Headache, vomiting, confusion, or decreased level of consciousness signal possible intracranial hemorrhage; report and treat immediately.
Before you read
Test yourself first: 2 questions
Question 1 of 2
A client with hemophilia A is missing which clotting factor?
Answer: A. Hemophilia A, the classic form, is caused by a deficiency of clotting factor VIII. Hemophilia B (Christmas disease) is caused by a deficiency of factor IX.
Question 2 of 2
A client with hemophilia A is admitted with a swollen, painful knee after a minor injury. Which is the priority nursing action?
Answer: A. Hemophilia A is a deficiency of clotting factor VIII causing prolonged bleeding; hemarthrosis (bleeding into a joint) is common. Priority care is to control bleeding by administering the prescribed factor VIII replacement, immobilizing and elevating the joint, and applying ice. Aspirin is contraindicated because it impairs platelet function. Vigorous range of motion would worsen joint bleeding.
Keep the streak going with more questions like these, each with a full rationale.
What is hemophilia?
Hemophilia A is factor VIII deficiency (most common) and hemophilia B (Christmas disease) is factor IX deficiency. It almost always affects males, with females being carriers. The hallmark is deep bleeding into joints (hemarthrosis) and muscles, and prolonged bleeding after injury or surgery.
Key points to remember
- X-linked recessive: males affected, females are carriers; type A = factor VIII deficiency, type B = factor IX deficiency.
- Hemarthrosis (bleeding into joints, especially knees, elbows, ankles) causes pain, swelling, warmth, and can lead to chronic joint damage.
- Platelet count and PT are normal; aPTT is prolonged because the intrinsic pathway factors are deficient.
- Treatment is IV replacement of the deficient clotting factor (factor VIII or IX concentrate); desmopressin (DDAVP) raises factor VIII in mild hemophilia A.
- Bleeding is not faster but is prolonged and harder to stop; prolonged oozing after dental work or circumcision is a common early sign.
- Aminocaproic acid may be used adjunctively to stabilize clots, especially for mucosal or oral bleeding.
Signs & symptoms
- Hemarthrosis (joint bleeds)
- Deep bruising, prolonged bleeding
Nursing priorities
- Control active bleeding: apply firm direct pressure, ice, immobilize and elevate the affected joint or limb.
- Administer prescribed clotting factor concentrate IV; have it readily available before any invasive procedure.
- Implement bleeding precautions: avoid IM injections, use the smallest gauge needle, hold venipuncture sites longer.
- Assess joints for hemarthrosis (pain, swelling, limited motion) and monitor for signs of internal or intracranial bleeding.
- Provide RICE (rest, ice, compression, elevation) and pain control with acetaminophen rather than aspirin or NSAIDs.
Labs & values
Patient teaching
- Teach factor replacement administration and to infuse at the first sign of bleeding (early treatment limits joint damage).
- Avoid contact sports and high-injury activities; choose swimming or other low-impact exercise to protect joints.
- Use a soft toothbrush and electric razor; avoid aspirin and NSAIDs, reading OTC labels carefully.
- Wear medical alert identification and inform all providers and dentists of the diagnosis before procedures.
- Genetic counseling is recommended; female carriers may pass the gene to offspring.
Red flags: report now
- Headache, vomiting, confusion, or decreased level of consciousness signal possible intracranial hemorrhage; report and treat immediately.
- Never give aspirin, NSAIDs, or other antiplatelet/anticoagulant drugs as they worsen bleeding.
- Avoid IM injections and rectal temperatures; signs of airway compromise from neck or throat bleeding require emergent attention.
References
Clinical references for this topic
