The School-Age Period: Enuresis

Enuresis (bedwetting) is a common problem in FXS. Although medication, particularly imipramine, can be helpful, as described below, other interventions should be tried first (Schmitt 1995; Moffatt 1997; von Gontard 1998). Decreasing fluids after dinner, urination at bedtime, waking the child to urinate again when the parents go to bed, building bladder musculature and size by intermittently stopping urine flow, and reinforcing the urination of larger and larger volumes can be helpful. However, further intervention is often necessary, and a trial of an alarm may be successful. Alarms are available and they include (1) the Potty Pager, a vibrating alarm from Ideas for Living in Boulder, Colorado, 1-800-497-6573; (2) Nytone alarm, a clip-on wet alarm from Nytone Medical Products, Salt Lake City, Utah, 1-801-973-4090; and (3) Wet-stop alarm with Velcro fasteners from Palco Labs in Santa Cruz, California, 1-800-346-4488. Most of these devices are $50 to $60. An alternative medication is desmopressin (DDAVP), a synthetic vasopressin analog, which is used nasally at bedtime. Although this has not been studied in FXS specifically, the response rate in enuretic children is 30-40% (Thompson and Rey 1995).

The School-Age Period: Ureteral Reflux

In our clinical experience we have seen 4 cases of significant and persistent ureteral reflux in approximately 350 children with FXS. In 3 cases this has led to nephrectomy because of renal complications, including hypertension. Perhaps the connective tissue dysplasia in FXS facilitates the ureteral dilation secondary to reflux. Severe reflux subsequently causes renal damage. Children with recurrent urinary infections and reflux should therefore be followed closely radiographically and treated aggressively to avoid kidney damage. Further studies regarding connective tissue abnormalities in the urinary system are needed in FXS.

Families who have ongoing difficulties with behavior in their child should be referred to a child psychologist with expertise in treating children with developmental disabilities. This therapist can provide ongoing support for the family and child with behavior modification programs and counseling (chap. 9).

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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