Why is joint disease a common complication in adults with hemophilia? Because there are many blood vessels in the synovium, or the lining of joints. The synovium is easily torn, which results in a bleed into the joint. When a lot of blood accumulates inside a joint, the synovium absorbs it. Iron from the blood builds up in the synovium, which thickens it and causes more blood vessels to form. This “thickening” is called synovitis, or joint swelling. One bleed makes another episode of bleeding more likely – starting a vicious cycle of joint damage.
Joint disease is much like arthritis: the joint, soft tissue, tendons and ligaments are damaged, and the damage gets worse over time. The result is pain, decreased range of motion, limited mobility and muscle atrophy (weakening). Because joint damage is progressive – the symptoms get worse as a person with hemophilia ages.
Some joints are more likely to be affected by bleeds than others, such as the 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, so bleed less often.
The lasting effects of joint disease
Joint disease, and the chronic pain that comes with it, can seriously impact a person’s quality of life. People may feel as though they can’t enjoy the same activities they used to, like riding a bike, going for a walk or doing everyday chores. Some people with more severe damage may need assistive devices. It’s important to talk to your doctor and HTC team about ways you can help prevent joint damage.
Preventing joint disease by managing bleeds
The most effective way to prevent joint damage is to treat bleeds immediately with clotting factor to stop the bleeding. Proper rest is also necessary, and follow up infusions may be needed, to help ensure that the joint properly heals.
It’s also possible to try to prevent bleeds from happening in the first place with regular prophylaxis therapy. People with moderate or severe hemophilia may receive factor concentrate, if they are a candidate for therapy, before they participate in sports or other activities which may place stress on joints and increase the risk of bleeds.
There are helpful tools available that can show you the impact nonadherence to a prophylaxis regimen can have on your joints. Talk to your doctor for more information about these tools.
Other ways to manage joint disease
In addition to treating bleeds, physiotherapy can be beneficial to keeping joints and muscles protected. Physiotherapy can help to strengthen muscles around the joints so they bleed less frequently, as well as 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. If you don’t already have one, your doctor or HTC can help you find a suitable therapist.
In some cases, joint disease progresses 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, including:
- Joint replacement, or the surgical removal of a damaged joint and insertion of a new, artificial joint.
- Joint fusion, which 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, and lessen pain.
Both procedures are performed by an orthopedic surgeon who works closely with a hematologist to manage bleeding during and after surgery. After surgery, physiotherapy is needed to help a patient strengthen the surrounding muscles and regain motion in the joint.
There may be other options your doctor recommends to help manage joint pain.
How to identify a Joint Bleed: QUICK TIPS
- A tingling, bubbling sensation (but no real pain)
- Tightness and pain (but no visible signs)
- Swelling at the joint and a hot feeling when the skin is touched
- Pain when bending or extending the joint
- Worsening swelling, and difficulty moving
REMEMBER: The earlier you treat a joint bleed, the better your chances of preventing serious damage.
Learning how to take care of yourself in the face of age-related issues is just as important to people with hemophilia as it is to the rest of the population.
Learn more about aging with hemophilia