South African Guidelines for Physiotherapy in Haemophilia

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.
   

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: 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.

• 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.

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.

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