. 2021 Mar 10;27(4):544–562. doi: 10.1111/hae.14282

TABLE 3.

Study sample characteristics, PA outcome measures and main findings

Author and sample size	Age (years)	Type	Severity	PA outcome	Main findings
Janco et al. (1996)²⁸ n = 96	4–17 (range)	Both	All	6‐month daily checklist of PA, that is any strenuous or out of school activity; time spent with friend for 30 min not at school; house‐hold tasks.	Higher clotting factor levels reported higher levels of strenuous PA (p < .04). When controlled for factor level, higher levels of strenuous activity had higher rates of spontaneous joint bleeding (p < .05) and higher rates of trauma‐related soft tissue bleeding (p < .03)
Heijnen et al. (2000)²¹ n = 293	<6 to >29 (range)	Both	All	Self‐administered PA questionnaire.	Participated in 1+ sports: 74%; ‘Not active’ = 26%; Sev: ‘Active’ = 71% ‘Not active’ = 29%; ‘As active’ as Dutch male population (survey in 1990/91 ‐ 32% ‘not active’, 45% were semi‐active and 23% were active).
Van der Net et al. (2006)²⁹ n = 13	6.6^a (range 8–14.6)	FVIII	Sev	Self‐report: Hrs of PA at home; school; extra‐curricular sports; leisure time in 1 wk.	245 (133.2; range: 90–540)^a mins/wk, that is Between ±60% and ±180% of the Dutch PA guidelines (Moderate PA for at least 420 mins/wk, including twice a week vigorous sport activities.).
Nazzaro et al. (2006)³⁰ n = 110	16.7^a (range 13–21)	NS	All	Survey: 2 questions adapted from IPAQ on strenuous PA and 30 mins moderate PA over 1 wk.	Avoided or limited PA: 60%; Exercised as a preventative measure: 27%; Did not engage in regular strenuous/moderate PA: 27%.
Fromme et al. (2007)³¹ n = 71 i.e. 44 youths, 27 adults	Youths 10.2 ± 3^a Adults 29.2 ± 12.5^a	NS	All	Self‐administered questionnaire (everyday activities/school sports/leisure sports)	Regular participation in school sports: 79.6% youths; 37% adults did during school days (significant at p < .05). Excused due to risk of injury: 33.3% adults; 13.6% youths Youths: 88.6% performed one or more leisure sports; Adults: 66.7% performed one or more leisure sports.
Tlacuilo‐Parra et al. (2008)²² n = 62 x 2 (HG and CG)	HG 9.02 ± 3.7^a CG 9.3 ± 3.7^a	FVIII	All	Self‐report on PA and inactivity (h/day spent in PA).	HG vs. CG: Grouped sedentary and low PA significant; Inactive: 77% vs. 51% Sedentary: 33% vs. 11% Low PA: 44% vs. 40%; (grouped‐ p = .003, OR 3.24, 95% CI, 1.36–7.79); Moderate PA: 23% vs. 38%; Intense PA: 0 vs. 11%.
Tiktinsky et al. (2009)³² n = 44	18±5^a (range 12–25)	Both	Sev	G&SQ, 1 unit = Minimum 15 mins exercise outside PE and not associated with organised athletics.	Strenuous PA at least once/wk^a: 56.8%; 5.0 ± 6.9 units/wk; Moderate PA^a: 4.5 ± 6.9 units/wk; Mild PA^a: 3.0 ± 4.3 units/wk; G&SQ total score^a: 77.9 ± 80.2 i.e. 9 METS (strenuous units/wk) +5 METS (moderate units/wk) +3 METS (mild units/wk).
Koiter et al. (2009)²³ n = 99	12.6^a (range 8–18)	Both	All	The Movement and Sport Questionnaire: 12 questions on participation in PE, sports and active lifestyle and list 3 sports (including duration and freq/wk).	1 sport minimum: All 99; 2+ sports: 80 (81%); Freq/wk: 5 ± 3.2^a; Soccer (42%); swimming (22%); tennis (21%); gymnastics (13%); cardio‐fitness (13%).
Ross et al. (2009)³³ n = 37	6–21 (range)	Both	Sev	Medical chart audit of athletic participation with telephone interview if data missing regarding PA type, prophylaxis use and injuries.	Athletic activities were organised and supervised by adults; occurred at least x2/7, minimum 30 mins of PA. Athletic activities classified by likelihood of impact by NHF: High impact PA: 73%; Low impact PA: 27%.
Sherlock et al. (2010)³⁴ n = 61	38^a (range 16–63)	NS	All	IPAQ and questionnaire regarding participation in sport.	High PA: 46%; Moderate PA: 28%; Low PA: 16%; Sport: 51%; Moderate mins/wk^a: 152.7 (±167.2); Vigorous mins/wk^a: 141.1 (±145.6); Walking mins/wk^a: 444 (±156.5); Sitting mins/wk^a: 2262 (±1326.8); Half as much time in moderate and vigorous PA vs. EU average.
Khawaji et al. (2010)³⁵ n = 30	30.5^a (range 20–57)	Both	Sev	MAQ	Weight‐bearing PA: 96.6%; Vigorous PA: 56.6% (eg jogging, wood chopping, hunting); Non‐weight‐bearing PA: 60% (eg cycling, swimming, strength); Leisure walking: 63.3%; 4+ physical activities: 80%.
Buxbaum et al. (2010)³⁶ n = 62 i.e. HG (17); CG (44)	HG:13.71 ± 2.1^a CG:13.28±2^a	Both (only FIX in severe group)	All	Biaxial accelerometer (ActiTraC; IM systems, Baltimore, MD, USA) on waist for 7 consecutive days.	HG vs. CG PA (h/wk)^a: Low: 70.24 (±7.1) vs.75.0 2 (±6) [p = .010]; Moderate: 18.35 (±3.4) vs. 15.89 (±3.3) [p = .012]; High: 11.44 (±6.3) vs. 9.13 (±3.8) [p = .086]; Vigorous: 1.96 (±2.6) vs. 1.54 (±1.4) [p = .409]; Both spent >70%/day sedentary.
Groen et al. (2011)²⁴ n = 36	12.5 ± 2.9^a (range 8.2–17.4)	NS	All	MAQ compared with data from a previous study of the general Dutch population.	1+ activities at competitive level: 83%; Met guidelines (1‐hr moderate PA/5‐8 METs/day): 27.8% (vs. 21% in general population); Inactivity: 8% (vs. 12% in general population).
González et al. (2011)³⁷ n = 66 i.e. HG (41); CG (25)	HG:12.78 (0.48)^a ^SEM CG:15.9 (0.18)^a ^SEM	FVIII	All	Triaxial accelerometer (ActiGraph GT3X, Fort Walton Beach, FL, USA) on right hip for 7 consecutive days.	HG vs. CG PA (mins/day) ^a ^SEM: Sedentary: 356.78 (16.6) vs. 479.41 (19.62) [p <.001] Light: 450.24 (18.68) vs. 479.41 (19.62) [p <.001]; Moderate: 8.48 (1.15) vs. 3.36 (0.86) [p = .001]; Vigorous: 0.25 (0.06) vs. 0.41 (0.10) (not significant); MVPA: 8.74 (1.19) vs. 3.77 (0.88) [p = .001]; Total PA (counts/min):652.63 (33.74) vs. 430.82 (30.63) [p <.001].
Khair et al. (2012)³⁸ n = 84	11.52 ± 3.4^a (range 5.83–17.86)	Both	All	Questionnaire regarding sporting activities (freq and duration of sport/wk)	Participation in sport: 90.5%; Number of sports per person: 4^a; With friends: 80%; At school: 80%; Team/club sports: 40%; Golf course/gym: 50%; Total h/wk^a: 4.9 (range 1–13); 1 hr/wk: 2.6%; 2–5 h/wk: 59.2%; 6–9 h/wk: 35.5%; 10–13 h/wk: 2.6%; Freq/wk: x1: 21.1% x2: 48.7% x3 27.6% x > 3: 2.6%.
Broderick et al. (2012)³⁹ n = 104	9.5±4^a (range 4–18)	Both	Mod/Sev	Self‐reported PA 3 days before a bleed, PA categorised by risk of collision using NHF criteria. PA in 8‐h immediately before the bleed and two 8‐hr windows at 24 and 48 h before the bleed.	Interviews conducted for 329 bleeds, there was exposure to: C2 ‐ Significant collisions might occur, for example basketball: 30.6% of bleed windows‐ 24.8% 1^st control windows, 21.4% 2^nd; C3‐ Significant collisions inevitable, for example wrestling: 7.0% of bleed windows‐ 3.4% 1^st control windows, 4.6% 2^nd.
Baumgardner et al. (2013)⁴⁰ n = 88	41 (31.9–52.4)^b	Both	All	Framingham PAI	PAI score: 30.8 (27.7–35.8)^b; ‘Active’ (score >38): 14%; ‘Sedentary’ (score <28): 25%
den Uijl et al. (2013)⁴¹ n = 199 i.e. HG (94); CG (105)	HG 25–27 (20–33)^b CG 24 (20–31)^b	Both	Mod/Sev	IPAQ and a self‐designed sport list specifying type of participation in sport during the preceding year.	IPAQ results (METs)^b: HG = 3276 (960–8640) vs. CG = 3023 (1493–6936) (p = .26). Participation in sport: HG: Sev: 47 (59%); Mod: 28 (70%) vs. CG: 92 (88%) (p < .01) High‐risk sport: HG: Sev: 27 (34%); Mod: 20 (50%) vs. CG: 64 (61%) (p < 0.01).
Broderick et al. (2013)²⁵ n = 104 (66 prospective diaries)	9.5±4^a (range 4–18)	Both	Mod/Sev	MAQ (METs/wk for past year) and a random 1‐week prospective record of PA during year. PA categorised by risk of collision using NHF criteria.	Total leisure‐time PA^b: 7.9 (4.6–13.0) h/wk; Vigorous PA (>6 METs)^b: 3.8 (1.6–6.4) h/wk; MVPA(>3METs)^b: 6.4 (3.7–10.0) h/wk; 1 sport minimum: 45% for all and 61% for boys >10 years; Inactivity/day: 20.7 h (86.3%); C2 or C3 PA: 1.5 h (6.3%). Less than half met guidelines (43%) (less than children without haemophilia−57–67%).
Niu et al. (2014)²⁶ n = 122 (Adults‐ IPAQ (n = 69): children CPAQ (n = 53)	5–14: 9.6 ± 2.6^a 15–64:35.2 ± 15.5^a	FIX	All	IPAQ and CPAQ (parental proxy report).	IPAQ: High PA: 62%; Moderate PA: 29%; Low PA: 9%; Walking^b: 210 mins; 79% achieved PA guidelines of 75–150 mins/wk MVPA.	CPAQ: No engagement in PA = 2 (n). 79% of parents reported their child participated in PA on at least 4 days/wk.
McGee et al. (2015)⁴² n = 48	14.3 ± 2.6^a (range 10–18.8)	Both	All	Chart review of participation in organised sport (ie participating in sport at least x2/wk for 30 mins). PA categorised by risk of collision using adapted NHF criteria.	Sport participation 1 season minimum of organised sport: 62.5% (30) Basketball: 12/30; Hockey: 2/30 (against the advice of the haemophilia treatment team); Number of sport participated in: 1 (0–3)^c.
von Mackensen et al. (2016)²⁷ n = 50	35.12 ± 14.7^a (range 17–66)	Both	All	Questionnaire regarding sports (freq and duration/wk).	Participation in sport: 64%; Number of sports per person: 2^a; With friends: 81.3%; Team/club sports: 37.5%; Golf course/gym: 50%; Total h/wk: 3.71 ± 1.7^a‐ 1 hr: 12.5%; 2–3 h: 34.4%; 4 h: 25%; 5–8 h: 28.1%; Freq/wk: x1/7: 25% x2/7: 53.1% x3/7 18.8% x4/7: 3.1%.
Cuesta‐Barriuso et al. (2016)⁴³ n = 104 i.e. HG (53); CG (51)	HG 10.08 ± 1.36^a CG 9.78 ± 1.22^a	Both	All	Participation in sport.	Days practicing sports (days/wk)^a ⁽ ^a ⁾:1.29; HG: 1.81 ± 1.75 (0–5); CG: 2.18 ± 1.22 (0–5). No significant differences between groups; Sports played included swimming, cycling, tennis and football.
Bouskill et al. (2016)⁴⁴ n = 66	11.52 ± 3.99^a	Both	All	Triaxial accelerometer (ActiGraph GT3X, ActiGraph Corp, Pensacola, FL, USA) on right hip for 7 consecutive days and 3DPAR.	ActiGraph data (mins/day) ^a: Sedentary: Sev: 633.4 (± 121.3); Mild/mod: 327 (±78.73); MVPA: Sev: 48 (±20); Mild/mod: 55 (±18); 3DPAR (METs/day) ^a: Sev: 3.54 (±2.17); Mild/mod: 4.71 (±2.86). Close to meeting guidelines of 1 hr/day.
Carneiro et al. (2017)⁴⁵ n = 100 i.e. BrG (50); CaG (50)	BrG 13 ± 2.9^a CaG 12.1 ± 2.8^a	Both	Mod/Sev	IPAQ	IPAQ vigorous METs^b [BrG (n−10) vs. CaG]: 480 (960) vs. 1200 (3120) (p = .0017). Overall activity BrG vs. CaG (n): High 18 vs. 28 (p = 0.0045); Moderate 13 vs. 16; Low 9 vs. 6.
Baumann et al. (2017)¹⁴ n = AG (299; 89/299 female); ChG (150; 29/150 female)	AG 29 (18–70)^c ChG 10 (0–18)^c	FIX	All	Survey on participation in recreational activities accounting for severity and treatment regimen, intensity and duration of activities.	Most common current recreational activities: AG: walking (44%), dancing (26%), fishing (19%), and bicycling (16%); ChG: walking (49%), swimming (18%), bicycling (11%), jogging/running (11%), and martial arts (8%); *Intensity and mean/median duration of PA provided in article.
Flaherty et al. (2018)¹⁵ n = 14	48^a (range 24–77)	FVIII	All	Semi‐structured interviews in person or by phone.	−11 reported daily PA, 2 reported being mostly sedentary; 2 reported current PA reduced from normal/desired routine due to injury—walking most common type of PA reported; 6 reported regular exercise, average 5 days/wk, 4 daily; large variety including walking, running, fitness class, cycling, hiking, kayaking, etc.; 8 infrequently exercised.
Kempton et al. (2018)⁴⁶ n = 339 (IPAQ completed)/381	34 (26.3, 47.2)^b	Both	All	IPAQ	166 (49.0%) reported PA in previous wk; Duration (mins/wk)^b: walking: 60 (30, 240); moderate PA: 90 (60, 180); vigorous PA: 105 (60, 180); MET (mins/wk)^b: walking: 346.5 (198.0, 660.0); moderate PA: 360.0 (160.0, 600.0); vigorous PA: 960.0 (360.0, 3360.0).
Pinto et al. (2018)⁴⁷ n = 146 AG (106); CTG (21); CPrG (6‐9y n = 11, 1‐5y n = 8)	AG 43.49 (13.89)^a CTG 14.00 (2.39)^a ChG:6‐9y: 7.73 (1.01)^a; 1‐5y: 3.38 (1.60)^a	Both	All	PA questionnaire which collected information on PA and sport participation.	Regular participation (n): AG = 29 (27.4%), swimming (16), walking (5), cycling (3); CTG = 12 (57.1%), swimming (5), football (3), dance (2), gym (2); ChG 6‐9y = 9 (81.8%), swimming (7), hockey (1), dance (1); ChG 1‐5y = 4 (50.0%), swimming (4), football (1).
Pinto et al. (2018)⁴⁸ n = 102	43 (18–74)^c	Both	All	Questionnaire on either regular or occasional PA (freq and types of PA).	65 (63.7%) practiced PA, no detail on frequency and type provided in article.
Versloot et al. (2019)⁴⁹ n = 144 i.e. DG (43); SG (28); DCG (46); SCG (27)	26 (23–30)^b	Both	Sev	IPAQ and a questionnaire listing 23 sports played during last 12 months. Freq. of sport performed/wk in May also asked. PA categorised by risk as per NHF classification.	High‐risk sports: 59.2% (DG 27.9%; SG 42.9%; p < .05); IPAQ DG vs. SG (×1000 METs/wk)^b: 18‐22y: 5.8 (1.1–15.1) vs. 3.5 (1.2–7.9); 23‐29y: 5.0 (0.7–14.9) vs. 4.5 (1.3–12.0); 30‐40y: 2.6 (1.1–12.1) vs. 1.8 (0.5–12.6); Number and freq of sports per group provided in article. Similar participation in sport between peers and PWH (raw data available upon request).
Goto et al. (2019)⁵⁰ n = 106	40.8 (12.1)^a (range 18–64)	Both	All	IPAQ and sport participation questionnaire	PA levels (MET‐mins/wk) = 1501.8 (3413.0)^a; 693.0^b Significantly lower PA than Irish patients p < .001 (Sherlock et al., (2010) had higher number of mild patients). Moderate PA (mins/wk) = 103.7 (372.1)^a; <0.1^b; Vigorous PA (mins/wk) = 53.4 (209.6)^a; <0.1^b; Walking (mins/wk) = 333.6 (1106.7)^a; 122.5^b; Low PA n = 63 (59.4%); Moderate PA n = 29 (27.4%); High PA n = 13 (12.3%). 0 mins/wk of vigorous PA, moderate PA and walking, n = 85 (80.2%), 81 (76.4%), and 32 (30.2%), respectively. Sport participation previous year n = 50 (47.2%).
Zanon et al. (2020)⁵¹ n = 40, ChG (12); AdoG (9); AG (19)	ChG = <12 AdoG = 12–18 AG = >18	FVIII	Sev	EPIC Norfolk PA Questionnaire	More PA/sport participation noted in highly adherent patients on prophylaxis. A difference between adolescents and adults in type, freq, and impact of PA was noted (raw data NR). Type of sport by category of adherence (None‐High): Hobby/leisure: None = 3 (15%); Min = 2 (10%); Low = 2 (10%); Med = 2 (10%); High = 11 (55%); Endurance sports: None = 3 (14.3%); Min = 2 (9.9%); Low = 2 (9.9%); Med = 3 (14.3%); High = 11 (52.4%); Athletic sports: None = 2 (13.3%); Min = 2 (13.3%); Low = 1 (6.7%); Med = 1 (6.7%); High = 9 (60%); Ball sports: None = 2 (16.7%); Min = NA; Low = 1 (8.3%); Med = 2 (16.7%); High = 7 (58.3%).
Timmer et al. (2020)⁵² n = 105	43 (30–54)^b	Both	All	Activ8 accelerometer carried in trouser pocket for 7 consecutive days.	Majority = Sedentary (n = 60); Walkers (n = 21); Bikers and runners (n = 24) Sitting (h/day): 9.2 (7.4–10.6)^b; Standing (h/day): 2.8 (2.0–3.6)^b; Walking (h/day): 1.9 (1.4–2.5)^b; Biking (mins/day): 14.2 (5.8–28.7)^b; Running (mins/day): 0.6 (0.2–1.9)^b; Frequency of active bouts /day: 10.0 (7.1–12.7)^b; Length active bout (mins): 11.8 (10.6–14.3)^b.
Taylor et al. (2020)⁵³ n = 72	44.5 ± 15.5^a (range 18–69)	Both	All (mod excluded from analysis)	IPAQ and questionnaire on types of activities involved in	High PA: Sev 17 (40%); Mild 15 (52%); Total 31 (43%); Moderate PA: Sev 19 (44%); Mild 9 (31%); Total 30 (42%); Low PA: Sev 7 (16%); Mild 5 (17%); Total 11 (15%) Total MET^a ⁽ ^a ⁾: Sev 3770 ± 3979 (219–20 739); Mild 4530 ± 4457 (33–18 339); Total 4075 ± 4164 (33–20 739); Vigorous and Moderate MET^a ⁽ ^a ⁾: Sev 2567 ± 3570 (0–18 660); Mild 3390 ± 3682 (0–16 260); Total 2899 ± 3613 (0–18 660); Self‐reported achieved UK guidelines for activity: Sev 15/43 (35%); Mild 19/29 (65%); Total 34/72 (47%); 85% met UK PA guidelines (higher than general population).
Bérubé et al. (2020)⁵⁴ n = 24	11.8 ± 3.3^a (range 6–18)	Both	Sev	Self‐report of PA/wk for safe and high‐risk PA in winter and summer‐ G&SQ wording used, parental proxy report taken for children <10y.	When those who practiced high‐risk vs. low‐risk PA were compared, those in the high‐risk category practiced more high‐risk PA vs. those in the low‐risk category (p < .05) (2.6 vs. 0.6 days/wk). No significant differences between categories with regards practice of lower risk PA.

Abbreviations: AdoG = adolescent group; AG = adult group; All = mild, moderate and severe; Both = FVIII and FIX deficiency; BrG = Brazilian group; C2 = category 2 activity; C3 = category 3 activity; CaG = Canadian group; CG = control group; ChG = children/caregivers of children group; CPAQ = Children's Physical Activity Questionnaire; CTG = children/teenager group; /day = Per day; DCG = Dutch control group; DG = Dutch group; FIX = FIX deficiency; freq = frequency; FVIII = FVIII deficiency; G&SQ = Godin & Shepard Physical Activity Questionnaire; HG = haemophilia group; hr(s) = hours; IPAQ = International Physical Activity Questionnaire; MAQ = Modifiable Activity Questionnaire; Med = medium; METs = metabolic equivalent of task; METs = metabolic equivalent of task; min = minimum; mins = minutes; Mod = moderate; MVPA = moderate‐vigorous physical activity; NA = not applicable; NHF = National Haemophilia Foundation; NR = not reported; NS = not specified; PA = physical activity; PAI = Physical Activity Index; PE = physical education; SCG = Swedish control group; SEM = standard error of the mean; Sev = severe; SG = Swedish group; 3DPAR = 3‐Day Physical Activity Recall Questionnaire; /wk = per week; /wk = per week HG = haemophilia group; y = years (age).

^{^a}

Mean±standard deviation

^{^b}

Median±(interquartile range)

^{^c}

Median±(range);