Moving beyond limitations: A narrative review exploring the intersection of haemophilia and physical activity

Low risk (1)

Low-moderate risk (1.5)

Moderate risk (2)

Moderate-high risk (2.5)

High risk (3)

Adapted PA in diverse settings with available resources

Includes those without access to prophylaxis

Form of benefit the patient is seeking

Ability for family to engage in activity together

Whether there is a target joint

If there is a target joint, whether specific sports are a risk to it

Best time to infuse factors relative to PA

General health

Bleeding history

Condition of joints

How joints respond to treatment

Patient recommendations:

Set goals

Ensure activities are supervised

Follow instructions to reduce injury

Warm-up prior to activity

Age-appropriate recommendations are provided for toddlers, preschoolers, school-age children, teens, and adults

Bone health

Muscle strengthening

Coordination

Physical function/condition

Ability

Preference/interests

Local customs/available resources

Healthy body weight

Positive self-esteem

Weight-bearing PA is encouraged to promote bone density and joint health

Health condition

Reflexes and coordination

Team versus individual sports

Supervised activity

Speed

Contact

Skill level

Position on the team

Plans for advancing in the sport

Protective gear

Training

Cost of activity/gear

Modifications to activity

Age

Family situation

Current activity level

Sport safety rating

Bleeding history

Joint health

Environmental factors

Improving muscle function, flexibility, strength, and endurance

Stretching, warmup, and cooldown

Gradual, targeted strength training

Non-contact sports (e.g., swimming, jogging, golf, rowing, etc.) over high-contact and collision sports (e.g., soccer, hockey, rugby, boxing, etc.) and high-velocity sports (e.g., skiing)

Organised sports over unstructured sports

Avoid high-contact, collision, and high-velocity sports unless patient has a sufficient prophylactic regimen and education on risks and protective measures

Identifying and managing bleeding episodes

Rehabilitation requirements and safe re-introduction of activity following a bleeding episode

Patient’s condition

Physical skills

Protective gear requirements

Appropriateness of specific sports

795 Cat I

193 Cat II

23 Cat III

29.8% Cat I

38.3% Cat II

86.6% Cat III

2 knee hemarthroses during fitness training; FVIII levels ~1% at start of PA

1 knee hemarthrosis during alpine ski fall; FVIII levels ~55% at start of PA

14 (54%) during high-risk sports

6 (23%) during high-intensity sports

Multicentre, prospective, 6-month observational study in PwHA (moderate to severe) aged 11–58 years

N=54

75.5% low

9.8% low-to-moderate

18.8% moderate

6.6% moderate-to-high

3.9% high

64.7% >24 hours

27.8% 1–2 days

37.3% 3–4 days

9.6% ≥5 days

1.26 (prophylaxis patients)

2.29 (on-demand patients)

Single-centre, retrospective cohort study in paediatric PwHA (moderate or severe) aged 3–15 years

N=105

57.6% low

42.4% medium

0% high

6.1% for low-risk PA

9.1% for medium-risk

2.7% during low-risk SIB

3.4% during medium-risk SIB

23 ankle

9 knee

2 elbow

Increase in number of patients and time (in minutes/week) engaging in moderate- and high-risk PA starting after first week of treatment initiation

Baseline PA levels were comparable to the general population and remained stable through Week 49

PA parameters remained stable after switching from FVIII prophylaxis (Week 24) to end of study (Week 49) including time spent in PA, daily step counts, and METs

The proportion of patients with zero PA-related bleeds increased from 39.3% with FVIII prophylaxis to 75.0% with emicizumab

Single-centre, retrospective chart review in PwHA (mild to severe) aged 0–80 years initiating emicizumab

N=68

55% of participants reported an increase in PA levels, 45% similar rates

25% reported trying new types of PA ranging from walking more to lifting weights or playing dodge ball

Interim analysis of the multicentre, prospective, observational study, TSUBASA, in PwHA (moderate or severe) aged 0–73 years

N=107

396 PA sessions reported, most commonly walking (32.4%), cycling (14.9%), and soccer (5.4%)

Two PA-related bleeds (0.5%) reported in one participant secondary to running and weight training

Exploratory analysis of the multicentre, randomised, open-label, Phase III explorer7 trial in PwHA/B with inhibitors (any severity) aged ≥12 years

N=52

Changes in percentage of awake time spent in moderate and moderate-to-vigorous PA from baseline to end of main part of trial favoured concizumab (estimated difference versus no prophylaxis: 4.2% for moderate PA, 4.4% for moderate-to-vigorous PA)

One patient (7.7%) in the concizumab group participated in each of low-to-moderate, moderate, and moderate-to-high-risk sports at end of main part of trial; one patient (9.1%) in the no prophylaxis group participated in low-to-moderate-risk sports (none in moderate or moderate-to-high-risk sports)

Changes in percentage of awake time spent in moderate and moderate-to-vigorous PA from baseline to end of main part of trial favoured concizumab (estimated difference versus no prophylaxis: 4.2% for moderate PA, 4.4% for moderate-to-vigorous PA) for PwH/A with inhibitors, but there was no significant difference for patients without inhibitors (estimated difference versus no prophylaxis: 1.3% for moderate PA, 0.8% for moderate-to-vigorous PA)

Between Weeks 52 and 156, 86–100% of patients reported doing the same or higher-intensity PA than at baseline

At Week 156, no patients reported a reduction in PA amount or intensity relative to baseline