Joint Disease

Joint damage (hemophilia arthopathy) is the most common complication of bleeding in hemophilia. Prophylaxis has been shown to reduce joint bleeding and prevent joint damage. Early treatment of each joint bleed reduces the risk of chronic joint disease, functional impairment, and disability. Factor concentrates, home treatment, physical therapy, and orthopedic surgery have contributed to decreased frequency and severity of joint disease among people with hemophilia during the past 25 years. Prevention remains the focus of hemophilia treatment centers (HTCs).

hemophilic knee

The frequency of joint disease has been further reduced as increasing numbers of individuals with severe hemophilia have participated in prophylactic programs. Unfortunately, most of these patients still develop at least one chronic joint in their lifetimes.

Prevention of Chronic Joint Disease

1. Recommend primary prophylaxis
  • Recommended for children to prevent recurrent joint bleeds
  • Initiated before the second or third episode of joint bleeding (hemarthrosis) in cases of severe hemophilia
  • Reduces joint bleeds and risk of chronic joint disease
2. Treat at first sign of hemorrhage

Treatment at the earliest sign of hemorrhage is the most important step in reducing the risk of chronic joint disease. Treatment must be accomplished before pain, swelling and limited motion are observed. The goal is to stop the bleeding before accumulation of blood in the joint space affects the synovium.

Splinting can be used for a short time to immobilize the affected joint during the acute phase of a bleed. In addition, assistive devices such as crutches can be used to rest an affected joint during this period. Patients should be encouraged to return gradually to normal activities.

After a bleed in which pain, swelling, and decreased motion are present, muscle atrophy can occur before activities return to normal. Physical therapy is important to increase muscle strength, range of motion and balance. The physical therapist can help to assess the musculoskeletal system and functional impairments and to address these with the appropriate treatment.

3. Encourage a healthy weight
  • Strong muscles can protect joints from injury.
  • Maintaining a healthy weight could be an important step towards preventing joint disease.
  • Careful monitoring of a patient’s weight and BMI, and routine evaluations by a physical therapist can help a patient develop a plan for overall fitness and maintenance of joint function.
4. Conduct physical assessments

Physical assessment of joint status can be aided through a routine evaluation by the physician, nurse, or physical therapist. Evaluation of joint status includes the following:

  • Patient history
  • Range of motion
  • Joint circumference or muscle girth
  • Muscle strength
  • Joint anatomy
  • X-rays or other diagnostic imaging if any abnormalities are noted
  • Magnetic resonance imaging (MRI) might be indicated in some cases

Bridging the Gap: Implications of Joint Disease

Bridge the gap icon

Many adults, especially older adults, grew up without the benefit of treating their bleeds early and adequately with factor replacement products. As a result, most have target joints and many have significant joint disease. Hemophilia arthropathy can affect patients both physically and emotionally; it can affect their gait, physical abilities, body image, relationships, perceptions of masculinity, feelings of vunerability, and loss of time from school and work. Arthropathy also causes chronic pain. As this population ages, the associated disabilities and pain from joint disease have become pressing concerns for many patients.

Previously, many adults readily disclosed their hemophilia diagnosis. However, when coinfection with viruses became an issue, many adults reconsidered the issue of disclosure. Most adults have shared their diagnosis, and often their associated complications, with friends and family. In terms of disclosing to their employers and coworkers, this has varied based on the individual’s preference, privacy issues, treatment needs, the degree of physical impairment, and past experiences with disclosure in the workplace.

In addition, hemophilia can continue  to have an effect on their dating relationships because it sometimes affects their mobility, appearance, and interests. Disclosure about HIV or HCV status can raise issues about trust, the ability to make plans for the future, and potential sexual transmission of HIV. For married men, there are similarities and differences in potential issues. Sexual transmission and safer sex are major concerns that can have an effect on family planning. For these men and their partners, support in exploring pregnancy alternatives is helpful. Men with HIV or hepatitis can also face concerns about disease progression, and complications of treatment, which can affect relationships. Adults with HCV or HIV, or both, also face issues related to disclosing their diagnosis to their children and often seek guidance with this issue from HTC staff.

As patients cope with multiple diagnoses, such as hemophilia, joint disease, HIV, and hepatitis, considerations for treatment become more complex.

Planning for health care in the event of progressive disability is an important task for an adult, again raising concerns about loss of independence. While some of this responsibility can be assumed by a spouse, partner, or family member if disability progresses, it is important for the adult patient to continue to maintain as much control as possible over his health care.

Adults with hemophilia have considerable concerns about financial stability and the cost of health care. The cost of treating hemophilia and its complications continues to rise in a climate of health insurance changes. Individuals might select jobs or keep unsatisfactory jobs to be able to obtain or retain insurance coverage. Those with insurance face rising co- payments and lifetime limits. Health insurance concerns, coupled with the uncertain ability to continue to work, can be devastating to patients and their families.