South African Guidelines for Physiotherapy in Haemophilia

Home/Physiotherapy
Physiotherapy 2017-04-17T16:23:59+00:00

These guidelines have been compiled by Mrs Ethelwyn Remmers, Chief Physiotherapist at Steve Biko 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).

This guide is intended for people with haemophilia and healthcare personnel who may not be familiar with particular physiotherapy protocols pertaining to haemophilia management.
A pdf version can be downloaded here.

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:

Mpumalanga 2017-04-17T10:18:34+00:00
Physician Hospital Telephone number
Dr M Terry Rob Ferreira Hospital 072 499 4069
Sr J Tshabalala Rob Ferreira Hospital 076 657 6729
North West 2017-04-17T10:15:11+00:00
Physician Hospital Telephone number
Sr D Mofulatsi Potchefstroom Hospital 072 046 2034
Dr C Radebe Potchefstroom Hospital 082 440 7760
Mthatha 2017-04-17T10:18:55+00:00
Physician Hospital Telephone number
Prof N Tonjeni Nelson Mandela Academic Hospital 083 378 0801
Sr F Ncapai Nelson Mandela Academic Hospital 073 201 6217
Polokwane 2017-04-17T10:12:34+00:00
Physician Hospital Telephone number
Dr C Sutton Polokwane Provincial 082 800 6778
Sr Frida Kopa Polokwane Provincial 082 701 6465
015 287 5043
Bloemfontein 2017-04-17T10:11:14+00:00
Physician Hospital Telephone number
Prof D Stones Universitas 051 405 2820
083 444 7233
Prof M Coetzee Universitas 051 405 3116
082 550 1968
Haemophilia Clinic Universitas 051 405 3069
Pretoria 2017-04-17T10:09:55+00:00
Physician Hospital Telephone number
Dr J Potgieter Steve Biko Academic Hospital 012 319 2543
Sr Kate Hill Steve Biko Academic Hospital 072 6734675
Rubine de Beer Steve Biko Academic Hospital 012 354 1645
082 655 4625
Durban 2017-04-17T10:08:17+00:00
Physician Hospital Telephone number
Dr N Rapiti King Edward VIII 031 360 3093
Dr R Thejpal Albert Luthuli 031 240 1536
082 562 4491
Dr Y Goga Albert Luthuli 082 787 5786
Haemophilia Clinic King Edward VIII 031 360 3680
East London 2017-04-17T10:07:03+00:00
Physician Hospital Telephone number
Dr R Mathew Frere Hospital 043 709 2511
083 381 5541
Sr Eurica Syce Frere Hospital 083 558 0084
Dr D Fleitas Frere Hospital 082 822 1579
Port Elizabeth 2017-04-17T10:05:35+00:00
Physician Hospital Telephone number
Dr Tsutsu Dora Nginza 082 411 1368
Dr N Littleton PE Complex 041 392 3218
Dr H Nel PE Complex 041 392 3268
Sr Sharon Jantjies PE Complex 084 480 8788
George 2017-04-17T10:02:44+00:00
Physician Hospital Telephone number
Sr Mary-Ann Rothman George Provincial Hospital 044 802 4408
Cape Town 2017-04-17T10:01:02+00:00
Physician Hospital Telephone number
Sr AL Cruickshank Groote Schuur Hospital 082 788 1038
Dr Cecile du Toit Groote Schuur Hospital 082 579 3136
Dr A van Eyssen Red Cross Children’s 021 658 5297 / 5185
Dr M Hendricks Red Cross Children’s 082 870 2260
Prof C Karabus Red Cross Children’s 072 521 1395
Sameer Rahim Red Cross Children’s 084 578 6684
Dr G Sissolak Tygerberg 084 676 8614
Dr F Bassa Tygerberg 083 231 4766
Tygerberg Paediatrc Clinic Tygerberg 021 938 4565
Dr A Van Zyl Tygerberg 082 372 8622
Johannesburg 2017-04-17T09:57:07+00:00
Physician Hospital Telephone number
Johannesburg
Dr J Mahlangu Johannesburg 083 644 5659
Dr R Schwyzer Johannesburg 011 488 3294
Sr B Mbele Johannesburg 011 488 3294/5
Prof A Krause (Genetics) Johannesburg 011 489 9223
Dr R Wainwright Chris Hani Baragwanath 011 933 9549
Prof M Patel Chris Hani Baragwanath 011 933 8368
Dr M Bassingthwaighte Chris Hani Baragwanath 082 461 4696
Dr MJ Rasesemola Dr George Mukhari 082 719 2718
Sr Vicky Sehube Dr George Mukhari 083 734 1220
Dr A Mc Donald Johannesburg – PVT 084 566 0838
Sr Alice Banze/ Sr S Tuswa Haemophilia Nurses Office 082 896 3833
011 787 6710

Physiotherapy

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:
    • Myofibrils (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.
  • 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 lightweight, durable material.
  2. Initially the splint is only removed for icing and measurement of joint/muscle circumference.
  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.
    Splinting Method

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.
    Icing methods: Ice applied around the entire area, Submerge the whole area of the bleed
  2. Cover the entire involved area with ice.
  3. Time: 5 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 2 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.
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;
  • 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 5 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 5 to 10 repetitions and progress to 15 at least 3 times 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 5 to 10, and again progress to 15, 3 times 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 5 to 10 repetitions. Hold this position for 10 seconds at a time.
    9. Lastly, it is necessary to improve the endurance (fitness) 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.
Guidelines on 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 under ‘Rehabilitation after a bleed’ section).
Free active phase walk with crutches, splint on, partial weight bearing on affected limb (see treatment plan no. 5 under ‘Rehabilitation after a bleed’ section).
Additional exercise phase walk with crutches, splint on, full weight bearing on affected limb (see treatment plan no. 6 under ‘Rehabilitation after a bleed’ section).
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.

WHY ICE?

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

It has been found that after ± 5 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 flow 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 5 minutes on and 10 minutes off.

WHY IMMOBOLISE AND REST?
Movement in the joint soon after a bleed will more easily drive blood into 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 this 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.

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.

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

Jones P, Buzzard B, Heijnen L. Go for It: Guidance on Physical Activity and Sports for People with Haemophilia and Related Disorders, Montreal, Canada: WFH, 1998.
Semple F. Exercise, Notes on Physiotherapy, Johannesburg General Hospital
Buzzard, BM. Protective training in haemophilia, Haemophilia 1998; 4(4): 528-531.
South African Practical Guidelines for Physiotherapy in Haemophilia

ACKNOWLEDGEMENTS
Fiona Semple, Physiotherapist, Johannesburg General Hospital
Physiotherapy Department, Steve Biko Academic Hospital

Bleeds in the upper limbs (arms)

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.

1. Free supination (turning palm up) and pronation (turning palm down)

1. Free supination (turning palm up) and pronation (turning palm down)

2. Pronation and supination using a ruler or a stick as leverage

2. Pronation and supination using a ruler or a stick as leverage

3. Stretching of muscle that bend the wrist and fingers

3. Stretching of muscle that bend the wrist and fingers

1. Free elbow flexion against gravity

1. Free elbow flexion against gravity

2. Free elbow extension against gravity

2. Free elbow extension against gravity

3. Elbow flexion with resistance (weights)

3. Elbow flexion with resistance (weights)

4. Elbow extension with resistance (weights)

4. Elbow extension with resistance (weights)

1. Free elevation of the arm

1. Free elevation of the arm

2. Elevation against gravity

2. Elevation against gravity

3. Shoulder and elbow extension with resistance (ball or weights)

3. Shoulder and elbow extension with resistance (ball or weights)

4. Shoulder and elbow extension with resistance rubber band / tubing

4. Shoulder and elbow extension with resistance rubber band / tubing

5. Wrists, elbow and shoulder with body weight as resistance

5. Wrists, elbow and shoulder with body weight as resistance

Bleeds in the lower limbs

The ilio-psoas muscle is the hip flexor 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.

1. Free hip extension

1. Free hip extension

2. Stretching ilio-psoas muscle with assistance

2. Stretching ilio-psoas muscle with assistance

3. Stretching ilio-psoas muscle

3. Stretching ilio-psoas muscle

4. Hip flexion against gravity

4. Hip flexion against gravity

5. Hip flexion against resistance (weights)

5. Hip flexion against resistance (weights)

6. Free hip abduction (away from the centre)

6. Free hip abduction (away from the centre)

7. Hip abduction against gravity

7. Hip abduction against gravity
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)

1. Static contractions of quadriceps muscle (knee extension muscle)

1. Static contractions of quadriceps muscle (knee extension muscle)
Pull kneecap – a
Push knee down onto bed – b
Pull toes up – c

2. Knee extension against gravity with a small range of movement

2. Knee extension against gravity with a small range of movement

3. Knee extension against gravity with a greater range of movement

3. Knee extension against gravity with a greater range of movement

4. Knee extension against resistance (weights) with a small range of movement

4. Knee extension against resistance (weights) with a small range of movement

5. Knee extension against resistance (weights) with a greater range of movement

5. Knee extension against resistance (weights) with a greater range of movement
These are the muscles that straighten and bend the knee. This muscle needs to be strengthened as soon as possible to reduce the risk of re-bleeds when walking or running.

1. Free knee flexion

1. Free knee flexion

2. Knee flexion against gravity

2. Knee flexion against gravity

3. Knee flexion against resistance (weights)

3. Knee flexion against resistance (weights)
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

1. Free ankle plantarflexion (foot down) and dorsiflexion (foot up)

1. Free ankle plantarflexion (foot down) and dorsiflexion (foot up)
Balancing and Stretching exercises (Following a muscle bleed)

1. Weight bearing for ankles

1. Weight bearing for ankles

2. Walking on toes or heels balancing exercise

2. Walking on toes or heels balancing exercise

3. Exercise on a ‘wobble board’ balancing exercise

3. Exercise on a 'wobble board' balancing exercise

4. Stretching of hamstring muscle

4. Stretching of hamstring muscle

5. Weight bearing exercise for calf muscles

5. Weight bearing exercise for calf muscles

6. Stretching exercise for calf muscle

6. Stretching exercise for calf muscle

7. Stretching exercise for calf muscle #2

7. Stretching exercise for calf muscle #2