A national assessment of standards of care for inherited bleeding disorders in Canada.

AIM: The Canadian Integrated and Comprehensive Care Standards for Inherited Bleeding Disorders were adopted in June 2020. In early 2023, a self-assessment of each of the 26 Canadian inherited bleeding disorder treatment centre's (IBDTCs) capacity to meet the Standards was conducted. The goal was to validate the standards by assessing appropriateness and adherence. As a result, centres can compare their own practices and capacity against those of all centres, identify barriers to adherence, identify opportunities for remedial actions and use the results locally as evidence for needed resources. METHODS: Healthcare providers (HCPs) in the 26 IBDTCs were provided with a checklist to assess adherence to each of the 66 standards of care. Centre participation was voluntary but strongly encouraged by the healthcare provider and patient associations. RESULTS: All 26 centres completed the self-assessments. Collectively, centres reported meeting 88.8% of the standards. Adherence to each standard ranged from 40% to 100%. Forty-one (41) of the standards were adhered to by 90% or more of the centres, 12 by 80%-89% of the centres and 13 by fewer than 80% of the centres. A report consolidating all the assessments was sent to the 26 centres. CONCLUSION: None of the comments received in the self-assessment reports indicated that a given standard was irrelevant, unrealistic or unnecessary. These data are strong indicators that the standards, as written, are appropriate. The self-assessments, however, reveal alarming deficiencies in staffing levels, notably in physiotherapy, psychosocial support and data entry and data management. These constitute a barrier to comprehensive care for many centres. The findings echo similar conclusions from a previous assessment conducted in 2015.

Developing clinical practice guidelines for physiotherapists working with people with inherited bleeding disorders.

INTRODUCTION: Several bleeding disorders are characterized by haemorrhage into joints and muscles. These conditions are best managed by interdisciplinary teams that include physiotherapists. In 1997, physiotherapists from haemophilia treatment centres in Canada formed the Canadian Physiotherapists in Hemophilia Care (CPHC). The guiding principles of the CPHC reflect a commitment to evidence-based practice, education and collaboration. AIM: To describe the process used by CPHC to develop evidence-based clinical practice guidelines to inform best practice, guide decision-making and help educate physiotherapists, students, and other team members about the physiotherapy management of people with bleeding disorders. METHODS: We followed the procedures outlined in the American Physical Therapy Association's Clinical Practice Guideline Process Manual (2018). Namely, we selected a working group, determined the scope of the guidelines, performed a literature search, selected and appraised the evidence, drafted the guidelines as practice statements, assigned a strength of recommendation to each practice statement and disseminated the guidelines. RESULTS: Thirty-nine practice statements were developed in nine practice areas. Strength of evidence was strong for two statements, moderate for one and weak for three. The remainder were graded as theoretical or best practice. CONCLUSION: To our knowledge, these are the first evidence-based clinical practice guidelines that cover all aspects of physiotherapy management of people with bleeding disorders. Some areas, such as exercise and manual therapy, have been well investigated. However, the overall low levels of evidence and low strengths of recommendations highlight the need for more rigorous research with this population.

Time to revisit tummy time: A commentary on plagiocephaly and development.

Many centres report receiving more referrals for deformational plagiocephaly since implementation of the Back to Sleep campaign. This commentary combines clinical experience, local quality improvement data and existing literature to highlight three points to help prevent and manage plagiocephaly: (1) communicating 'Back to Sleep, Tummy to Play', (2) the importance of early detection and (3) plagiocephaly as a marker of developmental risk. We recommend: (1) equal emphasis on the messages of Back to Sleep and supervised Tummy to Play, to start this messaging early and reinforce at every opportunity; (2) examination of skull shape and neck range of motion as a routine component of the newborn assessment so that caregivers can implement positioning and handling suggestions immediately and (3) physiotherapy referral for babies who have torticollis, or who show persistent or worsening plagiocephaly despite positioning and handling interventions, for further assessment and management.

Exercise for haemophilia.

BACKGROUND: Haemophilia is a bleeding disorder associated with haemorrhaging into joints and muscles. Exercise is often used to aid recovery after bleeds, and to improve joint function in the presence of arthropathy. OBJECTIVES: Our objective was to systematically review the available evidence on the safety and effectiveness of exercise for people with haemophilia. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register and electronic databases PubMed, OVID-Embase, and CINAHL. We hand searched abstracts from congresses of the World Federation of Hemophilia and the European Hematology Association, trial registries and the reference lists of relevant articles.Date of the last search of the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register: 14 December 2016. SELECTION CRITERIA: Randomized or quasi-randomized controlled studies comparing any exercise intervention considered relevant in haemophilia management including supervised, unsupervised, aquatic, strengthening, aerobic or cardiovascular, stretching, proprioceptive and balance training exercise programs in males of any age with haemophilia A or B of any severity (those with co-morbidities were not excluded). DATA COLLECTION AND ANALYSIS: Two authors reviewed the identified abstracts to determine their eligibility. For studies meeting the inclusion criteria, full articles were obtained. The two authors extracted data and assessed the risk of bias. Any disagreements were resolved by discussion. The authors contacted study investigators to obtain any missing data. MAIN RESULTS: Eight studies were included, which represented 233 males with all severities of haemophilia A and B, ranging in age from eight years to 49 years. Study duration ranged from four to 12 weeks. Exercise interventions varied greatly and included resistance exercises, isometric exercises, bicycle ergometry, treadmill walking and hydrotherapy; therefore, comparison between studies was difficult.None of the studies measured or reported adverse effects from the interventions. None of the studies reported outcomes regarding bleed frequency, quality of life or aerobic activity. Overall risk of bias across all studies was assessed as unclear.Very few studies provided sufficient information for comparison. None of the studies reported data that favoured the control group. One study reported that six weeks of resistance training improved joint health status (Colorado score) compared to controls. The addition of pulsed electromagnetic fields also improved ankle scores compared to exercises alone, but this was not seen in the elbows or knees.Two studies reported statistically significant improvements in pain intensity after exercise interventions compared to controls. Hydrotherapy exercises produced significant decreases in pain compared to controls and land-based exercise groups.Two studies found improvement in joint motion in the exercise group compared to controls. One study compared land- and water-based exercises; there was no difference in the range of motion between the two groups; however, the water-based exercise group did show improvement over the control group.One study, comparing joint traction and proprioceptive neuromuscular facilitation for the elbow to a control group, showed no differences in biceps girth or strength after 12 weeks of intervention.Some studies reported comparisons between interventions. In one study, treadmill training significantly improved balance in children compared to bicycle ergometry. Another study added partial weight bearing exercises to quadriceps exercises and showed improved walking tolerance.Four studies evaluated quadriceps or hamstring strength (or both). The addition of bicycle ergometry and exercises with weights was more effective than static exercises and treadmill walking for strengthening knee flexors and extensors. Partial weight-bearing exercises through range were more effective than static and short arc exercises for improving knee extensor strength. The addition of treadmill walking to ultrasound, stretching and strengthening exercises showed increased peak torque of knee flexors and extensors and decrease in knee effusion.The results should be interpreted with caution due to the quality of evidence (GRADE) as outlined in the summary of findings tables, which demonstrates that all but one of the outcomes assessed were rated as low or very low due to the small sample sizes and potential bias. AUTHORS' CONCLUSIONS: These results must be considered with caution. There is a lack of confidence in the results due to the small number of included studies and the inability to pool the results due to the heterogeneity of outcome measures. Most exercise interventions produced improvement in one or more of the measured outcomes including pain, range of motion, strength and walking tolerance. Hydrotherapy may be more effective than land exercises for pain relief in adults. Functional exercises such as treadmill walking and partial weight bearing exercises seem to be more effective than static or short arc exercises for improving muscle strength. These findings are consistent with the many non-controlled intervention reports in the haemophilia literature. No adverse effects were reported as a result of any of the interventions. However, some groups used prophylactic factor prior to exercise and other groups studied only subjects with moderate haemophilia. Therefore, the safety of these techniques for persons with severe haemophilia remains unclear.

Central intake to improve access to physiotherapy for children with complex needs: a mixed methods case report.

BACKGROUND: Children with complex needs can face barriers to system access and navigation related to their need for multiple services and healthcare providers. Central intake for pediatric rehabilitation was developed and implemented in 2008 in Winnipeg Manitoba Canada as a means to enhance service coordination and access for children and their families. This study evaluates the process and impact of implementing a central intake system, using pediatric physiotherapy as a case example. METHODS: A mixed methods instrumental case study design was used. Interviews were completed with 9 individuals. Data was transcribed and analyzed for themes. Quantitative data (wait times, referral volume and caregiver satisfaction) was collected for children referred to physiotherapy with complex needs (n = 1399), and a comparison group of children referred for orthopedic concerns (n = 3901). Wait times were analyzed using the Kruskal-Wallis test, caregiver satisfaction was analyzed using Fisher exact test and change point modeling was applied to examine referral volume over the study period. RESULTS: Interview participants described central intake implementation as creating more streamlined processes. Factors that facilitated successful implementation included 1) agreement among stakeholders, 2) hiring of a central intake coordinator, 3) a financial commitment from the government and 4) leadership at the individual and organization level. Mean (sd) wait times improved for children with complex needs (12.3(13.1) to 8.0(6.9) days from referral to contact with family, p < 0.0001; 29.8(17.9) to 24.3(17.0) days from referral to appointment, p < 0.0001) while referral volumes remained consistent. A small but significant increase in wait times was observed for the comparison group (9.6(8.6) to 10.1(6.6) days from referral to contact with family, p < 0.001; 20.4(14.3) to 22.1(13.1) days from referral to appointment, p < 0.0001), accompanied by an increasing referral volume for this group. Caregiver satisfaction remained high throughout the process (p = 0.48). CONCLUSIONS: Central intake implementation achieved the intended outcomes of streamlining processes and improving transparency and access to pediatric physiotherapy (i.e., decreasing wait times) for families of children with complex needs. Future research is needed to build on this single discipline case study approach to examine changes in wait times, therapy coordination and stakeholder satisfaction within the context of continuing improvements for pediatric therapy services within the province.

The Relationship Between Body Mass Index and the Magnitude of Curve at Diagnosis of Adolescent Idiopathic Scoliosis: A Retrospective Chart Review.

Purpose: To evaluate the relationship between body mass index (BMI) and spinal curvature in patients presenting with idiopathic scoliosis at a major pediatric tertiary care centre. Patients and Methods: Retrospective chart review (2015-2019). Data extracted from patient's first visit included age, sex, height, weight, spinal curvature (magnitude, location), referral source, physical activity participation (yes/no), pain (yes/no). Demographics were analyzed descriptively. The relationship between BMI and spinal curve magnitude was analyzed using Spearman correlation coefficient. Linear regression was applied to determine the relationship between BMI, curve magnitude, and curve location. Exploratory univariate analyses were conducted for BMI and referral source, pain, and skeletal maturity, and physical activity and pain. Results: A total of 206 patient charts were included (177 females, 29 males). Patients presented with double major (41.3%), thoracic (26.7%), thoracolumbar (22.8%), and lumbar (9.2%) curves. Mean (SD) BMI percentile was 48.3 (30.5). No relationship existed between BMI percentile and curve magnitude with curve locations combined. However, a test for interaction revealed a positive relationship between BMI percentile and curve magnitude for adolescents with double major curves, and a negative relationship for adolescents with thoracic curves. Exploratory analyses suggested a relationship between BMI percentile and presence of pain, and between referral source and curve magnitude. No relationship was observed between BMI and skeletal maturity, or physical activity and pain. Conclusion: The relationship between BMI varied by curve location within this cohort, in which most patients presented with a BMI <85th percentile. Findings highlight the importance of sensitive history taking and careful physical examination for early detection of scoliosis.