Tuesday, January 31, 2012

Surgery Scheduled

Nolan's surgeon called on Monday. Nolan's case had been presented to the GI team, and they reviewed all of his records. Nolan's reflux is very severe, but his records are a little confusing.

His initial tests (in 2009, prior to diagnosis) were:

Upper GI Barium X-Ray: Entirely Normal (normal anatomy, no reflux noted).
Gastric Emptying Scintiscan: Delayed Gastric Emptying (gastroparesis) noted, normal for reflux.

The other tests from the ENT's office included:

Pharyngeal pH probe, done ON medication (Nexium): significant airway reflux detected.
Pharyngeal pH probe, done ON medication (Nexium + Zantac): significant airway reflux detected.
Upper GI Barium X-Ray (2011): Normal anatomy, one episode of reflux noted.

Fortunately, the remainder of Nolan's tests from the GI doctor's office arrived. Those tests included:

pH Impedence Probe (dual channel): Severe reflux, slow bolus transit time.
Endoscopy with Biopsy and Pathology report: Reflux esophagitis with cellular dysplasia (Barrett's Esophagus).

Whether Nolan has delayed gastric emptying or not is completely insignificant now, because he has cellular changes in his esophagus - these cellular changes are very, very rare in children. The severity of Nolan's reflux, coupled with the respiratory symptoms, is enough to warrant a Nissen Fundoplication. With the cellular changes evident at the age of two, a Nissen Fundoplication is the only real way to proceed.

Barrett's Esophagus is a condition that predisposes people to cancer. The cells exhibit specific changes, and over the course of time, can turn into esophageal adenocarcinoma. The average age of diagnosis for Barrett's Esophagus is 55 years - in other words, the average person with Barrett's is a senior citizen.

Since children with this condition are so very rare, many GI doctors will not see a child who has these changes in the esophagus. I'm not sure how Nolan's original GI doctor missed it, or why she didn't ever do a follow-up endoscopy and surveillance biopsy, but I suppose the point is moot. The GI team at Golisano's saw it, and that is what matters. His GI care will be in Rochester now, anyway, so the little guy will be appropriately cared for.

The surgery (Nissen Fundoplication with Mic-Key button placement) has been scheduled for March 7 at Golisano Children's Hospital (part of Strong Memorial). The surgeon will do a repeat endoscopy with biopsy prior to the surgery.

If the biopsy shows that his esophageal cells have reverted to normal, then we can rest assured that the Barrett's is gone. If the biopsy shows that the dysplasia remains, then Nolan will be scoped and biopsied every six months for the rest of his life. If a scope shows changes in the pathology of the cells (advancing stages of this pre-cancerous condition), then further action would be taken at that time (I won't go into that now).

I'm still flabbergasted that our original GI doc never did a follow-up scope. I know the condition is very rare, but surveillance is mandatory with dysplasia - the risks are simply too high to ignore.

I'm also kicking myself, because this was one of the very few reports that we did not have a copy of. We still don't have an actual copy of the report - I only remember being told (verbally) that the pathology report showed "ectopic gastric mucosa" and "blunted villi," not having any idea of what those two things meant (and searching the interwebs for useful information with those words was futile).

The good news is that there is now a team in place to follow Nolan and to monitor the histological changes. I can't express how grateful I am that we made the decision to transition to Rochester for Nolan's care. And, hopefully, the new biopsy will show healthy, normal cells that haven't been altered by repeated acid exposure.

There are a few research articles on Barrett's Esophagus in children - I'm posting this here for my own reference:

Barrett's Esophagus

Barrett's Esophagus in Children: Diagnosis and Management

Endoscopic Surveillance of Children with Barrett's Esophagus

Barrett's Esophagus in Children: A Study of 31 Children:


TheSweetOne said...

Hooray for competent, informed and caring doctors! Hooray for Mom and Dad making a big move and getting another opinion and better care. Scary that all this is going on.
Re: the earlier GI not catching the cellular changes: if it's something seniors get, a pediatric GI may not be trained to see it. We face that for Lauren - but the other direction - for her cardiologist. When she's an "adult" we need to find a cardiologist who treats adults with congenital heart defects. Most cardiologists caring for adults are only trained for acquired heart disease... Not that it gives the GI an excuse, just an avenue for grace for everyone.

dlefler said...

To be fair, it is such a rare thing in a child. She may have NEVER seen it in a kid. Even the team in Rocheseter said it's rare. In fact, I think the term "Very Rare" was used about 15 times in one conversation. It was coupled with "low percentages." So really, I don't think I can fault our GI. Our ENT is top of the game and she knew to send us on- she's fantastic for getting us to Strong.

Herding Grasshoppers said...

Five more credits toward your medical degree... and more prayers for Nolan, March 7!


Julia said...

Heavy. It's gonna be a tough winter for you folks, warm temps notwithstanding, and I hope all goes well and the dysplasia is absent at the next biopsy. Does Nolan know about the surgery date? How's he coping with all this?

dlefler said...

We haven't told him about it yet. Child Life (the emotional support/psychological division of children's hospitals) don't recommend telling children until closer to the actual date. Since preschoolers don't have a great concept of how long a week or month is, they will worry that it is going to happen to them every single day until the actual event. We'll start talking about hospitals and surgeries about a week out from the surgery, and talk about the actual G-tube a little closer to the surgery itself.

We are being sent a "Tubie Friend" (stuffed animal with the same tube Nolan will get) to help him adjust. Hopefully he'll recover from the surgery, the apnea will begin to disappear, and we can ditch both the C-Pap and g-tube in short order.

Terena said...

wow. that's a lot, but I'm so glad the doctor's are now aware of it and staying on top of the issue. Has to be scary, though. Sending you some strong Mama support.

We don't tell Queen Teen about appointments or procedures too soon either. Usually I give her about a week, because even at the age of 17 she'll obsess and worry non-stop. I don't like to spring it on her either, or she'll wonder if I'm hiding something scary. Tricky balance.

Kyla said...

Glad surgery is on the calendar. It is always somewhat a relief when I can stop holding my breath and waiting for the other shoe to drop. Now you at least know when the show is dropping! ;)

As for the Barrett's, I would be SO upset! I know that the his GI isn't well-versed in these rare things, but I'd probably put a polite call into the office to let them know what they overlooked, so it might not happen to someone else in the future (not that they are likely to come across another 2 year old with Barrett's, but you know what I mean, LOL!).

dlefler said...

Kyla, I do want to get a copy of the pathology report. If for nothing else, to make sure that they realize surveillance has to be done for these kiddos. He's probably fine since it was only 2 years ago, but still... while rare, there should be some sort of standard protocol for monitoring these kiddos. I am glad we moved on to Rochester from Buffalo for his GI care, though!