Jane E Schneiderman

SickKids · Translational Medicine

Research has shown that there is a lack of confidence and understanding in how to use exercise for managing cystic fibrosis. This editorial discusses the key points of a consensus statement that highlights what is and is not known about the relationship between cystic fibrosis and exercise.

A better understanding of the pathophysiology of pediatric post-thrombotic syndrome (PTS) is needed to develop strategies to treat this condition. We investigated calf pump function, exercise capacity, balance in power output, and changes in limb muscle oxygen saturation (SmO2) and fluid content during exercise in ten pediatric patients with unilat...

Context: There are no clear pediatric guidelines on the return to physical activity following deep vein thrombosis (DVT) or pulmonary embolism (PE), particularly while being treated with anticoagulation. Objective: This scoping review aimed to examine the current literature on physical activity beyond simple ambulation for patients with DVT/PE b...

Importance Wearing a face mask in school can reduce SARS-CoV-2 transmission but it may also lead to increased hand-to-face contact, which in turn could increase infection risk through self-inoculation. Objective To evaluate the effect of wearing a face mask on hand-to-face contact by children while at school. Design, Setting, and Participants Thi...

Background The roles of physical activity (PA) and exercise within the management of cystic fibrosis (CF) are recognised by their inclusion in numerous standards of care and treatment guidelines. However, information is brief, and both PA and exercise as multi-faceted behaviours require extensive stakeholder input when developing and promoting such...

Persons with mild hemophilia A (HA) may use intranasal desmopressin prior to sports participation. Desmopressin is expensive, and can cause vomiting, headache, palpitation and occasionally seizures. Our group has previously documented a 2.3-fold increase in FVIII activity (FVIII:C) in adolescents with mild HA after moderate-intensity aerobic exerci...

Magnetic Resonance Imaging (MRI) is well‐suited for imaging peripheral blood flow due to its non‐invasive nature and excellent spatial resolution. Although MRI is routinely used in adults to assess physiological changes in chronic diseases, there are currently no MRI‐based data quantifying arterial flow in pediatric or adolescent populations during...

Background Youth with multiple sclerosis (MS) have high levels of disease activity, depression and fatigue, and lower moderate to vigorous physical activity (PA). PA participation is associated with lower disease activity, depression and fatigue as well as higher self-efficacy, goal setting and decreased barriers. These later constructs may be targ...

Exercise intolerance is common in people with CF (pwCF), but not universal among all individuals. While associated with disease prognosis, exercise intolerance is not simply a reflection of the degree of lung disease. In people with severe CF, respiratory limitations may contribute more significantly to impaired exercise capacity than in those with...

Background: Health care workers have a critical role in the pandemic response to COVID-19 and may be at increased risk of infection. The objective of this study was to assess the seroprevalence of SARS-CoV-2 immunoglobulin G (IgG) antibodies among health care workers during and after the first wave of the pandemic. Methods: We conducted a prospe...

Background Cystic fibrosis (CF) is a genetically inherited, life-limiting condition, affecting ~90,000 people globally. Physical activity (PA) and exercise form an integral component of CF management, and have been highlighted by the CF community as an area of interest for future research. Previous reviews have solely focused on PA or structured ex...

Background: Previous studies have shown evidence of hypoxemia and hypercapnia during cardiopulmonary exercise test (CPET) evaluation in children with congenital central hypoventilation syndrome (CCHS). However, there are no longitudinal studies which compared CPET findings to polysomnogram (PSG) or PHOX2B mutation, to date. Objectives: To descri...

Objective To evaluate the feasibility of studying creatine in juvenile dermatomyositis (JDM). Secondary objectives were to determine the effect of creatine on muscle function and metabolism, aerobic capacity, fatigue, physical activity and quality of life, as well as its safety. Methods We conducted a 6-month double-blind, randomized, multiple-bas...

Introduction: Exercise-induced laryngeal obstruction (EILO) affects 2-3% of the general population and 5.1% of elite athletes. Symptoms arise during high-intensity exercise and resolve at rest. EILO is often misdiagnosed as exercise-induced asthma as both conditions can present with dyspnea, chest tightness and cough. The purpose of this quality i...

Objective: This study aims to non-invasively quantify blood flow in the uterine arteries (UTA) and umbilical vein (UV) using phase-contrast magnetic resonance imaging (PC-MRI), and test whether these correlate with maternal fitness parameters. Method: Resting UTA and UV flows were measured in 23 healthy 30±3 year-old women who engaged in moderat...

Background Physical activity (PA) is associated with a diverse range of health benefits. International guidelines suggest that children should be participating in a minimum of 60 min of moderate to vigorous intensity PA per day to achieve these benefits. However, current guidelines are intended for healthy children, and thus may not be applicable t...

Rationale: The prognostic value of cardiopulmonary exercise testing (CPET) for survival in cystic fibrosis (CF) in the context of current clinical management, when controlling for other known prognostic factors, is unclear. Objectives: To determine the prognostic value of CPET-derived measures beyond peak oxygen uptake ( V . o2peak) following...

Rationale: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with CF may present an important determinant of aerobic exercise capacity in CF. Previous studies on the relationship between CFTR genotype and aerobic exercise capacity are scarce and con...

Background: Malignant hyperthermia (MH), a pharmacogenetic disorder of skeletal muscle, presents with a potentially lethal hypermetabolic reaction to certain anesthetics. However, some MH-susceptible patients experience muscle weakness, fatigue, and exercise intolerance in the absence of anesthetic triggers. The objective of this exploratory study...

Objectives: To investigate physical activity levels in youth with multiple sclerosis and monophasic acquired demyelinating syndromes ([mono-ADS], ie, children without relapsing disease) compared with healthy controls and to determine factors that contribute to engagement in physical activity. We hypothesized that greater physical activity goal set...

Introduction: Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells and CFTR dysfunction may present an important determinant of aerobic exercise capacity in CF. Previous studies on the relationship between CFTR genotype and aerobic exercise capacity are scarce and contradictory. Aims and object...

Unlabelled: This study investigated the impact of aerobic exercise on laboratory assessments of haemostatic activity in boys (5-18 years of age) with haemophilia A (HA) or B (HB), examining the hypothesis that laboratory coagulation parameters temporarily improve with exercise. Thirty subjects meeting eligibility criteria (19 HA; 11 HB; mean age:...

The health benefits of PA are well established for healthy and chronically ill children. This study objectively measures physical fitness and PA levels in children PLT and explores potential correlates and perceived barriers impacting their PA. A total of 23 children (16 females, mean 14.01 ± 2.49 yrs) >1 yr PLT were assessed for peak oxygen consum...

This study aimed to determine the validity of the Habitual Activity Estimation Scale (HAES) for assessing physical activity (PA) in children and adolescents with severe obesity. Data were obtained from participants (n=17) in the High Impact Strategies Toward Overweight Reduction in Youth study at the Hospital for Sick Children in Toronto. Objective...

To assess the criterion validity of existing accelerometer-based energy expenditure (EE) prediction equations among children with chronic conditions, and to develop new prediction equations. Children with congenital heart disease (CHD), cystic fibrosis (CF), dermatomyositis (JDM), juvenile arthritis (JA), inherited muscle disease (IMD), and haemoph...

To study the effects of L-arginine (L-Arg) on total body aerobic capacity and muscle metabolism as assessed by 31Phosphorus Magnetic Resonance Spectroscopy (31P-MRS) in patients with MELAS (Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like episodes) syndrome. We performed a case control study in 3 MELAS siblings (m.3243A>G tRNAle...

Introduction. We undertook a study to assess the impact of exercise on hemostatic parameters [FVIII, FIX, VWF, platelet count and function, thrombin generation time (TGT) and thromboelastography (TEG)] in boys with HA and HB. We did so to explore the hypothesis that subjects with non-severe HA and all subjects with HB would show an increase in plat...

Although peak aerobic capacity (VO2peak ) has been linked to outcome in patients with cystic fibrosis (CF), measuring is time consuming, and requires expensive equipment and expertise that is not readily available in all centers. Other fitness parameters such as peak anaerobic power, measures of power and strength may be simpler to deliver in the c...

Excess weight is often associated with insulin resistance (IR), and may disrupt fat oxidation during exercise. This effect is further modified by puberty. While studies have shown that maximal fat oxidation rates (FOR) during exercise decrease with puberty in normal weight (NW) and overweight (OW) boys, the effect of puberty in NW and OW girls is u...

Exercise is beneficial for patients with Cystic Fibrosis (CF) but long term effects of physical activity on lung function evolution are unknown. We evaluated the longitudinal relationship between changes in habitual physical activity (HPA) and rate of decline in lung function in patients with CF.We tracked HPA with the Habitual Activity Estimation...

Although physical activity may reduce lung function decline in youth with cystic fibrosis (CF), most patients are inactive. Little is known about why youth with CF are inactive or how to facilitate physical activity. This study explored perceptions toward physical activity in 14 youth with CF at a Canadian Hospital. Qualitative interviews were cond...

Survivors of childhood acute lymphoblastic leukemia (ALL) are at risk for overweight, predisposing them to long-term morbidity. We examined the relationship between body mass index (BMI) and demographic and lifestyle factors in a cohort of ALL survivors. We enrolled consecutive ALL survivors who attended the institutional AfterCare Clinic over a 1-...

Despite the benefits of physical activity for youth with cystic fibrosis (CF) and congenital heart disease (CHD), most are insufficiently active. More information is required on how to facilitate physical activity in these populations. Although there are no studies that provide information about participation in CF and CHD youth from the perspectiv...

Background/Objectives: The purpose of this study was to develop an activity energy expenditure (AEE) prediction equation for the Actiheart activity monitor for use in children with chronic disease. Subjects/Methods: In total, 63 children, aged 8–18 years with different types of chronic disease (juvenile arthritis, hemophilia, dermatomyositis, neuro...

Previous studies have reported differences in muscle function and metabolism between patients with cystic fibrosis (CF) and healthy controls (HC), but it is currently unknown whether these abnormalities are specific to CF or also seen in other airway diseases. In this study, we used magnetic resonance spectroscopy (MRS) during exercise to assess mu...

The purpose of this study was to develop an activity energy expenditure (AEE) prediction equation for the Actiheart activity monitor for use in children with chronic disease. In total, 63 children, aged 8-18 years with different types of chronic disease (juvenile arthritis, hemophilia, dermatomyositis, neuromuscular disease, cystic fibrosis or cong...

Children with arthritis face challenges when they try to increase their physical activity. The study's objective was to identify elements of a successful community-based exercise program for children with arthritis by investigating the perspectives of fitness instructors who led the program. This qualitative study used a phenomenological approach....

Regular exercise and habitual physical activity are important for patients with cystic fibrosis (CF). Research has demonstrated the benefits of aerobic, anaerobic, and strength exercise training programs for health and quality of life, however, the CF patient is faced with unique barriers and challenges to participation. Recently, increased levels...

To determine the feasibility of conducting a randomized controlled trial of a 12-week exercise intervention in children with fibromyalgia (FM) and to explore the effectiveness of aerobic exercise on physical fitness, function, pain, FM symptoms, and quality of life (QOL). FM patients ages 8-18 years were randomized to a 12-week exercise interventio...

Cystic fibrosis (CF) leads to pathological changes in organs that express the cystic fibrosis transmembrane conductance regulator (CFTR), including secretory cells of the digestive tract and the pancreas. Maintaining nutritional sufficiency is challenging for CF patients and therefore accurate monitoring is important for their clinical management....

To understand potential benefits of exercise in the cystic fibrosis (CF) population, there needs to be accurate methods to quantify it. The Habitual Activity Estimation Scale (HAES) questionnaire has been shown to be a feasible tool to measure physical activity however the reliability and validity have yet to be determined in the CF population. Fou...

To determine the reliability of formal exercise testing and the reliability of functional and activity questionnaires in children with juvenile idiopathic arthritis (JIA). Children with JIA of any subtype ages 8-16 years who were recruited to a randomized trial comparing different exercise therapies participated in 2 preintervention sessions of exe...

To examine the effectiveness of high-intensity aerobic training compared with low-intensity training in terms of energy cost of locomotion, peak oxygen uptake, peak power, and self-reported physical function in children with juvenile idiopathic arthritis (JIA). Eighty children with JIA, ages 8-16 years, were enrolled in a randomized, single-blind c...

Our objective was to study exercise capacity and cardiorespiratory response to exertion in survivors of congenital diaphragmatic hernia (CDH). This was a cross-sectional cohort study of 23 CDH survivors, aged 10-16 years, and 23 gender- and age-matched controls. Exercise testing was performed on a cycle ergometer, with cardiac output measurements m...

The purpose of this research was to develop a comprehensive normative database of the physiological characteristics of elite swimmers. Data were obtained from 195 elite swimmers (89 males and 106 females) ages 12 to 18 years. Six protocols were used to measure variables in the following categories: descriptive characteristics, cardiovascular, respi...

The purpose of this study was to determine the fetal response to submaximal maternal exercise at 22 to 26 weeks in pregnancies with abnormal uterine artery Doppler. This was a prospective comparison of singleton pregnancies with uteroplacental vascular insufficiency (UPVI) (mean uterine pulsatility index [PI] values >1.45 [n = 12]) and those with n...

To evaluate the feasibility of measuring habitual physical activity (HPA) in children with cystic fibrosis (CF) and to assess the relation between HPA and the rate of decline in FEV1 over a period of 2 years. At regular clinic visits, 109 patients (7 to 17 years; 56 girls) performed pulmonary function testing and completed the Habitual Activity Est...

Carbon dioxide (CO2) retention during exercise is uncommon in mild to moderate lung disease in cystic fibrosis (CF). The ability to deal with increased CO2 is dependent on the degree of airflow limitation and inherent CO2 sensitivity. CO2 retention (CO2R) can be defined as a rise in P(ET)CO2 tension of > or =5 mm Hg with exercise together with a fa...

Maximal voluntary ventilation (MVV) may be determined directly by the sprint method or calculated from pulmonary function data, using the functions MVV = forced expired volume in 1 sec (FEV1) × 35 or MVV = FEV1 × 40. The purpose of this paper was to test the validity of the equation over a wide range of lung function in children. Cystic fibrosis (C...

To evaluate the effects of a 3-year home exercise program on pulmonary function and exercise tolerance in mildly to moderately impaired patients with cystic fibrosis (CF) and to assess whether regular aerobic exercise is a realistic treatment option. Seventy-two patients with CF (7-19 years) were randomly assigned to an exercise group (a minimum of...