2 When a girl inherits a mutated gene from either her father or mother she

2 when a girl inherits a mutated gene from either her

This preview shows page 53 - 56 out of 65 pages.

2. When a girl inherits a mutated gene from either her father or mother, she becomes a carrier of hemophilia and can pass the disease to her sons. c. A and P Review i. Blood Clots 1. When an injury causes a blood vessel wall to break, platelets are activated. They change shape from round to spiny, stick to the broken vessel wall and each other, and begin to plug the break. They also interact with other blood proteins to form fibrin. Fibrin strands form a net that entraps more platelets and blood cells, producing a clot that plugs the break d. Pathophysiology i. Factor VIII deficiency 1. Necessary for thromboplastin formation in phase one of coagulation ii. Effect is prolonged bleeding 1. SQ and IM hemorrhages 2. Hemarthrosis bleeding a. Into the joints 3. Anemias 4. GI bleeding 5. Intracranial hemorrhage e. Diagnosis of Bleeding Disorders i. Medical and familial history? f. Diagnosis i. Circumcision ii. Toddler years iii. History of bleeding iv. Blood test PT/PTT bleeding time v. Fibrinogen level vi. Platelet count vii. Factor VIII and IX assays viii. DNA testing for carrier status g. Classification of Hemophilia i. Classified as: 1. Mild 2. Moderate 3. Severe
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54 ii. Average clotting activity level 100% 1. Normal range 60-100% iii. Every patient should know their clotting factor activity level iv. Degree of severity tends to be consistent within a given family v. The severity of hemophilia depends on the specific mutation in the Factor gene. If the mutation is small, like a one letter change in the gene, then the mutation is small. If the mutation is larger, like a backward chunk, the symptoms are usually severe. Classes Severe Moderate Mild Factor Level <1% 1-3% factor 6-25% factor Tendency to bleed Can bleed without injury Can bleed with slight injury Bleeds with severe injury, surgery, invasive procedures Joints Usually had join involvement May have joint involvement Rarely has joint involvement X/wk May bleed 1-2 x/week May bleed 1 x/month May never have a bleeding problem h. Complications i. Hematomas ii. Bleeding from circumcision iii. Minor trauma—large intramuscular hematoma iv. Hemarthosis-hallmark sign v. Intracranial hemorrhages vi. Bleeding in neck area vii. Spontaneous hematuria viii. Hematomas along spinal cord ix. AIDS x. Hepatitis i. Bleeding into Joints i. What should you do immediately in the patient with hemophilia? Why? j. Development of Arthritis
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55 i. k. Emergency Treatment i. Never make a hemophilia patient wait to be treated. 1. The longer he/she waits, the longer he/she will continue to bleed. 2. Early treatment can prevent complications, pain and death. ii. Bleeding can cause 1. Irreversible complications 2. Pain 3. Death iii. Listen to the patient and /or his family 1. Their information can be valuable iv. Important 1. Never give IM injections except with pretreatment 2. Never apply heat to a bleeding area 3. Never perform invasive procedures without pretreatment of clotting factor 4. Avoid arterial punctures v. Therapeutic Management 1. Apply pressure for 10-15 minutes 2. Immobilize 3. Elevate 4. Apply cold 5. Factor administration a.
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