Gear doesn't have an extension to hold on glasses, too!

New Glasses

Nolan needs a bigger nose. And ears.

We picked up the new glasses today. We put them on in Walmart, then I tried to get some shopping done: I had to put Nolan in the cart, because he kept walking along v-e-r-y slowly, looking at everything. He was quite pleased with how clear things were, and was really taking everything in.

I took the boys to the playground immediately after Walmart, and Nolan seemed to do well with the new spectacles. Right now, we only have two problems: one is his flat nasal bridge, which makes glasses very hard to fit. We have to have the saddle nose bridge piece and the cable arms to keep them in place.

They still slide down a lot, which leads to the second problem: Nolan keeps looking over the top of the lenses. The amblyopic eye needs to look through the lenses to help his brain recognize that vision is now possible in that eye. The glasses slip a little, and Nolan peeks.

Hey, NO PEEKING!

Otherwise, things are going really well. He's keeping them on, though there is quite a bit of peeking. The online pair I purchased are going to be way too big: they had a 130mm temple piece, and the straight-arm glasses at Walmart were WAY too big with this length. He really needs a 122mm-125mm temple length, so I'll go back online and try to find a smaller frame. Luckily I only spent $12 (with shipping) for the pair I bought!

Turning Green.

Dear Nolan,

When I suggest that you color while Mommy cleans the bathroom, I mean "color on some paper." Not "color on your mouth and see if you can turn your tongue green." Also, while coloring your hands green is very interesting, I would appreciate it if you could refrain from petting the wet dog.



Thank you,

Your Mom.

I ♥ Spring Break

The kids are both free from preschool (and speech therapy) for two glorious weeks. No rushing out the door every morning, and I don't feel like a taxi driver ferrying children between three different preschools. To top it all off, the weekend graced us with a 70 degree day. We all felt due a good day, since April 6 looked like this:

This week is going to be fairly unstructured. We have a "traveling nursery rhyme" project for Matt's pre-K class (he has "Hey Diddle Diddle"), so there are some craft items we are going to make for his school project. Otherwise, we are going to have fun: I'm taking the boys to see Hop at the theater, we're going to hit the playgrounds when it isn't raining, and we're going to play with the dog. Life is good!

We did order Nolan's glasses at Walmart on Saturday. We got the "Go Diego Go" frames (Habitat style), primarily because they were the only ones that fit. Nolan had a bit of a tantrum because he didn't want the "baby ones," but luckily he got past his frustration by the time we left the store. He needs a 125mm temple piece, and the infant Habitat frames were the only ones Walmart carried to fit the bill. They don't actually look "babyish," which is a relief. A picture of the ordered frames is below.

They should come in by this coming Saturday. It will be good to get that amblyopic eye working again!

I also ordered some $6.95 frames from an online source. I had trouble finding the exact measurements, but I did find a pair that will work as a good "backup" pair if his Diego glasses break or need maintenance for any reason. Unfortunately, the temples on these are 130mm, so they will probably be a bit too long for regular use. I'm not too worried, since they only cost $7 - if they don't work out, we didn't break the bank. The online "backup" glasses are below:


Nolan is quite excited for his glasses to come in. We'll see how he fares once he realizes he has to wear them full - time. We've been having trouble with his hearing aids - he throws them when he has tantrums. I hope this is a short phase!

Who You Callin' Lazy?

This picture may come in handy one day...

We had Nolan's vision appointment yesterday. His left eye read the eye chart with no problem. His right eye, however, couldn't see a thing. They tried several different lenses, and he couldn't get down to the "normal" line, even with correction. The verdict?

A lazy eye due to a difference in refractive power between the two eyes. Or, as the ophthalmologist put it: "he has anisometropic amblyopia." Anisometropia (literally, "unequal seeing") is more common among children than adults, and the result can be devastating for the affected eye if amblyopia develops and remains untreated. The brain doesn't like the poor vision it gets from the one eye, so it simply turns it off and relies on the better eye.

For Nolan, his right eye is myopic with a good deal of astigmatism. His left eye is very slightly farsighted, and also has a decent amount of astigmatism. His vision screen suggested 4 diopters of difference between the two eyes, but the real exam shows that he has 2 diopters of refractive difference between the eyes. This is a bit of a relief (the greater the difference, the greater the amblyopia). There is also a difference of about 1 diopter with regard to astigmatism.

The worst thing about amblyopia is that it is completely invisible. There is absolutely no way to tell if a child has amblyopia without an eye exam - there are no outward signs.* Your child could literally be going blind in one eye, and you would never know it.

My PSA: get a comprehensive vision exam before kindergarten. Especially if your child has a hearing loss - amblyopia can be treated very successfully if kids are caught in the preschool years.

I have his prescription in hand, and Nolan will get glasses very soon. Probably wire rims with cable arms, and they'll probably be blue. Nolan wasn't thrilled with the idea of glasses at first. I tried telling him that he would have glasses like Mommy and Daddy, but it didn't really sell him on the idea.

When we used one of his little friends from the John Tracy Clinic as a role model, however, he was right on board. Nolan loves Noah, and loves Noah's glasses. When we explained that he was going to get to wear glasses like Noah does, he was suddenly OK with the whole idea. In fact, he was more than "OK" with it - he wanted them badly. When we left the pediatric ophthalmologist's (PO's) office without a pair of glasses, he burst into tears! I promised him that we'll get his glasses very soon, and he was temporarily placated. I suspect we'll have to make our trip to Walmart over the weekend, or we're going to have a very disappointed little boy on our hands!

Of course, when I got home, Nolan went up to Matthew and declared, "I get to get GWASSES!" Then Matt burst into tears. "Now I'm the only plain one!" Oh, the plight of the older sibling who has no "accessories." We'll probably get Matt a pair of kid's sunglasses, since he feels a bit left out.

We are hoping that Nolan won't have to do any patching. We return to the PO in about six weeks for a follow-up check. If the vision in his right eye hasn't reached the normal level with his glasses, we'll have to start patching his good eye. Here's to a successful reawakening of his right eye before our June appointment - he relies on his vision for distinguishing some sounds (he speech reads the m and n sounds, for example), so the thought of taking away his good eye isn't very attractive.


*Many people confuse crossed eyes or wandering eyes with "lazy eye." Crossed or drifting eyes are strabismus, not amblyopia. While strabismus can lead to amblyopia, it is not the same thing. Most children who have amblyopia have straight eyes, with absolutely no outward sign of the condition. Children will not complain of poor vision in one eye, so the only way to catch amblyopia is to get a comprehensive eye examination.

Remarkably Unremarkable

Nolan came home singing on Tuesday. He hadn't sung a note in his entire life, but Tuesday seemed to be The Day of First Singing. He started singing, and didn't stop. All. Day. Long. Apparently, his classroom has been practicing for an Easter program, and he has been paying attention. I love this video for so many reasons:

1. He's singing (of course)
2. He learned the lyrics to the song without special prompting or attention.
3. He can vary his volume appropriately.
4. He does all the appropriate hand motions at the right time.
5. It's just too darned cute!



He sang with his entire preschool class today. Watching him with his class made my heart soar: just a typical kid in a typical class - remarkable for the fact that it is so very unremarkable. For all the therapy and hard work learning to listen... the fact that my boy is "just one of the kids" makes me happier than anyone could ever know.

Happy Applesauce Day

April Fool's Day ranks highly among the 3-5 year old demographic. Matt has been trying to trick me all day, stating things such as "your hair has fallen out, Mommy!" and then dissolving into giggles as he declares a hearty April Fool's Day to me.

Nolan, unfortunately, doesn't quite get the concept. He certainly gets the joke portion - he's been telling me it is warm outside all day long. Instead of shouting "April Fool's Day," however, he has been shouting, "Applesauce!"

Apparently, April Fool's sounds a lot like applesauce when you have a hearing loss. He has since modified his declaration to "criss cross applesauce!"

I tried to correct it a few times, but it is pretty cute. I sort of like Applesauce Day better than April Fool's Day.

In other news, Nolan's vision results are included on his screening form. This is just a screening test, so I'm not sure how accurate the results are.

His left eye appears to be farsighted, with very minimal astigmatism. The screening device shows the "sphere" to be +2.5, and the "cylinder" (astigmatism) to be +0.3. This is moderate farsightedness and mild astigmatism in that eye. They typically don't correct farsightedness under 4 diopters in preschoolers, since many will outgrow the condition. This eye isn't marked as having a problem on the screening form. The only odd thing about this reading is that his left eye used to be the more myopic of the two eyes - it's just strange that this eye went from being nearsighted to being farsighted.

His right eye appears to be nearsighted, with a "sphere" of -1.6. This is mild myopia, and not a huge deal in itself. The cylinder in this eye is +2.6, which is severe astigmatism. If the readings on this eye are correct, he will require a prescription (glasses) to correct the issue. A simulation of what his right eye can see is below - a little blurry, but it isn't horrible:


The real problem is the difference between the two eyes. Anisometropia is defined as a difference of more than 1.0 diopters between the eyes. Nolan has a difference of 4.1 diopters (if the screen is correct). This puts him at risk for amblyopia. A difference of 1.0 diopter for astigmatism also puts the eye at risk for amblyopia - Nolan has a difference of 2.3 diopters (cylinder) between his eyes.

This might explain why one eye wanders off every once in a while. With the vision in each eye differing to such a great extent, his brain might be "shutting off" the weaker eye.

The problem with amblyopia (if present) is that the eye can lose vision permanently if it isn't addressed. With the pre-existing hearing loss, we really don't want to lose an eye. I'm anxious to get into the ophthalmologist next week to get a more accurate reading on the eyes and to head off any problems.