Hemophilia

Overview of Hemophilia

Hemophilia is a bleeding disorder caused by abnormal plasma-clotting proteins. Clients with hemophilia have prolonged bleeding times and the risk of internal hemorrhaging into joints and muscles. This disorder is classified by the client's percentage of clotting factors: mild is 5% to 50%; moderate is 1% to 5%; severe is <1%. Patients with moderate and severe hemophilia will need factor replacement therapy to control and prevent excessive bleeding. Individuals with hemophilia may develop limited endurance from a combination of factors that include repeated joint bleeds, pain and limited joint mobility, muscle atrophy, and fear of injury. Limited endurance is associated with decreased muscle strength, joint stability, and proprioception.^1,2

Comorbidities to Consider

• Clients may contract hepatitis C from blood transfusions and develop liver disease.

Client Examination

Keys to Examination of Clients

• The stages of joint degeneration secondary to joint bleeds are determined by radiographs of joints using the Arnold-Hilgartner and Pettersson scales.³

• Discuss with clients if they have developed "target joints" from repeated bleeding episodes, which will need to be assessed carefully for mobility and signs of synovitis.

Arnold-Hilgartner Criteria for Stages of Hemophilic Arthropathy

0: Normal joint

1: Soft-tissue swelling, no skeletal abnormalities

2: Osteopenia and overgrowth of epiphysis, normal joint space

3: Changes in osseous contours, formation of chondral cysts

4: Narrowing of joint space, signs of articular cartilage destructions

5: Substantial disorganization of joint structures

Recommended Baseline Testing of Fitness Levels

• Aerobic fitness testing can be performed with walking or cycle ergometry methods.^4,5

• Assess for client risk of falling and the ability to control movements without risking overstressing joints and muscle groups.

• Client may need to take clotting factor replacement therapy before engaging in testing and new exercise programs.⁶

Exercise Prescription

Type: Recreational and fitness activities⁴

Intensity: Moderate intensities, 60%–80% of peak heart rate.

Duration: 30–60 minutes

Frequency: Three to five times per week

Getting Started

Individuals with hemophilia need to choose safe and appropriate activities that will not place them at risk for recurrent joint and muscle bleeds.⁴ Young people can be encouraged to participate in recreational and competitive swimming as these activities pose minimal risk for bleeding episodes, provide social interactions, and can be performed for a lifetime.² Some young people with hemophilia may rebel against restrictions on their participation in sports and recreational activities.⁵

Resistive exercise should be started at low levels with an emphasis on endurance activities of 15 to 20 ...