Introduction
Predicting the risk of sports injuries and sports‐induced bleeds (SIBs) in people with haemophilia (PWH) may support clinical counselling.
Aim
To assess the association between motor ...proficiency testing and sports injuries and SIBs and to identify a specific set of tests for predicting injury risk in PWH.
Methods
In a single centre, prospective study male PWH aged 6–49 playing sports ≥1x/week were tested for running speed and agility, balance, strength and endurance. Test results below −2Z were considered poor. Sports injuries and SIBs were collected for 12 months while 7 days of physical activity (PA) for each season was registered with accelerometers. Injury risk was analysed according to test results and type of physical activity (%time walking, cycling, running). Predictive values for sports injuries and SIBs were determined.
Results
Data from 125 PWH (mean ± SD age: 25 ± 12, 90% haemophilia A; 48% severe, 95% on prophylaxis, median factor level: 2.5 IQR 0–15IU/dl) were included. Few participants (n = 19, 15%) had poor scores. Eighty‐seven sports injuries and 26 SIBs were reported. Poor scoring participants reported 11/87 sports injuries and 5/26 SIBs. The current tests were poor predictors of sports injuries (Range PPV: 0%–40%), or SIBs (PPV: 0%–20%). PA type was not associated with season (activity seasonal p values > .20) and type of PA was not associated with sports injuries or SIBs (Spearman's rho < .15).
Conclusion
These motor proficiency‐ and endurance tests were unable to predict sports injuries or SIBs in PWH, potentially due to few PWH with poor results and low numbers of sports injuries and SIBs.
Introduction
The paediatric Haemophilia Activities List (pedHAL) was developed to measure activities and participation in children and youth with haemophilia. Results from international studies ...provide an opportunity to determine which items are universally important.
Aim
The aim of this study was to determine which items of the pedHAL are redundant to construct a shorter version of the pedHAL.
Methods
This study is a cross‐sectional multicentre secondary analysis on pooled data of published studies using the pedHAL (7 domains, 53 items, optimum score: 100) in children with haemophilia A/B aged 4–18 years. To identify redundant items, the following aspects were evaluated: floor and ceiling effects, proportions of missing and ‘not applicable’ responses, inter‐item correlations, component loadings in an exploratory factor analysis, internal consistency and item‐total correlations.
Results
Data on 315 patients with haemophilia from 6 studies were evaluated. Median age was 12.2 years) (range 4.0–18.0), 87.3% had severe haemophilia and 80.3% received prophylaxis. Median (IQR) pedHAL sum score was 96.7 (88.0–100). After a stepwise procedure, 31 items were removed, resulting in a pedHALshort of 22 items, representing all original 7 domains. Most remaining items belonged to the domains ‘sitting/kneeling/standing’ and ‘functions of the legs’. The pedHALshort sum score was similar to the original pedHAL sum score, with small differences in 5 domains.
Conclusion
This clinimetric study resulted in >50% reduction of the length of the pedHAL. The 22‐item pedHALshort reduces patient burden and is expected to capture the information on activities and participation. The pedHALshort needs validation in other populations.
Background
Improved treatment options for people with haemophilia (PWH) have increased the possibilities for sports participation, but the risk of sports‐induced bleeding (SIB) is still considered ...considerable by many.
Aim
To assess sports associated injury‐ and bleeding risk in PWH and to assess clotting levels associated with safe sports participation.
Methods
Sports injuries and SIBs were prospectively collected for 12 months in PWH aged 6–49 without inhibitors playing sports at least once weekly. Injuries were compared according to factor levels, severity, joint health, sports risk category and sports intensity. Factor activity at the time of injury was estimated using a pharmacokinetic model.
Results
125 participants aged 6–49 (41 children, 90% haemophilia A; 48% severe, 95% severe on prophylaxis) were included. Sports injuries were reported by 51 participants (41%). Most participants (62%) reported no bleeds at all and only 16% reported SIBs. SIBs were associated with factor levels at time of injury (OR: 0.93/%factor level (CI 0.88–0.99); p = .02), but not with haemophilia severity (OR: 0.62 (CI 0.20–1.89); p = .40), joint health, sports risk category or sports intensity. PWH with factor levels <10% during sports injury had a bleeding risk of 41% versus 20% in those with higher (>10%) factor levels.
Conclusion
The results of this study emphasize the importance of clotting factor levels in prevention of bleeds. This information is vital for patient counselling and tailoring prophylactic treatment with clotting factors and non‐replacement therapy.
Introduction
The Paediatric Haemophilia Activities List (pedHAL) assesses self‐reported limitations in activities and participation in children with haemophilia.
Aim
To assess longitudinal changes, ...child‐parent agreement and to identify which pedHAL domains yielded most information in boys with access to early prophylaxis.
Methods
The pedHAL (53 items, 7 domains, optimum 100) was completed annually at the Van Creveldkliniek by boys aged 4‐18 years with moderate/severe haemophilia and their parents. Development of the pedHAL in relation to bleeds, changes per domain over 3‐5 years, child‐parent agreement (% difference child‐parent≤|5|) per domain and domain scores (limitations defined as ≤ 95) were determined.
Results
Seventy‐three patients and their parents (92% severe haemophilia, median age 13.1 years range 5.4;18.0) completed ≥1 pedHAL. Median (IQR) pedHAL sum score was 99.5 (95.2;100.0) for children and 99.6 (95.8;100.0) for parents. If patients scored >95 and had no joint and/or muscle bleed, 90.9% of the patients scored >95 at the next assessment. The median change in sum score was 0.0 for both the 3‐ and 5‐year interval. Child‐parent agreement varied between domains from 92% (‘self‐care’) to 71% (‘sitting/kneeling/standing’). Most limitations were reported in the domains ‘sitting/kneeling/standing’, ‘functions of the legs’ and ‘leisure activities and sports.’
Conclusion
In routine clinical practice in Dutch children on prophylaxis, pedHAL scores were high and remained stable in 3‐5 years at group level. In individual patients without joint and/or muscle bleeds, pedHAL scores remained high after 1 year. Child‐parent agreement was not optimal which indicated that both child report and parent proxy should be reported.
Introduction
Although sports participation is advocated in people with haemophilia (PWH), detailed data concerning sports participation in Dutch PWH is lacking.
Aim
to assess sports participation in ...Dutch PWH (6‐65 years) compared to the Dutch general population (GP).
Methods
Data from a nationwide, cross‐sectional study in PWH were analysed. Sports participation (type, duration, frequency) was assessed by the Modifiable Activities Questionnaire (MAQ), limitations in activities using the (Paediatric) Haemophilia Activities List ((Ped)HAL). Sports in the two highest categories according to the National Hemophilia Foundation classification were considered high‐risk sports. Groups were compared using Chi‐square testing.
Results
A total of 524 Adult PWH (median age: 45 (IQR: 30–55); 37% severe) and 126 paediatric PWH (median age: 11 (IQR: 8–14); 52% severe) were included. Sports participation was higher in adults (70%) than the GP (58%) and similar to the GP in children (PWH: 68%, GP: 72%). High‐risk sports participation decreased with age in PWH: from 65% (6‐12 years) to 17% (50‐65 years), which was also observed in the GP. Sports participation in children was independent of severity (non‐severe: 67% vs. severe: 65%; P = 0.97), but not in adults (non‐severe: 75%, severe: 62%; P < 0.01). Non‐severe PWH played more high‐risk sports than severe PWH: children at 65% vs. 48% (P = 0.05), adults at 25% vs. 15% (P = 0.07).
Discussion
These results suggest that sports participation in PWH was comparable to the GP. Sports participation was dependent of haemophilia severity in adults. Children were more involved in high‐risk sports than adults. More studies on sports‐related injury‐risk are needed for adequate counselling.
Introduction
The Haemophilia Activities List (HAL) was developed to measure activities and participation in persons with haemophilia (PWH). Shortening the questionnaire may facilitate use of the HAL.
...Aim
The aim of this study was to determine which items of the HAL are redundant, to construct a shorter version of the HAL, and to determine the construct validity of the HALshort.
Methods
A secondary analysis was performed on pooled data of two published studies using the HAL (seven domains, 42 items, optimum score: 100) in adults with haemophilia A/B. Data were divided into a derivation (62%) and a validation set (38%). Redundant items were identified by evaluation of: floor and ceiling effects, proportions of missing and ‘not applicable’ responses, inter‐item correlations, component loadings in an exploratory factor analysis, internal consistency, and item‐total correlations. Correlations with the SF‐36 and EQ‐5D‐5L were used to determine construct validity of the HALshort.
Results
Data on 680 PWH were evaluated. In the derivation dataset (n = 420), median age was 30 years (range 18–80), 43% had severe haemophilia and 61% received prophylaxis. Median (IQR) HAL sum score was 65.0 (55.7–88.8). The stepwise procedure resulted in a HALshort of 18 items with a median sum score of 63.3 (54.4–86.7). Construct validity was similar for the HAL and HALshort in the validation dataset (n = 260).
Conclusion
This clinimetric study resulted in a >50% shortening of the HAL. The 18‐item HALshort reduces patient burden and is expected to capture the information on activities and participation. The HALshort needs further validation.
Introduction
The Haemophilia Activities List (HAL) and paediatric HAL assess self‐reported limitations in various daily activities. To reduce patient burden, shorter versions of the pedHAL (22 items) ...and HAL (18 items) have been developed.
Aim
This study aimed to determine the agreement between the pedHAL/HALfull and pedHAL/HALshort and construct validity and internal consistency of the pedHAL/HALshort in persons with haemophilia (PWH).
Methods
A cross‐sectional secondary analysis of the Hemophilia in the Netherlands‐6 national survey was performed. Adult and paediatric PWH completed the original pedHAL/HALfull, from which pedHAL/HALshort were derived. Score differences between the original and short versions were calculated. Construct validity was studied by testing hypotheses regarding the relationship of the pedHAL/HALshort with the pedHAL/HALfull, Haemophilia & Exercise Project Test‐Questionnaire (HEP‐Test‐Q), Canadian Haemophilia Outcomes‐Kids’ Life Assessment Tool (CHO‐KLAT) and RAND 36‐item Health Survey (RAND‐36) (convergent/discriminant validity) as well as its ability to discriminate between subgroups (known‐group validity). Internal consistency was assessed with Cronbach's α.
Results
We included 113 children (median 10y range 4–17, 53% severe haemophilia) and 691 adults (median 51y range 18–88, 35% severe). Scores of the pedHAL/HALfull and pedHAL/HALshort were similar with high correlations (>0.9). Construct validity was confirmed for the pedHAL/HALshort. The HALshort was able to discriminate between different disease severities and ages. Cronbach's α of the pedHAL/HALshort was 0.95–0.97.
Conclusion
This study confirmed the agreement between the pedHAL/HALfull and the pedHAL/HALshort and the construct validity of the pedHAL/HALshort. The next step is to study construct validity of the pedHAL/HALshort when administered as short forms.
Introduction
Sports participation in children with hemophilia is generally considered to be associated with increased injury risk, which is generally considered highest in severe hemophilia.
Aim
To ...assess sports participation according to age and severity in children with hemophilia and its association with sports injuries.
Methods
In a retrospective single‐center study, sports participation, injuries, and bleeding data from three consecutive annual clinic visits were collected for young patients with hemophilia (PWH, aged 6‐18). Sports in categories 2.5 and 3 of 3 according to the National Hemophilia Foundation classification were considered high‐risk. Groups were compared using chi‐square testing.
Results
105 PWH (median age: 13(IQR 10‐14); 53% severe; bleeding rate: 1/y) were identified; three were unable to perform sports and were excluded. The majority of PWH (77%) played sports weekly, of which 80% high‐risk sports. Sports participation (median 3.0x/wk), and the proportion of injured PWH was similar in severe (42%) and non‐severe (33%) PWH. Sports injuries were rare (65% no injuries in 3 years, median 0/y (IQR 0‐1)). Annually, PWH did not report more injuries (15%) than age‐matched boys (28%). Sports injuries were not associated with frequency and type of sports.
Discussion
This retrospective study showed high sports participation (including high‐risk sports) and low injury rates. Sports participation was similar across severities and injury rates were not higher than among the general population. Injuries were not associated with frequency or type of sports. A prospective study with objective assessment of sports participation and injuries is warranted to confirm these findings and avoid recall bias.
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