Introduction: Throughout life, a patient with severe haemophilia is confronted with many treatment-related challenges. Insight into self-management and non-adherence could improve the quality of care for these patients. The aim of this study was to provide an overview of the current evidence on self-management and adherence to prophylaxis in haemophilia. Method: Based on series of studies and published literature, aspects of treatment were explored: learning and performing self-infusion, achieving self-management skills in adolescence, adherence issues and coping with haemophilia. Evidence-based and age-group-specific recommendations for haemophilia professionals were formulated. Results: Nearly, all severe haemophilia patients and parents were able to perform self-infusion and the quality level of infusion skills was acceptable. Learning self-infusion was generally initiated before the onset of puberty and full self-management was obtained 10 years later. Adherence was defined using a Delphi consensus procedure and was determined by skipping, dosing and timing of infusions. Adherence levels varied according to age, with highest levels in children (1–12 years) and the lowest among 25–40 years. Adherence to prophylaxis was acceptable (43%), yet 57% of the population struggled with prophylaxis. Qualitative research showed that the position of prophylaxis in life is the main driver of adherence. This position is influenced by acceptance and self-management skills. Regarding coping with haemophilia, the majority of patients used a problem-focused approach. Conclusion: Self-management and adherence to prophylaxis vary during the life span. Acceptance of the disease and self-management skills were important aspects that may require tailored professional support.
Objective: To evaluate the preliminary effectiveness of a goal-directed movement intervention using a movement sensor on physical activity of hospitalized patients. Design: Prospective, pre-post study. Setting: A university medical center. Participants: Patients admitted to the pulmonology and nephrology/gastro-enterology wards. Intervention: The movement intervention consisted of (1) self-monitoring of patients' physical activity, (2) setting daily movement goals and (3) posters with exercises and walking routes. Physical activity was measured with a movement sensor (PAM AM400) which measures active minutes per day. Main measures: Primary outcome was the mean difference in active minutes per day pre- and post-implementation. Secondary outcomes were length of stay, discharge destination, immobility-related complications, physical functioning, perceived difficulty to move, 30-day readmission, 30-day mortality and the adoption of the intervention. Results: A total of 61 patients was included pre-implementation, and a total of 56 patients was included post-implementation. Pre-implementation, patients were active 38 ± 21 minutes (mean ± SD) per day, and post-implementation 50 ± 31 minutes per day (Δ12, P = 0.031). Perceived difficulty to move decreased from 3.4 to 1.7 (0-10) (Δ1.7, P = 0.008). No significant differences were found in other secondary outcomes. Conclusions: The goal-directed movement intervention seems to increase physical activity levels during hospitalization. Therefore, this intervention might be useful for other hospitals to stimulate inpatient physical activity.
Non-intubated patients with acute respiratory failure due to COVID-19 could benefit from awake proning. Awake proning is an attractive intervention in settings with limited resources, as it comes with no additional costs. However, awake proning remains poorly used probably because of unfamiliarity and uncertainties regarding potential benefits and practical application. To summarize evidence for benefit and to develop a set of pragmatic recommendations for awake proning in patients with COVID-19 pneumonia, focusing on settings where resources are limited, international healthcare professionals from high and low- and middle-income countries (LMICs) with known expertise in awake proning were invited to contribute expert advice. A growing number of observational studies describe the effects of awake proning in patients with COVID-19 pneumonia in whom hypoxemia is refractory to simple measures of supplementary oxygen. Awake proning improves oxygenation in most patients, usually within minutes, and reduces dyspnea and work of breathing. The effects are maintained for up to 1 hour after turning back to supine, and mostly disappear after 6–12 hours. In available studies, awake proning was not associated with a reduction in the rate of intubation for invasive ventilation. Awake proning comes with little complications if properly implemented and monitored. Pragmatic recommendations including indications and contraindications were formulated and adjusted for resource-limited settings. Awake proning, an adjunctive treatment for hypoxemia refractory to supplemental oxygen, seems safe in non-intubated patients with COVID-19 acute respiratory failure. We provide pragmatic recommendations including indications and contraindications for the use of awake proning in LMICs.
