Well, Actually...

We had our little visit with the ENT today to discuss the whole Nolan-doesn't-eat-anything issue. Since this has put us quite on edge (the total consumed today was 2 teaspoonfuls of yogurt in addition to some milk), we wrote down every issue that we've come across in the past six months (our "worry list"):

• Head lag (that's always been there)
• Problems eating
• Problems gaining weight
• Random "fevers" (99-100 degrees)
• Elevated AST and ALP (liver enzymes)
• Clutching his neck while eating or drinking
• Posterior urethral valves (that have been taken care of, thank God)

Our perplexed ENT did note there was head lag, and observed him pull at his throat while drinking.

Nolan pulling at his neck while drinking

I told her I didn't know what to make of all of this. That he wasn't eating, and if it was behavioral then we needed some help. Because he's getting worse, and not better with the current course of action. Then she asked what made me think it was behavioral. I told her that the scintiscan was negative. Her response was:

"Well, actually...."

Nothing good starts out with that qualifier. It turns out the scintiscan was negative for reflux. Yippee! It was also positive for delayed gastric emptying (DGE for short, or gastroparesis). It is borderline, because there are currently two standards used for DGE. One standard says that if only 25% of the stomach contents are emptied after one hour, then it is DGE. The other says that if only 30% of the stomach contents are emptied after one hour, then it is DGE. Nolan was at 27%, smack in the middle of those categories. By one, he has gastroparesis. By the other, he is borderline. She did diagnose him with gastroparesis, as he has symptoms consistent with the disorder and the test results indicate an issue with gastric emptying.

The next decision was whether we should be sent to neurology or to gastroenterology first. Since the head lag has always been there, we were lobbying for the gastro consult. The not-eating thing has our stress levels sky high.

She checked his weight (22 pounds, 12 ounces at 22 months of age) and agreed that we should see the GI doc. She also ordered a repeat of the liver function tests to see if those had normalized and an upper GI to be completed before we see the GI specialist. We had the liver panel redrawn today, so hopefully those numbers will come out as normal. It would be nice to wipe those off the "worry list."

Off we go to the GI specialist to find out what is going on with the little guy, and to get him eating again.

Official Audiology Report

I received the official audiology report in the mail from Nolan's latest hearing test. Everything looks great, we only had one significant drop in the last six months (at 500Hz for the left ear, by 10dB). Everything else was about the same, a 5dB loss for many frequencies, but this is considered simple variability and not a "true" loss.

They also took a reading at 1500Hz for the first time- 50dB for the left ear and 45dB for the right ear.

Because audiograms can vary by a slight amount, it is good to compare audiograms taken quite some distance apart. So, here is the left ear, with one year's difference:

His high frequencies have remained at the same mild level.

A fluctuation to 55dB at 4kHz was observed in Aug. 2008, but did not stay at that level!

And here is the right ear, with one year's difference:

The right ear has dropped a tad in all frequencies.

The shaded area are the sounds of speech that Nolan cannot hear unaided. Which is pretty much... all of them. He actually can't hear Dennis unaided at a short distance at all, though he can hear me fairly well. My voice must resonate at a fairly high frequency!

All in all, it is still good news because his hearing is helped greatly by his hearing aids. Since hearing ability is calculated by the "better ear," he is moderately severe rising to mild. Pay no attention to the categories on this teaching audiogram, because they have 55dB-80dB listed as "severe." Most terminology I have seen labels this hearing level as "moderately severe," and that is the terminology our audiologist uses.

Audiology Visit/Booth Test

I am so ecstatic to have a fixed hearing aid. Our electrical tape supply was beginning to run a tad low! Lucky for us, the aid is still under warranty, so all repairs are free. This repair would have been free, anyway, because our wonderful audiologist had battery doors hanging out in the office. It is amazing how much easier it is to work with an aid that is fully functional. The loose battery door and constant re-application of electrical tape was getting old!

Nolan also had a booth test. He did lose some low frequency hearing (we've never really had two audiograms that look exactly the same), but his high frequencies are stable. From what I remember (which isn't much-I was totally focusing on the good/stable high frequency numbers), he lost about 10dB in the lows (in his "good" ear- the "bad" ear stayed about the same).

I think his "good" (left) ear is now 60dB at 500Hz and 1000Hz, rising to 35dB at 2000Hz and 4000Hz. So moderately severe rising to mild- there's a reverse slope for you! His "bad" ear was 65dB rising to 45-50dB, so moderately severe rising to moderate. His hearing aids are still working well for him, and I'm thrilled that his high frequencies are mild in his good ear. It is harder to amplify the softer sounds of speech that occur at the high frequencies, so having a mild loss there is definitely a plus. The low tones are not unimportant, though. Consonants like /m/ /j/ and /z/ reside there. Plus, most of the vowels hang out in the low frequency range.

This does explain why Nolan has been unable to hear my husband's voice recently. He used to hear Dennis unaided, and now doesn't respond unless Dennis gets REALLY loud. He has a rather deep voice and a 60-65dB threshold is just below the speech sounds in that range. He still hears his mama just fine, though!

We took new impressions and will pick up earmolds on the 14th of July. His hearing aids will also be reprogrammed on that day, to add some amplification to the lower end of the spectrum.

An Unexpected Call

The phone rang today and caller I.D. said "Kaleida Health." Since we don't have any scheduled procedures at Buffalo Children's, I figured there must be a billing problem or something of that nature. I picked up the phone, and it was the speech language pathologist from the feeding clinic.

I had left her a message a couple of weeks ago stating the scintiscan was negative for reflux (per her request) and didn't really expect a call back.

"Well, have they done an upper GI? Did they start him on a trial of reflux meds anyway?"

Ummm... no! The ENT and pediatrician didn't really seem to want a follow up. The feeding clinic SLP told me that I needed to call the ENT now, because dysphagia in a 2 year old is not "normal." She is also calling our pediatrician with an urgent message that Nolan needs to have the cause of his swallowing issues figured out.

All righty then.

I figured it was just behavioral if the scintiscan was negative, but the SLP said that routinely refusing solids and spitting food out of the mouth is usually indicative of a medical issue. His diet is almost exclusively liquid now- he did have some oatmeal for breakfast (about 1/8 cup) and ate some yogurt for lunch (about 1/4 container). Other than that, it's just milk. If anything, his issue with solid food is getting worse, not better.

I called the ENT's office and made an appointment for July 1. If we can't find a medical cause for the refusal to eat solid foods, then we'll start attending the feeding clinic. Either way, we've got to get this kid eating. I might be able to fatten him up on Pediasure, but he will eventually need to eat regular food.

Post- Op Appointment

Mr. Nolan on the deck

We had a nice, uncomplicated visit with the urologist today, concluding our little encounter with posterior urethral valves. He confirmed that the VCUG was completely normal, and I reported that the post-operative period has been fairly uneventful. Except for a low-grade fever of about 100 degrees, but Nolan seems to almost always have that. We don't have to see the urologist again for an entire year, and when we do it will be for a bladder ultrasound. No big deal, and we'll just monitor the little guy over time to make sure his bladder doesn't show evidence of thickening (something that can happen with PUV boys). Nolan's in a really low risk category since he has no apparent kidney or bladder damage, and normal creatinine levels.

The only complicated thing is the travel. When Nolan was an infant, trips to Buffalo meant a screaming baby and a frustrated toddler. Then things got easy for a little while- Matt was old enough to entertain himself and Nolan was older and less fussy.

With a toilet trained 3 1/2 year old and an almost-2 year old, things have gotten "complicated" again. Just past Fredonia (and long past any bathrooms), Matthew announces he has to go pee pee. While I am thrilled that he is completely toilet trained, I wasn’t thrilled at the location of his urgent request. There is NOTHING between Fredonia and Cassadaga. A few minutes later. Matthew-with-the-yellow-eyes announces that perhaps he could go in the woods. I was tempted. The shoulders were narrow, the traffic fairly busy, and there was NO bathroom in sight. I told him to hold it “a little bit longer.”

We make it to Fredonia and the most blessed Sunoco on earth. I rush inside with Nolan (who has conveniently lost his shoes in the car) and a scissor-legged Matt. We got the key to the bathroom, and Matt walks inside to see a… URINAL. Wide eyed, he says (de sotto voice): “I want to pee in that.” Unfortunately, Nolan also spied it and managed to get his hands into it before I could stop him. ACK! I pull Nolan away and hold Matt up so that he can use the thing. Nolan thinks the “fountain” is funny and tries to stick his hands in the stream- EGADS! He has no ears on, of course, because I left them in the car in my haste. I don’t know what the Quickie Mart patrons could have been thinking, with me yelling, “Nolan! Don’t touch the PEE!” I get Matt cleaned up, wash Nolan’s hands in the dirty gas-station sink, and rush both boys back out to the car. Luckily I had some wet wipes in there.

I think I am going to invest in a little porta-potty. No one ever tells you about gas station bathrooms and toddlers before you have kids. Blech.

Puppet Therapy

Nolan has developed a deep love of puppets, probably because he thinks they are real. The boy will come up and pet them, hug them, and treats them as if they are alive. I have decided to use this puppet-love to my advantage, and have turned "whale feeding time" into a therapy activity.

Our whale likes to eat all sorts of strange items- from blocks to toy cars! I can make the activity as simple or as difficult as I want. For basic vocabulary, I could have Nolan feed the whale a "cow" or a "block." For positional words, I place blocks around the room and have him feed the whale the block that is under the chair or on top of the bookcase. Or I can have the whale eat big blocks, or things of a certain color. For really advanced listeners, I could have the whale eat things that are not red or things that are not made of wood. For my older son, I have the whale eat things that begin with a specific letter.

The best thing is that both my boys think this is just a game. They have no idea they are learning anything!

When You First Find Out

Baby Nolan- 1 day old. Or when we knew.

Some parents are told when their baby is no more than a day old. Some find out when a speech delay is found at the age of two. And others don't know until an elementary school screening reveals a problem. Pediatric hearing loss isn't something anyone is really prepared for. It's not in the "What to Expect" books. Most parents have never noticed a child wearing hearing aids or cochlear implant- and at an incidence of 3/1000, there simply aren't that many children out there who need them.

Then the diagnosis comes. The screening audiologist in the newborn center tells you that your baby has "referred" on the screen. It might be fluid- it might not. You take your baby home and wonder if he can hear any of the songs you sing to him. It takes a few months to get the full diagnosis and hearing aids, so in the meantime, you are left to wonder. Or your child with a speech delay is sent in for a hearing test, and the audiologist finds the cause of the speech delay- in the form of a hearing loss. You worry about how much your child has missed out on. How long it will take him to catch up.

For any parent, there is a whirlwind of medical appointments and audiology appointments following the diagnosis. There is little time to catch one's breath, or to process what you have been told. Scary statistics on reading levels and language scores pop up on every google search you perform. What is a parent of a newly diagnosed child to do?

• Find other parents who have children with hearing loss. This will ease your mind greatly, and give you greater resources and emotional support. The list at listen-up.org is wonderful for parents of deaf and hard of hearing children. If your child has been recommended for a cochlear implant, CI Circle is a great resource. They can also tell you how to use electrical tape to temporarily fix your kid's hearing aid (thanks, Tammy)!
• Buy a binder. And lots of dividers. All of your referrals, early intervention materials, doctor appointment information, audiology reports, etc. can be nicely stored inside. I also print out calendar pages from Word and include them, so I don't get appointments mixed up. I also included many clear page protectors. They're great for sliding appointment cards into or any other odd-shaped things (like CD's from CT scans).
• Expect a few medical appointments. After a diagnosis of permanent hearing loss, you will be referred to an ENT. The ENT will then order a CT scan, MRI, or both. They may also order a urinalysis, kidney ultrasound, EKG, vision exam, and other medical tests as deemed appropriate. Don't freak out about these tests- there is a rather standard protocol for kids with hearing loss.
• If your newly diagnosed child is an infant, toddler, or young preschooler- buy a pilot's cap. They help keep those little fingers from turning those expensive hearing aids into teething toys. Since Nolan has impressive fine motor skills and the pilot's cap is now useless, we use critter clips to keep them from getting lost when he pulls them out.
• If your child pulls out their aids, it doesn't mean they hate them. For younger toddlers, it is often curiosity. In Nolan's case, he gets bored and pulls them out to play with them. There is also an adjustment period to wearing the aids, so start out with a few hours per day and slowly increase it to having the child wear them during all waking hours. I've been told that by the time they hit preschool age, they're pretty good about leaving them in. Since we're not quite there yet, I can only hope!
• You have to walk before you can run. And you have to listen before you can speak. For any child learning to hear, consider their "hearing age." Children with normal hearing have been listening in the womb for several months before birth. Then they listen and hear for another year before producing their first word. Since Nolan received his hearing aids at four months old, his "hearing age" is technically four months after his birth date. For a child who first receives full access to sound via a cochlear implant at one year of age, their hearing age will be a year younger than their date of birth. Do therapy, narrate everything, and have patience. It will come. There are great therapy ideas at the Listening Room.

I would also recommend the books Choices in Deafness, Children with Hearing Loss: A Family Guide, and (for hard of hearing children) Not Deaf Enough. And ANYTHING by Carol Flexor.

Relax. Keep your sense of humor. Rejoice in your beautiful child, and know that everything really will be just fine.