As the causative factor for haemophilic arthropathy is the spontaneous bleeding, the aim of treatment is to prevent the bleeding. his is done by replacement of deficient factor with concentrate of the clotting factors obtained from fresh frozen plasma.

The use of concentrates allows administrationof adequate amounts of factor in a small volume, thereby avoiding the dangers of circulatory overload.

Factor VIII deficiency (hemophilia A and von Willebrand’s disease) the concentrate used contains factor VIII and fibrinogen. Commercially preparedcryoprecipitates are available.

For factor IX deficiency (hemophilia B) the concentrate used contains a high level of factor IX and low levels of factors, II, VII, and X. It is manufactured as the prothrombin complex from human plasma.

Both types of concentrates have the potential for transmission of the infectious virus via the infused blood products. Other associated complications are hepatitis, fever, allergic reactions, headache, abdominal pain, and occasional development of disseminated intravascular coagulation.

The dosage required to replace a factor deficiency depends on the patient’s weight and plasma volume.20 to 30 minutes after administration of the antihemophilic factor the plasma level will rise. The biologic half-life of factor VIII is 6 to 12 hours, whereas that of factor IX is 8 to 18 hours.

In case of the management of bleeding into joints, muscles, and soft tissue, the dose of factor VIII or IX is calculated to raise the plasma level to 30 percent of normal.

In severe hemarthrosis it may be desirable to raise the plasma level to 40 percent of normal.

Body develops inhibitors of factor VIII and IX as a result of the immunologic response.

A low titer of inhibitors may be circumvented by high dosage of factor VIII infusion. Other methods are the administration of prednisone and cyclophosphamide or the use of concentrations of prothrombin-activate material or of plasmaphoresis.

Early Bleeding into Muscles and Soft Tissues

• Self-administration of factor VIII or IX by the hemophiliacs or their parents at home
• The part is splinted in comfortable neutral position in foam pillows or soft appliances.
• Weight-bearing is restricted if lower limbs are involved.
• Gradual mobilization as soon as the acute symptoms of pain and muscle spasm have subsided the affected limb is gradually mobilized under cover of factor replacement.

With early treatment the hemorrhage in the muscles will usually resolve within three to five days. Hemorrhage in the quadriceps femoris and biceps brachii take the longest time to resolve

Acute Hemarthrosis

Note: Delay in adequate treatment is the primary cause of joint deformities in hemophilia.

Acute bleeding into joints should be considered an emergency requiring immediate attention.

Immediate treatment of bleeding into joints results in less arthropathy and minimizes the extent of joint destruction.

Home care therapy permits factor replacement as soon as a bleeding episode takes place. This type of care has following disadvantage

• Inadequate follow-up
• Risk of transmission of hepatitis to a family member
• Increased risk of infection due to lack of appropriate sterile technique

The family should be instructed that in case of severe and there is marked distention of the joint, to bring the child to the hospital immediately.

In case of minimal or moderate Pain is not very severe, and the child continues to bear weight on the affected limb. This causes more bleeding into the joint.

This continues and within a few days the joint will become markedly swollen, very painful and will develop fixed flexion contracture. Initially, in the event of associated bleeding into the peri-articular tissues and muscles, pain and muscle spasm will be marked from the onset; the patient will be apprehensive of moving the limb and will be forced to rest and to seek medical attention.

The affected joint is temporarily immobilized in a position of rest and minimum intraarticular pressure.

A compression helps to create tamponade and reduce further bleeding but distal circulation should be carefully watched.

Under no circumstances should a circular plaster cast be used. The swelling will obstruct the distal circulation and cause gangrene.

The limb should be elevated and Cold compresses are applied over the affected joint. The clotting defect is corrected by administration of antihemophilic factor.

Analgesics

It is best to avoid anlgesics or sedative. NSAIDS are contraindicated due to inhibitory effect on platelets. With opoid drugs as addiction can be a problem with repetitive use. If patient is under effect of analesia he will be unable to get proper warning of continued bleeding.

Decrease of severity of pain is the first indication of cessation of hemorrhage. Monitoring the circumference of the joint at intervals also tells the progress.

However, in case of need opoid drugs may be given but they should be used conservatively. If the pain is intolerable and does not respond to factor replacement and splinting, pain medications that can be given are propoxyphene, acetaminophen, codeine.

Aspiration

The joint should be aspirated and decompressed if there is severe hemarthrosis with marked distention of the joint capsule.

Aspiration of the joint should be performed under strict aseptic conditions in the operating room and under local anesthesia. Following that Factor VIII or IX is administered intravenously. After the aspiration the compression dressing and posterior splint are reapplied. Administration of the factor is continued for three to seven days following cessation of bleeding.

Physiotherapy

After cessation of bleeding, physical therapy to mobilize the joint is initiated. Isometric muscle exercises, gentle active assisted exercises are carried out intermittently. The range of motion of the affected joint is progressively increased. Full weight-bearing is not permitted as necessitated by limitation of joint motion and muscle weakness. Transition to activity be gradual.