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 Injury
- Risk for Injury
May be related to
- Decreased clotting factor (VIII or IX)
Possibly evidenced by
- [not applicable]
- Client will not experience injury.
|Assess signs and symptoms of bleeding;
hemarthrosis (stiffness, tingling, or
pain); subcutaneous and intramuscular
hemorrhage; oral bleeding; epistaxis
(is not a frequent sign); petechiae
|Early detection of bleeding episodes will delay initiation of factor replacement therapy and will minimize complications; oral bleeding is often caused by trauma to the gums; petechiae is caused by low platelet function versus a deficient clotting factor.|
|Advise adolescents to use an electric shaver versus manual razor devices (with blades).||High risk of bleeding is related to use of razor blades; minimal risk of bleeding is associated with the use of electric shaver.|
|Utilize appropriate toys (soft, not pointed
or small sharp objects); for infants, may need to use padded bed rail sides on crib; avoid rectal temperatures.
|All of these recommendations will minimize and/or prevent bleeding episodes due to trauma.|
|Provide appropriate oral hygiene (use of
a water irrigating device; use of a soft toothbrush or softening the toothbrush with warm water before brushing; use of sponge-tipped toothbrush).
|Implementation of appropriate oral hygiene will minimize trauma to the gums.|
|Substitute the subcutaneous route for
intramuscular injections; utilize
venipuncture blood drawing technique
for all required blood testing samples
versus the use of a finger or heel puncture.
|Both of these measures are associated with less bleeding after implementing subcutaneous injection or venipuncture blood sample.|
|Recommend non-contact sports activities such as swimming, hiking, or bicycling.||These activities are considered a safe activity by the Hemophilia Foundation.|
|Avoid contact sports such as football,
soccer, ice hockey, karate.
|Contact sports will predispose the child to injury and bleeding episodes.|
|Limit use of helmets and padding of cause joints during participation in contact sports activities.||Daily use of these measures may the child to feel ostracized or may create emotional discomfort.|
|Maintain close supervision during play time to minimize injuries.||Prevent bleeding related to trauma in the child’s environment.|
|Institute the following measures to control and halt all bleeding episodes:|
||Allows clot formation.|
||Decrease blood flow to control bleeding.|
||Promote vasoconstriction, but use caution with small children to avoid tissue damage.|
||Control and stop bleeding episode; Prevent crippling effects from joint bleeding.|
||Minimize hemorrhage in muscles of the lower spine.|
||These values determine current hemodynamic status and factor replacement therapy guidelines or protocols|
||Avoid injury to the affected extremity or joint and to avoid recurrence of bleeding to these.|
|Educate the family and affected child about signs and symptoms of bleeding, and appropriate interventions to
control bleeding at home.
|Empowers others with accurate information to identify and manage bleeding episodes.|
|Instruct parents to include iron-rich foods
|Maintains iron level to prevent anemia.|
|Advice to wear a medical alert bracelet.||Facilitates accurate diagnosis and treatment in case of an emergency.|
|Teach parents related to home health maintenance:|
||Protect the child from childhood communicable diseases.|
||Minimize oral trauma.|
||Minimize emotional distress during the child’s progression through the different developmental stages.|
||Minimize risk of trauma in the home by falls; infants and toddlers frequently fall or sustain injuries.|
|Instruct parents and child, if age
appropriate, to administer factor VIII
via IV if signs and symptoms appear,
or before dental visits or other possible invasive procedures; instruct in mixing the precipitate, drawing into syringe, venipuncture, and application of pressure following IV, and allow for return
|Prevents or manages bleeding by factor replacement.|
|Teach parents and child of possible
reactions to IV concentrate administration and that blood is tested for AIDS.
|Decreases anxiety caused by risk of infections such as hepatitis and AIDS from replacement products.|
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Hematologic and Lymphatic Care Plans
Care plans related to the hematologic and lymphatic system:
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- Deep Vein Thrombosis | 5 Care Plans
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