1000 Families Study, a UK multiwave cohort investigating the well-being of families of children with intellectual disabilities : cohort profile

Purpose: The 1000 Families Study is a large, UK-based, cohort of families of children with intellectual disability (ID). The main use of the cohort data will be to describe and explore correlates of the well-being of families of children with ID, including parents and siblings, using cross-sectional and (eventually) longitudinal analyses. The present cohort profile intends to describe the achieved cohort. Participants: Over 1000 families of UK children with ID aged between 4 and 15 years 11 months (total n=1184) have been recruited. The mean age of the cohort was 9.01 years old. The cohort includes more boys (61.8%) than girls (27.0%; missing 11.1%). Parents reported that 45.5% (n=539) of the children have autism. Most respondents were a female primary caregiver (84.9%), and 78.0% were the biological mother of the cohort child with ID. The largest ethnic group for primary caregivers was White British (78.5%), over half were married and living with their partner (53.3%) and 39.3% were educated to degree level. Findings: to date Data were collected on family, parental and child well-being, as well as demographic information. Wave 1 data collection took place between November 2015 and January 2017, primarily through online questionnaires. Telephone interviews were also completed by 644 primary caregivers. Future plans: Wave 2 data collection is ongoing and the research team will continue following up these families in subsequent waves, subject to funding availability. Results will be used to inform policy and practice on family and child well-being in families of children with ID. As this cohort profile aims to describe the cohort, future publications will explore relevant research questions and report key findings related to family well-being

Perfusion Methods and Modifications to the Cardiopulmonary Bypass Circuit for Midline Unifocalization Procedures

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare form of congenital heart disease. The midline unifocalization procedure has been developed for the treatment of PA/VSD/MAPCAs. These are complex and very lengthy procedures that require an entirely different method of perfusion. The purpose of this study was to review our perfusion modifications to support these unifocalization procedures. Sixty-four unifocalization procedures have been performed at our institution during the past 3 years. The median age was 4.1 months (range 1 month–3.5 years) and the median weight at surgery was 4.5 kg (range 3.5–19 kg). The median duration of cardiopulmonary bypass was 352 minutes (range 128–629 minutes), and the median duration of cross-clamp was 24 minutes (range 14–72 minutes). The conduct of surgery included cooling to a rectal perfusion temperature of 25° and a flow rate of 100 mL/kg/min. A pH-stat strategy and del Nido cardioplegia were used. Modifications to the cardiopulmonary bypass circuit include upsizing the oxygenator, reservoir, cannulae, vent catheter, and tubing. All circuits were modified to include the capability of performing an intraoperative flow study. This study is used to determine whether the VSD can be closed during surgery. A collateral flow study circuit is also set up for first-time operations to measure the residual collateral flow after all of the MAPCAs have been harvested. Patients who require midline unifocalization will invariably require very lengthy periods of support on cardiopulmonary bypass. We have adapted our perfusion circuitry to prepare for the demands on the bypass circuit to meet the requirements of this patient population. Our institution has developed a systematic approach for the conduct of perfusion to best serve our patients presenting with PA/VSD/MAPCAs