Haemophilia type A Treatment

Haemophilia type A
Treatment
Treatment consists of preventing and combating hemorrhagic accidents. For this purpose specific substitution factor is deficient according to the duration of its life, all life possible.
Curative treatment of bleeding provides combat casualties reported. Prophylactic therapy includes avoiding high physical exertion. Prophylaxis are provided in compliance with the following contraindications: - Medical maneuvers bleeding: intraarterial, intramuscular hematomas or haemarthroses puncture, cauterization; - Restriction of ENT surgery, dental care (only with special training); - Eviatarea administration of aspirin or nonsteroidal anti-inflammatory (NSAID) that causes bleeding from the gastrointestinal mucosa, in particular; - Tetanus vaccination, BCG, DPT, polio are normal; - Avoid prolonged immobilization as leading to ankylosis; - Avoid circular plaster device; - Limiting hospitalization. Substitution therapy is essential to assessing the haemorrhagic accident: - Light bleeding when factor VIII has a value of 20%; - Average bleeding when factor VIII has a value of 30%; - Severe bleeding when the factor VIII has a value of 40-50%. - Severe bleeding when the factor VIII has a value of 100% and over.
If the amount of clotting time (CT) exceeds 40 minutes, when factor VIII is less than 0. 1%. When the amount of clotting time is in the range 18-40 minutes, factor VIII is the percentage of 1%.
A special importance is given knowledge of factor VIII concentration of the preparation used. Thus, whole blood has a concentration of factor VIII 5. 8UI/ml, is a lyophilized plasma concentration 1UI/ml and cee antihemofilica shows a 2UI/ml concentration of factor VIII, cryoprecipitate has 4UI/ml, vials containing human antihemofilica 25-50UI/ml with globulin, and the animal - 200UI/ml.
Half-life knowledge is required to treatment, whose value is 10-16 hours: 3-4 doses / day and 2 severe informele doze/12 hours later. Fever is known to increase the need for vitamin D. Prevention of isoimmunization is the intra-venous infusion 15-30 minutes fast. Factor VIII transfusion requirements 1UI / kg increased by 2% concentration of factor VIII in medium forms - the factor VIII dose is administered 20UI/kgc necessary.
Control bleeding if haemarthroses 1-3 provides for the transfusion factor VIII if the existence of muscle hematomas are suficienete 1-2 transfusions, fecal nerve compression transfusions are 3-6, 3-6 retroperitoneal-transfusion-retropharyngeal 3-6 transfusions, intracranial, 7-14 transfusions. Adjuvant corticosteroid treatment provides for the administration of prednisone at a dose of 0. 5-1mg/kgc/zi per-orally for 3-4 days, when there haemarthrosis, haematuria, bleeding post-dental extractions. Epsilon aminocaproic ACID is administered at a dose of 100-400mg/kgc/zi, if per-oral gingival bleeding, epistaxis, bleeding alveolo-dental (contraindicated in haematuria). It aims to avoid aspirin, indomethacin has, of novocaine, the morphine, papaverine.
Local hemostasis is made by: compression, administration of thrombin and GELASPON ice pack.
It is recommended inteventia surgery, orthopedic surgery, functional rehabilitation and gym. Nosebleeds can be treated locally by performing compression lavage with saline and ten minutes, mesa with thrombin. Substitution therapy is contraindicated cauterization, is used only in extreme cases. Post-extraction bleeding stops by: cleaning the socket, the administration of thrombin GELASPON local, 1-2 make Factor VIII transfusion, Epsilon aminocaproic ACID administration for 10-15 days (protects the clot) and prednisone for a period corticoterpie 3-4 days. Tracking is indicated for 5-9 days (daily) because there is risk of delayed bleeding.
Treatment for hematuria consists of: rest, diuresis cure, substitution treatment.
If haemarthrosis patient is immobilized in physiological position, place ice on the affected joint and is substitution treatment.
Take a series of physical and psychosocial measures: homes 5-10 years, educational and vocational training for professions without exercise, mental health: restrictions limiting school activities (tailored to their abilities) and expansion of home care.
Prophylactic treatment consists 16-32U/kgc/saptamana substitute in 1-3 home doses per week to make a better quality of life, accident avoidance repetitive bleeding, functional sequelae. This treatment provides a number of risks: isoimmunization, in severe cases increased cost factor VIII less than 1% bleeding frequency of 3-4 accidents per month. Bear in mind that genetic counseling is as radical.
Mortality is present in a rate of 3%. Functionality influence prognosis: - Disabling in haemophilia arthropathy leading to chronic stiffness; - Muscle: motor defeat; - Sensing: deafness, blindness; - Hemiplegia, epilepsy.
Disease complications are: - Mechanical compression that causes paralysis, pathological bone fractures; - Iatrogenic - HIV, HBV, HCV circulating antibodies against factor VIII (10-20%), posttransfuzionale.