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Questions and Answers
Which modality should be excluded during the subacute phase of physical therapy after 48 hours?
Which modality should be excluded during the subacute phase of physical therapy after 48 hours?
What type of exercises should start once the joint is pain free through its full range?
What type of exercises should start once the joint is pain free through its full range?
Which of the following is NOT a basic element of a physiotherapy exercise program in hemophilia?
Which of the following is NOT a basic element of a physiotherapy exercise program in hemophilia?
What is the preferred type of exercise for initial strengthening in an open chain?
What is the preferred type of exercise for initial strengthening in an open chain?
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What is the recommended duration for holding a stretch in static stretching?
What is the recommended duration for holding a stretch in static stretching?
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What is the primary cause of Hemophilia A?
What is the primary cause of Hemophilia A?
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Which type of bleeding is most commonly associated with hemophilia?
Which type of bleeding is most commonly associated with hemophilia?
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What is the role of factor II, known as prothrombin, in hemostasis?
What is the role of factor II, known as prothrombin, in hemostasis?
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Which coagulation factor is referred to as the Christmas factor?
Which coagulation factor is referred to as the Christmas factor?
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What percentage of the hemophilia population does Hemophilia A represent?
What percentage of the hemophilia population does Hemophilia A represent?
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What is a rare type of hemophilia caused by a deficiency of factor XI?
What is a rare type of hemophilia caused by a deficiency of factor XI?
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Which of the following factors is not assigned a specific role in blood clotting?
Which of the following factors is not assigned a specific role in blood clotting?
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What is the major cause of death in the hemophilic population?
What is the major cause of death in the hemophilic population?
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Which muscles are most commonly affected by bleeding in hemophilia?
Which muscles are most commonly affected by bleeding in hemophilia?
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What severe condition can result from bleeding into muscles?
What severe condition can result from bleeding into muscles?
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Which of the following is NOT a psychological impact faced by children with hemophilia?
Which of the following is NOT a psychological impact faced by children with hemophilia?
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What can recurring bleeds into a growing child's joint lead to?
What can recurring bleeds into a growing child's joint lead to?
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How does hemophilia potentially affect bone health?
How does hemophilia potentially affect bone health?
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What aspect of proprioception is notably impaired in people with severe hemophilia?
What aspect of proprioception is notably impaired in people with severe hemophilia?
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Which symptom is NOT commonly noticed in young children with hemophilia?
Which symptom is NOT commonly noticed in young children with hemophilia?
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During the assessment of hemophilia, what historical information is essential to gather?
During the assessment of hemophilia, what historical information is essential to gather?
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What physical consequence can occur due to hyperemia from bleeding into a joint?
What physical consequence can occur due to hyperemia from bleeding into a joint?
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What is one possible indicator of limited movement in young children with hemophilia?
What is one possible indicator of limited movement in young children with hemophilia?
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What genetic condition is characterized by a defect in the gene responsible for the production of clotting factors VIII, IX, or XI?
What genetic condition is characterized by a defect in the gene responsible for the production of clotting factors VIII, IX, or XI?
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Hemophilia is inherited in what manner?
Hemophilia is inherited in what manner?
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Which of the following best classifies severe hemophilia based on clotting factor levels?
Which of the following best classifies severe hemophilia based on clotting factor levels?
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Which joints are most commonly affected by hemarthrosis in individuals with hemophilia?
Which joints are most commonly affected by hemarthrosis in individuals with hemophilia?
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What is a common initial sign of hemophilia observed after specific medical procedures?
What is a common initial sign of hemophilia observed after specific medical procedures?
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What can untreated joint bleeds lead to in individuals with severe hemophilia?
What can untreated joint bleeds lead to in individuals with severe hemophilia?
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How is soft tissue bleeding, such as in muscular tissue, commonly referred to?
How is soft tissue bleeding, such as in muscular tissue, commonly referred to?
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Which of the following factors is often assessed to classify hemophilia severity?
Which of the following factors is often assessed to classify hemophilia severity?
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Which of the following can be a symptom of joint bleeds associated with hemophilia?
Which of the following can be a symptom of joint bleeds associated with hemophilia?
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What is the term for chronic bleeding into the joint that can lead to hemophilic arthropathy?
What is the term for chronic bleeding into the joint that can lead to hemophilic arthropathy?
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What is the primary treatment for hemophilia aimed at addressing bleeding episodes?
What is the primary treatment for hemophilia aimed at addressing bleeding episodes?
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Which assessment method is NOT included in the evaluation for hemorrhage causative factors?
Which assessment method is NOT included in the evaluation for hemorrhage causative factors?
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What is the purpose of prophylactic treatment in hemophilia?
What is the purpose of prophylactic treatment in hemophilia?
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What is a recommended first step in managing an acute haemarthrosis?
What is a recommended first step in managing an acute haemarthrosis?
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What can be a consequence of delayed treatment of acute haemarthrosis?
What can be a consequence of delayed treatment of acute haemarthrosis?
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Which assessment technique helps to evaluate balance and coordination?
Which assessment technique helps to evaluate balance and coordination?
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Which of the following is a goal of physical therapy for patients with hemophilia?
Which of the following is a goal of physical therapy for patients with hemophilia?
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How often can ice packs be applied during treatment for acute bleeding episodes?
How often can ice packs be applied during treatment for acute bleeding episodes?
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What role do splints or compression bandages play in acute haemarthrosis treatment?
What role do splints or compression bandages play in acute haemarthrosis treatment?
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What type of physical therapy may be utilized for managing pain in patients with hemophilia?
What type of physical therapy may be utilized for managing pain in patients with hemophilia?
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Study Notes
Hemophilia
- Hemophilia is an X-linked congenital bleeding disorder
- Caused by deficiency of coagulation factor VIII (in hemophilia A) or factor IX (in hemophilia B)
- People with hemophilia have lower clotting factor levels or impaired activity of coagulation factors needed for normal clotting
- Hemorrhages occur frequently in joint cavities, but also in muscles and mucous membranes (mouth, gums, nose) and central nervous system
- Central nervous system hemorrhages are uncommon, but a major cause of death in hemophilia patients
- Hemophilia A is the most common type (85% of cases), caused by a deficiency of factor VIII
- Hemophilia B is caused by deficiency of factor IX
- Hemophilia C (rare type) is caused by a deficiency of factor XI
Normal Hemostasis
- In normal hemostasis, blood cells and substances form clots, stopping bleeding
- Clotting factors are proteins produced by cells lining blood vessel walls
- Key components include platelets, vitamin K, and fibrinogen
- Platelets are blood cells produced in bone marrow, essential for blood clot formation
Blood Clotting Factors
- Factor I - fibrinogen
- Factor II - prothrombin
- Factor III - tissue thromboplastin (tissue factor)
- Factor IV - ionized calcium (Ca++)
- Factor V - labile factor or proaccelerin
- Factor VI - unassigned
- Factor VII - stable factor or proconvertin
- Factor VIII - antihemophilic factor
- Factor IX - plasma thromboplastin component, Christmas factor
- Factor X - Stuart-Prower factor
- Factor XI - plasma thromboplastin antecedent
- Factor XII - Hageman factor
- Factor XIII - fibrin-stabilizing factor
Types of Hemophilia
- Hemophilia A is the most common type, with a deficiency of factor VIII
- Hemophilia B is caused by a deficiency in factor IX
- Hemophilia C is a rare, milder form of the condition, and presents with a factor XI deficiency
Hemophilia and Genetics
- Hemophilia is an inherited genetic condition
- Caused by a defect in the gene that determines how the body produces factors VIII, IX, or XI
- The gene is located on the X chromosome, making it an X-linked recessive disorder
- The condition is passed down genetically
Clinical Classification of Hemophilia
- Hemophilia is classified as mild, moderate, or severe based on the degree of clotting factor deficiency
- This classification is compared to the general population's levels and the bleeding stimulations
Mild Hemophilia
- Predisposition to bleeding with injury
- Haemoarthrosis (bleeding into joints) is rare
- Factor level is greater than 5-40% of normal
Moderate Hemophilia
- Predisposition to bleeding with slight injury
- Haemoarthrosis occurs sometimes
- Factor level is 1-5% of normal
Severe Hemophilia
- Predisposition to spontaneous bleeding with little or no trauma
- Haemoarthrosis is very frequent
- Factor level is less than 1% of normal
Clinical Manifestation of Hemophilia
- The most common manifestation is hemarthrosis (bleeding into joints), with ankles, knees, and elbows commonly affected
- Shoulders, wrists, and hips may also be affected
Initial Signs of Hemophilia
- Persistent bleeding after circumcision, other surgeries, or blood tests
- Unexplainable recurrent bruises
- Hesitation to use a limb
- Bleeding from cuts and scratches that takes a long time to stop
- Swelling of a joint after injections (e.g., immunizations)
- Frequent nosebleeds
Joint Bleeds (Hemarthroses)
- Spontaneous or trauma-induced joint bleeds
- Main causes of chronic pain and disability in severe hemophilia
Symptoms of Joint Bleeds
- Symptoms may not be apparent immediately
- Chronic bleeding breaks down the joint lining (synovium), causing damage
- Leads to hemophilic arthropathy (painful arthritic condition)
Soft Tissue Bleeding
- Bleeding in muscles (hematomas)
- Iliopsoas, quadriceps, gastrocnemius-soleus, and forearm muscles are commonly affected
- Bleeding into muscles can cause severe problems, including compartment syndrome
- Bleeding into joints, soft tissues, and muscles is more common after age two
Psychological Disturbance
- Children with hemophilia may feel different and isolated
- Daily fluctuations in disease symptoms may affect mood and coping abilities
Growth Abnormalities
- Recurrent bleeds into the same joint of a growing child can lead to growth abnormalities
- Epiphyses (bone growth plates) are open in children, making them susceptible to growth abnormalities
- Bleed into a joint causes hyperemia, which stimulates growth
Lower Bone Mineral Density
- Hemophilia can result in lower peak bone mass and bone mineral density (BMD)
- This is often due to avoidance of weight-bearing physical activity during childhood and adolescence
Proprioception
- Proprioception is impaired in people with hemophilia
- Static proprioceptive performance (measured through single limb stance) is significantly decreased in patients with severe hemophilia compared to healthy subjects
Signs in Young Children with Hemophilia
- Irritability
- Crying
- Favoring a limb
- Refusing to walk
- Limited movement (mobility) of the joint
- Refusing to straighten, weight bear, or use the limb
Assessment of Hemophilia
- Examination should include historical information about bleeding frequency and location, and the patient's normal activities
- Essential for identifying causative factors and designing a program to reduce hemorrhage frequency and severity
Assessment Includes
- Muscle tests
- Leg length measurement
- Girth measurement (joints and muscles)
- Gait assessment
- Pain assessment
- Palpation
- Balance and coordination assessment
- Gross motor development
Management of Hemophilia
- I. Medical management includes intravenous factor replacement (either on demand or prophylactically)
- II. Physical therapy treatment focuses on maintaining strength and range of motion (ROM) in all joints, and preventing or diminishing disability
Physical Therapy of Acute Haemarthrosis (PRICE)
- Protection
- Rest (bed rest for 1 day)
- Ice (20 minutes, every 4-6 hours)
- Compression (splints, slings, bandages)
- Elevation (3-4 days)
Treatment Stage Includes
- TENS (transcutaneous electrical nerve stimulation)
- Movement in pain-free ranges
- Hydrotherapy
Subacute Phase Management (after 48 hours)
- Modalities (ice, TENS, splints)
- Progressive movement and exercises
- Progressive weight bearing (within range of motion)
- Proprioceptive exercises
- Hydrotherapy (gait training)
Basic Elements of Physiotherapy Exercises Program in Hemophilia
- Strength training (isometric, isotonic, isokinetic) with progressive resistance
- Flexibility exercises (static, dynamic, PNF)
- Balance and proprioceptive training (starting with stable surfaces and progressing to unstable ones)
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Description
This quiz explores the concepts of hemophilia, an X-linked bleeding disorder, and the process of normal hemostasis. Delve into the causes, types, and effects of hemophilia, alongside understanding the essential mechanisms involved in clot formation and maintenance. Enhance your knowledge of clotting factors and their significance in cardiovascular health.
