Review Hemophilia

Pathophysiology of the Hematologic System > Pathophysiology

Exam Review

Hemophilia

Share This Link

Share on social media

Facebook

Messager

WhatsApp

Share URL
Copy

Experience unlimited access to all questions, exams, video tutorials and study guides. Subscribe now to access all questions.

Total Questions : 15

Showing 15 questions, Sign in for more

Question 1:

A nurse is providing education to a group of parents about hemophilia.
Which statement by one of the parents indicates a need for further teaching?

A. "Hemophilia A is more common than hemophilia B.” B. "Hemophilia is inherited through a defective gene on the Y chromosome.” C. "Males inherit hemophilia from their mothers who carry the defective gene.” D. "Females can also have hemophilia, but it is very rare.”

Answer and Explanation

Choice A:

Hemophilia A and hemophilia B are both types of hemophilia, but hemophilia A is actually less common than hemophilia B. The prevalence of hemophilia A is about 1 in 5,000 males, whereas hemophilia B occurs in about 1 in 25,000 males. Therefore, this statement is incorrect.

Choice B:

Hemophilia is not inherited through a defective gene on the Y chromosome. Hemophilia is an X-linked recessive genetic disorder, which means it is caused by a mutation in genes located on the X chromosome, not the Y chromosome. This statement is incorrect.

Choice C:

This statement is correct. Males inherit hemophilia from their mothers who carry the defective gene on one of their X chromosomes. However, it's important to note that females can also be carriers of the gene and can pass it on to their sons. This statement does not indicate a need for further teaching.

Choice D:

This statement is incorrect. While it is very rare for females to have hemophilia, it is not impossible. Females can inherit hemophilia if they have two affected X chromosomes or if they inherit one affected X chromosome and one affected Y chromosome. Therefore, this statement does not indicate a need for further teaching.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 2:

A client with hemophilia A asks the nurse about the cause of the condition.
Which response by the nurse is accurate?

A. "Hemophilia A is caused by a mutation in the F8 gene, which encodes factor VIII.” B. "Hemophilia A is caused by a mutation in the F9 gene, which encodes factor IX.” C. "Hemophilia A is caused by a mutation in the Y chromosome.” D. "Hemophilia A is caused by a deficiency of vitamin K.”

Answer and Explanation

Choice A:

Hemophilia A is indeed caused by a mutation in the F8 gene, which encodes factor VIII. Factor VIII is essential for blood clotting, and mutations in this gene lead to a deficiency or dysfunction of factor VIII, resulting in hemophilia A. This response provides accurate information about the cause of the condition.

Choice B:

Hemophilia A is not caused by a mutation in the F9 gene, which encodes factor IX. Factor IX deficiency is associated with hemophilia B, not hemophilia A. This statement is incorrect.

Choice C:

Hemophilia A is not caused by a mutation in the Y chromosome. As mentioned earlier, it is caused by a mutation in the F8 gene on the X chromosome. This statement is incorrect.

Choice D:

Hemophilia A is not caused by a deficiency of vitamin K. Vitamin K deficiency can lead to bleeding disorders, but it is not the cause of hemophilia A. This statement is incorrect.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 3:

A nurse is assessing a client with hemophilia.
Which clinical manifestations should the nurse expect to find? (Select all that apply.)

A. Easy bruising. B. Hematuria. C. Joint pain and swelling. D. Elevated factor VIII levels. E. Spontaneous nosebleeds.

Answer and Explanation

Choice A:

Easy bruising is a common clinical manifestation of hemophilia. Patients with hemophilia have difficulty forming blood clots, which makes them prone to bleeding into the soft tissues, leading to easy bruising. This choice is correct.

Choice B:

Hematuria (blood in the urine) is not a typical clinical manifestation of hemophilia. Hemorrhaging in the urinary tract is uncommon in hemophilia, so this choice is incorrect.

Choice C:

Joint pain and swelling are characteristic clinical manifestations of hemophilia. Bleeding into the joints can cause pain, swelling, and limited range of motion. This choice is correct.

Choice D:

Elevated factor VIII levels are not an expected clinical manifestation of hemophilia. In fact, individuals with hemophilia have reduced levels of factor VIII due to the genetic mutation that causes the condition. This choice is incorrect.

Choice E:

Spontaneous nosebleeds are a common clinical manifestation of hemophilia. The fragile blood vessels in the nose can rupture easily, leading to spontaneous nosebleeds. This choice is correct.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 4:

A client with hemophilia is at risk for complications.
Which complication should the nurse prioritize in the client's care plan?

A. Hypertension. B. Deep vein thrombosis. C. Hemarthrosis. D. Hyperlipidemia.

Answer and Explanation

Choice A:

Hypertension is not a common complication of hemophilia. While bleeding disorders like hemophilia can lead to bleeding in various body systems, hypertension is not directly associated with hemophilia. Therefore, it is not a priority in the care plan for a client with hemophilia.

Choice B:

Deep vein thrombosis (DVT) is a condition characterized by the formation of blood clots in deep veins, which can lead to serious complications. While individuals with hemophilia are at an increased risk of bleeding, they are not at an increased risk of DVT. In fact, individuals with hemophilia often have difficulty forming blood clots, making DVT less likely in this population. Therefore, it is not a priority in the care plan for a client with hemophilia.

Choice C:

Hemarthrosis is a critical complication that should be prioritized in the care plan for a client with hemophilia. Hemarthrosis is the bleeding into joint spaces, which can lead to severe pain, swelling, and reduced range of motion in the affected joint. It is a common and serious complication in individuals with hemophilia because bleeding into joints can cause long-term damage and disability. Therefore, the nurse should prioritize assessing and managing hemarthrosis in the client's care plan to prevent further complications.

Choice D:

Hyperlipidemia refers to high levels of lipids (fats) in the blood, such as cholesterol and triglycerides. It is not a common complication of hemophilia, and there is no direct link between hemophilia and hyperlipidemia. Therefore, it is not a priority in the care plan for a client with hemophilia.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 5:

A nurse is educating a group of nursing students about the treatment options for hemophilia.
What intervention should the nurse include in the teaching?

A. Administering anticoagulants to promote clot formation. B. Encouraging regular physical activity to prevent bleeding episodes. C. Administering factor replacement therapy to replace deficient clotting factors. D. Administering iron supplements to improve hemoglobin levels.

Answer and Explanation

Choice A:

Administering anticoagulants to promote clot formation is not appropriate for the treatment of hemophilia. Anticoagulants are medications that thin the blood and prevent the formation of blood clots. However, in individuals with hemophilia, the issue is not the formation of excessive clots but rather the inability to form effective clots due to a deficiency in clotting factors. Therefore, anticoagulants would worsen the bleeding disorder and are not a suitable intervention.

Choice B:

Encouraging regular physical activity to prevent bleeding episodes is a reasonable recommendation for individuals with hemophilia. Regular, low-impact physical activity can help strengthen muscles and joints, which may reduce the risk of bleeding episodes and joint damage. However, this alone is not a treatment for hemophilia but rather a preventive measure.

Choice C:

Administering factor replacement therapy is the primary treatment for hemophilia. Hemophilia is characterized by a deficiency in specific clotting factors (Factor VIII for Hemophilia A and Factor IX for Hemophilia B) Replacement therapy involves infusing the missing clotting factor to achieve normal or near-normal levels, allowing the blood to clot properly. This intervention is crucial in managing and preventing bleeding episodes in individuals with hemophilia.

Choice D:

Administering iron supplements to improve hemoglobin levels is not a direct treatment for hemophilia. Hemophilia is a clotting disorder, and iron supplements are typically used to treat conditions related to iron deficiency anemia. While individuals with hemophilia may experience anemia as a result of chronic bleeding, the primary treatment for hemophilia involves addressing the clotting factor deficiency through factor replacement therapy. .

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 6:

A nurse is caring for a client with hemophilia.
The client reports pain, swelling, and reduced range of motion in their joints.
Which of the following statements by the nurse is appropriate?

A. "It's just a minor issue, and the pain will go away on its own.” B. "Let's apply ice to the affected joint to reduce the swelling.” C. "I'll assess your joint and provide pain management as needed.” D. "You should perform strenuous exercises to improve joint mobility.”

Answer and Explanation

Choice A:

"It's just a minor issue, and the pain will go away on its own." This statement is not appropriate because joint pain, swelling, and reduced range of motion in a client with hemophilia, known as hemarthrosis, can lead to long-term joint damage and disability if not properly managed. Downplaying the issue is not in the best interest of the client and may lead to inadequate care.

Choice B:

"Let's apply ice to the affected joint to reduce the swelling." While applying ice to an inflamed joint can help reduce swelling in some cases, it may not be the best immediate approach for a client with hemophilia. Ice application should be done with caution, as it can potentially worsen bleeding in individuals with hemophilia. The primary focus should be on assessing the severity of the bleeding, providing appropriate pain management, and consulting with a healthcare provider.

Choice C:

"I'll assess your joint and provide pain management as needed." This is the most appropriate response. The nurse should assess the client's joint for signs of hemarthrosis, such as swelling, warmth, and reduced range of motion. Prompt assessment allows for early intervention to manage the bleeding and alleviate pain. Providing pain management as needed, which may include analgesic medications, is essential to improve the client's comfort and prevent further complications.

Choice D:

"You should perform strenuous exercises to improve joint mobility." Encouraging strenuous exercises in a client with hemophilia who is already experiencing joint pain and swelling is not advisable. Strenuous exercise can exacerbate bleeding and joint damage. Instead, the focus should be on gentle range-of-motion exercises and physical therapy, guided by a healthcare provider, to improve joint mobility without increasing the risk of bleeding.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 7:

A client with hemophilia presents with symptoms of bleeding into muscles, soft tissues, and gastrointestinal tract.
Which statement by the nurse is correct?

A. "It's essential to limit your fluid intake to prevent further bleeding.” B. "Let's administer aspirin to help with the pain and swelling.” C. "I'll monitor your condition closely and provide appropriate treatments.” D. "This bleeding is normal and should resolve on its own.”

Answer and Explanation

Choice A:

"It's essential to limit your fluid intake to prevent further bleeding." Rationale: This statement is incorrect. Limiting fluid intake will not prevent bleeding in a client with hemophilia. In fact, maintaining adequate hydration is important to prevent complications and maintain overall health.

Choice B:

"Let's administer aspirin to help with the pain and swelling." Rationale: This statement is also incorrect. Aspirin is not recommended for individuals with hemophilia as it can further increase the risk of bleeding due to its antiplatelet effects. Administering aspirin would be contraindicated in this case.

Choice C:

"I'll monitor your condition closely and provide appropriate treatments." Rationale: This is the correct statement. For a client with hemophilia experiencing bleeding into muscles, soft tissues, and the gastrointestinal tract, close monitoring and appropriate treatments are essential. This may include administering clotting factor concentrates, pain management, and supportive care.

Choice D:

"This bleeding is normal and should resolve on its own." Rationale: This statement is incorrect and potentially dangerous. Bleeding in a client with hemophilia is not normal and should not be left untreated. Without intervention, it can lead to severe complications and even life-threatening situations.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 8:

(Select all that apply):
A nurse is educating a group of clients with hemophilia about diagnostic evaluation.
Which tests are typically used to diagnose hemophilia?

A. Genetic testing for the F8 or F9 gene mutations. B. Prothrombin time (PT) C. Partial thromboplastin time (PTT) D. Platelet count. E. Fibrinogen levels.

Answer and Explanation

Choice A:

Genetic testing for the F8 or F9 gene mutations. Rationale: Genetic testing for mutations in the F8 (Factor VIII) or F9 (Factor IX) genes is a critical diagnostic evaluation for hemophilia. Hemophilia A is caused by mutations in the F8 gene, while hemophilia B is caused by mutations in the F9 gene.

Choice B:

Prothrombin time (PT) Rationale: PT is not typically used to diagnose hemophilia. It primarily evaluates the extrinsic and common coagulation pathways, which are not directly related to hemophilia, which is a disorder of the intrinsic coagulation pathway.

Choice C:

Partial thromboplastin time (PTT) Rationale: PTT is one of the key tests used to diagnose hemophilia. It assesses the intrinsic coagulation pathway, and prolonged PTT results may indicate a bleeding disorder, including hemophilia.

Choice D:

Platelet count. Rationale: Platelet count is not a specific test for diagnosing hemophilia. Hemophilia is a deficiency in clotting factors, not a platelet disorder. Platelet counts are typically normal in individuals with hemophilia.

Choice E:

Fibrinogen levels. Rationale: Fibrinogen levels are not typically used to diagnose hemophilia. Hemophilia is characterized by deficiencies in specific clotting factors (Factor VIII or Factor IX), and fibrinogen levels are not directly related to these factors.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 9:

A nurse is obtaining a history from a client suspected of having hemophilia.
What information should the nurse include in the history assessment?

A. Family history of bleeding disorders. B. Recent traumatic injuries. C. Medications affecting coagulation. D. Allergies to latex products.

Answer and Explanation

Choice A:

Family history of bleeding disorders. Rationale: In the assessment of a client suspected of having hemophilia, it is essential to inquire about a family history of bleeding disorders because hemophilia is a genetic disorder, and a positive family history can be a strong indicator.

Choice B:

Recent traumatic injuries. Rationale: Inquiring about recent traumatic injuries is important because individuals with hemophilia are at increased risk of bleeding following injuries. Knowing about recent trauma helps assess the risk of bleeding episodes.

Choice C:

Medications affecting coagulation. Rationale: It is crucial to inquire about medications that can affect coagulation, such as anticoagulants or antiplatelet agents, as these medications can increase the risk of bleeding in individuals with hemophilia.

Choice D:

Allergies to latex products. Rationale: While allergies to latex products should be assessed for safety reasons, it is not directly related to the diagnosis of hemophilia. This information is important for overall patient safety but is not a specific factor in diagnosing hemophilia.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 10:

A nurse is assessing a patient with hemophilia.
Which aspect of the assessment should the nurse prioritize to determine the type and severity of hemophilia?

A. "Can you describe the triggers for your bleeding episodes?” B. "What is the frequency of your joint pain?” C. "Do you have a family history of hemophilia?” D. "Have you been prescribed antifibrinolytic agents?”

Answer and Explanation

Choice A:

"Can you describe the triggers for your bleeding episodes?” This is the correct choice. Understanding the triggers for bleeding episodes can help determine the type and severity of hemophilia. For example, frequent spontaneous bleeding without apparent triggers may suggest severe hemophilia, while bleeding triggered by trauma or surgery may indicate a milder form.

Choice B:

"What is the frequency of your joint pain?” This choice is relevant to assessing the impact of hemophilia on the patient's quality of life but does not directly help determine the type and severity of hemophilia.

Choice C:

"Do you have a family history of hemophilia?” While family history is important in assessing the risk of hemophilia, it alone does not provide information about the type and severity of the patient's condition.

Choice D:

"Have you been prescribed antifibrinolytic agents?” This question pertains to treatment rather than the assessment of the type and severity of hemophilia. It is important to know the treatment history but does not provide direct insight into the condition's severity.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 11:

A client with hemophilia reports joint pain.
What nursing intervention should the nurse prioritize for managing the client's pain?

A. "Administer acetaminophen as prescribed.” B. "Apply pressure, ice, and compression to the affected joint.” C. "Refer the client to genetic counseling services.” D. "Encourage the client to perform vigorous physical exercises.”

Answer and Explanation

Choice A:

"Administer acetaminophen as prescribed." While pain management is essential for clients with hemophilia, acetaminophen is not the first-line choice because it does not have anti-inflammatory properties. Additionally, in hemophilia, there is a risk of liver damage from excessive acetaminophen use.

Choice B:

"Apply pressure, ice, and compression to the affected joint." This is the correct choice. Applying ice and compression to the affected joint can help reduce pain and inflammation in clients with hemophilia. It is a safe and effective nursing intervention.

Choice C:

"Refer the client to genetic counseling services." Genetic counseling is important in hemophilia for family planning and risk assessment. However, it is not the priority nursing intervention for managing acute joint pain.

Choice D:

"Encourage the client to perform vigorous physical exercises." Encouraging vigorous physical exercises is not advisable for clients with hemophilia, especially when they are experiencing joint pain. It can increase the risk of bleeding and further joint damage.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 12:

(Select all that apply):
A nurse is providing education to a patient with hemophilia about self-care measures.
Which recommendations should the nurse include? (Select all that apply.)

A. "Avoid invasive procedures and injections.” B. "Use nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief.” C. "Engage in gentle exercise to maintain joint function.” D. "Monitor for signs of anemia and thrombosis.” E. "Apply heat to bleeding joints to alleviate pain.”

Answer and Explanation

Choice A:

The nurse should recommend avoiding invasive procedures and injections for a patient with hemophilia. Hemophilia is a bleeding disorder characterized by a deficiency in clotting factors, and any invasive procedures or injections can lead to bleeding episodes. This recommendation is essential to prevent unnecessary bleeding and complications.

Choice B:

The use of nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief is not a suitable recommendation for a patient with hemophilia. NSAIDs can increase the risk of bleeding due to their antiplatelet effects. It is crucial to avoid medications that can worsen bleeding in individuals with hemophilia.

Choice C:

Engaging in gentle exercise to maintain joint function is a valuable recommendation for patients with hemophilia. Hemophilia often leads to joint problems due to repeated bleeding episodes, and gentle exercise can help maintain joint mobility and prevent contractures. It is essential to recommend exercise within the patient's tolerance to promote joint health.

Choice D:

Monitoring for signs of anemia and thrombosis is important for patients with hemophilia. Anemia can occur if there is excessive bleeding, leading to a decrease in red blood cell count. Additionally, patients with hemophilia are at risk of developing thrombosis due to treatment with clotting factor concentrates. Therefore, regular monitoring for these complications is necessary to ensure timely intervention if needed.

Choice E:

Applying heat to bleeding joints to alleviate pain is not a recommended practice for patients with hemophilia. Heat can increase blood flow to the affected area, potentially exacerbating bleeding. Cold therapy, such as ice packs, is often recommended to reduce inflammation and pain while minimizing the risk of bleeding.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 13:

A nurse is caring for a patient with mild to moderate hemophilia

A. Desmopressin acetate (DDAVP) B. Factor VIII concentrates. C. Tranexamic acid. D. Gene therapy.

Answer and Explanation

Choice A:

Desmopressin acetate (DDAVP) is a medication used to stimulate the release of factor VIII and von Willebrand factor from endothelial cells. It is particularly effective in patients with mild to moderate hemophilia A who have a functional von Willebrand factor. DDAVP works by promoting the release of stored clotting factors from the endothelium, temporarily increasing their levels in the bloodstream. This medication is administered intranasally, subcutaneously, intravenously, or orally, making it a versatile option for treatment in different clinical settings.

Choice B:

Factor VIII concentrates are used for the replacement of factor VIII in patients with hemophilia A but do not stimulate the release of factor VIII and von Willebrand factor from endothelial cells. Factor VIII concentrates are typically administered intravenously to replace the deficient clotting factor.

Choice C:

Tranexamic acid is an antifibrinolytic agent used to prevent the breakdown of fibrin clots. While it can help in managing bleeding episodes in patients with hemophilia, it does not stimulate the release of factor VIII or von Willebrand factor from endothelial cells.

Choice D:

Gene therapy is an emerging treatment approach for hemophilia, but it does not stimulate the release of factor VIII and von Willebrand factor from endothelial cells. Gene therapy aims to provide a long-term solution by introducing functional clotting factor genes into the patient's body.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 14:

A client with hemophilia experiences mucosal bleeding episodes such as epistaxis and oral bleeding.
Which drug, used as adjunctive therapy to inhibit the breakdown of fibrin clots, should the nurse consider?

A. Factor replacement therapy. B. Desmopressin acetate (DDAVP) C. Aminocaproic acid. D. Gene therapy.

Answer and Explanation

Choice A:

Factor replacement therapy, such as the administration of factor VIII concentrates, is the primary treatment for managing bleeding episodes in patients with hemophilia. However, it does not specifically inhibit the breakdown of fibrin clots, which is the primary role of aminocaproic acid.

Choice B:

Desmopressin acetate (DDAVP) is used to stimulate the release of factor VIII and von Willebrand factor from endothelial cells. While it can help in some cases, it may not be the most effective choice for mucosal bleeding episodes like epistaxis and oral bleeding, where aminocaproic acid is more commonly used.

Choice C:

Aminocaproic acid is an antifibrinolytic medication that inhibits the breakdown of fibrin clots. It is particularly useful in managing mucosal bleeding episodes in patients with hemophilia, such as epistaxis and oral bleeding, by preventing the premature dissolution of clots and promoting hemostasis.

Choice D:

Gene therapy is an emerging treatment for hemophilia, but it does not directly address the breakdown of fibrin clots. It aims to provide a long-term solution by introducing functional clotting factor genes into the patient's body, but it may not be the immediate choice for managing acute bleeding episodes.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Question 15:

A client with hemophilia has prolonged partial thromboplastin time (PTT) and normal prothrombin time (PT)
What does this laboratory result indicate about the client's condition?

A. The client has a normal coagulation profile. B. The client may have a vitamin K deficiency. C. The client likely has hemophilia. D. The client is at risk for thrombosis.

Answer and Explanation

Choice A:

The client has a normal coagulation profile.
This choice is incorrect because the client's prolonged PTT suggests a problem with the intrinsic pathway of coagulation, which is typical in hemophilia.
A normal coagulation profile would show normal values for both PTT and PT.

Choice B:

The client may have a vitamin K deficiency.
This choice is not applicable in this context.
A vitamin K deficiency primarily affects the extrinsic pathway of coagulation, leading to an elevated PT, but it does not typically affect the PTT, which is prolonged in hemophilia.

Choice C:

The client likely has hemophilia.
This is the correct choice.
Hemophilia is characterized by a deficiency or dysfunction of clotting factors, most commonly factor VIII (hemophilia A) or factor IX (hemophilia B)
Prolonged PTT with normal PT is a classic laboratory finding in hemophilia.

Choice D:

The client is at risk for thrombosis.
This choice is incorrect.
Hemophilia is associated with bleeding tendencies, not an increased risk of thrombosis.
Prolonged PTT indicates a reduced ability to form clots, making thrombosis less likely.

Discussion Section

Posting as Anonymous or Login Here

0 Pulse Checks

No comments

Sign Up or Login to view all the 15 Questions on this Exam

Join over 100,000+ nursing students using Naxlex’s science-backend flashcards, practice tests and expert solutions to improve their grades and reach their goals.

Exam Review

Hemophilia Share this Share This Link Share on social media Facebook Messager WhatsApp Share URL Copy Close

Total Questions : 15

Sign Up or Login to view all the 15 Questions on this Exam

Hemophilia

Share This Link

Share on social media

Facebook

Messager

WhatsApp

Share URL
Copy