Children with SMA can be troubled by gastroesophageal reflux disease (or GERD). GERD most often affects those with type 1, the most severe form of SMA, although it is also common in children with type 2 and less so in those with SMA type 3.
GERD causes acid reflux and may be associated with silent aspiration (food or drink that enters the respiratory tract without any warning signs, such as coughing or choking), which can lead to pneumonia, respiratory distress, or other life-threatening events.
Signals such as frequent “spitting up” or vomiting after meals, complaints of chest or abdominal discomfort, bad breath, or regurgitation of feeds may indicate the presence of GERD.
How is GERD diagnosed?
A routine upper gastrointestinal series, which is an X-ray examination of the upper gastrointestinal tract, is the first step to evaluating reflux and identifying the possible need for a gastrostomy tube placement. In this test, the esophagus, stomach and part of the small intestine are made visible on the X-ray using a liquid suspension such as barium, or a water-soluble contrast.
Motility studies, such as scintigraphy, may help document delayed gastric emptying, which may be the cause of GERD. The test creates a two-dimensional picture of the body through the use of safe radioisotopes. A camera is then placed that follows the radioisotopes to allow an examination of the digestive tract.
Management of GERD
Before considering medication, there are some measures that can be used to help manage GERD. These include:
- Eating small portions throughout the day instead of large meals.
- Reducing the fat content of the diet since children with SMA are more likely to have trouble processing fat. Children above age 2 should not be getting more than 30% of calories from fat, and less during an illness (no more than 15-20% of total energy).
- Avoiding irritating foods that may cause reflux, such as chocolate, peppermint, spicy foods, and acidic foods like soft drinks, citrus, tomatoes, etc.
- Inclining after meals.
Medication for GERD involves short-term use of acid neutralizers (magnesium or calcium carbonate) and/or inhibitors of acid secretion such as histamine blockers and proton pump inhibitors (famotidine, ranitidine, and omeprazole).
For serious cases of acid reflux, a Nissen fundoplication may be recommended. This is a surgical procedure that corrects GERD by creating an improved valve mechanism at the bottom of the esophagus, limiting the upward flow of acids. Depending on the individual, this procedure may be performed laparoscopically, or using a camera and several tiny cuts, or via an “open” technique, through a regular surgical incision. A gastrostomy tube may be temporarily placed in the stomach to allow for feeding after the surgery.
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