Hemophilia, an X-linked disorder, is a congenital inherited bleeding disorder of the clotting mechanism cause by an abnormal gene that produces a defective clotting factor protein with little or no clotting ability. The two most common forms of this disorder are Classic hemophilia (type A) which is caused by the lack of factor VIII and Christmas disease (type B) is caused by the deficiency in factor IX. Since both of these disorders are X-linked, the female is the carrier and the disorder is manifested only in males.
Hemophilia is classified into the following three groups, based on the severity of factor deficiency, mild (5-50%), moderate (1-5%) and severe (1%). Hemophiliacs are at risk for prolonged bleeding or hemorrhage as a result of minor trauma. Individuals with severe hemophilia, or less than 1% clotting factor, are also at risk to suffer from spontaneous bleeding without trauma or more severe prolonged bleeding after trauma. Bleeding can
occur at any part of the body. Hemarthrosis, or bleeding into the joint spaces, is the most common complication of severe hemophilia. The knee joint is the most frequent joint involved.
Nursing Care Plans
The nursing care plan goals for a client with hemophilia may include absence of complications and pain, prevention of injury and bleeding, improved physical mobility, and understanding of the disease condition and its management.
Impaired Physical Mobility
May be related to
- Pain and discomfort with the onset of bleeding episodes
Possibly evidenced by
- Pain in affected joint
- Decreased ability to move the joint
- Immobilized joints (first 24 to 48 hours after a bleeding episode)
- Potential contractures in affected joints
- Child will maintain optimal physical mobility as evidenced by normal range of motion (ROM) and activities of daily living within ability.
|Assess for limited ROM, contractures, and bony changes in the joints when bleeding has stopped.||Recurrent bleeding of the joints can lead to bone destruction, permanent deformities, and crippling. This data gives the baseline for evaluating further constraints from bleeding.|
|Provide gentle, passive ROM exercise when the child’s condition is stable.||Clients who are experiencing active bleeding have restricted mobility or splinting. However, later ROM exercise can help maintain optimal muscle and joint movement.|
|Assist with the progression to active exercise as tolerated.||Non-weight bearing exercise facilitates optimal ROM without stimulating rebleeding. Extra weight should be avoided until swelling has subsided.|
|Educate on preventive measures, such as the application of protective gear and the administration of factor products.||Avoiding injury and hemarthrosis is essential for maintaining joint and limb mobility and use.|
|Provide assistive devices when needed.||The chronic joint deformity is a common complaint.|
|Refer for physical therapy, occupational therapy, and orthopedic consultations, as required.||Electrical stimulation of the muscles around the joints prevents muscle atrophy. Active physiotherapy helps in regaining joint movement and preventing fibrous build up.|
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