Nocturnal enuresis in sickle cell disease

Abstract

Nocturnal enuresis is a prevalent and challenging problem in children and young adults with sickle cell disease (SCD). Limited progress has been made in elucidating etiology and pathophysiology of nocturnal enuresis in individuals with SCD. Among adults with SCD ages 18–20 years, approximately 9% report nocturnal enuresis. Nocturnal enuresis contributes to decreased health related quality of life in people with SCD, resulting in low self-esteem and sometimes social isolation. Postulated non-mutually exclusive causes of nocturnal enuresis in individuals with SCD include hyposthenuria leading to nocturnal polyuria, decreased bladder capacity or nocturnal bladder overactivity, increased arousal thresholds, and sleep disordered breathing. No evidence-based therapy for nocturnal enuresis in SCD exists. This review is focused on describing the natural history, postulated causes and a rational approach to the evaluation and management of nocturnal enuresis in children and adults with SCD.

Keywords::

• clinical evaluation
• etiology
• management
• nocturnal enuresis
• sickle cell disease
• sleep disordered breathing
• tonsillectomy and adenoidectomy

Acknowledegments

The authors would like to acknowledge M Rodeghier for his help in creating Figure 1 and the Vanderbilt Medical Scholars Program and NIH grant UL1 RR 024975.

Financial & competing interests disclosure

This publication was supported by CTSA award No. UL1TR000445 from the National Center for Advancing Translational Sciences. Its contents are solely the responsibility of the authors and do not necessarily represent official views of the National Center for Advancing Translational Sciences or the National Institutes of Health. The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Nocturnal enuresis is more common in individuals with sickle cell disease (SCD) than in the general population.

• Etiology for increased incidence of nocturnal enuresis in individuals with SCD is unknown.

• Nocturnal enuresis in SCD is most likely multifactorial.

• Postulated causes include hyposthenuria, nocturnal bladder overactivity, sleep disordered breathing and neurological dysfunction.

• Increased incidence of sleep disordered breathing in individuals with SCD may require therapy such as tonsillectomy and adenoidectomy to decrease the incidence of nocturnal enuresis.

• Clinical evaluation for nocturnal enuresis in SCD is the same as for the general population and includes a thorough assessment.

• First-line treatment for nocturnal enuresis in SCD is similar to that in the general population and includes behavior modification through alarm therapy.

Notes