Exercise plays an important role for anyone in maintaining a strong and supple body and, for people with haemophilia, when this is combined with specialist physiotherapy it can help prevent chronic joint and muscle problems. This is vital to anyone with haemophilia, as they are more likely to have periods of enforced rest or immobilisation due to bleeds. Therefore, there is an emphasis on the need to recover well, not just from the bleed but from the loss of muscle tone, which in some muscles will start to break down after only 24 hours of bed rest.
The key for those with haemophilia is to make sure they get good, individualised advice from an expert. A physiotherapist who specialises in haemophilia can give advice on what to do to maintain or improve health and fitness regardless of a person’s age or their joint problems. They can tailor an exercise programme that will maximise activity without doing damage to problem joints or muscles. Specialist physiotherapists also play an essential role in giving advice and prescribing exercises or specific treatment, such as a hydrotherapy pool or gym, to speed up recovery from bleeds and to help protect the joints and muscle from the effects of a bleed.
If you do have a bleeding episode, the PRICE or RICE regime is very useful. The letters stand for:
Protection – try to take the weight off your joints, perhaps by using a sling or crutches Rest – this helps the healing process. Try not to use the injured joint or muscle too much.
Ice – ice can help with pain and reduce swelling and bleeding. Wrap an icepack in a towel and place over the affected area for 10 to 15 minutes. Do not leave the ice pack on for more than 20 minutes, or repeat it more often than every two hours, or put the ice pack directly on your skin.
Compression – sometimes an elasticated bandage can help reduce swelling
Elevation – this can help reduce swelling by draining the blood away from the affected joint or muscle. If it is your ankle or knee, you could lie on the sofa with your leg on some cushions. If it is your elbow or hand, you could rest it along the back of the sofa or put it in a sling.
But these techniques are really only first aid in preventing a bleed from progressing too quickly, by slowing down or reducing the amount of blood that leaks into a joint or muscle and need to be used alongside factor replacement or other treatment to stop the actual bleeding.
After the acute phase of the bleed has ended, getting the joint or muscle moving again is critical. Especially important is striking the right balance of doing enough to restore physical movement and not overdoing it and risk re-bleeding. This is best achieved with advice from a specialist at your Centre and ideally a specialist physiotherapist – they have a very good understanding of body movement and the healing process.
The role of a Haemophilia Physiotherapist is essentially divided into 3 areas, Fiona Hall, Haemophilia Physiotherapist at Cardiff explains:
Following bleeds and/or injuries, where the musculo-skeletal system is involved, (bleeds into joints and muscles form the majority, around 85% of the problems that we see.) physiotherapy is essential to help to restore the joint or muscle to its pre- bleed status, and to help reduce the long-term consequences of bleeding. Therefore, restoration of Range of Movement, muscle strength length and tone, and restoration of balance and coordination are essential for aiding recovery and helping to prevent recurrent bleeds due to weakness and inadequate rehabilitation. This treatment needs to be appropriately timed, so that patients do not have to wait on waiting lists for physiotherapy, this could result in further muscle wasting, weakness and recurrent bleeding, not to mention the possible increase in use of blood products due to delay and further bleeding.
On-going monitoring / musculoskeletal surveillance
Patients are seen on a regular basis for the physiotherapist to carry out a full and comprehensive Musculo-skeletal review on all severe and moderately affected patients. These assessments follow the H.C.P.A. (Haemophilia Chartered Physiotherapist’s) guidelines, and form part of the recognised level of care to be received by these patients according to National Service Specification for Haemophilia and related disorders and the recognised Care Pathway. As a result of carrying out these assessments and discussion with multidisciplinary team, appropriate referrals to Orthopaedic clinics can be made. Due to multiple joint arthropathies and the musculoskeletal nature of the problems experienced by this group of patients, many of them are routinely referred to an Orthotic or Podiatry service in their own area. This is initially for assessment and then provision of specialist insoles or alteration to foot wear.
Preparation of patients with inherited bleeding disorders for Orthopaedic surgery
This involves standard pre and post-operative management of the patient, but with the specialist understanding of the complications of operating on such patients. Close liaison with the Therapists managing the patients on- site (usually Llandough), and liaising with other members of the MDT is essential, as factor replacement, pain management and post-operative protocols are complex and differ with each patients. To this end, we have developed close links with Orthopaedic surgeons and their teams involved in the most common areas of joint surgery undertaken in Haemophilia care, i.e. Total knee replacements and revision surgery, Ankle Arthroscopy and Arthrodesis, and Total hip replacement.
It is usual in Cardiff for the Physiotherapist to accompany the patient to Orthopaedic clinics where possible, and to help to keep the close liaison between the teams and the patients right through to final rehabilitation and recovery to ensure continuity, optimum care and outcome.