It may be necessary to use appropriate pain relief for chronic pain caused by arthropathy. Options that are often prescribed for people with haemophilia are:
- Paracetamol (used cautiously in patients with concomitant liver disease).
- COX-2 inhibitors (these are an anti-inflammatory drug that has minimal effect on platelet function).
Note: Paracetamol and COX-2 inhibitors can cause bleeding in the stomach and it is important that their use is monitored carefully by your treatment centre.
Other methods of pain relief, such as rest-ice-compression-elevation (RICE), splinting and walking aids (e.g. crutches, canes, orthotics, etc.) are reliable and can provide some help when carrying out daily activities. Also, light daily exercise and physiotherapy are important with joints that have severe or repeated bleeds.
Certain drugs affect the way platelets plug holes in blood vessels. A person with haemophilia should never take drugs containing:
- Aspirin (ASA) and any drugs containing aspirin
- Non-steroidal anti-inflammatory drugs (e.g. ibuprofen)
Orthopedic interventions can be very effective in managing pain and restoring joint function. Acute pain from recurrent bleeding into target joints can be helped by procedures such as synovectomy. Chronic pain from an irrevocably damaged joint can be relieved by procedures such as joint replacement. All invasive procedures must be performed under the protection of factor replacement, even for those on emicizumab prophhylaxis. The haemophilia doctor must be involved to ensure that adequate levels of replacement are provided for the appropriate time post-operatively. Factor replacement may be recommended prior to post-operative physiotherapy sessions.
Three techniques can be used to remove a swollen synovium:
- Radioactive synovectomy: A radioactive isotope, such as 32P or 90Yttrium is injected into a target joint, usually under flouroscopic guidance in the radiology Within the joint, the radioactivity reduces the amount of swollen synovium. This technique has not been shown to increase the risk of developing cancer although this is a theoretical risk.
- Arthroscopic synovectomy: Using small surgical incisions a tiny camera is inserted into a joint to guide the removal of the synovium through the other This is usually done under general anesthetic and can be used for ankles, knees and elbows. Physiotherapy may be necessary post-arthroscopy for 2 to 4 weeks.
- Open synovectomy: Under a general anesthetic, the joint is opened surgically and the synovium removed. Physiotherapy will be necessary for at least 4 weeks.
Chronic joint damage produces pain and decreased range of motion. When the pain is severe and interferes with the activities of daily living, joint replacement is an option. Knee and hip replacements are the most common. Elbow, shoulder and ankle replacements are done less commonly due to the complexity of the joints. Newer techniques and materials are expanding indications.
The damaged joint and adjacent bone are removed and replaced with plastic and metal components (knee) or with a metal ball and a plastic cup (hip). Factor replacement is extremely important, as this can be a bloody surgery even in non-haemophiliac patients. Clotting factor levels are kept at 100%, usually by continuous intravenous infusion, for 10 days or more. Specific management must be done by the haemophilia doctor.
Pain control is critical during the recovery period so that early mobilization and physiotherapy can occur. Most patients are walking within 2 days (hip and knee) and are discharged within 7-14 days. Improvement continues for up to 6 months.
Most people are left with a pain-free joint. Range of motion usually is better with hip than with knee replacements. Ninety percent of hip and knee replacements should last 10 years, and even longer in people who are not running or jumping. Replacement of the artificial joint is sometimes necessary as the artificial joint can wear out or become loose. The success rate is usually not as good as for first time replacements.
Risks associated with joint replacement
There are very low risks associated with general anesthetic. Your anesthetist can best assess these. Intra-operative and post-operative bleeding should be limited by factor replacement. In cases where there is significant blood loss, transfusion with red blood cells may be necessary after surgery. Blood products are extremely safe these days. Infection may complicate surgery. This may be superficial or in the deep tissue and bone. Infection may occur early or develop weeks or months after surgery. Infection requires antibiotic therapy usually by intravenous route and in hospital. An infection may not clear up until the artificial joint is removed. The new joint may dislocate. The components may become loose. If the joint fails, the surgeon may need to perform further surgery.
Joint fusion (arthrodesis)
This operation is often performed on the ankle when chronic pain makes walking painful. Pins are inserted into the ankle so that one or more of the ankle joints is stopped from bending. This is very effective in reducing and even eliminating pain. In many cases, the joint damage had already caused the ankle to lose almost all flexion, so a person walks just as well after the surgery as before, minus the pain. This surgery can be performed arthroscopically in some cases, so is much less invasive than a joint replacement. However, the ankle needs to be completely immobilized for several weeks, with no weight-bearing, so the ankle bones can permanently fuse.
Other surgeries to help manage pain
Other surgeries might be considered to manage pain from damaged joints. These are:
- Removal of small bony growths around the joint margins (cheilectomy).
- Removal of the radial head in the elbow to improve rotation of the forearm.
- Removal of the ball part of the femur to allow a fibrous union to This may be done if a hip replacement fails (Girdlestones Procedure).
- Removal of a wedge of bone from the femur or tibia to realign the leg and reduce pain (osteotomy).
Less invasive options for managing pain
Injection of a corticosteroid, e.g. methylprednisolone, into an affected joint can be used in the short to medium term to decrease inflammation and resultant pain. This could be used while awaiting surgery.