The child with hematological dysfunction Defect in hemostasis hemophilia

Defect in hemostasis: hemophilia Hemostasis is the process that stops bleeding when a blood vessel is injured. The coagulation process is complex, clotting depend on three factors: 1. Vascular influence. 2. platelet role. 3. clotting factors

Defect in hemostasis: hemophilia Hemophilia: refer to group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of the blood. The symptomatology is similar regardless of which clotting factor is deficient The identification of specific factor deficiencies allows definitive treatment with replacement agents.

Hemophilia In about 80% of all cases of hemophilia ,The inheritance pattern is demonstrated as X-linked recessive. Forms of Hemophilia: Hemophilia A :Factor VIII Deficiency, or classic Hemophilia Hemophilia B: factor IX Deficiency, or Christmas disease. Van Willebrand disease (vWD) a deficiency ,abnormality, or absence of the protein called von Willebrand factor (vWF) and deficiency of factor VIII, affected male and female

Hemophilia: Pathophysiology Factor VIII is produced by the liver and is necessary for the formation of thromboplastin in phase 1 of blood coagulation. Individuals with Hemophilia have two of the three factors required for coagulation ,so they may bleed for longer periods, but not at a faster rate. Bleeding in SQ and IM IS COMMON. Hemarthrosis: bleeding into joint space is the most frequent type of internal bleeding. Bleeding in the neck, mouth, or thorax is serious,. Intracranial hge can have fatal consequences. Hge in GI can lead to anemia. Hge into retroperitoneal cavity is especially hazardous. Hge in the spinal cord lead to paralysis.

Hemophilia: diagnostic evaluation Prolonged hge is apparent: Hge from any trauma-loss of deciduous teeth, circumcision, epistaxic, injections Excessive bruising. SQ and IM hge. Hemarthrosis Hematomas. Spontaneous hge History of bleeding. Evidence of X-linked inheritance. Laboratory findings. PTT DNA testing

Hemophilia: therapeutic management The primary therapy for hemophilia is replacement of the missing clotting factor: 1. factor VIII concentration from pooled plasma . 2. DDAVP a synthetic form of vasopressin that increases plasma factorVIII and vWF levels 3. other drugs: - Corticosteroids for hematuria, acute Hemarthrosis, chronic synovitis. NSAIDs

Hemophilia: therapeutic management 4. Regular program of exercise and physical therapy 5. Treatment without delay results in more rapid recovery and decreased complication by: The family is taught the technique of venipuncture and to administer the AHF.

Hemophilia: prognosis There is no cure for hemophilia. May exposed to HIV and hepatitis transmission. Gene therapy may prove to be a treatment option in the future.

Hemophilia: nursing considerations 1. Prevent bleeding: Decrease risk of injuries: the environment should be safe as much as possible. Use of a protective equipment during sports. Dental care. These children should wear medical identification Neither aspirin nor any aspirin-containing compound should be used.

Hemophilia: nursing considerations 2. Recognize and control bleeding: Supportive measures may be implement as RICE: 1. rest. 2.ice. 3. compression. 4. elevation. 3. prevent crippling effects of bleeding: During bleeding episodes the joints is elevated and immobilized. Active range of motion exercises are usually instituted after the acute episode. Diet is also an important consideration

Hemophilia: nursing considerations 4.Support family and prepare for home care: Genetic counseling is essential as soon as possible after diagnosis Children are taught to take responsibility for their disease at an early age . Family should meet the health team frequently. Parents group discussion are beneficial in meeting those needs often best met by similarly affected families