Sleep disorders in Charcot-Marie-Tooth disease type 1.

INTRODUCTION: Obstructive sleep apnoea (OSA) and restless legs syndrome (RLS) have been reported in Charcot-Marie-Tooth disease (CMT) type 1A and axonal subtypes of CMT, respectively. The aim of this case-control study was to investigate both prevalence and severity of OSA, RLS and periodic limb movements in sleep (PLMS) in adult patients with genetically proven CMT1. PATIENTS AND METHODS: 61 patients with CMT1 and 61 insomnic control subjects were matched for age, sex, and Body Mass Index. Neurological disability in patients with CMT was assessed using the Functional Disability Scale (FDS). RLS diagnosis was based on a screening questionnaire and structured clinical interviews. All participants underwent overnight polysomnography. RESULTS: OSA was present in 37.7% of patients with CMT1 and 4.9% of controls (p<0.0001). The mean Apnoea Hypoponea Index (AHI) was significantly higher in patients with CMT1 than in control individuals (9.1/h vs 1.2/h). RLS was present in 40.9% of patients with CMT1 and in 16.4% of controls (p<0.001). In the CMT1 group, OSA was significantly more common in men and RLS in women. The AHI correlated with both age and the FDS score, the latter being a significant independent predictor of OSA. PLMS were found in 41.0% of patients with CMT1, but were not correlated with measures of sleep quality. CONCLUSIONS: In addition to known risk factors, CMT may predispose to OSA. RLS is highly prevalent not only in axonal subtypes of CMT but also in primarily demyelinating subforms of CMT. PLMS are common in CMT1, but do not significantly impair sleep quality.

A multidisciplinary approach to reduce central line-associated bloodstream infections.

BACKGROUND: Stony Brook University Hospital (SBUH) joined a Critical Care Learning Collaborative in fall 2004. The collaborative incorporated application of central line and ventilator bundles, multidisciplinary rounding, and daily goal sheets to improve patient outcomes. In a two-year period, the initiative spread to the medical, pediatric, cardiac, and neonatal ICUs. METHODS: Despite some success, the goal of eliminating central line-associated bloodstream infections (CLABSIs) was not initially realized. In response, SBUH developed a standardized central line insertion credentialing program for residents. After further review of the residual central line infection data, it was evident that many of the lines became infected after day 7 of insertion. Evaluation of the line maintenance process revealed that nursing staff were not accessing the lines using the same level of sterile technique as used during insertion. As a result, a central line maintenance protocol was developed and deployed. RESULTS: After cumulative efforts were undertaken, SBUH's overall CLABSI rate decreased by 59% in a five-year period and by more than 80% in the most recent 12 months. CONCLUSIONS: A critical feature of the approach that SBUH followed was to establish buy-in and oversight from the SICU leadership through a multidisciplinary team, which became the "learning laboratory" for many of the subsequent changes in practice. Also, the fundamental role of the Continuous Quality Improvement (CQI) Department's quality management practitioner as facilitator cannot be overstated. "Hardwiring" of process changes augmented sustainability of improvements, as did a change in the health care team's perception of central line infections--that is, from an "unavoidable complication" to "a failure."