Rare Dysmotility Syndrome (RDS)

Overview: Rare Dysmotility Syndrome is a rare gastric disorder (having to do with the gastrointestinal track).  This disorder causes the motility of the stomach and intestines to be extremely slow which often leading to not being able tolarate food.  It also causes slow gastric emptying -  taking a long time to be digested.  It's not certain if the cause is prematurity or genetic.  There is also belief that ascites may be related to this condition.  There are not many cases of RDS registered in the US.
Symptoms:

• Some of the known symptoms with RDS include:
• slow digestion
• weight loss
• failure to thrive
• swallowing problems and poor oral motor skills
• distended abdomen
• excessive gas or inability to pass gas
• reflux or GERD
• anorexia
• chronic constipation or diarrhea
• vomiting
• unable to tolerate feeding without distention.
• laboratory chemistries, unbalanced electrolytes

Testing/Diagnosis:  To diagnose RDS a doctor may order an upper or lower GI (an image study to observe the body and how it digests food.  Genetic testing as well as testing for Cystic Fibrosis is often order.  In some cases doctors may also request a biopsy of the intestines.  The best test to document delayed gastric emptying is a nuclear scintigraphy test (gastric emptying study). In this test a radiolabeled meal is ingested and the radioisotope is followed so that a time curve can be plotted to determine how long it takes for the stomach to empty. This is compared to a "normal" curve. Generally, a t1/2 (time it takes to empty half the meal) of greater than 90 minutes is considered abnormal.

Treatments:  Some medications may help with RDS, such as different types of anti bacterials to help kill the bacteria along the intestinal wall.  Feeding tubes (often J tubes in order to bypass the stomach completely) can help with the feed intolerance.  Often doctors will suggest/prescribe elemental formula's.  An elemental formula is one that is already broken down as it would be during normal digestion.  There are many different types of elemental formulas, some are more broken down than others. There are medications that can be used for treating these diseases, for example metoclopramide or cisapride, but these usually are of benefit in the milder cases. Because of the rarity of these diseases the management of patients usually is turned over to "sub - subspecialists," gastroenterologists with special expertise in disorders of motility.

Resources:http://www.nationwidechildrens.org/motility-center
http://www.pedsgi.org/
https://www.facebook.com/pages/PEDS-Pediatric-Digestion-and-Motility-Disorders-Society-Inchttp://www.inspire.com

Aizen's Story
 
We went through so much trying to figure out why our preemie, Aizen,  wouldn't eat.  We could not figure out why his tummy was so distented.  He was put through so many tests and lab work, all coming back negative.  The doctors finally diagnosed Dysmotility Syndrome. Even though they are not completely sure about the diagnosis, they are treating Aizen for this syndrome.  So far treatment has been successful.

Contributed by MOM Ashley Lembert