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  Young smiling boy Medication can be important in the treatment of fragile X related behavior problems
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Infancy: Vomiting and Gastroesophageal Reflux

Although many babies with FXS do well in the newborn period, others have been described as stiff, unable to cuddle, irritable, and unable to feed well. Recurrent vomiting with feeding is not uncommon. Gastroesophageal reflux (GER) has been diagnosed in several infants with FXS, and it may be related to the connective tissue abnormalities, hypotonia, dysfunction of the gastroesophageal sphincter, or a hypersensitive gag reflex (Goldson and Hagerman 1993). The vomiting in patients with GER usually resolves with positioning upright after meals or with thickening of the feedings (Sondheimer 1994). Occasionally medication is needed to decrease reflux and improve gastric emptying time. The medications commonly used in GER include metoclopromide (Reglan), which is related to neuroleptic medication, and bethanacol, a cholinergic drug that stimulates intestinal smooth muscle contraction. Both medications will enhance the lower esophageal sphincter pressure, and metoclopromide will improve gastric emptying time. Both, however, can cause side effects in the central nervous system (CNS), including irritability, and metoclopromide can cause dystonic reactions. Cisapride (Propulsid), a prokinetic agent, has been used to treat reflux, but a recent association with arrhythmias has discouraged its use in young children.

Recurrent GER can cause significant esophagitis and pain. These problems can be treated with an H2 blocker, such as ranitidine (Zantac), or a proton-pump inhibitor, omeprazole (Prilosec), if ranitidine is not effective (Karjoo and Kane 1995). Rarely surgery is needed to tighten the sphincter area with a Nissen or Thal fundoplication procedure and only after medical intervention has failed.

Some children with FXS will have habitual vomiting when they are upset, overwhelmed, or frustrated. Behavioral intervention to decrease vomiting and replace it with a more acceptable behavior can be helpful. We have noted several cases of failure to thrive related to difficulties in sucking, GER, tactile defensiveness, or aversion to food textures (Goldson and Hagerman 1993). If feeding difficulties are a problem, particularly sucking or intolerance of certain food textures, consultation with an occupational therapist and/or a speech and language therapist and subsequent work or oral desensitization, oral stimulation, and oral motor coordination can be helpful (chap. 10).

This article is not intended to give medical advice for individual cases.  Any change in medical treatment should be done in consultation with appropriate medical personnel. This article is written for medical professionals.  Some of the terms will be unfamiliar to those who are not trained in medical fields.

*This article is from the chapter on treatment in the 3rd edition of Fragile X Syndrome: Diagnosis, Treatment, and Research edited by Randi Jenssen Hagerman, M.D. and Paul Hagerman, M.D., Ph.D., to be published May 2002.  It is included with permission from The Johns Hopkins University Press. References to other chapters refer to chapters in the book which are not included as part of this website.

The complete 3rd edition of Fragile X Syndrome: Diagnosis, Treatment, and Research can be ordered from the National Fragile X Foundation by calling 1-800-688-8765 or from The Johns Hopkins University Press at 1-800-537-5487.

 

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References: A, B, C, D, EF, G, H, IJ, K, L, M, NOP, QR, S, T, UVWXYZ
 

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