Sleep disturbance in children with mucopolysaccharidosis (MPS) types II and III; a review of medication usage

It is widely recognised that sleep difficulties are common in children with MPS II and MPS III. Observation of clinical practice noted children were prescribed a range of medications of varying doses. The purpose of this review was to look at current prescribing practice and therapeutic outcome in MPS II and III in a single tertiary unit. A retrospective review of medications used to aid sleep disturbance in those with a diagnosis of MPS II and MPS III was undertaken. Data collected included current medication, previously prescribed medication including doses and whether single therapy or dual therapy was used. The time interval before adding a new medication was documented and whether current therapy was efficacious. 44 patient records were reviewed, (34% MPS II/66% MPS III), of which 26 (59%) patients had sleep disturbances, 33% of MPS II (all of whom had neurological phenotype) and 72% of MPS III. 22 out of 26 patients were prescribed medication to help sleep. Melatonin was used as first line for all 22 patients, 11 patients required additional therapy. The most commonly prescribed second line agent was an antihistamine. Chloral hydrate was used occasionally, benzodiazepines were not used. Sleep problems were a prominent management issue in this group of patients’; more than one drug was needed in half of the group. There was variation around decisions to escalate treatment. Further information is needed on efficacy of medication in this setting including parents’ perspective in order to establish single standard of practice.