‘Joint damage’ is the damage caused by the repeated bleeding in and around the joint cavity. Permanent damage can be caused by one serious joint bleed (hemarthrosis).
Normally, however, the damage is the result of many bleeds into the same joint over a period of years. The greater the number of bleeds, and the more serious the bleeding, the greater the damage. Doctors call joint damage in hemophilia hemophilic arthropathy.
The damage to the joint of a hemophiliac is similar to the damage to the joint of a person with arthritis.
It occurs in two places in the joint: the synovium and the cartilage. The synovium is a thin lining on the inside of the joint. It has three functions:
- to lubricate the joint
- to feed the cartilage
- to remove fluid and debris from the joint.
There are a very large number of blood vessels in the synovium. This is one of the reasons why joint bleeding is so common in hemophilia.
When there is bleeding in the joint, the synovium absorbs the blood in an attempt to remove it. The iron in the blood accumulates in the synovium. Doctors think the iron causes the synovial lining to get thicker. The thicker the synovium is, the more blood vessels it contains. This, in turn, makes subsequent bleeding more likely.
There are two types of cartilage. The most important is the one which forms a smooth hard cap on the ends of the bones which form the joint. They allow the bones to rub and glide over each other without friction. This cartilage gets eaten way by enzymes from the swollen synovium. The other type of cartilage, like a shock absorber, is only found in the knees, and is often injured in sports. It is not so important in hemophilic joint damage. In this way, the joint bleeding becomes a vicious circle. As the joint is damaged, bleeding happens more often. Damage gets worse and worse. As well as damage to the joint itself, there is often shrinkage of the soft tissues, tendons and ligaments around the joint so that the person loses some of the range of movement in the joint.
Almost all severe adult hemophiliacs in Canada suffer from arthropathy in one or more joints. Many have arthropathies in knees, ankles and elbows. The older the hemophiliac, the greater the chance he suffers from multiple severe arthropathies. This is because hemophilia treatment was less advanced when these adults were children – the joint damage began then.
Happily, many children today are growing up with normal or nearly normal joints. The use of prophylactic treatment (factor replacement therapy several times a week to prevent bleeding) has improved their chances of reaching adulthood without the development of arthropathies.
Mild and moderate hemophiliacs suffer fewer joint bleeds than severe hemophiliacs. As a result, they suffer less from hemophilic arthropathies. However, as stated above, joint damage can begin after one serious bleed. Therefore, it is just as important to prevent these joint-damaging bleeds in mild and moderate hemophiliacs as it is in severe hemophiliacs.
The joints most often damaged by bleeding are the hinge joints. These are the:
These hinge joints have little protection from side-to-side stresses. As a result they bleed more often.
The ball-and-socket joints, which are better supported, bleed less often. These are the:
Joints in the wrist, hand and foot bleed occasionally. However, these bleeds do not often lead to serious joint damage.
Joint damage can affect a hemophiliac in his daily activities in a number of ways. In general, joint damage leads to:
- repeated bleeding into the same joint
- a loss of range of motion in the joint (the joint cannot be fully extended nor flexed)
- a loss of strength in the muscles around the joint
- pain when using the joint
- pain even when the joint is at rest.
When the damage is in the knee or ankle, and depending on the severity, a hemophiliac may:
- be unable to ride a bicycle, run or dance
- be unable to walk without pain
- be unable to carry heavy loads
- be unable to kneel
- have trouble going up, and especially down, stairs
- have difficulty getting in and out of a car
- need to use a cane or crutches
- need to get around in a wheelchair.
When the damage is in the elbow, and depending on the severity, a hemophiliac may:
- be unable to carry heavy loads
- be unable to do up shirt buttons, or tie a tie
- have difficulty shaving, or eating
- have pain writing.
It is much easier to prevent joint damage than to repair it after it has happened. In fact, once a joint is damaged, doctors may be able to slow down or stop additional damage, but they cannot make the joint like new.
The only way to completely prevent joint damage is to prevent bleeds into the joints. This is done with prophylactic factor replacement therapy.
When bleeds do occur, they need to be treated immediately with factor concentrates. This will stop the bleeding and limit the amount of blood in the joint.
Other things can also be done to limit the permanent effects of a bleed. These are:
- rest (to allow the bleeding to stop)
- elevation of the limb (to lower the blood pressure and allow the blood to drain away)
- non-steroidal anti-inflammatory drugs (to reduce swelling)
- electrical stimulation of the muscles around the joint (to avoid wasting of the muscles while the joint is recovering)
- active physiotherapy (to regain movement in the joint and avoid the formation of fibrous build-ups in the joint).
When a hemophiliac has repeated bleeds into the same joint, he and his doctor may decide that prophylactic treatment for a period of three to six months is necessary. The goal is to break the vicious cycle of bleeding and allow the synovium to return to normal. If this does not work, other treatments will be necessary.
Orthopedic interventions can be very effective in managing pain and improving 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. The hemophilia 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.
One treatment for damaged joints is called synovectomy. This is the removal of the synovium. Its main goal is to reduce the number of bleeding episodes and break the vicious cycle of hemorrhage and joint damage. After removal, the synovium grows back, but it is no longer thick and engorged with blood vessels, as it was before.
Three techniques can be used to remove swollen synovia:
A radioactive isotope, such as 32P or 90Yttrium is injected into a target joint, usually under flouroscopic guidance in the radiology department. 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.
Using small surgical incisions a tiny camera is inserted into a joint to guide the removal of the synovium through the other incisions. 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.
Under a general anesthetic, the joint is opened surgically and the synovium removed. Physiotherapy will be necessary for at least 4 weeks.
None of these operations makes the joint like new. Nor do they restore the range of motion which has already been lost. However, they do have some major benefits:
- pain is reduced
- the joint is more functional (walking is easier in the case of a knee or ankle)
- the number of bleeds is reduced
- the degeneration of the joint is stopped or slowed.
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-hemophiliac 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 hemophilia 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 10-14 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 replacement.
Ninety percent of hip and knee replacements should last 10 years. 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.
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. Transfusion with blood products may be necessary. Most hospitals performing joint replacements have autologous blood donation programs for patients to store their own blood preoperatively in case a transfusion is needed. Alternatively, blood products from anonymous donors can be used.
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.
Other surgeries might be considered to manage pain from damaged joints. These are:
- Removal of small bony growths around the joint margins (cheilectomy)
- Fusion of the joint to leave a painless immobile joint (arthrodesis)
- Removal of the radial head to improve rotation of the forearm
- Removal of the ball part of the femur to allow a fibrous union to develop. 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).