Hemophilia A: Causes and Clinical Manifestations

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Questions and Answers

What is the correct sequence of the hemostasis process?

Coagulation, Vascular Spasm, Platelet Plug Formation

Coagulation, Platelet Plug Formation, Vascular Spasm

Vascular Spasm, Platelet Plug Formation, Coagulation (correct)

Platelet Plug Formation, Vascular Spasm, Coagulation

Which component is NOT a key element in the hemostasis process?

Fibrin

Clotting Factors

Red Blood Cells (correct)

Platelets

What is the purpose of fibrinolysis in the hemostasis process?

To promote vascular spasm

To enhance fibrin formation

To initiate platelet activation

To dissolve the clot once the vessel is healed (correct)

What is a primary risk factor associated with clotting disorders?

Advanced age Signup and view all the answers

How should nursing care for clients with cellular regulation disorders be characterized?

Evidence-based and culturally sensitive Signup and view all the answers

What is a common clinical manifestation of Polycythemia Vera?

Severe hypoxia Signup and view all the answers

Which of the following lab results would indicate a diagnosis of Polycythemia Vera?

Hematocrit > 55% Signup and view all the answers

Which factor deficiency is associated with Hemophilia A?

Factor VIII Signup and view all the answers

What condition is characterized by an abnormality of the von Willebrand Factor?

Von Willebrand Disease Signup and view all the answers

Which of the following symptoms can indicate Hyperkalemia as seen in Polycythemia Vera?

Muscle weakness Signup and view all the answers

What is the primary cause of prolonged bleeding in hemophilia A?

Deficiency of factor VIII Signup and view all the answers

Which of the following is NOT a common clinical manifestation of hemophilia A?

Swollen lymph nodes Signup and view all the answers

What is involved in the diagnosis of hemophilia A?

DNA testing for inheritance patterns Signup and view all the answers

What type of therapy is primarily used to treat deficiency of factor VIII in hemophilia A?

Factor VIII concentrates Signup and view all the answers

Which drug should be avoided in the treatment of hemophilia A due to its impact on bleeding risk?

Aspirin Signup and view all the answers

How do individuals with hemophilia typically express bleeding severity?

Based on the degree of bleeding severity Signup and view all the answers

What is the role of regular exercise and physical therapy in patients with hemophilia A?

To prevent chronic joint bleeding effects Signup and view all the answers

What factor affects the likelihood of spontaneous bleeding episodes in individuals with hemophilia A?

Severity of factor deficiency Signup and view all the answers

What is the primary goal of prophylactic therapy in hemophilia?

Reduce the frequency of joint bleeds Signup and view all the answers

Which of the following treatment strategies is NOT part of bleeding management for hemophilia?

Regularly scheduled imaging tests Signup and view all the answers

Which type of thrombocytopenia is typically associated with autoimmune disorders?

Immune Thrombocytopenia Purpura Signup and view all the answers

What is the platelet count threshold that defines thrombocytopenia?

150,000 platelets per microliter Signup and view all the answers

Which complication is associated with the presence of antiplatelet antibodies in thrombocytopenia?

Tissue ischemia in major organs Signup and view all the answers

What should be monitored as part of preventing complications in patients with hemophilia?

Signs of bleeding complications Signup and view all the answers

How does heparin-induced thrombocytopenia typically manifest?

5-14 days after starting heparin Signup and view all the answers

What is the prognosis for hemophilia patients without any treatment?

Poor, potentially leading to shortened life expectancy Signup and view all the answers

What is one potential future treatment for hemophilia being researched?

Gene therapy Signup and view all the answers

Which of the following is not a cause of thrombocytopenia?

Increased oxygen levels in blood Signup and view all the answers

What is a characteristic of the preferred conflict resolution style for nurses?

Values win-win resolutions Signup and view all the answers

In which scenario should a nurse say ‘no’ assertively?

If it conflicts with patient care plans Signup and view all the answers

What is the best approach for a chair dealing with a member who dismisses group ideas?

Encourage positive group membership from the member Signup and view all the answers

What emotional tone should be avoided in an e-mail to maintain professionalism?

Hostility and aggression Signup and view all the answers

What trait is linked to resilience when nurses assertively refuse requests?

Maintaining clear boundaries for patient care Signup and view all the answers

Which conflict resolution style focuses on mutual problem-solving and cooperation?

Collaboration Signup and view all the answers

What is the first step in effective conflict resolution?

Identifying conflict management styles Signup and view all the answers

What technique involves rephrasing a speaker's message to enhance understanding?

Paraphrasing Signup and view all the answers

Which listening step refers to making judgments about the information received?

Judging Signup and view all the answers

Which communication technique is essential for effectively managing interpersonal conflict?

Using 'I' messages Signup and view all the answers

What type of conflict occurs between different groups or organizations?

Inter-group or Organizational Signup and view all the answers

What method should be avoided to promote a positive environment during conflict resolution?

Interruption and distraction Signup and view all the answers

Which technique focuses on expressing feelings without assigning blame during communication?

Reflecting emotions Signup and view all the answers

What factor is most likely to undermine patient trust in communication?

Providing conflicting messages Signup and view all the answers

How does body language influence communication?

It can create confusion if inconsistent with speech Signup and view all the answers

What is a major consequence of offering unwarranted reassurance in nursing communication?

Undermines patient trust Signup and view all the answers

Which response is most likely to be considered nontherapeutic in the context of patient statements about feelings of failure?

You have everything to live for. Signup and view all the answers

What communication skill can enhance the effectiveness of e-mails to a supervisor?

Crafting a clear subject line Signup and view all the answers

Which of the following best describes a logical fallacy in communication?

Making flawed arguments that lead to misunderstandings Signup and view all the answers

What does filtering refer to in the communication process?

The selective perception of information by the receiver Signup and view all the answers

Which communication technique is least effective in resolving conflicts?

Responding with generalized answers Signup and view all the answers

Study Notes

Hemophilia A

Deficiency of factor VIII (antihemophilic factor AHF)
Factor VIII is produced in the liver and is needed for the formation of thromboplastin in phase I of blood coagulation.
Individuals with hemophilia had ⅔ of the factors required for coagulation.

Hemophilia Pathophysiology

Vascular Influence
Platelets
Bleeding time may be longer in individuals with hemophilia but the bleeding rate is not faster.
Expression of bleeding severity varies from severe, moderate to mild.

Hemophilia Clinical Manifestations

Prolonged bleeding anywhere from or in the body.
Hemorrhage from trauma including loss of deciduous teeth, circumcision, cuts, epistaxis, injections.
Excessive bruising, even from slight injury, such as a fall.
Subcutaneous and intramuscular hemorrhages.
Hemarthrosis, especially knees, ankles, elbows.
Hematomas: pain, swelling, limited movement.
Spontaneous hematuria

Hemophilia Diagnostics

History of bleeding episodes.
Evidence of X-linked inheritance.
⅓ of cases are new mutations.
DNA Testing
Laboratory findings:
- Bleeding Time
- Factor Assays
- PTT
- Platelets
- PT
- Thrombin Time

Hemophilia Treatment

Primary treatment is replacement of missing clotting factors.
- Factor VIII concentrates
1-deamino-8-d-arginine vasopressin (DDAVP)
Antifibrinolytic Therapy
- Tranexamic acid (Cyklokapron)
Aggressive factor concentrate replacement therapy
- Prevent chronic crippling effects from joint bleeding.

Hemophilia Treatment - Other Drugs

Corticosteroids
Avoid aspirin and traditional NSAIDs
Cyclooxygenase-2 (COX-2) inhibitors, selective NSAIDs
E-aminocaproic acid (Amicar)

Hemophilia Treatment - Prognosis

No cure for hemophilia.
Symptom management.
With treatment, individuals can have an average life expectancy.
Prior to 1975, patients may have been exposed to HIV
Gene therapy may be a future option.

Hemophilia Nursing Interventions

Bleeding prevention and management.
Monitor for signs of bleeding complications.
Administration of clotting factor replacement therapy.
Injury prevention and self-care measures.
Pain management during bleeding episodes.
Promote physical mobility and joint protection.
Ensure compliance with treatment plans and medication regimens.
Educate and support the family.
Prepare for Home Care

Thrombocytopenia

A reduction of platelets < 150,000
Caused by:
- Impaired platelet production
- Increased platelet destruction
- Abnormal platelet distribution

Thrombocytopenia - Types

Immune Thrombocytopenia Purpura (ITP)
Thrombotic Thrombocytopenia Purpura (TTP)
Heparin- Induced Thrombocytopenia Purpura (HIT)

Thrombocytopenia - Causes

Immune Thrombocytopenia Purpura (ITP)
- May be autoimmune-related or d/t drug toxicities
- Usually acute in children, chronic in adults.
- Decreased platelets
- Sometimes related to autoimmune disease
Thrombotic Thrombocytopenia Purpura (TTP)
- Increase in platelet aggregation
- Platelets in circulation decrease
Heparin-Induced Thrombocytopenia Purpura (HIT)
- Usually 5-14 days after starting heparin.
- Unexplained platelete decrease by >50%, OR decrease in platelets in circulation
- Can be fatal
- Increased risk for kidney damage, myocardial infarction, stroke

Thrombocytopenia - Clinical Manifestations

Common for all types:
- Petechiae
- Purpura
- Easy bruising
- Epistaxis
- Heavy menses
- Gum bleeding

Thrombocytopenia - Clinical Manifestations

Presence of antiplatelet antibodies in blood
Small microclots block capillaries causing tissue ischemia in major organs.

Thrombocytopenia - Specific Pathophysiology

Immune Thrombocytopenia Purpura (ITP)
- Usually acute in children, chronic in adults.
- Decreased platelets
- Sometimes related to autoimmune disease
Thrombotic Thrombocytopenia Purpura (TTP)
- Increase in platelet aggregation
- Platelets in circulation decrease

Thrombocytopenia - Heparin-Induced Thrombocytopenia Purpura (HIT)

Usually 5-14 days after starting heparin.
Unexplained platelete decrease by >50%, OR decrease in platelets in circulation
Can be fatal.
Increased risk for kidney damage, myocardial infarction, stroke
Small microclots block capillaries causing tissue ischemia in major organs.

Communication Process

All communication contains information that is filtered out by the receiver.
The receiver's response is dynamic, circular, involving interpretation and filtration.

Verbal vs Non-Verbal Communication

Verbal communication includes talking and listening.
Non-verbal communication includes facial expressions, body language, dressing style, lifestyle and material possessions.
Non-verbal communication can confirm or deny the spoken word often conveying conflicting messages.

Blocking Communication

Blocking communication may be achieved through noncommittal or generalized answers.
False assurances can undermine patient trust.
Conflicting messages can confuse patients and hinder patient care.
Logical fallacies can lead to misunderstandings and poor decision-making.

Email Communication

Ensure accuracy, attention to detail, thoroughness and conciseness in email communication.

Communication Styles

The passive communication lacks decisiveness, avoids confrontation, and is inhibited.
The aggressive communication style is very decisive, often creating or escalating conflict.
The assertive communication style is the preferred communication style for nurses. It approaches conflict directly and respectfully, shows self-confidence and value in others' views, and seeks win-win resolution.

Saying “No” Assertively

Saying “no” assertively can be a stress management technique linked to resilience.
Saying "no" is appropriate when tasks are outside your scope of practice, when requests are unreasonable, when you feel unsure, if saying yes will negatively affect your shift plan and impact patient care, when you don’t want to, or if the request is not consistent with the values of the organization.

Conflict Resolution

Conflict occurs when interaction becomes stressful, taking on a competitive, or oppositional nature.
The first step in conflict resolution is recognizing how individuals manage conflict.

Conflict Resolution Styles

Avoidance style is the most common conflict resolution style.
Accommodation, force, compromise, collaboration, and avoidance styles are all commonly used in conflict resolution.

Active Listening Techniques

Position and attention, paraphrasing, reflecting emotion, questioning, acknowledging, summarizing, framing, and reframing are active listening techniques.

Interdisciplinary Communication

Interdisciplinary communication involves the application of critical thinking skills and an understanding of the levels of discipline among other professionals.
Interdisciplinary communication is essential for clear and effective communication.

Conflict Description

Conflict can be classified by the level of stress and hostility involved.
Conflict can be inevitable and either positive or negative

Types of Conflict

Intrapersonal/Individual conflict occurs within the person.
Interpersonal Conflict occurs between two or more people and can contribute to burnout and work-related stress.
Inter-group or Organizational conflict occurs between two or more groups of people, departments or organizations.

Conflict Resolution Techniques:

Use “I” messages, maintain eye contact, pay attention to body language, explain, not excuse, and follow through.

Therapeutic Communication Techniques

Paraphrasing, reflecting, open questioning, acknowledging, summarizing, framing, and reframing are all therapeutic communication techniques.

Assertive Communication

Utilize an open, honest, and direct problem-solving approach.
Focus on the cause of the conflict, not personalities.
Gather all the information needed.
Differentiate between fact and opinion.
Maintain a private setting and neutral territory
Establish ground rules.
Listen carefully, avoid prejudging, and avoid dwelling on the conflict itself.

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Description

This quiz covers the essential aspects of Hemophilia A, including its pathophysiology, clinical manifestations, and diagnostic criteria. It will help you understand the role of factor VIII, the severity of bleeding in affected individuals, and the various symptoms that may arise. Test your knowledge on this important blood coagulation disorder.