Recommendations for the management of gastrointestinal comorbidities with or without trofinetide use in Rett syndrome

Figures & data

Table 1. Summary of recommended treatments for gastrointestinal comorbidities in RTT. First-line treatment options are noted in boldface.

Management strategy/treatment	GI comorbidity
	Consti pation	Diarrhea	Vomiting	Aspiration	Dysphagia	Gastroesophageal reflux	Nausea	Gastroparesis	Gastritis	Abdominal bloating
Feeding strategies
Dietary fiber, fiber supplements	x	x	x
Upright, seated position for feeding			x	x	x	x
Thickening agents, pureed foods				x	x	x
Monitor hydration	x	x	x		x
Smaller feeds/meals			x	x	x	x
Strategic placement of food in the mouth to aid chewing/swallowing				x	x
Small bites of food followed by liquids				x	x
Modification of diet to reduce foods that cause gas										x
Pharmacotherapy
Laxatives	x									x
Suppositories	x
Enemas	x
Discontinue/reduce laxatives		x
Loperamide		x
Antiepileptic drugs*			x
Proton pump inhibitors			x			x			x	x
H2 receptor blockers			x			x			x
Prokinetic agents^†			x			x		x		x
Ondansetron^†			x				x
Antacids						x			x
Antibiotics										x
Triple or quadruple therapy for H. pylori infection									x
Digestive aids^‡										x
Anti-gas medications										x
Specialists
Gastroenterologist	x	x	x	x	x	x	x	x	x	x
Feeding therapist				x	x
Occupational therapist				x	x
Speech therapist				x	x
Surgery
Fundoplication						x
Gastrostomy tube placement				x	x
Gastrojejunostomy tube placement			x	x	x	x		x
LINX Reflux Management System placement						x
Other
Gastrostomy tube venting										x
CPR training for caregivers				x
Herbal remedies							x			x
Raising head of bed						x
Specific to trofinetide treatment
Reduce, divide, or titrate trofinetide dose		x	x
Switch any concomitant medications with sugar alcohols to pill form if possible		x
Reduce liquid volume before and after trofinetide administration			x
Experiment with timing trofinetide dosing closer to or farther from mealtimes			x				x

*To maintain control of seizures.

^†Should be avoided in individuals with long QT syndrome.

^‡Lactaid, Beano.

CPR = cardiopulmonary resuscitation; GI = gastrointestinal; RTT = Rett syndrome.