Primary Nursing Diagnosis

Risk for injury related to altered hemostasis and adverse effects of treatment

OUTCOMES. Risk control; Safety behavior: Fall prevention; Knowledge: Personal safety; Safety status: Physical injury; Knowledge: Medication; Safety behavior: Home physical environment

INTERVENTIONS. Bleeding precautions; Bleeding reduction; Fall prevention; Environmental management; Safety; Health education; Surveillance; Medication management

U PLANNING AND IMPLEMENTATION Collaborative

Replacement therapy and drug therapy may be used prophylactically or to control mild or major bleeding episodes. Desmopressin will raise factor VIII levels two- to threefold. Factor VIII replacement therapy is indicated for active bleeding or preparation for multiple tooth extractions or major surgery. Cryoprecipitate contains high levels of factor VIII and fibrinogen. Purified plasma-derived factor VIII concentrates are derived from large pools of plasma donors. Recent methods of screening and heating of these concentrates have greatly diminished the risk of contamination with the human immunodeficiency virus (HIV) but have little effect on the risk of hepatitis transmission. High-potency factor VIII preparations (those that are highly purified) are considered to be virtually virus-free. Recombinant factor VIII contains less risk of viral transmission but has a relatively high cost. A rule of thumb is that for every 1 unit/kg infused, factor VIII levels will increase 2%. In an emergency, a 50 unit/kg IV bolus will increase levels to 100%.

Factor VIII, also found in fresh-frozen plasma (FFP), is usually reserved for hemophilia A patients who are actively bleeding. Bleeding episodes in hemophilia B can be treated with FFP or purified factor IX. Hemophilia C rarely requires intervention. Prophylactic replacement therapy for factor VIII or factor IX deficiency has been found to be beneficial in preventing spontaneous bleeding episodes and in minimizing bleeding complications such as joint disease. Any incidence of head trauma should receive immediate therapy that raises the factor VIII or IX levels to 100% normal before any diagnostic tests are performed. Intracranial hemorrhage is the most common cause of death in hemophiliacs; approximately 50% of these are associated with acute head injury.

Pharmacologic Highlights

Medication or Drug Class

Dosage

Description

Rationale

Desmopressin, 1-deamino-8-D-arginine vaso-pressin (DDAVP)

0.3 mcg/kg IV diluted in 50 mL normal saline solution; intranasal: 300 mcg, 1 spray in each nostril

Antidiuretic

Stimulates rapid release of von Willebrand factor (vWF) into the blood; treatment of choice in hemophilia A patients who do not have life-threatening bleeding problems; avoided with infants; does not play a role in treatment for hemophilia B

Other Drugs: Analgesia for hemarthrosis; avoid products containing aspirin and nonsteroidal anti-inflammatories; Antifibrinolytic agents: Aminocaproic acid (Amicar) can be used in oral surgery or bleeding.

Independent

To prevent trauma that may precipitate bleeding episodes, avoid intramuscular injections and minimize the number of venipuncture attempts. Alert other health team members about the patient's high risk for bleeding. Avoid sources of mucosal irritation such as rectal temperatures, urinary catheters, and suppositories. Use only sponge sticks and nonalcoholic rinses for oral care. Assure that tourniquets or blood pressure cuffs are applied no longer than necessary. Perform nasopharyngeal or oropharyngeal suctioning very gently and only when needed. Prevent skin breakdown through the use of frequent turning and preventive skin care.

When bleeding occurs, apply firm, direct pressure for at least 5 minutes or until bleeding has stopped completely to sites of subcutaneous injections and venipuncture sites. Use sandbags and pressure dressings to maintain pressure on large puncture sites after hemostasis has been established. Initially, provide rest and elevation to a bleeding joint. Initiate mobilization within a few days after the bleeding is controlled to facilitate restoration of normal joint range of motion. Apply ice packs to control epistaxis, hematoma formation, and hemarthrosis.

Evaluate the family's current coping mechanisms and the level of anxiety. Encourage the patient and family members to verbalize their feelings openly and clearly with staff and with each other.

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