ENT Visit/Sinus CT Results/Fundoplication Referral

Why does the flurry of doctor appointments always seem to come over the winter months?

On Monday, we went to see Nolan's ENT to discuss his sinus CT results. We had been given a heads-up that the CT results showed sinusitis, so that wasn't really a surprise. His sphenoid and maxillary sinuses show evidence of chronic sinusitis, a result of the ever-present acid reflux that inflames the area. The mucus can't drain, so it becomes infected. Since the sinusitis is caused by the reflux, we can't really treat the sinus disease until we get the reflux under control.

We also discussed Nolan's ridiculous ear infections. We now know why the antibiotic drops can't cure the infections. The child has stomach acid coming out of his ears - there is no amount of antibiotic therapy that is going to work in that scenario. You can clear the resulting infection, but the inflammation and drainage are going to remain.

Of course, this means that our attempts to treat the symptoms of Nolan's acid reflux are failing. We can treat the apnea with a C-Pap. That is, when Nolan can tolerate the C-Pap mask and isn't too nauseated at night. We have removed his tonsils and adenoids, trimmed his lingual tonsils, had his larynx trimmed and his epiglottis tacked up. We have had four sets of ear tubes inserted, and supplement his diet so that he can maintain his weight, even when he won't eat. We have him on adult dosages of two different anti-reflux drugs. And none of it is really working.

And then there is the nausea and stomach pain. We have a four year old child who begs us for more medication. Who asks for a bowl to hold onto at bedtime. Who says, "I feel sick" several times each day. Who gags and coughs from reflux on a regular basis.

With his lungs, airway, ears, sinuses, and nutrition affected, avoiding a fundoplication is no longer feasible. He has been referred to Strong Memorial Hospital in Rochester for a Nissen Fundoplication evaluation. We don't have any appointments yet, so I have no idea when the ball will get rolling. I don't know what the evaluation process involves, or what the overall success rates are for a child with Nolan's particular form of acid reflux.

We do have a bazillion questions for the surgeon, and I am busy formulating a list.

On the plus side, the ENT did call over the audiogram from his hearing test on Friday, and his hearing is definitely stable. We'll take all the good news we can get around here!

Stable

No more pictures, Mom!

Nolan has been having a lot of trouble hearing lately, so we were concerned about his test on Friday. As it turns out, his hearing is stable - changes of 5dB here and there, but the audiologist was happy to report a similar audiogram to the one he had in July. 

I suppose his difficulty in hearing simply comes from the deep end of a moderately severe hearing loss - he still can't hear his father (even aided) unless Dennis repeats words over and over again. His distance hearing is abysmal - he can hear me if I'm within 6 feet (aided) but outside of that he is lost. Frustrating, but something we'll just have to live with. Thank goodness for the FM system, because he wouldn't hear a thing in school without it. 

I should get the report sometime next week. I didn't actually see the audiogram, so I want to see if the "5dB" was a 5dB loss across the board, or if there were 5dB variations up and down. It makes a difference, because Nolan has lost hearing very slowly: 5dB + 5dB + 5dB adds up over time. If the 5dB is up and down, then I won't be concerned - that's just normal variation.

He does have granulation tissue on his left eardrum - not that surprising considering the recent infection. Otherwise, his tubes are open. We took new impressions for earmolds, since the right earmold had a small rip. He hasn't actually outgrown them (the last set were made before we went to John Tracy, over a year-and-a-half ago). With all the infections, however, I wanted new earmolds. I don't know if the old ones are harboring any infection, but why take the chance? We'll pick up his new molds on January 6 - for the first time, we didn't get blue-and-white swirls. Nolan wanted something different!

We're off to the ENT tomorrow to discuss the "abnormal" sinus CT scan results. I also have to ask about additional medication for the reflux, since Nolan is frequently nauseous throughout the day and begs for relief. I'm going to ask if he can have children's Tums (on top of his high-dose Nexium and Zantac). 

In the meantime, we're happy for hearing stability. We take good news where we can get it!

Party Time (and sinus CT results)

I was able to volunteer in Nolan's class today for his Thanksgiving feast - it was a lot of fun! Nolan was a Pilgrim, and the children sat down with all of their friends for some "friendship soup" (everyone brought a can of alphabet soup to cook together, with the extra cans donated to a food bank).

The little guy's ear drops have started causing agony again - he is one of those kids who has extreme pain with the application of the Ciprodex drops. I have no idea why - I thought we were going to be fine this round, but now that a lot of the fluid/infection has started to dry up, the drops are actually penetrating his tubes and reaching his middle ear space.. and causing pain. I hate to see him in that level of pain - you know it is bad when a child asks for a shot instead of a few ear drops! Unfortunately, we can never get the same doctor twice at our pediatric group, so no one has any idea about this problem. And convincing a new doc that your kid has intractable pain with the application of ear drops is impossible. The paradigm is: "patent tubes = ear drops." I have to talk to our ENT about it, because the little guy has too many infections each year to go through this every time.

Nolan is still without an ear, and unfortunately the infected ear is his "good ear." While both ears were the same on his audiogram last July, we know the right ear has been having some trouble lately - and the left ear is infected. He is coping fairly well, though he is getting none of the Ling sounds correct. He can discriminate (or figure out) whole words, though, so he's struggling along. We have been using sign language to communicate since he can't hear well with the one ear - our sign isn't very good, but at least we can ask him if he needs to go to the bathroom or clarify basic instructions that he can't hear. To top it all off, his right aid keeps getting impacted with "gunk," which causes it to quit working. His last ear infection lasted for 6 weeks, and that is a long time to go without adequate hearing. Hopefully this one will heal up faster.

Our little pilgrim's sinus CT report came back, and the nurse practitioner at our ENT's office called with the results. They show sinus disease (chronic sinus infections, likely from the reflux), so we will discuss that at our appointment on December 5. I am very glad they gave us a heads-up, because I hate getting bombarded with new information and having to make decisions on the same day. We can review the information and do some research before making any surgical or medical decisions.

Our medical insurance is changing, too, and the cost of Nolan's hearing tests will increase. The new insurance will only pay for one per year, and Nolan has been having them more often due to decreasing hearing levels. He is due for a test in January, but I am going to call our audiologist to see if we can get that bumped up to December, before the new insurance takes effect. Of course, with the chronic ear infections, it will probably be a moot point - we'll probably have to repeat the test in January, anyway. I hope and pray the ear infection(s) dry up and his tubes stay clear so that we can get an accurate reading before the end of the year.

A Nice, Easy Appointment

Nolan's ENT ordered a CT scan of his sinuses since they have been infected for the past two years (another consequence of airway reflux). We headed up to Buffalo Children's today, with Nolan complaining heavily about missing school.

It took a lot of convincing to get him to lie still on the table - once he saw the papoose board, he started to wig out. No surprise there, since that apparatus has always been connected to painful procedures in his experience - the technician walked him through the procedure with his hearing aids and glasses on, and then I took his "ears" and "eyes" off for the actual procedure. The little guy did great!

Listening to the radiology technician before the actual scan.

The entire scan probably took 2 minutes - sinuses are faster than imaging the inner ear, and I was extremely thankful that he can lie still and cooperate. Kids who can't lie still have to be sedated for the procedure, and that adds a lot of stress to a simple test! Nolan did beautifully and I put his hearing aids back on as soon as I was allowed back into the room. It took longer for the radiology department to burn the CD of his CT scan images than it took to actually perform the test!

We left Buffalo, waved goodbye to Canada (across the Niagara River) and stopped by the McDonald's on I-90 for lunch. Nolan loves watching the trucks pass under the bridge, and a nice man stopped by and showed him how to pump his arm to get the trucks to blow their horns. Nolan thought this was really fun - I had to drag him away to get lunch!

McDonald's is a post-doctor treat for the little guy,  and he ate his apples with gusto. He wasn't very interested in the chicken nuggets, but was very happy to have some one-on-one time with Mommy.

I have the CD with the images of Nolan's sinuses - it is sort of weird to see your kid's sinuses on an X-Ray!

I still have to make the follow-up appointment to discuss the results (and to get the ENT to look at Nolan's ears - they've been bothering him, probably due to wax). Hopefully we'll get a week or two off before we trudge back up to Buffalo. I don't mind the trips, but I am less fond of traveling when the snowy weather hits!

CT Scan Results

Nolan’s temporal bone CT scan came back as totally normal, which is really good news. This means that his cochlea (the inner ear hearing organ) is normal, his vestibular aqueducts are normal (if these were abnormal, he would have a progressive hearing loss), and all other osseous (bony) portions of his inner and middle ear are normal. If his hearing loss ever does progress, this means that he would be a cochlear implant candidate.

I also got the genetics report in the mail today (I had already written that those tests came out as normal). The genetics department wrote into the letter that they observed no abnormalities in Nolan suggestive of a syndrome, and that while these three tests came back as negative, it does not mean that Nolan’s hearing loss isn’t genetic. The empiric data show that we have a 18-25% risk of having another deaf/hard of hearing child (if we were to have more children) and Nolan has an empirical 10% chance of having a deaf or hard of hearing child. Matthew’s chance would be far less than this (the same as the normal population), assuming a recessive gene is at play. These are empirical values, however, and not actual risk numbers (they’re just based off the averages from other kids with unknown causes of deafness).

They don’t feel that Nolan’s deafness was caused by environmental factors or illness.

When talking to the ENT, she said she was no longer concerned with his head shape, since it appears to be rounding out and the geneticist was unconcerned. No additional mention was made of the midface hypoplasia, either in the geneticist’s report or by the ENT. They were supposed to do a head CT scan, but I don’t think they did this. I’ll ask again tomorrow with the ENT at Nolan’s ear tube surgery to see if they did the head CT in addition to the temporal bone CT. So far, though, his head appears to be normalizing and doesn’t appear to be causing any problems.

Dr. Brodsky (the ENT) said she would present his CT scan before her radiology rounds tomorrow to discuss the next step. She has not seen the actual scan herself, but has received the report. The hospital was supposed to give us a disc of the scan (we asked several times and they wouldn’t). Unfortunately, this means she hasn’t had the chance to look at the scan herself. She always double checks the results (we don’t expect her to find anything the radiologist didn’t, however). She was quite surprised that they ran only a limited genetics panel. She said that they typically run a lot of markers on deaf/hard of hearing kids and they only ran 3 on Nolan.

We are not quite sure what the next step is. His ear tube surgery is tomorrow (he has visible fluid in both ears, worse in his left) and then we have a follow-up (post-op) on April 30. Dr. Brodsky does want him to have a follow-up hearing evaluation (he’ll have one at least every six months during his early childhood). Since we don’t know the cause, we don’t know if his loss is progressive or stable and he’ll take continual monitoring for a while to see how stable he is. Configured losses (like Nolan’s) are more likely to progress, but that doesn’t mean that all configured losses progress. He will have an eye exam at some point, but probably after he is a year old or so. Other than that, we’re pretty much at the end of the diagnostic process- we’ll probably have more genetic markers run and an eye exam, but after that we’re done. He’ll be monitored by the ENT until he is no longer a pediatric patient (or we move) and will be monitored by the audiologist.

We may never know what caused his hearing loss, but we know what DIDN'T:

• Jervell Lange Nielson Syndrome
• Branchi-Oto-Renal Syndrome
• Alport Syndrome
• Large Vestibular Aqueduct Syndrome
• Mondini's Dysplasia (or any other cochlear dysplasia)
• Connexin 26
• Connexin 30
• Mitochondrial A1555G

So now we just watch and wait!

March Doesn't Look Any Better Than February...

I was really, really hoping that the doctor appointments would slow down once we hit early March (I only had Nolan's CT scan and six month well-baby visit scheduled). I should have known better!

I went to the ENT today and she checked Nolan's ears for fluid. Apparently, my mantra of "fluid be gone" did no good and he has fluid in his left ear. Since he's had fluid in at least one of his ears since January 9th, and one ear infection to boot, the ENT scheduled same-day surgery on March 27th to place PE tubes. We'll go in on the 26th of March, and if by some great and wonderful miracle the fluid is gone, we'll cancel the surgery. If not, we proceed.

The one thing that makes my mommy-freak-o-meter begin to ring is the fact that Nolan will be sedated twice in a short period of time: once on March 18th for the CT scan and again on March 27th for the PE tubes.

I also had the genetics bloodwork done today- they are testing exactly what I thought they would be testing (thank you, Dr. Google) and his blood has been sent to Boston University for Connexin 26 testing, Connexin 30 testing, and the mitochondrial A1555G mutation testing. It will take 4-6 weeks to get results back, so we should know by about the time Nolan has his tubes placed.

One thing the receptionist at the ENT mentioned was that the guys doing the CT should give us a CD of images, and to bring that to the pre-op appointment on March 26th. Don't think I'm not burning us a copy of that bad boy! Nolan's medical file is beginning to take over the better part of a file cabinet drawer!

The schedule as it now stands:

February 29: Earmold impressions
First week of March: Pick up earmolds
March 7: Nolan's 6 month well-baby check
March 18: CT Scan (sedated)
March 26: ENT appointment
March 27: PE tube placement (sedated)

Maybe April will be a nice, quiet month...

Back to Square One

I received the approval letter in the mail for Nolan's CT scan. This would normally be a happy event (since it took some argument from the ENT's office to get it covered). Unfortunately, I looked at the dates of coverage: January through March 10, 2008. Nolan's CT isn't scheduled until March 18!

I called our lovely insurance company to get an extension, and was told that the ENT's office would have to reapply for a coverage extension. Essentially, we're back to square one. I have to call the ENT's office (once they're back from lunch) to get them to apply for the extension.

The hospital only does CT scans under general anaesthesia once per month, so we don't have a choice as to the scan date. I just hope this doesn't push us back to April (especially with the frontal bossing).

I am full of ire toward insurance companies at the moment.

In other (good) news, the microphone covers arrived in the mail today. That was fast! I love Buffalo Hearing and Speech Center- the audiologists are WONDERFUL and they have cutting-edge equipment.

Head X-ray Results

The pediatrician's office called and Nolan's head X-rays were normal. This greatly reduces the chance of craniosynostosis (a head CT is required for confirmation). This is good/bad news- good news because it means that we probably don't have major surgery in the near future. Bad news because it means we have no idea what is causing the frontal bossing. I guess the CT scans and the trip to the geneticist will set us on the path to find out more. I am really glad we were able to get into the geneticist so quickly. I REALLY don't handle waiting very well- when you are worried about your baby, you want to know what it is, and you want to know NOW!

Still waiting for news from the ENT on the approval/denial of the temporal CT and the scheduling dates for both the head and temporal bone CT scans...

UPDATE: The ENT's office called and insurance has approved the temporal bone CT! This means we will only have to sedate once for both scans. Buffalo Children's should call next week to set up the date for the CT scans.

And the Marathon Continues...

I thought I'd post an update from the ENT visit yesterday. We've been on a bit of a medical marathon this week, and I will be glad when Saturday arrives and we get a break from all the doctors' offices!

The ENT told us the EKG and urinalysis were normal (which I already knew, but was glad to hear again). She noted Nolan had fluid in his right ear, so we have to go back to her office at the end of February to check on that ear. She was also quite concerned about the frontal bossing(unlike the pediatrician) and is sending us in to see a geneticist and to get a full head and temporal bone CT scan.

I took Nolan down for his head X-rays this morning. I should hear back from the pediatrician on Friday or Monday. X-rays are not the diagnostic standard for craniosynostosis, but are a good first step. We're getting a full head CT anyway, so that will definitely let us know if his sutures are closing prematurely.

The geneticist called and we have an appointment for February 8. I'm glad we got in so quickly! We'll start the process of looking for syndromes associated with hearing loss and frontal bossing.

The bad news is that our insurance company is balking at the temporal bone CT. This is a standard part of the diagnostic process for kids with hearing loss- to visualize the cochlea and vestibular system. Oddly enough, they don't require approval for the full head CT. Because of the difference in what they are willing to cover, we may have to do two separate CT scans. This means Nolan will have to be sedated twice, which is frustrating at best. The ENT's office is currently arguing with the insurance company to get them to cover the temporal bone CT. If the ENT's office doesn't prevail, we'll go ahead with the CT scan and begin the appeal process to get the temporal bone CT covered. Insurance companies can be quite terrible to deal with. They're great if you never need medical coverage, but if you have any medical issues be prepared for many fights!

Anyway, we should know by tomorrow whether or not we get to fight Univera for coverage of the temporal bone CT. It's a necessary part of Nolan's diagnostic process. Sheesh.