South African Practical Guidelines for Physiotherapy in Haemophilia

  • Practical guidelines for physiotherapy in haemophilia

    This guide is also available to be downloaded in .pdf format here.

    These guidelines have been compiled by Mrs Ethelwyn Remmers, Chief Physiotherapist at Pretoria Academic Hospital on behalf of the National Working Group on Physiotherapy of the South African Haemophilia Foundation Medical and Scientifc Advisory Council (MASAC). The aim of these guidelines is to give a practical approach to the management of people with haemophilia (PWH).

    Editorial committee:
    Anne Gillham Ethelwyn Remmers
    Johnny Mahlangu Rosemarie Schwyzer
    David Stones Cyril Karabus
    Glynn Wessels Mike du Toit

    This booklet is intended as a guide for people with haemophilia and healthcare personnel who may not be familiar with particular physiotherapy protocols pertaining to haemophilia management.

    If you are uncertain about anything, please contact Mrs Ethelwyn Remmers on 072 247 7352 or your nearest Haemophilia Treatment Centre to be referred to a Haemophilia Physiotherapist.

    Haemophilia Treatment Centres and Treaters
    Haemophilia Foundation

  • Physiotherapy in the haemophilia patient

    The role of the physiotherapist is to help reduce and prevent muscular and joint problems in the person with haemophilia.

    Muscle bleeds can be a consequence of:

    • Direct trauma
    • Sudden stretch
    • Overstretch

    Some patients can bleed spontaneously without any of the above causes.

    Joint bleeds are caused by:

    Trauma to the synovial membrane (joint lining)

    Signs and symptoms of a bleed:

    • Pain
    • Reduced range of movement (ROM)
    • Increased temperature
    • Swelling

    Dangers of a bleed:
    1. Increased pressure in a confined space may cause damage to sensitive structures such as:
    • Myofbrils (muscle fibres);
    • Nerves;
    • Hyaline cartilage (smooth bone-end covering);
    • Synovial membrane of joints (joint lining).
    2. This leads to decreased function of muscles or joints.
    3. Increased pain, which again decreases function of muscles or joints.
    4. With the repeated bleeds the muscles may not have regained full length or strength by the next bleed, this leads to a vicious cycle of bleeds as the joints are not adequately supported by the muscles.
    5. This is the beginning of arthropathy and eventually osteoarthritis.

  • Management of an acute bleed

    Replace the missing clotting factor by infusion
    Immobilise the muscle or the joint in the straightest comfortable position
    Ice the area of the bleed
    Rehabilitation of the affected area

    Immobilisation methods
    1. Joints like the knee, elbow and ankle can easily be splinted using a fairly-light weight, durable material.
    2. Initially the splint is only removed for icing and measurement of joint/muscle circumference.
    Splinting
    3. The areas posing problems to splinting are bleeds in the hip joint or the iliopsoas muscle.For these, bed rest is the only good form of immobilisation, with the hip as straight as possible.

    Ice
    1. Apply wet ice in a damp towel or submerge in ice water. If ice is unavailable a packet of frozen peas or sweetcorn from the freezer may be used.
    Ice
    2. Cover the entire involved area with ice.
    3. Time: Five minutes with ice on; 10 minutes with ice off - Repeat this until ice has melted
    4. This procedure should be repeated as often as necessary. In a severe bleed, repeat every two hours.
    5. Always measure the swollen area.
    To evaluate the effect of treatment, measurement should be:
    • with the same tape measure;
    • in the same spot; and
    • at the same point in the procedure, i.e. before or after icing, or both.
    6. Note down the measurement at each treatment.

  • Rehabilitation after a bleed

    After controlling the acute bleed, rehabilitation starts in order to return to or improve on the pre-bleed state.
    The rate of progress depends on several factors:
    • Severity of the bleed – the more severe, the slower you can progress;
    • Length of the rest or immobilisation – the longer it has been, the longer it takes to get back to the pre-bleed stage; and
    • Target joints/muscles (previously damaged) are less likely to return to the pre-bleed state.

    Treatment plan:
    1. Rest the joint muscle as straight as comfortably possible.
    2. Stretching after muscle bleeds – start with fve stretches and increase to 10 stretches, holding the muscle on stretch for 10 seconds at a time A muscle that can comfortably reach full stretch over a joint, will help reduce the likelihood of a bleed due to a sudden jerk or stretch of the muscle.
    3. Gentle static muscle contractions (tightening of the muscles without causing any movement of the joint) as soon as the pain allows. No more than 5 to 10 contractions twice daily are necessary.
    4. Strong static muscle contractions – start these exercises as soon as
    • pain improves;
    • the swelling is reduced; and
    • the temperature at the site of the bleed, is down.
    • Do fve to 10 repetitions and progress to 15 at least thrice daily.
    • Do exercises when splint is removed for icing and measuring.
    5. As symptoms improve (less pain, swelling and temperature), the splint can be left off for longer periods. One can now move to free exercise:
    • movement with gravity eliminated and
    • movement against gravity.
    6. Finally, exercise against resistance (weights).
    Important: Always use light weights and high repetitions when exercising.
    When progressing from one exercise to the next, it is wise to reduce the repetitions to five to 10, and again progress to 15 thrice daily.
    7. Once the pre-bleed state has been reached, strengthen the muscles further by additional resistance (weights).
    Strong muscles around a joint support and protect the joint, and this reduces the risk of a bleed.
    With a muscle bleed, full stretch (elasticity) must be regained or improved to reduce risk of a bleed due to overstretching.
    8. Full range of movement of the joint must be aimed for. Apply gentle stretches at the end of the movement, do five to 10 repetitions. Hold this position for 10 seconds at a time.
    9. Lastly, it is necessary to improve the endurance (ftness) of the muscles to reduce the risk of injury due to early tiring of the muscles. This is achieved by regular exercise using low weight load and high repetitions. E.g. cycling, swimming, walking.

  • Rehabilitation after a lower limb bleed

    Guidelines of the use of walking aids

    In lower limbs, walking aids are generally used in the rehabilitation stage. These may include walking frames, crutches or walking sticks.

    Acute and early phase

    Walk with crutches, splint on, non-weight bearing on the affected limb (see treatment plan no 4).

    Free active phase

    Walk with crutches, splint on, partial weight bearing on affected limb (see treatment plan no 5).

    Additional exercise phase

    Walk with crutches, splint on, full weight bearing on affected limb (see treatment plan no 6).
    Splint off (still sleeping with splint on), partial weight bearing on affected limb
    Splint off, crutches, full weight bearing on affected limb
    Finally walking without any aid.

  • Common questions

    Why Ice?

    • For the analgesic effect (pain relief)
    • For maximal vasoconstriction (narrowing the blood vessels) thus reducing the blood volume to the affected area for the duration of the icing.

    It has been found that after ± five minutes of ice, the vessels in the area are maximally constricted. Should the ice remain on longer, the vessel will start dilating beyond their normal state and thus increase the blood fow to the area. Once the ice has been removed, it takes ±10 minutes before the vessels return to their normal state.

    Ice is applied for five minutes on and 10 minutes off.

    Why immobolise and rest?

    • Movement in the joint soon after a bleed will easily drive blood to the smooth cartilage covering the bone ends, and thus cause damage to it.
    • Muscle action across a joint increases the pressure in the joint, which could lead to joint damage.
    • To minimise the damage to muscle fibres – bleeding and muscle contraction increase the intramuscular pressure.
    • To reduce pain, thus decreasing muscle inhibition or guarding.
    • To minimise the risks of another bleed at the stage.

    Why strengthen muscles?

    • Muscles are weaker than normal after rest and/or immobilisation.
    • Weak muscles cannot support and protect joints adequately thereby joints are more susceptible to recurrent bleeds.
    • Retain muscle contractability and regain or improve muscle elasticity, thus reducing the risk of a bleed due to overstretch.
    • Regain muscle bulk and improve the appearance of the limb.

    Why improve balance?

    • Balancing exercises re-educate movement patterns and posture automatically.
    • Maintaining good balance would reduce the risk of a muscle or joint bleed in the event of stumbling.

  • Bleeds in the upper limbs (arms)

    Elbow

    Bending and straightening (flexing and extending) as well as rotating movements (pronation and supination) are to be maintained or improved.

    Another aspect closely connected to exercising, is the development of good veins in order to facilitate infusion procedure. This can be achieved by squeezing a soft ball or similar object.

  • Exercises for various joints - Upper limbs
    • Shoulder
      1. Free elevation of the arm
      Free elevation
      2. Elevation against gravity
      Elevation against gravity
      3. Shoulder and elbow extension with resistance (ball or weights)
      Extension with resistance
      4. Shoulder and elbow extension with resistance rubber band / tubing
      Extention with resistance rubber band / tubing
      5. Wrists, elbow and shoulder with body weight as resistance
      Body weight as resistance

    • Elbow
      6. Free elbow flexion against gravity
      Flexion against gravity
      7. Free elbow flexion against gravity
      Flexion against gravity
      8. Elbow flexion with resistance (weights)
      Flexion with resistance (weights)
      9. Elbow flexion with resistance (weights)
      Flexion with resistance (weights)

    • Wrist
      10. Free supination (turning palm up) and pronation (turning palm down)
      Supination (turning palm up) and pronation
      11. Pronation and supination using a ruler or a stick as leverage
      Pronation and supination using a ruler or a stick as leverage
      12. Stretching of muscle that bend the wrist and fingers
      Stretching of muscle

  • Exercises for various joints - Lower limbs
    • Hips and Psoas muscle

      The ilio-psoas muscle is the hip fexor muscle. For these bleeds, the only good form of immobilisation is bed rest with the hip as straight as possible. It may be necessary to use a pillow to support the leg in the very acute stage, but as soon as possible this must be removed. It is advisable to lie on the stomach (prone lying) for periods of the day – this is to stretch the hip muscles.

      After a hip bleed/psoas bleed it is advisable to also do quadriceps exercises.

      Hip Flexion (Psoas muscle) and Extension
      13. Free hip extension
      Free hip extension
      14. Stretching ilio-psoas muscle with assistance
      Stretching ilio-psoas muscle with assistance
      15. Stretching ilio-psoas muscle
      Stretching ilio-psoas muscle
      16. Hip flexion against gravity
      Hip flexion against gravity
      17. Hip flexion against resistance (weights)
      Hip flexion against resistance (weights)
      18. Free hip abduction (away from the centre)
      Free hip abduction (away from the centre)
      19. Hip abduction against gravity
      Hip abduction against gravity

    • Knee joint, Quadriceps and Hamstring muscles

      These are the muscles that straighten and bend the knee. The medial (inner) part of the quadriceps muscle helps to "lock" the knee when straightening it. This muscle needs to be strengthened as soon as possible to reduce the risk of re-bleeds when walking or running.

      Quadriceps (Extention muscle)
      20. Static contractions of quadriceps muscle (knee extension muscle)
      Static contractions of quadriceps muscle (knee extension muscle)
      Pull kneecap – a
      Push knee down onto bed – b
      Pull toes up – c
      21. Knee extension against gravity with a small range of movement
      Knee extension against gravity with a small range of movement
      22. Knee extension against gravity with a greater range of movement
      Knee extension against gravity with a greater range of movement
      23. Knee extension against resistance (weights) with a small range of movement
      Knee extension against resistance (weights) with a small range of movement
      24. Knee extension against resistance (weights) with a greater range of movement
      Knee extension against resistance (weights) with a greater range of movement

    • Hamstrings (Flexion muscle)
      25. Free knee flexion
      Free knee flexion
      26. Knee flexion against gravity
      Knee flexion against gravity
      27. Knee flexion against resistance (weights)
      Knee flexion against resistance (weights)

    • Ankle joint and Calf muscle

      A bleed in the calf muscle would affect knee and ankle movement. Position the ankle in mid-position, and the knee as straight as possible.

      The same applies for an ankle bleed.

      Ankle and Calf muscle
      28. Free ankle plantarflexion (foot down) and dorsiflexion (foot up)
      Free ankle plantarflexion (foot down) and dorsiflexion (foot up)

      Balancing and Stretching exercises (Following a muscle bleed)
      29. Weight bearing for ankles
      Weight bearing for ankles
      30. Walking on toes or heels balancing exercise
      Walking on toes or heels balancing exercise
      31. Exercise on a 'wobble board' balancing exercise
      Exercise on a 'wobble board' balancing exercise
      32. Stretching of hamstring muscle
      Stretching of hamstring muscle
      33. Weight bearing exercise for calf muscles
      Weight bearing exercise for calf muscles
      34. Stretching exercise for calf muscle
      Stretching exercise for calf muscle
      35. Stretching exercise for calf muscle
      Stretching exercise for calf muscle

  • Sport

    Participation in sport is encouraged. This is both enjoyable and ensures regular exercises.

    It is necessary to ensure full elasticity and strength of your muscles and full mobility of your joints before taking part in sport.

    Discuss the type of sport with your physiotherapist before starting. A wide range of sporting activities is regarded as "low risk" for persons with haemophilia.

  • Glossary
    Arthritis Infammation of a joint. In haemophilia caused by irritation due to excess blood within the joint space
    Arthropathy Chronic arthritis. In haemophilia long-term damage due to repeated bleeds into the joint
    Contraction Tightening of muscle
    Elasticity Stretchability of muscle
    Haemarthrosis Joint bleed
    Haematoma Tissue bleed. Blood clot may involve muscle and other soft tissue
    Hayline cartilage Smooth pearly covering of bone-ends
    Immobilise Prevent or reduce movement to a minimum
    Myofibrils Muscle fibres
    Prophylaxis Treatment given to prevent bleeding
    Synovial membrane Smooth joint lining
    Synovial fluid Lubricating joint fluid
    Synovitis Infammatory response in joint resulting in swelling
    Extension Straightening a joint
    Flexion Bending a joint
    Dorsiflexion Pulling the foot and toes down
    Plantarflexion Pointing the foot and toes down
    Pronation With elbow bent, turning palm of hand down
    Supination With elbow bent, turning palm of hand up
    Ilio-psoas Hip flexion muscle
    Hamstrings Knee flexion muscle
    Quadriceps Knee extension muscle
    NWB Not putting any pressure on the leg when walking, although you may place the foot on the ground
    PWB Putting some pressure through the leg, initially very little, gradually increasing the pressure
    FWB Putting equal pressure through both legs when walking

  • References

    Go For It – WFH 1998
    Management of the haemophiliac – various notes. Fiona Semple, Physiotherapist, Johannesburg General Hospital
    Exercise. Fiona Semple, Physiotherapist, Johannesburg General Hospital, Physiotherapy Department, Pretoria Academic Hospital
    Buzzard, BM. Protective training in haemophilia. Haemophilia 1998; 4(4): 528-531.