The most common complication of hemophilia is joint disease. A joint is an area where two bones come together. People with hemophilia can bleed into the joint space after an injury or, at times, without obvious cause. The pressure of blood filling the joint cavity causes significant pain and can lead to chronic swelling and deformity. Joint damage can occur after repeated bleeding into the same joint or after one serious joint bleed. The joints typically affected include the elbows, ankles and knees.
How does joint damage occur?
Joint damage in people with a bleeding disorder is similar to joint damage of a person with arthritis. The damage occurs in the synovium and the cartilage around the bones.
The synovium is a lining that lubricates and feeds the joint; it also removes fluid and debris from the joint. There are blood vessels in the synovium and that is why bleeding into the joints is common in people with a bleeding disorder. One of the synovium’s functions is to remove fluid from the joint. When there is blood in the joint, the synovium absorbs it. Blood has iron and it is believed that the iron in the blood causes the the lining to get thicker. As the synovium gets thicker, it contains more blood vessels and therefore subsequent bleeding is more likely.
Joints have two types of cartilage, cartilage around the ends of the bones and cartilage to absorb shock. The cartilage affected when bleeding occurs is the cartilage around the bone area. This cartilage is a smooth surface on the ends of the bones and allows the two bones connecting in that joint to move without friction on each other. When bleeding occurs, enzymes from the swollen synovium destroy the cartilage that covers the ends of the bones. As the cartilage erodes and becomes pitted, the rubbing of bone on bone is very painful. The cartilage that absorbs shock in a joint is usually not affected by bleeding, but rather by injuries in sports or other physical activities.
As described, joint bleeding can become more likely even after one joint bleed. Joints that bleed often are usually referred to as “target joints”. As joint damage progresses, movement may become restricted in that joint.
What are the symptoms of a joint bleed?
- Tingling inside the joint
- Loss of motion
Small children may not be able to describe the symptoms above. Below are some signs to watch for in small children:
- Favoring a limb – a baby may hold bottle with opposite hand than usual, toddler may use opposite hand to eat
- Refusing to walk – child may not want to move or may walk trying to avoid bearing weight on the affected leg
What should be done when a joint bleed is suspected?
It is very important not to ignore the signs of a joint bleed. Early treatment with factor concentrate is crucial to reduce the risk of joint damage. If you suspect a joint bleed, call your doctor immediately to avoid complications.
In addition to factor treatment, the following can help a bleeding joint feel better and minimize damage:
- Apply ice to the area
- Rest the joint
- Elevate the limb
What is the treatment for damaged joints?
Over time, joints can become severely damaged and the person suffers from acute pain and restricted range of motion in that joint. Surgery can be effective in managing pain and improving movement of the joint. It is important to note that a hemotologist must be involved in the planning of all invasive procedures to ensure proper clotting levels during and after surgery. The following are common surgical procedures used to aleviate pain and improve function of damaged joints:
A syncovectomy is the removal of the synovium. Removing the synovium stops the bleeding cycle caused by the thick synovium. The procedure doesn’t make the joint “like new”, but it helps aleviate pain and improve function of the joint. There are three synovectomy techniques used:
- Radioactive – A radioactive fluid is injected into the joint, which reduces the swelling in the synovial membrane.
- Arthroscopic – The synovium is removed through surgical incisions in the area. A small camera is inserted into the joint to help guide the removal of the synovium.
- Open – The joint is opened surgically and the synovium removed.
Joint replacement is a surgical procedure that helps people who suffer from chronic pain that interferes with their daily activities. During this surgical procedure, the damaged joint and bone are removed and replaced with plastic and metal components. Joint replacement surgery is more common on knee and hip joints than on elbows, shoulders and ankles. After surgery and physiotherapy, most people are left with a pain-free joint and improved range of motion. Replacement of the artificial joint is sometimes necessary as it can wear out or become loose. Ninety percent of hip and knee replacements should last 10 years.