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Explanation

1) Think long term.

As progress can be slow for PWH, thinking in longer timeframes can help (i.e. tracking progress at 2 - 4-week intervals rather than from day to day).

2) When assessing musculoskeletal function and joint health, hemophilia-specific tools can be used.

Tools such as the HJHS and the Hemophilia Activities List have been developed specifically to measure joint health and functional limitations in PWH, and have been validated [69] . These (and other)

hemophilia-specific tools may be helpful to assess and improve

outcomes, although PWH tend to prefer short tools [29] [70] . Hemophilia-specific tools should only be used by PTs trained in their use.

3) When assessing pain in PWH, consider if there may be joint bleeding.

Differential diagnosis is difficult in hemophilia, as there are several pathologies with similar symptoms, and a hemophilic flare-up may also mask pain from a bleed. However, there are several characteristic signs and symptoms that indicate a bleed, including a prodromal sensation of aura, inability to load the joint, tingling sensation, lessening pain after clotting factor treatment, painful palpation of swelling, no locking joint and a feeling of fullness in the joint (see Figure 4) [51] [52] .

4) Include teaching of supportive postures while sitting and focus on joint protection in those with complications from established joint disease.

Use of pillows, bolsters, and chairs can encourage a comfortable, ergonomic position and reduce stress on the joints, along with common joint protection techniques, equipment, and devices, as employed

in the treatment of OA and rheumatoid arthritis.

5) Encourage PWH to make creative use of their adaptive equipment and devices.

PWH may use standard equipment and devices (e.g. a mobile walker), with or without modifications, in unconventional ways because they are unable to bear weight through upper extremity joints, owing to blood-induced joint damage. This approach helps PWH live their daily lives in the most effective way for them.

6) Work on functional strength (e.g. taking sweater on/off, putting on shoes, sit to stand, steps and stairs) in addition to classic muscle strengthening.

These functional strength exercises can be modified as needed (e.g. repetitions of bicep curls at a specific weight), at a level appropriate for the individual with hemophilia [41] .

7) Isometric strengthening within a comfortable range may be considered the safest type of strengthening.

Isometrics focus solely on the muscle, without placing undue stress on joint structures, which may be damaged in PWH. Owing to the static nature of isometrics, it is commonly believed that there is little risk of impinging on hypertrophied synovial tissue, as could be the case during isotonic exercise. There should be a focus on a gradual progression from isometric to isotonic, within a pain-free ROM, and finally progressive, resistive exercises. Closed-chain versus open-chain formats should be considered to minimize risk.

8) Day-to-day functional postures can be assessed in the clinical examination room.

This can be measured by examining how PWH conduct activities such as rising from a chair and standing at the sink to wash. Hints, tips, and prompts can be taught to enhance stability and control in such positions.

9) Ensure PWH have sufficient hemostatic cover before commencing each physical therapy session, as recommended by their HTC.

This treatment may or may not be necessary depending on the severity of hemophilia and the factor infusion regimen prescribed by the HTC. This may involve clotting factor treatment before each physical therapy session (for example in post-surgery cases), which most PWH will typically administer themselves.

10) It is essential to highlight the importance of adhering to the prescribed factor replacement regimen and the consequences of non-adherence (e.g. the consequences of bleeding).

This is particularly useful for groups in which adherence is often problematic, for example in adolescents or in older adults with poor venous access from years of infusing.