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

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.

• 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.
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.
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.

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.

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.

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.

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.

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

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.