Lull in the Storm

The lake effect snow stopped for a brief period of time- just long enough to get up to Buffalo, place the pH probe, and get back home. It is coming down fast and furious again, so I am very glad we managed to get home before it started coming down again!

The other weather cat indicates the snow has stopped falling- for now

As far as the placement goes, this one was harder in some ways and easier in others. Nolan knew what was coming, and was inconsolable in the examining room. Once they shoved the lidocaine coated cotton up is nose, he was howling. In case you can't guess, this is about a 15 on an awfulness scale of 1-10.

Once we took him to the procedure room, he was clinging to me and screaming. Then it took a couple of failed tries in the right nostril, more cotton/lidocaine in the left nostril, and a final successful placement of the probe on that side. Sidenote: if you can avoid having to do a pH probe, I highly recommend avoiding it. Frankly, it sucks.

The "better" part is that Nolan calmed down while we were trying to place it and actually listened to the nurse practitioner telling him to swallow. He would cry, gulp, cry, gulp, and finally the probe was taped in place. We were done, and I could retrieve my boy from the blue board of doom papoose board.

The nurse told him he could have three stickers for being such a good boy. She held up three fingers, and Nolan immediately brightened. He held up his fingers in a similar configuration and said, "This many?" She nodded, and he squealed with glee. THe traditional "three" used by most people is the sign language number for six. Of course, he raided the sticker box and took six stickers- the nursing staff cracked up once I explained the whole "six vs. three" thing.

It was an easy drive home, with no gagging or choking this time. The nurse practitioner pulled the probe up a bit, so it is not right on his gag reflex. Mighty nice of her, I might add.

Watching How to Train Your Dragon

The numbers need to stay above 5.5- I've seen several drops to 5.6 (and one to 5.2). On average, they are much better than the last run, though. He's due for his Nexium in about ten minutes and he'll get more Zantac at 8:00pm. Hopefully this will be enough to keep his pH numbers above that dratted 5.5 number!

We will return the probe tomorrow, and determine if Tuesday's supraglottoplasty surgery is a go/no-go for launch.

A Weekend of Birthdays and Christmas

The Christmas and Advent season is in full swing at our house. I stole an idea from Julie over at Herding Grasshoppers, and have wrapped our winter and Christmas books. Every night, the boys take turns and unwrap a book, and I read this for their bedtime story. So far, we've read a beautiful pop-up version of The Nativity, a couple of Elmo Christmas books from the dollar store (hey, I was running low on winter/Christmas books), and Snowmen At Night. I still don't have quite enough books, so I am going to find the Polar Express and a few other books to add to the pile. This is a really fun way to count down the days until Christmas!


Saturday, Matthew had his fifth birthday party. He won't be five until the 22nd, but we needed to have his party on the early side, since the preschool kids go on winter break soon. We went bowling, and all the kids had a blast!


On Sunday, we went to a local Christmas tree farm and cut down our tree. The boys really loved this, since we were running through the plantation in lake effect snow.


After slaying the Christmas tree, I took the boys to see Tangled while Dennis went to his hockey game. Nolan was really into the movie, and upon exiting the theater, he said, "The horse- HA HA! He funny!"

I was really hoping for a snow day, since all of the other local districts are closed today. Our school district, however, is very dedicated and remains open despite the nasty road conditions. I suppose it will be good to get back into some sort of a routine!

The Weather Cat indicates we should all have a snow day.

Revising a Diagnosis: Acid Reflux

When Nolan had his first pH probe done, it was placed all the way down by his stomach- just above the lower esophageal sphincter (LES). The doctor had to verify its placement with an X-ray, since you can't visually detect if the probe is in the right location. The probe demonstrated that Nolan had severe gastro-esophageal reflux disease, or GERD. His LES was opening at inappropriate times, allowing stomach acid to reflux up into his esophagus. Symptoms typical of GERD are (Nolan's symptoms are in red):

Burning sensation in the throat
Vomiting
Difficulty Swallowing (Dysphagia)
Sensation of a lump in the throat
Reflux is worse when lying down
Esophagitis
Slow esophageal motility
Delayed Gastric Emptying
Difficulty gaining weight/failure to thrive

Since starting the Nexium, the dysphagia has disappeared and he has been gaining weight. We thought the GERD was resolved with the addition of proton-pump inhibiting medication: the Nexium has been a literal Godsend for his growth and weight gain.

I thought it was very strange, then, when the ENT placed a probe that was very short, and sat behind Nolan's uvula. It had a red LED light that blinked, and I could see it blinking when he opened his mouth. How could a probe placed that high detect reflux in the esophagus?

As it turns out, they weren't looking for typical GERD. Instead, the probe was a new type created by the Resmed corporation, to detect a type of reflux called laryngopharyngeal reflux disease (LPR). This is the same condition as extra-esophageal reflux disease (EEGD), but is not the same as isolated GERD.

LPR is a distinct diagnosis, because it involves the dysfunction of the upper esophageal sphincter (UES). Acid reflux is normally stopped by the UES, and does not enter the lungs or the pharynx space where the voice box is located. Unfortunately, in Nolan's case, the upper esophageal sphincter isn't functioning, so the reflux comes up and hits his larynx/pharynx. The diagnosis used to be based on clinical evidence (see symptoms below: Nolan's symptoms are in red).

Chronic cough
Hoarseness
Stridor
Croup
Reactive airway disease (asthma)
Sleep disordered breathing
Frank spit up
Feeding difficulty
Turning blue
Aspiration
Pauses in breathing (apnea)
Apparent life threatening event (ALTE)
Failure to thrive
Reflux is worse when upright, during the day
Sinusitis
Chronic otitis media
Laryngomalacia
Normal esophageal motility

Children with LPR often lack the esophagitis that occurs with GERD, because the acid shoots past the esophagus and pools in the pharynx.

The Resmed probe can be placed visually, because it is placed above the upper esophageal sphincter. The probe detects small droplets of reflux in the air, as opposed to liquid reflux in the lower esophagus.

While LPR is a distinct diagnosis from GERD, the treatment is the same. Proton pump inhibitors are always required for LPR (sometimes GERD can be handled with H2 blockers alone). LPR tends to be more difficult to control, as we have seen in Nolan's case. Children over the age of three do not generally outgrow LPR, since it is caused by a permanent neuromuscular or anatomical problem. If medication fails to control LPR, surgery is sometimes recommended.

It is highly likely that Nolan's LPR is the cause of his laryngomalacia, and we have to control the reflux before we try to do any sort of airway surgery. Since his pharynx is getting sprayed with acid droplets, the laryngomalacia will likely recur if we don't get the reflux under control.

This is why we have our fingers (and toes) crossed that the Zantac + Nexium combination will be sufficient to keep his pH levels in the right range (above 5.5 for daytime readings). Once we can get this reflux under control, we can fix his airway and hopefully many of his other symptoms will disappear!

3 Year Old Well-Child Visit

Nolan turned three in August, but we're just now getting in for his well-child visit. Visits to the pediatrician are generally unhelpful, since we only see them for the required vaccinations (we see his ENT nearly every month; the pediatrician, once per year). We haven't seen Nolan's actual primary care provider in over two years: the problem with a group practice is that the child is punted to any available medical professional, which means we have to re-educate the staff on his history every time we visit.

The lab techs came in before the nurse arrived, and poked his finger for blood. This was not a good way to start off the office visit, since Nolan was understandably upset after this procedure. They weighed him (30 pounds!!!) and measured him (36.5 inches), and then the nurse asked the question.

"Have there been any updates since your last visit?"

I took a deep breath and said:

"He had a sleep study that found severe obstructive and central sleep apnea and recurrent ear infections. He had a tonsillectomy and adenoidectomy in April, along with his third set of PE tubes. Then he had an 8 week long ear infection that wouldn't clear and they cultured it and it was fungal, and his tubes fell out. Then he had another sleep study and his obstructive apnea got WORSE since the tonsillectomy so the ENT did a bronchoscopy and found severe laryngomalacia. He had a fourth set of PE tubes placed in October along with the bronchoscopy. They want to do surgery to correct the laryngomalacia, but won't do it until we verify his reflux is under control. So they did a pH probe in November and he failed it and they diagnosed him with laryngopharyngeal reflux disease.* So now they've added Zantac and we are going to do another pH probe next week to see if it is helping. We don't know what to do if he fails it. Oh, and his feet turn out when he runs and he trips and falls a lot."

The nurse smiled and left, then the random-nurse-practitioner-of-the-day walked in.

"So, I heard he has some reflux and it is under control. That's great! Oh, your bandaid matches your earplugs!"

And this, folks, is why we stay away from the pediatrician's office at nearly all costs.

After I corrected her, and went back over the same history I gave the nurse, she wrinkled her brow and said, "well, it sounds like the specialists are on top of everything."

Since we haven't really addressed the whole feet-turning-out thing (which might be perfectly benign), I mentioned it. She told me they don't worry as long as their feet are in alignment when they are walking and standing. She wanted to see him walk, then furrowed her brow some more.

Then she put him back on the table and checked out his hips, and told us that it is probably nothing, but we should keep a sharp eye on him. If his gait gets worse (or doesn't get any better) over the next year or so, we'll be referred to Shriner's Hospital to some orthopedic specialists. Since we're perfectly happy to ignore this issue for the moment, I'm happy to watch and wait. Unfortunately, since Nolan's issues have only gotten worse over time and not better, I have a feeling we'll be seeing an orthopedist sometime next year.

We left the pediatrician's office after the little guy got three shots (Prevnar 13, Hepatitis A, and the flu shot).

The great news from today: his iron levels are normal, his urine sample was fine, and his growth is slow-but-steady (with a small dip in height).



*Laryngopharyngeal Reflux: a whole 'nother blog post.

On the Bright Side

Unexpectedly canceled appointments always lead to a lot of stress: all of that angst in gearing up for a stressful procedure is heightened when the appointment is canceled and puts a whole chain of medical events in limbo. Then we have the sitters lined up (for Matt) that we have to contact, the day of school missed for both boys, and the hours of work missed by my husband.

On the other hand, talking to the ENT office personnel every 15 minutes this morning did give me access to Nolan's echocardiogram/EKG results: totally normal. While the ENT has not reviewed the results herself, the nurse practitioner looked at the report and gave me the information. His heart is fine, despite year(s) of severe apnea.

Nolan originally had a well-child appointment scheduled for this Friday, and I forgot to cancel it. Good thing, since the pH probe has been delayed until next Tuesday. We will go to the pediatrician tomorrow and do the routine height/weight/how-is-he-doing checkup. They also do a quick blood count at the pediatrician visits, which is important because Nolan is at risk for anemia (and calcium deficiency and B12 deficiency) from prolonged use of high-dose proton pump inhibitors.

Since we have no school or doctor's appointments today, we are going to play in the snow and finish decorating for Christmas. If nothing else, living in Western New York with a medically complex kiddo keeps us flexible!

Buffalo Trips #2 and #3 Canceled

Lake effect snow: the bane of our medical-travel existence. Nolan's pre-op appointment (and pH probe placement) was in Amherst. A very strong band of lake effect snow pummeled the south towns of Buffalo last night, resulting in the closure of the I-90. 20 inches of snow fell last night on the highway, and another foot is expected today.

I called the ENT's office and we are trying to move his appointment to next Tuesday. This may impact his scheduled surgery on the 14th, but we won't know until we get the pH probe results back (on Thursday or Friday). If the surgery gets canceled, we literally won't know until a day or two before the scheduled date. This sucks, because we have family scheduled to fly in the Sunday before the procedure. I am waiting for the nurse practitioner to call me back to verify whether the data will be read by Friday the 10th of December. We need to know if the surgery is a go/no-go so that flights can be canceled or rearranged.

Drat this weather. Hopefully we won't get pummeled by another storm next Tuesday.

Every Vote Counts

The John Tracy Clinic is amazing: they offer amazing services to families of deaf and hard of hearing children for no charge. When we attended JTC this summer, we heard Nolan whisper for the first time, received recommendations for a personal FM system, and finally received information to nail down Nolan's hearing loss type and levels. Without John Tracy, we would still be wondering if Nolan had a progressive hearing loss, we would not have the FM system, and we would not have been as empowered.

Unfortunately, the Clinic struggles for funding and relies on generous donations from the community.

Please vote for John Tracy in the Pepsi Refresh challenge: imagine what they could do with $250,000! Every child deserves a voice.

http://www.refresheverything.com/jtc