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.
Risk for Bleeding
May be related to
- Decreased concentration of clotting factors circulating in the blood (factor VIII and factor IX)
Possibly evidenced by
- [not applicable]
- Child’s risk for injury from possible bleeding is decreased through the use of appropriate prophylactic measures.
|Monitor the coagulation assays for factor VIII AND IX.||Decreased value indicate that factor replacement therapy is subtherapeutic.|
|Monitor the partial thromboplastin time (PTT).||PTT is prolonged because of a deficiency in the clotting system factors. The prothrombin time will be normal.|
|Assess for any signs of bruising and bleeding (note the extent of bleeding). Assess for prolonged bleeding after minor injuries.||Usual sites of external bleeding may include the bleeding in the mouth from a cut, bite, or from cutting or losing a tooth; nosebleeds for no obvious reasons; heavy bleeding from a minor cut, or bleeding from a cut that resumes after stopping for a short time. Hemophiliacs do not bleed faster or more frequently. Instead, they bleed longer due to a deficiency of clotting factor. Clients are often aware of bleeding before clinical manifestation. Bleeding can be life-threatening to these clients.|
|Assess for any pain and swelling over the entire body.||A headache, in the presence of a trauma history, may suggest intracranial hemorrhage. Abdominal pain may indicate an internal bleeding. Bleeding into a joint is usually reported as a peculiar tingling sensation felt well before pain or swelling is detected.|
|If spontaneous or traumatic bleeding is evident, monitor vital signs.||Hypovolemic shock can happen due to decreased circulatory volume with blood loss. Signs include hypotension and tachycardia.|
||Hgb and Hct are monitored as indicators of blood loss.|
||Clients who require frequent transfusions may develop inhibitor antibody and require a subsequent change in coagulation therapy to factor VIIa.|
|Anticipate or instruct in the need for prophylactic treatment before high-risk situations, such as invasive diagnostic or surgical procedures, or dental work.||Treatment may include cryoprecipitate, which contains factor VIII and fibrinogen, and factor VIII concentrate, or it may include desmopressin (DDAVP) for treating mild to moderate hemophilia A.|
|Perform the following measures to control bleeding:
||Controlling bleeding is a nursing priority. Nasal packing should be avoided, because the subsequent removal of the packing may precipitate further bleeding.|
|If bleeding is in a joint (hemarthrosis), elevate and immobilize the affected limb. Apply ice packs to control bleeding.||Repeated hemarthrosis can result in severe and crippling deformity.|
|Provide replacement therapy of deficient clotting factors.||Replacement of factors is the primary treatment for bleeding. Treatment includes factor VIII, which is an essential clotting factor needed to convert prothrombin to thrombin. This treatment can also be provided in the home. DDAVP is the treatment of choice for mild hemophilia. It is an analogue of vasopressin and is available intravenously and intranasally. Recombinant DNA factors are available. Because they are not produced from humans, they should reduce the risk for infectious transmission.|
|Administer plasma-derived factor VIIa for clients with antibodies against factor VIII.||Antibodies (inhibitors) to the clotting factors can destroy it before it is able to work and defeat the effect of replacement therapy. New therapies are available to neutralize the antibodies.|
|Administer tranexamic acid or epsilon aminocaproic acid as indicated.||These are second-line antifibrinolytic drugs that do not actually form clots but help hold clots in place by stopping plasmin activity. They do not replace factor therapy. They are commonly used before dental work, during bleeding episodes, and, in the rare case of a woman with hemophilia, during heavy menstrual bleeding.|
|Anticipate the need for blood replacements.||Volume expanders and O-negative blood should be immediately available in the event of life-threatening hemorrhage.|
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