Hemophilia & von Willebrand Disease
Hemophilia A (factor VIII deficiency) High-yield Verified · Sep 2026Hemophilia is usually an inherited bleeding disorder caused by low levels of clotting factor VIII (hemophilia A) or factor IX (hemophilia B), causing spontaneous bleeding and bleeding after injury or surgery. Von Willebrand disease is a disorder in which von Willebrand factor (VWF) — the protein that helps platelets stick and carries factor VIII — is low or does not work.
Factor VIII versus IX versus VWF; X-linked inheritance and why females can still bleed; joint bleeds that start as tingling; the head injury that gets factor before the CT; isolated long aPTT in hemophilia but often normal screens in VWD; desmopressin with fluid restriction; and no aspirin, no NSAIDs.
What’s going wrong in the body
The normal physiology, where it fails, and why the body’s compensation backfires.
01 A weak clot — factor VIII, factor IX and von Willebrand factor
"People with hemophilia A or B have low levels of either factor VIII (8) or factor IX (9), respectively." "Factor VIII normally forms a complex with activated factor IX to convert factor X to Xa; its absence results in unstable clot formation and delayed hemostasis." "This defect primarily affects secondary hemostasis." In hemophilia B, "A deficiency in factor IX leads to a defective coagulation cascade and inadequate formation of a fibrin mesh."
Severity is the factor level. "The lower the amount of the factor, the more likely it is that bleeding will occur." StatPearls bands: "Severe hemophilia A: <1% normal factor VIII activity, associated with spontaneous bleeding, particularly hemarthroses." "Moderate hemophilia A: 1% to 5% activity, with bleeding typically following minor trauma." "Mild hemophilia A: 5% to 40% activity, with bleeding typically occurring after significant trauma or surgical procedures." Hemophilia B uses the same bands.
VWF is platelet glue and a factor VIII carrier. "Normally, when a person is injured and starts to bleed, the VWF in the blood attaches to small blood cells called platelets. This helps the platelets stick together, like glue, to form a clot at the site of injury and stop the bleeding." It "functions as a carrier for factor VIII to maintain its levels and help in platelet adhesion." So VWD bleeds from mucosa — "prolonged bleeding from mucosal surfaces (epistaxis, dental extractions, menstruation)" — while type 3, with "very little or no VWF and low levels of factor VIII," can bleed like hemophilia.
02 Inheritance — X-linked hemophilia, autosomal VWD
"Hemophilia A follows an X-linked recessive inheritance pattern." "A carrier mother has a 50% chance of transmitting the pathogenic variant to each child, resulting in affected sons or carrier daughters." "An affected father transmits the variant to all daughters, who become obligate carriers, but not to his sons." "Approximately one-third of cases arise from de novo mutations in individuals without a known family history."
Females can bleed too. "Female carriers may exhibit reduced factor VIII levels and bleeding symptoms due to skewed X-chromosome inactivation." "Females can also have hemophilia, but this is much rarer." NHS: carriers "can have symptoms of haemophilia, but this is less common and the symptoms are less severe than in men."
VWD affects both sexes equally. "VWD can be passed down from either the mother or the father, or both, to the child." "VWD is the most common bleeding disorder, found in up to 1% of the U.S. population," and "VWD occurs among men and women equally" — though women notice it more, through "heavy or abnormal bleeding during their menstrual periods and after childbirth." Hemophilia A is the most common hemophilia — "approximately 80% to 85% of all hemophilia cases" — and "Inherited hemophilia occurs in about 1 of every 5,000 male births."
03 Joints bleed, then bleed again — treat early and replace what is missing
"Recurrent hemarthrosis is the hallmark presentation in older children and adults, typically affecting large joints such as the knees, ankles, and elbows." "Recurrent bleeding, particularly into joints and muscles, leads to iron deposition, synovial inflammation, and progressive tissue damage," and synovitis sets "the area up for rebleeding." "Hemophilic arthropathy is the leading cause of chronic disability."
Treat at the first sign. Factor is given "at the first sign of joint bleeding—including the prodromal phase of stiffness or tingling before pain and swelling." StatPearls (hemophilia A): "Prompt treatment is essential, ideally within 1 hour of symptom onset"; the hemarthrosis chapter says "ideally within 2 hours of bleed identification." "The standard way to treat both hemophilia A and B is to replace the missing blood clotting factor." "Prophylaxis is now the standard of care for patients with severe hemophilia A," and in hemophilia B "Patients with severe hemophilia should receive prophylaxis regardless of bleeding episodes."
Head injury gets factor first. "Intracranial hemorrhage remains the most serious complication and requires immediate treatment without delay for confirmatory testing." The major treatment complication is an inhibitor — "an antibody that develops against an infused factor and hinders its proper functioning" — suspected when a patient shows "poor response to factor replacement."
Causes & risk factors
Modifiable factors are teaching targets — the patient can act on them.
Signs & symptoms
Each finding tied to the mechanism that produces it — and the triage it calls for.
Diagnostics
Complications
Medications for this condition
Why each class is prescribed here — every card links to its full pharmacology page.
Desmopressin (DDAVP) — scoped use. In mild hemophilia A "desmopressin (DDAVP) can be used as a first-line therapy for minor to moderate bleeding"; in VWD it treats "milder forms of VWD (mainly type 1)." It releases stored factor VIII and VWF. Not for hemophilia B; "Type 3 von Willebrand disease does not respond"; StatPearls trials it in "type 1, some type 2 (except type 2B)." A trial is needed first. Watch sodium: "Hyponatremia is the major adverse effect; therefore, fluid restriction is required." "tachyphylaxis typically occurs after 3 to 4 doses"; "DDAVP is contraindicated in children younger than 3 due to seizure risk"; it carries a thrombosis risk.
"Acetaminophen remains the preferred analgesic for most patients" (StatPearls, hemophilia B).
"selected opioid analgesics such as codeine may be used for acute bleeding-related pain" (StatPearls, hemophilia B).
AVOID. NHS: "do not take aspirin, ibuprofen or other non-steroidal anti-inflammatory drugs (NSAIDs)." In VWD, NSAIDs also "can worsen hyponatremia" with desmopressin.
AVOID — aspirin and antiplatelet drugs impair hemostasis.
AVOID unless "specifically recommended after careful risk-benefit assessment" (StatPearls, hemophilia B).
VWD heavy periods. "Birth control pills can increase the levels of VWF and factor VIII in the blood and reduce menstrual blood loss." NHS also names the intrauterine system.
VWD anemia from heavy bleeding — NHS: "your doctor may also suggest taking iron supplements."
"vaccination against hepatitis A and B, particularly in individuals exposed to blood products" (StatPearls).
Target joints. "a short course of glucocorticoids may help reduce pain and swelling from synovial inflammation, provided there are no signs of infection."
Beyond medications
- Factor replacement (no class page). Clotting factor concentrates are infused into a vein — episodically "to stop a patient’s bleeding episodes" or as prophylaxis "to prevent bleeding episodes from occurring." Home infusion means "bleeds can be treated more quickly, resulting in less serious bleeding and fewer side effects." Recombinant factors "cannot spread any bloodborne viruses." Prophylaxis frequency depends on the product (StatPearls) — NHS: "up to 3 times a week" in severe haemophilia.
- Emicizumab (hemophilia A) — given under the skin; it "bridges activated factor IX and X, mimicking the cofactor function of activated factor VIII." "Breakthrough bleeding and surgical procedures still require factor VIII supplementation." For persistent inhibitors, StatPearls recommends emicizumab over bypassing agents for prophylaxis.
- Inhibitors — bypassing agents (recombinant activated factor VII, aPCC) for acute bleeds and immune tolerance induction with regular high-dose factor VIII "over months to years."
- Gene therapy — "an option for people with hemophilia A and B" (CDC).
- Antifibrinolytics (tranexamic acid, aminocaproic acid) for mucosal bleeding — "oral, nasal, gastrointestinal, and uterine bleeding" — but "tranexamic acid is contraindicated in upper urinary tract bleeding due to the risk of obstructive clot formation."
- VWF replacement — for "more severe forms of VWD or people with milder forms of VWD who do not respond well to the nasal spray"; preferred over desmopressin "in patients with cardiovascular or cerebrovascular disease." Type 3 patients can form alloantibodies with "an increased risk for life-threatening anaphylactic reactions."
- Joint bleeds — factor first, then "immobilization, ice, and compression"; aspiration is not routine and only "after factor replacement has raised specific factor levels"; then "a structured rehabilitation program" with a hemophilia-experienced physical therapist.
- Activity — "avoid high-risk activities and contact sports while remaining physically active through low-impact exercises"; StatPearls (hemophilia B) lists swimming, walking, cycling, golf, tennis, archery and table tennis.
Nursing considerations
The RN-specific layer — each action paired with the reason it matters.
How a real nurse thinks
The clinical-judgment loop, worked for a typical presentation — reasoning, not reference.
- 1 Recognize cues
A head injury in a child with severe hemophilia A, with vomiting and headache, and no factor given since the fall. He looks well, but NHS lists "have a head injury" for haemophilia under "Call 999 or go to A&E," and headache with vomiting "could be a sign of bleeding in the brain."
- 2 Analyze cues
Intracranial hemorrhage "may occur spontaneously or after minor trauma" and "requires immediate treatment without delay for confirmatory testing." A normal neurological exam now does not rule it out.
- 3 Prioritize
Factor replacement comes first — before the CT. Notify the provider immediately so factor is ordered and given without waiting for imaging.
- 4 Generate solutions
Obtain the factor order and give it immediately; then accompany him to CT; start neurological observations; contact the hemophilia treatment team; avoid intramuscular injections; give the prescribed analgesic — not an NSAID — for his headache.
- 5 Take action
SBAR: 8-year-old boy, severe hemophilia A, head injury 30 minutes ago, vomited once, headache, scalp swelling; GCS 15. No factor since the fall. Requesting factor replacement now, before the CT, and hemophilia team notification. Factor is given, then the CT is done, with neuro checks continuing.
- 6 Evaluate
The CT shows no intracranial bleed. He is observed with neuro checks and further factor per the hemophilia team. His mother is taught that for a head injury she should treat with his usual factor straight away if possible and call 999 — "Do not drive to A&E."
Scenario — you’re the nurse
Work each question before revealing the answer. Rationales explain the wrong options too.
An 8-year-old boy with severe hemophilia A on home prophylaxis fell from his bicycle 30 minutes ago and hit his head. He wore no helmet. His mother says he ‘seems fine’ but has vomited once and complains his head hurts. She did not give factor before coming.
Q1 Multiple choice A child with severe hemophilia A has a head injury and a headache. What should happen first? - A. Obtain a head CT to confirm bleeding before giving factor
- B. Give factor replacement immediately, without waiting for imaging
- C. Give ibuprofen for the headache and observe
- D. Apply ice and discharge with head-injury instructions
Reveal answer & rationale
Q2 Multiple choice Which laboratory pattern is expected in hemophilia A? - A. Prolonged PT with normal aPTT
- B. Isolated prolonged aPTT with normal PT/INR and platelet count
- C. Low platelet count with normal clotting times
- D. Prolonged PT and aPTT with low fibrinogen
Reveal answer & rationale
Q3 Select all that apply Which findings should a parent treat as possible bleeding and report to the hemophilia team straight away? Select all that apply. - A. Tingling or stiffness in a knee
- B. A swollen, painful calf
- C. Pink or dark brown urine
- D. A small bruise that is fading
Reveal answer & rationale
Q4 Multiple choice A patient with type 1 von Willebrand disease is starting desmopressin. Which instruction is most important? - A. Drink extra fluids to prevent dehydration
- B. Follow the fluid restriction and have sodium levels checked as ordered
- C. Take ibuprofen for any menstrual cramps
- D. Expect the medicine to work every time, however often it is used
Reveal answer & rationale
Q5 Select all that apply Which statements about hemophilia and VWD are accurate? Select all that apply. - A. Female carriers of hemophilia can have bleeding symptoms
- B. Screening tests are often normal in von Willebrand disease
- C. Desmopressin treats hemophilia B
- D. Aspirin should be avoided
Reveal answer & rationale
Common questions
What causes hemophilia & von willebrand disease?
What are the early signs of hemophilia & von willebrand disease?
Which findings should a nurse report immediately in hemophilia & von willebrand disease?
Can girls and women have hemophilia?
Why give factor before the CT after a head injury?
Why are aspirin and ibuprofen avoided?
Sources
- Hemophilia A — StatPearls — StatPearls (NCBI Bookshelf)
- Hemophilia B — StatPearls — StatPearls (NCBI Bookshelf)
- Von Willebrand Disease — StatPearls — StatPearls (NCBI Bookshelf)
- Hemarthrosis — StatPearls — StatPearls (NCBI Bookshelf)
- About Hemophilia — CDC — Centers for Disease Control and Prevention
- Treatment of Hemophilia — CDC — Centers for Disease Control and Prevention
- About von Willebrand Disease — CDC — Centers for Disease Control and Prevention
- Diagnosing von Willebrand Disease — CDC — Centers for Disease Control and Prevention
- Haemophilia — NHS. Contains public sector information licensed under the Open Government Licence v3.0 (https://www.nationalarchives.gov.uk/doc/open-government-licence/version/3/) — NHS (UK)
- Von Willebrand disease — NHS. Contains public sector information licensed under the Open Government Licence v3.0 (https://www.nationalarchives.gov.uk/doc/open-government-licence/version/3/) — NHS (UK)
Reviewed by Hae Suk Lee, RN
Educational summary for nursing students. Scenarios are fictional teaching cases. Always verify against current guidelines and your institution's protocols before acting in practice. Not medical advice.