Improvements in long term phototherapy for patients with Crigler - Najjar syndrome Type I

Abstract

Patients with Crigler - Najjar syndrome Type I are being treated with long-term blue-light phototherapy into childhood, adolescence and beyond. Phototherapy systems adapted from sunbed-type bases fitted with blue-emitting fluorescent tubes have been described. These systems provide higher irradiances and improved patient compliance compared with overhead therapy systems used in neonatal phototherapy. The acrylic bases of such units are, however, not designed to provide adequate levels of comfort for prolonged treatment in the long term. Previous work has shown that layer(s) of transparent `bubble-wrap' can be used to address this problem, although the material absorbs light and provides lower levels of comfort for older or larger patients. We have used designs of transparent plastic lilos that provide better cushioning, although tend to puncture, and share with bubble-wrap a low porosity leading to patient discomfort. We have investigated the use of standard mesh and high-transmission fabrics stretched over an adjustable-tension frame. This method in particular combines a high degree of comfort with a clinically effective blue-light irradiance level, and hence appears to provide a satisfactory method of phototherapy delivery. The development of higher transmission materials offers further potential for improvement.