Symptoms of dysmotility can look a lot like GERD.
- Abdominal distention and pain
- Refusal to eat more than a small amount/feeling full quickly
- Intolerance of bolus feeding
- Or any combination of these
More often than not in kids, dysmotility means food is traveling too slow. The most common is delayed gastric emptying. Check out this video from John Hopkins of how a stomach is supposed to empty vs. when there is dysmotlity.
Gastroparesis (from Traci, Lucas' Mom)
Lucas was 13 days old when he started to projectile vomit about 2-3 feet. He wasn't a good eater from the start. He would take a couple of ounces at best. He was spitting up frequently. By one month old he could swat a bottle out of my hand. He cried a lot, he slept poorly, he arched and squirmed. Reflux was an understatement. Our GI said it was the worst reflux she had ever seen.
Shortly after we began tube feeding at 2 months we realized that bolus feeding was not working at all. Lucas would vomit before the feed was finished. He would vomit an hour or two after a feed. Slowing it down didn't work. We moved to continuous feeding. While it was a big improvement, there was still a lot of discomfort.
First things Lucas was tested for:
- Structural abnormalities - Upper GI series (8 weeks old)
- Swallow Study - checking for aspiration and dysphagia (difficulty swallowing) (8 weeks old)
- Endoscopy with biopsy - testing for food allergies, Eosinophilic Disorders, they also checked down to the duodenum for structural abnormalities (3 months old)
- Lower GI series/Colonoscopy with biopsy (13 months old)
- (non GI) Cystic Fibrosis, Heart conditions, Metabolic Conditions, Endocrine Issues
All these tests came back normal, except he aspirated thin liquids, which has nothing to do with his motility. During this time we really tried oral feeding - we would stop feeds for about an hour before an oral feed and then leave him off feeds for 2 hours after. Because we had to keep him off feeds for so long, his weight gain suffered. Oral feeds often yielded vomit. We didn't want to create more oral aversions, so we slowed it down.
Ironically, we never performed a Gastric Emptying Scan (see Tests). There was so much clinical evidence that the stomach wasn't emptying properly, we didn't think it was necessary. Moreover, we weren't confident in the ability of Lucas' stomach to handle the volume needed to conduct the test.
By the time he was 6 months old he moved to a G tube. The first thing we noticed was the large residuals we saw when we vented his stomach. We could see an ounce or more when he was running at about an ounce an hour. He also continued to vomit large amounts for the low rates we were running him on. Now, we are talking 8 - 13 times a day, 24 hours a day and one vomit event can have 4-5 retches. Looking back I am amazed he continued to gain weight. But, he was definitely low level dehydrated. We struggled to get in and keep in what he needed.
We moved to a GJ at 13 months and it was like I got a new child. He was so much happier, he slept better, he gained weight, he made more progress on milestones. Bypassing his stomach was fantastic, all the formula stayed in. (See About Feeding Tubes for more on GJ tubes). We regret not doing it sooner. About 2 days after getting the GJ, Lucas started to projectile vomit bile at the rate and frequency that he was vomiting formula. We were told to start continuous venting. We had to figure out how to do it with an active almost toddler. Farrell bags are traditionally used, but don't really work when the stomach won't re-feed what empties out. G output - the combo of bile, stomach juices and possibly what was orally fed, should lessen in a couple of weeks of the transition to a GJ tube. We continued to see a lot of G output, so we moved to get manometry testing done. (write up in Tests) Manometry measures the muscle movements and coordination. About the same time we did genetic testing. Lucas was 19 months old. Essentially when you run out reasons for dysmotility, you may end up at genetic testing. Lucas was low-tone, had developmental delays, including speech delays. But, so many of his issues were GI related, none of our docs (including specialists) considered a genetic cause likely.
The manometry showed there was no peristalsis. His stomach muscles weren't reacting to the presence of food. Because we were seeing so much G output, there was some concern that there was dysmotility in his intestines, too. But that wasn't the case. Genetic testing revealed he is missing 4 genes on his 17th chromosome (17q21.31 microdeletion, now called Koolen de Vries Syndrome). The missing genes are the root cause. Chromosome abnormalities often cause low muscle tone. Lucas' stomach is very low-tone. His brain sends the signals to start digestion, but the muscles aren't strong enough to respond correctly.
As kids grow and have muscle development, motility can improve. Lucas' long-term outlook is good. We were told that as his muscles strengthen they should begin to work correctly. He has moved from a GJ to a G tube, but is still continuously fed.
Multiple Areas of Dysmotility (from Katy, Peyton’s Mom)
For the first three years of Peyton’s life, other than a poor latch and delays in adaptive skills, she ate fine. She loved food. She was 50th percentile; her growth from being an IUGR baby was fantastic. Her bowel movements would often switch from infrequent to diarrhea, but it never seemed to bother her. Peyton has a progressive disease, and unfortunately all of the above mentioned would change, drastically. I will start from the top and work my way down.
Peyton’s swallow had always been off. She gagged, she coughed, she would put her head back and fuss. We used to think that it was due to her GERD. Following her third difficult to treat pneumonia it was suggested that Peyton have a swallow study. Her esophageal dysmotility was an incidental find, but it made total sense. Her peristalsis was so slow that eating solids was painful and difficult. While watching the swallow study take place, there were pieces of banana just sitting in her throat. I could not imagine the feeling. This was one of the many reasons Peyton naturally gravitated towards purees. Their advice was to have Peyton take a sip of drink in between bites. This obviously made eating a bit of a chore.
Early 2010 when Peyton was three in a half, eating became a huge issue, getting her to drink liquids was worse. We started keeping track of exactly what she took in. Most days she would eat breakfast, pick at lunch, and avoid dinner. Her average intake of fluids was 12 oz per day. She was getting dehydrated all the time. We were all baffled. We thought at first that it was due to the fact that she was getting her liquids thickened due to airway penetration. We were wrong. As time went by a clear pattern was established. She would not drink on the same days she ate. She was not drinking or eating enough to sustain herself. The tube talk started coming up. Eventually a NG tube was placed for fluid intake. As we were forcing 40 oz of fluids in a day, it became very clear what the issue was. There was either room for food, or liquid, but not both.
She had an upper GI with small bowel follow through in July of 2010. They did not find a structural issue, but what they did find was that it took over four hours for her pylorus to open up and allow the barium through. It was then she received a clinical diagnosis of GP. Over time more of her supplementation was given through tube and we eventually switched to full formula nutrition and a G tube. Even then she would back most of her feeds into a Farrell bag unit and be very distended. She recently had a gastric emptying study done that showed severely delayed gastric emptying. That was done on a diluted elemental formula. It became clear we could no longer use her stomach. She has just started J feeds.
Chronic Intestinal Pseudo Obstruction
There were times things seemed to work ok, and times when things wouldn’t work at all. At times Peyton would put more into her Farrell bag than she was even being fed. At this point she was already being continuously fed. During these “episodes,” which sometimes included vomiting, but mostly would be backing up, stomach x-rays would show “moderate to severe illeus in the small intestine and colon dilation.” The way this was originally was explained to us is that she was developing “fluid air pockets” throughout her intestines. This showed them that her bowels were stopping in areas due to wrong signals showing there is an obstruction when there is not. After this happened on numerous occasions she was labeled with Chronic Intestinal Psuedo Obstruction. These episodes were treated with complete gut rest and IV fluids. When the obstructions resolve, they will slowly work her formula back up to rate. We never know when these episodes will come. Sometimes they are sparked by illness, other times they come out of nowhere. We are currently waiting on manometry studies to hopefully shed some more light.
As mentioned before, Peyton began having difficulty with eating early in 2010. By May of 2010 after she had gone through months of feeding therapy, it became apparent that it was not oral aversion. No one was suspecting gastroparesis at this point. Our pediatrician sent her for a stomach x-ray. He explained that sometimes when kids do not want to eat, it is due to stool burden. I thought it was a far cry, but we agreed to the x-ray.
What the x-ray showed was that she had stool through all three pieces of her large intestine. There was not even an area that was not packed with stool. We thought for sure this was our answer. First we were to add Miralax which softened her stool. She started stooling frequently, but her x-ray would show no change. After a month we started adding in Dulcolax suppositories and pills. This would produce large amounts of stool, but her x-ray would show no change, the same story with pediatric fleet enemas. It seemed no matter how soft we got her stool, or however frequent her bowel movements were, we just could not clean out her colon.
At this point we had met with GI who had ultimately decided that she needed to be admitted for a cleanout. The placed a NG tube (her first) and ran a substance called Nulytely through it. It basically is an extremely strong version of Miralax. This process took three days and got her cleaned out. Most children would have the NG tube removed after the procedure, but because they could not get her to sufficiently eat and drink after the procedure, it was decided at that time to leave it in place. Since Peyton’s first clean out her colon has continued to give her lots of trouble. It shuts down when she gets pseudo obstructions causing it to dilate with air, and she often gets stool burden that require clean outs. She is currently on a large bowel regimen including; Miralax, Dulcolax, Senokot, and Lactolose daily. These often keep things up and running, but if her colon wants to stop, it is going to stop regardless. We recently did a sitzmark study. Peyton had to swallow a pill with 24 radiological markers. This is normally done off bowel regimen, but they specifically wanted to see how Peyton’s was working for her. Five days after the procedure she had to have an X-ray. All of the markers should be gone after 2-3 days. For a child has functional constipation there will be markers down at the rectum. In someone like Peyton, who has a colon that does not move well, all 24 markers were strung throughout her colon. There are several procedures being considered including; cecostomy, or illeostomy, once again we will need to have manometry studies to decide which procedure would be best for her.
Dysmotility due to Structural Abnormalities (From Annabella's Mom, Jamie)
My daughter, Annabella, was born at term. She was a healthy 7lbs15oz. She took a bottle fine. She has had issues with constipation since early on, but otherwise she was a typical, slightly fussy, but typical infant. She never ate a lot and never really took more than 4-5oz of a bottle at a time.s She had some very minor motor skill delays. Once she started sitting up on her own she caught up with the rest quickly....standing, cruising, and she started walking soon after her first birthday.
Around her first birthday we also switched pediatricians due to a few issues I had that concerned me that he wouldn't take seriously. At that time I received copies of their medical records which included their growth charts. This pediatrician didn't share percentiles etc with parents because he felt people put to much weight on them. But I was shocked to see the nose dive my daughter had taken. I knew she was very small for her age, but he always assured me that I just had small children and that it was nothing to worry about. At our new pediatrician for her 1yr check she weighed 16lbs and the pediatrician was very concerned. He ran some bloodwork that all came back fine except for IgA portion of her Celiac Panel. She is considered clinically IgA deficient, although currently non-symptomatic. He sent us to see a pediatric GI at Children's Hospital of Philadelphia.
The GI ordered a battery of tests. The first test that we had scheduled was an Upper GI. Immediately the radiologist noticed that even though she hadn't eaten since the night before there was a nearly full tummy. Then he saw clearly that something was constricting her small intestine just below the stomach. She was scheduled for an MRI a few days later and it was confirmed that she had an Annular Pancreas. Which means her pancreas had grown around her intestine and was constricting it closed. She was scheduled for surgery a few weeks later where they created a bypass around the constricted area. She had a longer and more difficult recovery than expected and wound up in the hospital for 17 days (we were expecting 4-7 days) and unable to eat anything by mouth for that time period and was on TPN via Picc. The Picc wound up getting infected etc etc.
By the time we left the hospital and she was ALLOWED to eat she would take almost nothing by mouth. This was October, in December she was evaluated by the feeding team at CHOP and was accepted into their 4 week intesive feeding program, but she didn't weigh enough to qualify at the time. So she was admitted back into CHOP where she had an NG tube placed. Things were complicated by us all coming down with a stomach bug so it wasn't clear if her intolerance of feeds and puking was from an issue with the feeds or from the stomach virus she was getting over (again a 2 day admission turned into a week). She is supposed to be at 48mls/hr over 10hrs while she sleeps, but anything above 29mls causes her to wake vomitting. So even with the NG tube she is gaining just minimally and barely keeping up with herself. She now weighs enough to qualify for her feeding program, but because of her intolerance right now she is disqualified pending reevaluation. She just had a milk scan to see how she is emptying. The test showed only a mild to moderate emptying problem, but the GI felt that since the test only uses 2oz of formula it is not that accurate of a test for Anna and that at higher volumes it is a more severe issue. She also had several reflux episodes, but is not aspirating.
We will go back to see her surgeon to discuss and schedule her for a G tube. We are starting with the G tube with the possibility of having to switch to G/J tube in the future. The hope with Anna is that her motility will improve with time once her body retrains itself now that annular pancreas is corrected.
Continuous Venting the G port - with a G or GJ tube
Farrell bags allow for venting the stomach while feeding. Moreover, it gives food a place to go until the stomach is better able to handle it. The food enters the bag and then gravity fed back into the stomach.
Continuous Draining the G port (with a GJ or separate Gs and Js)
With gastroparesis and other motlity disorders like Chronic intestestinal pseudo obstruction is sometimes necessary drain the G. Farrell bags only work well if what is vented can be re-fed into the stomach on its own. However, that may be less likely to happen with motility disorders.
Many parents devise their own system to deal with continuous draining. Here are some options.
This system is made from what you already have. It is best to test out continuous draining.
Cut the tubing from a used feeding bag, wrap a diaper around the cut end. Place the diaper in a plastic bag and twist-tie it closed.
You can keep it lower the stomach, and on the floor while sleeping. The diaper can be weighed to measure output.
Very clever mommas have figured out a way to use a feeding set as an alternative to a farrell bag. Cut out the part that goes into the pump and use the pliable tubing to connect the bag to the rest of the tubing.
Another solution if you need to re-feed bile, may be urinary collection bags.
A number of parents use Hollister urinary collection bags. The tubing that comes with the collection system is bulky and some parents find it too short. Here the bag is shown connected it to the feeding set tubing with heavy duty sports tape. It likely could be connected using the tubing from the feeding set that fits into the pump (as shown in the Zevex bag example above). It has an easy to use valve. The bag can be used up to a week with daily (or more) rinsing.
Tip from a Momma:
If you are going to be draining overnight, put a plastic container near the crib or bed and put the bag in it, so that if it leaks it doesn't ruin the floor. Bile will remove the polyurethane off of wood floors and will stain carpeting.
To Re-Feed or Replace Bile?
This is for you and your docs to decide. Bile is important for hydration and weight gain. Sometimes doctors recommend replacing g output with pedialyte. Blood work may be done to make sure chemistries are OK.
If the volume of output is considerable, it may be necessary to re-feed. This sounds so much worse than it is. Bile should be collected and re-fed several times a day. Bile can be mixed with formula and run through the feeds to the J.
Check with your doctor on their recommended procedure. Some parents will re-feed in addition to the full daily formula in-take. Children who are prone to dehydration may benefit from re-feeding all that is drained out.
Tip from a Momma:
Use different extension sets for the G and J ports so you can tell them apart in the dark when you are administering medication.
Ex. MicKey for the J and AMT for the G port.