Questions and Answers
Preventing bleeding episodes primarily involves strengthening muscles and joints through appropriate exercises.
True
It is important to restrain children from mastering motor skills to prevent injuries.
False
Close supervision during playtime is not necessary if the environment is safe.
False
The family often requires assistance to prepare a child with hemophilia for school.
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Only boys can have hemophilia, which affects their participation in certain sports.
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Protective equipment is discouraged during athletic activities for children with hemophilia.
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Children with severe hemophilia can engage in contact sports like boxing and football.
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Swimming and golf are examples of noncontact sports suitable for children with hemophilia.
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Kneepads and helmets are examples of protective equipment for children participating in sports.
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Maintaining physical activity is not important for children with hemophilia.
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Study Notes
Overview of Hemophilia
- Hemophilia encompasses a range of bleeding disorders due to congenital deficiencies or dysfunctions of coagulation proteins.
- Approximately 80% of hemophilia cases follow an X-linked recessive inheritance pattern.
- The two primary types are hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency), with von Willebrand disease being another significant hereditary bleeding disorder.
Pathophysiology
- Hemophilia A is characterized by a deficit in factor VIII, essential for thromboplastin formation in blood coagulation.
- The severity of hemophilia correlates with the amount of factor VIII present; lower levels result in more severe symptoms.
Clinical Manifestations
- Bleeding severity can vary widely, with around 60-70% of affected children having severe forms.
- Common symptoms include prolonged bleeding, especially after minor injuries, and hemarthrosis (bleeding into joint cavities) typically affecting knees, elbows, and ankles.
- Early hemarthrosis signs include joint stiffness and pain; severe cases present with swelling and limited mobility.
- Spontaneous bleeding can occur, including hematuria and less frequently, epistaxis.
- Internal bleeding risks include GI hemorrhage, retroperitoneal bleeding, and intracranial hemorrhages, which can be fatal.
Diagnostic Evaluation
- Diagnosis relies on a history of bleeding episodes, evidence of X-linked inheritance, and abnormal laboratory tests, including a prolonged PTT.
- Specific assays are used to measure factor VIII and factor IX levels.
- DNA testing is available for carrier detection in families with hemophilia.
Therapeutic Management
- Treatment focuses on bleeding prevention and prompt intervention to mitigate tissue damage.
- Factor VIII replacement can be administered through fresh blood or concentrated forms; dosing is calculated based on plasma volume.
- Repeat infusions may be needed to maintain factor VIII activity levels.
Adjunct Therapies
- Management strategies depend on the site of bleeding:
- Joint: Rest, ice, elevation, immobilization, physiotherapy.
- Soft tissue: Ice and elevation.
- Muscle: Similar to soft tissue with additional bed rest for iliopsoas muscle injuries.
- Mucous membranes: Pressure application for nosebleeds, topical agents, or nasal packing.
Nursing Care Management
- Prevention of bleeding includes reducing injury risk through safe environments and supervision during play.
- Encourage age-appropriate exercises to strengthen muscles and joints while allowing for natural motor skill development.
- Support families in preparing children for school integration regarding activity limitations.
- Recommend protective gear for physical activities and guide families toward noncontact sports while advising against high-risk sports like boxing and football.
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Description
This quiz explores the various types of hemophilia, their genetic inheritance patterns, and the underlying coagulation factors involved. Learn about hemophilia A and B, along with Von Willebrand disease, and understand how these disorders affect individuals. Test your knowledge on this critical subject in hematology.
