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.
- Cape Town
- East London
- North West
- Port Elizabeth
|Dr M Terry||Rob Ferreira Hospital||072 499 4069|
|Sr J Tshabalala||Rob Ferreira Hospital||076 657 6729|
|Sr D Mofulatsi||Potchefstroom Hospital||072 046 2034|
|Dr C Radebe||Potchefstroom Hospital||082 440 7760|
|Prof N Tonjeni||Nelson Mandela Academic Hospital||083 378 0801|
|Sr F Ncapai||Nelson Mandela Academic Hospital||073 201 6217|
|Dr C Sutton||Polokwane Provincial||082 800 6778|
|Sr Frida Kopa||Polokwane Provincial||082 701 6465
015 287 5043
|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|
|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
|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|
|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|
|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|
|Sr Mary-Ann Rothman||George Provincial Hospital||044 802 4408|
|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|
|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
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
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:
- Reduced range of movement (ROM)
- Increased temperature
Dangers of a bleed:
- Increased pressure in a confined space may cause damage to sensitive structures such as:
- Myofibrils (muscle fibres);
- Hyaline cartilage (smooth bone-end covering);
- Synovial membrane of joints (joint lining).
- This leads to decreased function of muscles or joints.
- Increased pain, which again decreases function of muscles or joints.
- 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.
- 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
- Joints like the knee, elbow and ankle can easily be splinted using a fairly lightweight, durable material.
- Initially the splint is only removed for icing and measurement of joint/muscle circumference.
- 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.
- 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.
- Cover the entire involved area with ice.
- Time: 5 minutes with ice on; 10 minutes with ice off (Repeat this until ice has melted)
- This procedure should be repeated as often as necessary. In a severe bleed, repeat every 2 hours.
- 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.
- Note down the measurement at each treatment.
- 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.
- Rest the joint muscle as straight as comfortably possible.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
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.
- 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|
|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|
|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
Fiona Semple, Physiotherapist, Johannesburg General Hospital
Physiotherapy Department, Steve Biko Academic Hospital
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 elevation of the arm
2. Elevation against gravity
3. Shoulder and elbow extension with resistance (ball or weights)
4. Shoulder and elbow extension with resistance rubber band / tubing
5. Wrists, elbow and shoulder with body weight as resistance
1. Free elbow flexion against gravity
2. Free elbow extension against gravity
3. Elbow flexion with resistance (weights)
4. Elbow extension with resistance (weights)
1. Free supination (turning palm up) and pronation (turning palm down)
2. Pronation and supination using a ruler or a stick as leverage
3. Stretching of muscle that bend the wrist and fingers
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
2. Stretching ilio-psoas muscle with assistance
3. Stretching ilio-psoas muscle
4. Hip flexion against gravity
5. Hip flexion against resistance (weights)
6. Free hip abduction (away from the centre)
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)Pull kneecap – a
Push knee down onto bed – b
Pull toes up – c
2. Knee extension against gravity with a small 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
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
2. Knee flexion against gravity
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