Most adults with moderate to severe haemophilia never forget the feeling: a painless but distinct sensation, a tingling within a joint... the first inkling that a joint bleed has begun its predictable and potentially destructive course.

If this episode of joint bleeding happens to be among the first, joint function will most likely return to near normal afterwards. But unfortunately, once one joint bleed occurs, others tend to follow. It's as if the first episode predisposes the joint to future ones. And repeated episodes of bleeding into the joints can lead to advanced degenerative arthropathy.

In an ideal world where factor replacement was started the minute a joint bleed was detected, this downhill course toward joint deformity could be avoided. And, indeed, the percentage of people with haemophilia who reach an advanced stage of joint dysfunction is diminishing. But even with today's norm of early, effective treatment/prevention of joint bleeds, joint haemorrhage remains the most common bleeding manifestation of haemophilia A and B.

Eventually independent daily living is threatened by these problems:

• pain
• loss of range of motion
• muscle wasting and weakness
• changes in movement patterns of the arms and legs
• abnormal gait or walking pattern

In many cases, the problems can be handled by conservative treatment. This includes factor replacement as needed, therapeutic exercises, medication to relieve pain and inflammation, and restriction of activity. For some other people with haemophilia, though, the problem becomes more severe even with these measures. The person reaches his thirties or forties facing considerable incapacity and disabling pain. This is when joint replacement surgery may be necessary to improve quality of life.

As orthopaedic surgeon Thomas Sculco of the Hospital for Special surgery in New York explains, "Pain is the greatest determinant. When we see extreme pain coupled with stiffness, deformity, loss of function, and x-ray confirmation that cartilage is destroyed, we consider joint replacement." Sandy Ganz, PT, physical therapist in the Rehabilitation Services Department of the same hospital, has helped many people with haemophilia through the recovery period after joint replacement. She shares the generally held view that surgery should be done "only when there is disabling pain that doesn't respond any longer to medical treatment." She adds that "people shouldn't seek joint replacement just to improve gait or reduce joint deformity."

John Stewart (not his real name), a 27-year-old with severe haemophilia, suffered terrible pain in his left knee because the cartilage was destroyed. Whenever he tried to exercise, he was incapacitated for several days afterward. At first, when he asked about the possibility of knee replacement surgery, doctors told Stewart that the surgery was more appropriate for older people, that for now he should continue his regimen of therapeutic exercises and medication to relieve pain. His reaction: "When I'm older, I'll probably be slowing down anyway. Now is the time that I need to be most active." Ultimately his doctors agreed and the procedure was done. Although Stewart was younger than most patients who undergo joint replacement surgery, his medical history and level of motivation made him a prime candidate for the operation.

WHICH JOINTS ARE AFFECTED AND WHY?

The joints that most often suffer frequent bleeds and consequent arthritic deterioration are hinge-type joints such as the knee, elbow and ankle. These joints are more susceptible to bleeding and subsequent joint damage for several reasons. They have a relatively large amount of synovium, the membrane that lines the capsule in which the joint resides. The more synovium there is in a joint, the more "rubbing" there is within the joint as it moves. These joints also lack a protective muscle coverage and aren't as able to withstand the stresses of rotary and angulatory motion. Socket-type joints like the hip and shoulder are involved with moderate frequency. Wrists, fingers, toes and vertebral joints are only rarely affected.

Once bleeding into a joint becomes a recurrent event, joint damage progresses through three stages:

Acute Stage

• the 24-48 hours following a bleeding episode

Subacute Stage

• joint may be mildly swollen and warm, slightly stiff and may lack full range of motion
• muscle wasting may be present

Chronic Stage

• joint capsule and its lining are chronically swollen
• progressive loss of joint motion, increased pain, joint deformity
• chronically boggy swelling not relieved by factor replacement
• if target joint is a knee or ankle, person may walk with a limp
• if target joint is an elbow, activities of daily living such as driving a car or combing hair may be difficult

WHO ARE LIKELY CANDIDATES?

Joint replacement surgery is never a simple undertaking in someone with haemophilia; it requires aggressive factor replacement pre- and postsurgery, a long hospital stay, and considerable rehabilitation.

The best candidates are those who are like John Stewart -- basically healthy, motivated and mentally ready for the discomfort and demands that will be placed on them by the surgery and the intensive postoperative physical therapy and rehabilitation. They must have already exhausted the appropriate nonsurgical methods of preserving joint function and mobility, and must be patient enough to work through an extended recovery period. Looking back on his own experience, the young man points out: "You need to be motivated to make it a success, because it requires a lot of work to build up strength. What you get out of it depends on how much you put in."

Since aggressive factor replacement is necessary to prevent excessive bleeding during surgery, people with factor inhibitors are not generally considered good candidates for replacement surgery. Especially if a person with inhibitors is a high-level responder, it may be impossible to give enough factor to control bleeding during and after surgery.

People who are HIV positive may also be candidates for joint replacement surgery. In these individuals, as in others, the decision is based on the risk versus the benefit they are likely to get from the operation. As always, no decision is made without a comprehensive team evaluation and multiple discussion among team members and with the candidate and his family.

Finally, the cost of joint replacement surgery is significant; a person must have insurance that covers some or all of the cost, or he or his family must be able to pay.

WHERE SHOULD THE SURGERY BE DONE?

A recent textbook describes total knee replacement in a person with haemophilia as a "technically formidable" procedure. Dr. Sculco points out that such a procedure should be done only at an institution where orthopaedic surgeons have done many such replacements and every member of the treatment team has experience with the special demands of haemophilia.

Sandy Ganz, PT, whose institution does joint replacement on patients with haemophilia from all over the United States and the world, believes that it is not just the experience of the surgeon that is critical. "A haemophilic total joint is very different from the average total joint that you work with in physical therapy," she points out. "At a centre, the physical therapist is an experienced member of a team that includes the orthopaedic surgeon and the haematologist. It's not uncommon for a joint bleed to occur during physical therapy. Only a therapist with special experience knows what to look for and what to do."

SUCCESS RATES

Overnight miracles do not occur, as Stewart is quick to point out. "It took about a year of rehabilitation before I stopped being concerned about my knee all the time," he recalled. "At first I could just exercise a little at a time. Eventually I worked up to walking four or five miles." The "ultimate achievement" was when he recently walked a full eight miles in a fund-raising event. Dr. Sculco adds that complications, including infections, are also a possibility. Some patients have circulating anti-coagulants and continue to bleed into the muscle despite surgery.

In general, the goals by which health care providers and patients measure the success of joint replacement surgery in haemophilia are as follows:

1. Decrease in pain at the affected joint
2. Decrease in pain and stress at joints surrounding the affected joint
3. Decrease in frequency and number of joint bleeds
4. Increase in FUNCTION and mobility

These goals are long-term and should not be assumed that they will be reached quickly. After surgery, a staged exercise and physical therapy program will begin as soon as possible -- before the patient leaves the hospital -- and will become part of an ongoing process of rehabilitation. The overall goal is to make the person able to function independently with little or no disability. Prevention of joint bleeds is optimal, but is not always possible.

The success of total joint replacement depends on many factors, including such things as the type of artificial joint implanted, prior surgery on the joint and the surgeon's experience. The experience of several institutions follows, according to the joint involved.

KNEES

The knee joint is most likely to cause severe pain and disability and is the joint most frequently replaced. Maintaining range of motion presents a particular challenge, which appears to be met better by some of the newer techniques and prosthetic materials in use today. As part of a 20-year (1967-1987) study, 46 knee replacements were done in people with haemophilia at the University of Southern California and the Haemophilia Centre, Orthopaedic Hospital in Los Angeles. These were the reported failure rates:

• Problems with prosthetic implants -- 2,4%
• Need to reoperate (1-14 years after first operation) due to problem with prosthetic implant -- 6,8%

Of 24 total knee replacements done in 14 people with haemophilia at the Hospital for Special surgery in New York, 21 (88%) had a good or excellent result in terms of pain relief and improvement in the joint's range of motion.

A report on total knee replacements done between 1983 and 1992 at a haemophilia centre in England included a comparison of the number of joint bleeds and the factor units required before and after joint replacement. In 10 people with severe haemophilia who had a total of 15 knees replaced, the average number of bleeds in the operated knees fell from ten to two a year; the average yearly amount of factor needed fell from 15,500 units to 2,200 units per knee.

ANKLES

The ankle is a close second to the knee as the joint most frequently involved in destructive joint bleeds. But only a small portion of people who suffer repeated ankle bleeds wind up with joint pain and deformity serious enough to warrant ankle replacement surgery. In an overview of the 20-year California study mentioned above, only one ankle replacement was reported compared with 46 knee replacements. Eleven years after his surgery, the patient who received the ankle replacement was symptom-free, without pain or bleeding into the joint.

HIPS

Hip replacement is moderately frequent in people with haemophilia. Its success can be affected by the fact that PWHs so often have knee and ankle problems as well, giving them a stiff-legged gait. Without the normal shock absorption of the knees and ankles, the hip is put under additional stress. Over time, the increased stress of a stiff-legged gait can cause the artificial hip to loosen. This and other complications can necessitate reoperation.

At the two California hospitals, investigators rated their long-term success with hip replacement as "fair", with reoperation becoming necessary in about 60% of people over the 20-year period. They noted, however, that all these people were doing well, were free of hip pain and able to walk without assistance. They were all doing substantially better than before their initial hip surgery.

A NEW LEASE ON LIFE

Complications to hip replacement surgery can occur, and some, such as infection, can be serious. But thanks to today's advanced surgical techniques and improved artificial joint materials, problems associated with the procedure occur less often than in the past. The overwhelming majority of people with haemophilia who reach the point of needing this surgery function significantly better with their new joint than with their old one. Pain is relieved or reduced, the number of joint bleeds goes down, and they can walk more normally and enjoy life more fully. As Stewart describes it, "Even now, four years after my surgery, every time I take a long walk, I realise it's a very special thing."

For more information about joint replacement, contact your local Haemophilia Treatment Centre

Susan Roessner Dodson and Susan Roher are healthcare writers living in Southampton and Chappaqua, New York.

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