On Motility Issues

When we had our consultation with the pediatric surgeon, we brought up the fact that Nolan had shown delayed gastric emptying on an earlier test (called a scintiscan). The surgeon reassured us that the majority of kids with reflux appear to have delayed gastric emptying because the food goes up as well as down, resulting a a delayed time. Once the fundoplication is performed, the food can only go down and motility "improves." This was a reassuring thought and we figured the delayed emptying was simply an artifact on the test because of his reflux.

When Dr. Wonderful called me at home the other night, he told me to forget everything he told me about motility. The aforementioned is true for kids with regular, straightforward severe reflux. It is not true for Nolan.

Motility issues have brought everything to a grinding halt.

From the few tests that have been performed, it is apparent that Nolan has two motility issues (one definite, the other possible):

1) Delayed Gastric Emptying, or Gastroparesis
2) Esophageal Motility Problems

Nolan's scintiscan showed 0 episodes of reflux, but significant delayed gastric emptying. In this case, there was no reflux to create a "false positive" result. He truly has delayed gastric emptying, and it is possibly the underlying cause to the ridiculous levels of reflux.

Nolan's 24 hour impedance probe (from when he was 2 years old) showed a slow "bolus transit time" in his esophagus. This means that food took too long to get from his mouth to his stomach.

So, why are motility problems important?

If Nolan had a Nissen Fundoplication without addressing the motility, several things could go awry. His stomach would not empty fast enough, and with the top of the stomach "tied off," he could get painfully bloated. The potential esophageal motility issues are more worrisome - with a Nissen, a child could actually lose the ability to swallow food if there are esophageal motility problems.

We are going to have a repeat gastric emptying scintiscan done at Golisano Children's Hospital. If he still shows gastroparesis, then a pyloroplasty will be performed alongside the fundoplication. A pyloroplasty is the same surgery as performed on children with pyloric stenosis - it is a cut into the bottom sphincter of the stomach to allow food to empty faster. This comes with its own set of risks and benefits, which is why they don't perform this routinely with Nissen Fundoplications - it is reserved for kids with gastric emptying problems. A g-tube is always used for kids with delayed emptying with a fundoplication, to allow venting of stomach contents or air, in the event that the stomach can't empty itself fast enough.

There isn't much to be done about esophageal motility problems. This is usually an absolute contra-indication to a Nissen fundoplication. There is a test for esophageal motility, but it is quite invasive (the test is called esophageal manometry).

Anyhow, there are a few decent articles on motility problems in children. I'm linking them here for my own reference, but also to help others understand the difference between "typical reflux" and "reflux with motility issues."

http://www.articles.complexchild.com/00005.pdf: A great, plain-English description of various motility problems in children.

http://www.articles.complexchild.com/00028.pdf: A great article about the difference between "regular" reflux and reflux with motility issues.

http://www.nature.com/gimo/contents/pt1/full/gimo46.html: An article about the difference between LPR and GERD types of reflux (Nolan has LPR reflux)

Article on gastroparesis: http://www.usagiedu.com/articles/gastrop/gastrop.pdf

And then there is this little gem, about understanding the surgical treatment of reflux in the presence of delayed gastric emptying: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1422000/

"the existence of delayed gastric emptying should not be a contraindication for a Nissen fundoplication. Second, those who do operations other than a Nissen fundoplication for the treatment of reflux may wish to consider a fundoplication in the group of patients found to have abnormal emptying, as this operation is the only one that clearly improves gastric emptying. Third, in patients with delayed emptying the surgeon should not advocate a pyloroplasty, but simply a fundoplication. This recommendation, while indirectly supported by the results of this study, stems from a number of other observations. First, pyloroplasty increases duodenogastric reflux, which may damage the gastric mucosa or worsen esophagitis if the cardia remains incompetent. Second, pyloroplasty without vagotomy is ineffective in the treatment of idiopathic or diabetic gastroparesis regardless of its effects on gastric emptying. Lastly, this study and the preponderance of evidence from other studies suggest that there is a good chance that gastric emptying will be normalized after a Nissen fundoplication. If that does not occur, there is always time to reassess the situation and devise a new strategy to deal with the problem."

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.