Strategies to encourage physical activity in patients with hemophilia to improve quality of life

Figures & data

Table 1 Summary of previous research about PA in PWH

References	Journal	Year	PWH group		Control group		Method	Results
			N	Average age, years (SD; range)	N	Average age, years (SD; range)
Falk et al^Citation10	Med Sci Sports Exerc	2000	30	12.0 (3.17)	16	11.9 (2.8)	Godin Leisure-Time Exercise Questionnaire	PA level of the PWH group was significantly lower than that of the control group Activity scores 31.8±22.7 (control group 83.4±64.6) Strenuous PA (times/wk) 1.38±2.06 (control group 6.31±5.38)
Tlacuilo-Parra^Citation25	Br J Haematol	2008	62	9.02 (3.7)	62	9.30 (3.7)	Validated questionnaire	Sedentary PA 33% (control group 11%) Low-level PA 44% (control group 40%) Moderate-level PA 23% (control group 38%) Intense-level PA 0% (control group 11%) PA level of the PWH group was significantly lower than that of the control group
Engelbert et al^Citation11	J Pediatr	2008	34	12.9 (3.2; 8.2–17.4)	–	–	Modifiable Activity Questionnaire	No significant differences for PA in the severity of hemophilia were found A substantial proportion of PWH were overweight, without showing a reduction in PA PA of total patients 57.5 (MET-hours/wk) (38.1; 5.8–181.3; n=34) PA of patients with mild hemophilia 53.5 (MET-hours/wk) (23.8; 15.0–91.9; n=16) PA of patients with moderate hemophilia 65.4 (MET-hours/wk) (19.5; 50.3–94.0; n=4) PA of patients with severe hemophilia 59.9 (MET-h/wk) (54.0; 5.8–181.3; n=14)
Tiktinsky et al^Citation22	Haemophilia	2009	44	18 (4; 12–25)	–	–	G&S questionnaire	Strenuous PA (units) 5.0±6.9 Moderate PA (units) 4.5±6.9 Mild PA (units) 3.0±4.3 Total PA (units) 12.6±10.8 G&S total score 77.9±80.2 Moderate correlation was found between PA and age (R=−0.45, P<0.002) Significant difference in the cause of bleeds was found: those who exercised strenuously showed higher proportion of bleeds due to traumatic reasons (P<0.01) 25 participants (56.8%) performed strenuous PA at least once a week
Buxbaum et al^Citation26	Haemophilia	2010	17	13.71 (2.1;11–18)	44	13.28 (2.0; 10–16.5)	Activity monitor (biaxial accelerometer [ActiTrac; IM Systems, Baltimore, MD, USA])	PA level (low and moderate) of the PWH group was significantly higher than that of the control group Low-level PA 70.24% (SD, 7.1) (control group 75.02% [SD, 6.0]) (P=0.010) Moderate-level PA 18.35% (SD, 3.4) (control group 15.89% [SD, 3.3]) (P=0.012) High-level PA 11.44% (SD, 6.3) (control group 9.13% [SD, 3.8]) (P=0.086) Vigorous PA 1.96% (SD, 2.6) (control group 1.54% [SD, 1.4]) (P=0.409)
Fearn et al^Citation27	Haemophilia	2010	20	39.4 (33.7–45.1)	20	40.3 (34.3–46.3)	The HAP	Adjusted activity score by the HAP 66.15 (59.38–72.92) (control group 87.10 [84.14–90.06]) PA level of the PWH group was significantly lower than that of the control group
Sherlock et al^Citation23	Haemophilia	2010	61	38 (16–63)	–	–	IPAQ	High-level PA: 28 patients (45.9%) Moderate-level PA: 17 patients (27.9%) Low-level PA: ten patients (16.4%) Moderate PA (minutes) 152.7±167.2 (European average 318.7±115.5) Vigorous PA (minutes) 141.1±145.6 (European average 281.7±631.15) Walking (minutes) 444.0±156.5 (European average 600±878.3) Sitting (minutes) 2,262.0±1,326.8 (European average 2,033.0±1,115.7) Total MET (minutes) 4,319.7±3,491.2 (European average 6,505.9±7,332.54)
González et al^Citation17	Haemophilia	2011	41	12.78 (8–18)	25	15.90 (8–18)	Activity monitor (triaxial A digraph) GT3X accelerometer (ActiGraph LLC, Fort Walton Beach, FL; USA)	PA (light, moderate, and moderate to vigorous) of PWH was higher than their healthy counterparts Healthy participants spent more time in sedentary behaviors than PWH Sedentary behavior (minutes/d) 356.78 (16.6) (control group 479.41 [19.62]) (P<0.007) Light PA (minutes/d) 450.24 (18.68) (control group 339.63 [19.93]) (P<0.007) Moderate PA (minutes/d) 8.48 (1.15) (control group 3.36 [0.86]) (P<0.007) Vigorous PA (minutes/d) 0.25 (0.06) (control group 0.41 [0.10]) Moderate-to-vigorous PA (minutes/d) 8.74 (1.19) (control group 3.77 [0.88]) (P<0.007)
Groen et al^Citation28	Haemophilia	2011	47	12.5 (2.9)	–	–	Modifiable Activity Questionnaire	PA levels were similar across PWH and comparable with the general population Total PA time (hours/wk) severe 8.7±3.8; non-severe 8.6±7.8 PA (MET-hours/wk) severe 54.9±30.3; non-severe 55.6±53.4) Vigorous PA (hours/wk) severe 6.2±4.2; non-severe 5.4±6.3
Khawaji et al^Citation29	Blood Coagul Fibrinolysis	2011	34	Group A: 27.1 (1–2; 19–35); group B: 44.1 (2.1; 33–56)	Group A: 20; group B: 42	Group A: 29.9 (1–0); group B: 43.0 (1.1)	Modifiable Activity Questionnaire	Group A: patients who started prophylaxis at the age of ≤3 years Group B: patients who started prophylaxis at the age of >3 years The age in group A was lower than that in group B (P<0.001) Total joint score in group A was lower than group B (P<0.001) Vigorous activity (MET-hours/wk): group A: 4.76 (1.59); group B: 0.57 (0.37) Leisure activity (MET-hours/wk): group A: 21.15 (3.19); group B: 8.45 (2.35) Total activity (MET-hours/wk): group A: 25.24 (3.59); group B: 9. 05 (2.41) PA (vigorous, leisure, and total) in group A was higher than group B PA (vigorous, leisure, and total) in group B was higher than the control group
Czepa et al^Citation24	Haemophilia	2012	48	44(11)	43	42(11)	Five-point Likert scale	PA level of PWH group was significantly higher than that of the control group Activity level (times/wk) 1.3±1.3 (0.0–4.0) Control group 0.7±0.9 (0.0–3.0)
Broderick et al^Citation21	Haemophilia	2013	104	9.5 (4–18)	–	–	Modifiable Activity Questionnaire	Leisure-time PA: all boys: 7.9 hours/wk; boys >10 years; 8.5 hours/wk Vigorous PA (>6 METs); all boys: 3.8 hours/wk; boys >10 years of age: 4.5 hours/wk Moderate and vigorous PA (>3 METs); all boys: 6.4 hours/wk; boys >10 years; 6.8 hours/wk
Goto et al^Citation30	Haemophilia	2014	32	42.5 (9.5; 26–61)	–	–	Activity monitor (triaxial accelerometer [HJA-350IT; Omron Co., Ltd, Kyoto, Japan])	PA for locomotive activities (MET-hours/wk) 12.8 PA for non-locomotive activities (MET-hours/wk) 3.7 Total PA (MET-hours/wk) 16.5
Goto et al^Citation31	J Jap Prim Care Assoc	2014	22	37.2 (10.4)	–	–	Activity monitor (triaxial accelerometer [HJA-350IT; Omron Co., Ltd])	No subject reached recommended PA, and all were physically inactive PA for locomotive activities (MET-hours/wk) 7.0 PA for non-locomotive activities (MET-hours/wk)2.8 Total PA (MET-hours/wk) 9.8

Notes: Previous research has investigated PA using a variety of methods, and characteristics of the subjects were different in these studies. PA can be measured in two ways – self-reporting and activity monitoring.²⁹ The former is widely used, because it is simple and low cost, whereas the latter has possibilities for goal setting and quantified monitoring. We focused on four studies that used an activity monitor. Studies by Buxbaum et al²⁶ and González et al¹⁷ showed that PA of PWH was higher than that of the control group. On the other hand, two studies by Goto et al^30,31 showed physical inactivity in PWH. However, regarding the subjects of research by Buxbaum et al, half of the number of subjects had severe hemophilia, average age was 13.71 years, and status of arthropathy was unknown. In addition, regarding the subjects of research by González et al, almost all the subjects had mild hemophilia, average age was 12.78 years, and status of arthropathy was 1.09–3.83 using the Gilbert score.⁷⁰ On the other hand, regarding the subjects of research by Goto et al, almost all the subjects had severe hemophilia, average age was 42.5 years and 37.2 years, and almost all had severe arthropathy. Therefore, the comparison of PA for PWH based on previous research is difficult at present.

Abbreviations: G&S, Godin and Shephard; HAP, human activity profile; IPAQ, International Physical Activity Questionnaire; MET, metabolic equivalent; N, number; PA, physical activity; PWH, patients with hemophilia; SD, standard deviation.

Table 2 Classification of exercise motivation based on self-determination theory

1.	Intrinsic motivation example: Because there is fun in exercise itself
2.	Integrated regulation example: Because my exercise is my top priority
3.	Identified regulation example: Because I think exercise is important for me
4.	Introjected regulation example: Because guilt occurs if I do not exercise
5.	External regulation example: Because the doctor told me that I should exercise
6.	Amotivation example: Reason for lack of exercise is unknown

Note: Motivation is listed in the order in which it is possible to exercise continuously.

Table 3 Source of self-efficacy information

1.	Enactive attainment (eg, discuss challenges depending on the ability and successful experiences of the patients)
2.	Vicarious experience (eg, share successful experiences with patients who have same abilities or problems)
3.	Verbal persuasion (eg, physician praises the successful experience of the patient)
4.	Physiological states or emotional arousal (eg, awareness of improvement in physical function to the patient by comparing previous and present PA levels)

Abbreviation: PA, physical activity.

Table 4 Information necessary for adaptation to the behavior change stage based on the transtheoretical model

Stage	Definition	Characteristics of patient	Goal	Strategies to support change	Inappropriate approach
Pre-contemplation	No intention of changing behavior in the near future, usually at the next 6 months	Not exercising at recommended level No intention to exercise May verbalize an inability to exercise or sustain exercise based on prior unsuccessful attempts May be uninformed or under-informed about the consequences of inactivity in terms of functional activity initiated at any point in the life cycle Tend to avoid reading, talking, or thinking about their inactivity View that the cons outweigh the pros	Increase awareness about the need to change	Encourage thinking about change Discuss feelings and perceptions of exercise/lack of exercise behavior –What in their life contributes to it? What meaning does activity/inactivity have for the patient/family? Give personalized feedback about the risk of the current behavior (loss of muscle strength, endurance, flexibility resulting in decreased functional ability) Personalize information about the benefits of initiating an exercise program beginning as an older adult Dialog about how inactivity affects role implementation, social activities	Intrusive behavior Unilaterally provide knowledge Persuade or argumentative discussion
Contemplation	Intention of changing behavior within the next 6 months	Seriously contemplating exercising/physical activity change in the next 6 months but not within the next month More aware of the pros of changing, but the cons still outweigh the pros, resulting in difficulty progressing to next stage May verbalize an understanding of the need for exercise but does not have the energy or readiness to change the behavior May verbalize: “I know I should, but I can’t now”	Motivate and increase confidence for the ability to change	Help patient set realistic expectations reinforced by small success Identity questions about exercising and continue to provide education about personal risks and benefits Decrease the barriers to exercise Encourage reflection on the ways inactivity affects their life Point out people who included regular exercise in their lives Help to make a definite commitment to change	Criticize or condemn the patient for failure to achieve behavior change Ignore benefits so that the patient continues with a sedentary lifestyle
Preparation	Intention of changing behavior within the next month	Intend to exercise in the near future (within the next 30 days) Pros grow stronger and outweigh cons Has more confidence that they can successfully exercise	Negotiate a plan for exercise	Create a new self-image as an exerciser Make a public commitment to exercise Identity alternatives for exercise and make a plan Facilitate involving supportive others Encourage a component of behavior change where they can experience success	Underestimate the ability of patient who does not reach the desired level of behavior change Creates many problems
Action	Lifestyle modifications for <6 months	The individual has made changes in activity and exercise level within the past 6 months and is meeting recommended exercise levels Relapses are frequent	Reaffirm commitment to exercise and implement plan	Frequent positive reinforcement with rewards for exercising regularly Initiate walking clubs Mobilization of social supporters to participate with the process Introduce additional exercise as an alternative Check off each time you exercise	Make the patient feel relieved by behavior change to the desired level Provide problems one after another
Maintenance	Exercising regularly and has done so for >6 months	Characteristics of patient Exercising regularly for >6 months Exercise has become an established pattern Increasingly more confident that he/she can continue to sustain activity	Problem-solve to prevent relapse	Plan for resisting temptations to skip exercise sessions Join support groups or have exercise buddies	Scold or blame the patient for physical limitations

Figure 1 Step count after 15 months of self-monitoring intervention for PWH.

Abbreviation: PWH, patients with hemophilia.

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