Pronto 7

Each participant was asked to sit on a chair, roll up one sleeve, and relax for five minutes, so that the measurements were made at rest. Subsequently, they underwent an electrocardiogram and spirometry to verify normal results. A second stage consisted of serial measurement of vital signs, including heart rate, systolic blood pressure, diastolic blood pressure, tympanic temperature, total hemoglobin, perfusion index, and baseline lactic acid levels.
Blood pressure and heart rate were measured with the use of oscillometric sphygmomanometry (Schiller BP 200 Plus; Schiller, Baar, Switzerland). For measuring tympanic temperature we used a Braun Thermoscan Pro 6000 (Welch Allyn, Inc., New York, USA) with ExacTemp™ technology. Total hemoglobin values and perfusion index were monitored with a multiparameter monitor Pronto 7 (Masimo, California, USA), software version b99e80000004ef796 (2.2.15), and sensor revised version a83f90f0000c53f2. For the determination of lactic acid levels we used an Accutrend Plus meter (Roche Diagnostics, Mannheim, Germany), with a measurement range of 0.8-21.7 mmol/L.

Subjects self‐assessed their sexual maturity using a validated form (Morris and Udry 1980) corresponding to Tanner's five pubertal stages. Certified pediatric Respiratory Therapists measured each subject's height (seca 217, seca GmbH & Co. KG., Hamburg, Germany), weight (Scale‐Tronix, White Plains, NY), and noninvasive hemoglobin (via CO‐oximetry; Pronto‐7, Masimo Corp., Irvine, CA), and performed the pulmonary function test consisting of spirometry, lung volumes, and diffusion capacity (MasterScreen™ PFT system, Jaeger, CareFusion Corp., San Diego, CA). Maximal inspiratory (PI_MAX) and expiratory (PE_MAX) pressure maneuvers (Mouth Pressure Meter, Micro Direct, Inc., Lewiston, ME) were then performed. Resting baseline data were collected while seated on a cycle ergometer (Excalibur Sport, Lode BV, Groningen, Netherlands), which was followed by the incremental cycling test. After the second visit, physical activity levels were self‐assessed with a modified version of a validated physical activity questionnaire (Kowalski et al. 2004). Training details were collected from each swimmer's coach.

Consultants discussed the study recruitment with the caregivers of the children living with SCA during sickle clinics, or contacted them via phone or letter. Families who consented to the study completed cognitive testing at the clinic visit or came to the university campus. All testing was completed in a quiet room and took approximately 2 hours; tests were conducted between 2010 and 2016. Additionally, pulse oximeter (Masimo Pronto-7) measurements (i.e., oxygen saturation) were obtained on the day of testing and took 5 min to complete. Steady-state hemoglobin levels taken at the closest clinical visit were obtained from the patient’s medical records. Caregivers of children under 16 completed the executive function questionnaire while their child was being tested.