So Incredibly Busy - Taking a Blog-cation

Nolan is doing fairly well, though his stomach is acting up quite a bit. It might have something to do with the insane amount of excitement that surrounds Christmas.

He did sleep two full nights with his C-Pap, however, which is wonderful. We're picking up my in-laws today and will be celebrating for the remainder of the holiday break. I'm taking a blog-cation until Christmas is over. In the meantime, here is a really cute rendition of the Christmas Story:

Annual Eye Exam

Still lacking stereoscopic vision - but the glasses are cool!

Nolan had a dilated eye exam yesterday. Things are looking pretty good! As far as the amblyopia goes, there is still a line's difference between the two eyes - one eye is correcting to 20/25 and the other is correcting to 20/30. That is a really good improvement for Mr. Lazy Eye!

The exam itself shows a slight prescription change. His right eye became 0.5 diopter more nearsighted while his left eye became 0.25 diopter more farsighted. I am very glad we caught the amblyopia through last year's vision screening program, because his eyes are becoming more divergent as far as the prescription goes. This is known as "anisometropia" - when it develops in adults, it is not a big deal. In children, however, it causes blindness in one eye (unless treated).

A little sensitive to light with dilated eyes!

We are able to hold off on patching indefinitely, which is great news. I need to order new glasses to fit the stronger prescription, and he has to continue wearing them during all waking hours (or else the amblyopia will worsen).

The best news is that we don't have to return for a full six months - and the next exam will be free from eye drops!

Pediatric Surgeon Consultation Appointment Made

It is crisp and sunny outside - I am VERY thankful that this winter is starting off on a mild note. I certainly hope it stays this way, at least through the beginning of January!

I made an appointment for Nolan's pediatric surgery consultation. We have an appointment for January 9th at Strong Memorial Hospital to discuss the potential Nissen Fundoplication. Rochester is a good three hours from our house, so we'll probably go up the day before and stay the night. The science museum looks like a fun side-trip, so we're thinking of taking both boys to the museum on the Sunday before the big appointment.

I am not sure what 'happens' at the consultation appointment, but I suspect we'll review his history and go over prior test results. It is possible that a few tests will be ordered (a gastric emptying scan and possibly esophageal manometry), and we will be able to ask our questions. I found a great article on Complex Child that suggests 10 questions every parent should ask before proceeding with a fundoplication:

1. Does the child have motility problems?

This is the biggest question we can't quite answer. Kids with motility problems do worse with fundoplications than kids who have no motility issues. Another gastric emptying scan and esophageal manometry will be able to help answer this question. Nolan had one GES over two years ago, which showed "borderline" gastroparesis (27% emptying time after one hour). The problem is that there is no national standard for pediatric gastric emptying times. In some clinics, Nolan's time would be considered "normal." In others, it would be considered flat-out delayed. We need to re-examine this angle.

2. Have all medical treatments been tried?

Yes. Nolan has been on adult dosages of a proton pump inhibitor and an acid blocker, and the reflux persists. When on certain antibiotics (which should improve potential motility problems), the reflux worsens or remains the same. He has failed medical therapy for reflux.

3. Has my child had the appropriate testing to rule out other conditions that mimic reflux?

Yes. There are many other conditions that mimic reflux (like cyclic vomiting syndrome, eosinophilic esophagitis, etc.). Other than the potential gastroparesis, Nolan has had all of the recommended and required tests:

a) A pH probe to demonstrate the presence of acid reflux, even on high doses of anti-reflux meds (this is the gold standard for diagnosing reflux).
b) Upper endoscopy with biopsies to rule out eosinophilic esophagitis. Nolan's upper endoscopy was performed two years ago, showed reflux damage, ectopic gastric mucosa, and blunted villi in the esophageal tract. Eosinophilic esophagitis was not present.
c) Gastric Emptying Scan. Yes. The gastric emptying scan was performed over two years ago and showed borderline delayed gastric emptying. This is a question that remains, which fits in with the first question regarding motility problems. He has never had manometry performed.
d) Upper GI Barium to rule out anatomical problems: Yes. Nolan has had two upper GI series performed, both of which showed normal anatomy. There is no malrotated intestines or hiatal hernia present.

4. Does my child have a history of vomiting, gagging, or retching?

Possibly. Nolan occasionally goes through vomiting cycles, similar to what is seen in kids with delayed gastric emptying. He often gags and/or chokes on reflux. He does not retch. Again, this ties into possible motility issues that will have to be investigated prior to consenting to a fundoplication. Since Nolan's type of reflux is not typical GERD but is LPR (extra-esophageal), motility medications will probably not help his situation greatly - both sphincters malfunction and the reflux ends up in his airway. In this case, ruling out motility issues will simply help us decide if the fundoplication will cause more harm than good, though when the lungs are affected, a fundoplication is nearly always indicated.

5. Does my child aspirate his secretions or food?

No. Nolan does not have aspiration problems with his saliva or with food. Kids who aspirate their own secretions have a harder time following a fundoplication - and a fundoplication cannot "fix" this problem since the aspiration is of secretions above the level of the reflux. This is not an issue with Nolan, fortunately.

6. Does my child have a history of neuro-irritability, visceral hyperalgesia, or chronic abdominal pain?

No. Nolan is often nauseated, but doesn't seem to be in acute pain very often. This is often seen in children who have neurological impairments, and a fundoplication may worsen abdominal pain in these children. This doesn't apply to Nolan.

7. Has a GJ Tube been tried?

This question was written primarily for children who are receiving a fundoplication for reflux associated with failure-to-thrive issues. In these children, a G-tube is often already present, and the child is not feeding orally.  For these kids, a GJ tube can bypass the stomach entirely and prevent reflux, with fewer side effects than a fundoplication, particularly for children with neurological issues.

In Nolan's case, this is non-applicable. While on the low end of the weight charts, he is not failure-to-thrive. His reflux causes apnea and airway issues, but we have the weight issue under control. He eats orally and does not have a gastrostomy tube, so trying a GJ tube is inappropriate in his case.

8. Does my child have a history of esophageal atresia?

No. Kids with esophageal atresia often perform poorly with fundoplications. This is non-applicable to Nolan.

9. Does my child have dysphagia or feeding problems?

Not really. When in the middle of a bad reflux flare, Nolan will avoid solid food. He was dysphagic as a toddler (difficulty swallowing) due to the reflux, but this has improved greatly with medication. In general, he doesn't have feeding difficulties. There are still questions about his esophageal motility, which ties in to the first question on this form.

10. Is my child under two years of age?

No. Kids under the age of two often "outgrow" reflux. Children over the age of 3 or 4 are nearly all severe, chronic, life-long refluxers. Children under the age of two should not have a fundoplication unless the reflux is life-threatening (apnea, airway, or severe failure-to-thrive are present). At the age of 4 years, 4 months, Nolan's reflux has only gotten worse with time. He will not outgrow the reflux, and faces lifelong high-dose medical therapy, which has proven to be ineffective for him.

The questions provided by this article are great, because it helps us to narrow down the relevant questions prior to consenting to the surgery. Really, the biggest outstanding question is regarding motility issues - we need to get more definitive information on this problem, particularly since Nolan had a questionable gastric emptying scan and a longer-than-normal bolus transit time on his last pH probe.

Well Child Visit/Some Good Days/Phone Calls to Make

Last Tuesday, Nolan had his four-year-old well child visit.Yes, he turned four in August... but insurance mandates that physicals be 365 days apart, so they have been slipping farther and farther away from his actual birthday!

The little guy is doing well. The vital stats:

Age: 4 years, 4 months
Height: 39.5"
Weight: 32 pounds
Clothing Size: 3T

He's meeting all developmental targets, and at this appointment his nose and ears were clear. His lungs were a little "wet" sounding, but he has been coughing a lot with reflux lately. His weight percentile is down a bit from last year. At his peak, we had managed to get him to the 24th percentile. Now he's in the 14th - still great, but obviously we keep a sharp eye on his weight and supplement with DuoCal in yogurt when he stops eating solid foods. His height is great - 22nd percentile.

The rest of last week was wonderful. His reflux seemed to settle a bit, and he had a clear nose and ears. We don't often see him "clear" anymore, and the difference in energy level between a healthy Nolan and a refluxing Nolan is astonishing. When he has energy, he doesn't know what to do with it and is absolutely HYPER.

Saturday night, he complained of a headache. He was in a lot of distress, so I put him to bed with Tylenol, and left the C-Pap off. With a full-face mask, we don't take any risks if he might get sick in the night.

He woke up in the morning and was fine, but coughing and gagging a lot. We're now measuring the "good days" in shorter and shorter time increments.  Reflux, welcome back.

We went to the Christmas Tree Farm and Nolan was extremely difficult the entire time. His energy was gone and he wanted nothing to do with the process.

And then there is the sinusitis. His nose is running like a leaky hose again. At least his ears are clear.

He almost never naps, and he slept on the way home. We were hoping he'd get a good nap in, but he woke as soon as we got home from the tree farm.

Mr. Cranky spent the rest of the day watching cartoons. We left the C-Pap off again last night, so that we don't exacerbate the reflux (in Nolan's case, the blowing air just forces the infected material in his sinuses into his lungs and ears). Hopefully this will be a short bout and he'll be "clear" again soon.

In the meantime, I have to call the pediatric surgeon today to set up an appointment. I just hope that we get an appointment on a day without snow - I have no desire to drive to Rochester in a blizzard!

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!

Finally, Dry Ears

Watching the Grinch Who Stole Christmas

Nolan's ear dried up on Wednesday. His hearing still seemed "off" in that ear (he should still hear unaided in that ear if you are within three feet or so and speaking loudly). His hearing seems to have improved today, though, which is wonderful. He has a hearing test slated for tomorrow afternoon, and we really wanted clear ears and a return to his standard level of hearing. His hearing is pretty crummy anyway, but we'd rather see his normal moderately-severe level than the severe-profound levels that an infection incurs.

The Teacher of the Deaf (TOD) for the elementary school called and asked about budgetary requests for next year. Nolan already has his FM system, so I couldn't really think of anything to add to his IEP. She did put in some extra money for batteries, since the FM system drains batteries fairly quickly. The only "snag" that we might hit is if we change hearing aids and need different receivers for the FM system, but I am pretty sure the receivers we have will work with nearly all hearing aid brands. Plus, the next set of aids will probably be Phonak, which happens to be the same brand as his FM system - they should function seamlessly.

I did have a laugh when she said that Nolan could just use a speaker soundfield system if his ears are infected. The school's only other child with a hearing loss has a very mild loss in the high frequencies. If Nolan has an ear infection and is unaided, he can't hear anything. Period. He drops down past the 90dB range and is functionally deaf - there is nothing to help that situation! Even when his ears are clear, he can only understand words projected at a 90dB level (unaided) - his last test showed 88% accuracy with words at 90dB. Unless she's going to set up a speaker to blast out rock-concert levels of sound, it won't work. I have a feeling the school will need a bit of an education to deal with his level of loss - he has to use his personal FM system at all times in the classroom. Nothing else will allow him to hear in noise (and if he's unaided, he simply can't hear conversational speech at all).

In any case, hopefully Nolan's hearing test tomorrow will show stability. I'm worried about that right ear, which has been having difficulty for the past two months. It was really a problem when the little guy (temporarily) lost the rest of his hearing in the left ear - let's just say that we have been using a lot of sign language in the classroom over the past 2 weeks, because he simply couldn't hear a thing. His right ear was also the first to drop the high frequencies back in 2008 (the left ear fluctuated up and down and then finally followed suit in 2010). The right ear seems to precede the left ear when we do see changes in hearing, so I will be greatly relieved if that right ear is nice and stable!