Joint Disease
Many adults with severe hemophilia have permanent damage in one or more joints, which is caused by bleeding into the joint cavity. In some cases, joint damage can begin after only one major joint bleed. More often, however, joint disease is the result of many bleeds over a period of years. Joint disease is a serious complication that can lead to a loss of range of motion, muscle atrophy (weakening), pain, and limited mobility.
Some joints are more likely to be affected by bleeds than others, such as knees, ankles and elbows because they are hinge joints and have little protection from side-to-side stresses. Ball-and-socket joints like the shoulder and the hip are well protected by large muscles, and are designed to move in many directions without being injured.
What Is a Target Joint?
When one joint bleeds over and over, or when it bleeds more than all the other joints, it is called a target joint. Target joints are more susceptible to joint disease.
Joint disease can affect two parts of the joint: the synovium and the cartilage. Inflammation of the synovium is called synovitis. Erosion and damage to the cartilage is called arthritis.
What Is Synovitis?
The insides of joints are covered by a thin lining called the synovium or synovial membrane. It has many blood vessels that lubricate the joint, feed the cartilage, and carry debris away. The synovium is easily torn, which results in a bleed into the joint. Once a joint has had a bleed, it may take several weeks for the blood to be removed completely and the joint may be vulnerable to repeat bleeds. If there have been many bleeds in a short period of time, the synovium will become thicker and inflamed, and the joint will grow new blood vessels to help with the clean-up. This is called synovitis.
Synovitis looks and feels different from a single, acute bleed. The joint looks "puffy" or swollen all the time, and the swelling does not go away after a factor treatment infusion. If the joint has become permanently stretched from chronic swelling, it may not be painful. While a bleeding joint will quickly lose motion as the blood fills the joint cavity, joints that are swollen from synovitis usually continue to move without pain. However, they may lose motion over months or years.
What Is Arthritis?
Arthritis means "inflammation of the joint." In addition to inflammation of the synovial lining, there is also damage to the cartilage and bones of the joint surfaces. There are two types of cartilage in joints. The most important is the one that forms the smooth hard cap on the ends of the bones and allows the joints to move with very little friction. When a lot of blood accumulates inside a joint, after either a very large bleed or repeated bleeds, the old blood and the fluid that the synovium makes to try to heal itself begin to eat away at the cartilage. Some of the symptoms of arthritis include stiffness in a joint, pain, and muscle weakness that may affect movement such as walking or climbing stairs.
Preventing Joint Damage by Managing Bleeding
The best way to prevent joint damage is to treat bleeds immediately with clotting factor to stop the bleeding. Proper rest is necessary, and follow up infusions may be needed, to ensure the joint's proper healing after a bleed.
It's also possible to try to prevent bleeds from happening in the first place. Clotting factor can be given before bleeding actually occurs as a preventative measure—this is called prophylaxis, or prophylactic treatment. People with severe hemophilia may receive prophylactic factor infusions on a regular basis, like two to three times per week, to prevent bleeding from starting. People with moderate hemophilia may receive factor concentrate before they participate in sports or other activities which may place stress on joints and cause bleeds. Be sure to talk to your doctor or Hemophilia Treatment Centre for guidance on managing bleeds and preventing joint damage.
Other Treatment Options
In addition to treating bleeds, physiotherapy can be a helpful adjunct to keeping joints and muscles protected. Physiotherapy can help to strengthen muscles around the joints so they bleed less frequently, as well as identify movements and positions to help prevent further injury. Because each patient's situation is unique, a licensed physiotherapist who has experience treating people with hemophilia should prescribe specific exercises based on a detailed assessment of the patient's condition. Your doctor or Hemophilia Treatment Centre can help you find a suitable therapist.
In some cases, joint disease can progress so far that it becomes painful or difficult to walk or do other daily activities. When this happens, it may be necessary to undergo one or more kinds of joint surgery:
- Joint replacement is the surgical removal of a damaged joint and insertion of a new, artificial joint.
- Joint fusion uses special surgical tools to help mend the damaged section of a joint. This procedure fuses, or attaches, two bones on each end of a joint, eliminating the joint itself. Joint fusion helps a joint to regain its stability, bear weight better, as well as removing the pain.
Both procedures are performed by an orthopedic surgery, working closely with a hematologist to ensure adequate factor treatment is infused to manage bleeding during and after surgery. After surgery, physiotherapy helps the patient strengthen the surrounding muscles and regain motion in the joint.