Haemophilia type A - Treatment

Treatment consists of preventing and combating hemorrhagic accidents. For this purpose specific substitution factor is deficient according to its lifetime, it is possible lifetime.
Curative treatment provides control bleeding accidents reported.Prophylactic therapy includes avoidance of high physical effort.Prophylactically are provided subject to the following contraindications:- Medical maneuvers bleeding: intra-arterial, intramuscular, or haemarthroses hematoma puncture, cauterization;- Restriction surgery ENT, dental (only with special training);- Eviatarea administration of aspirin or nonsteroidal anti-inflammatory (NSAID) that causes the lining of digestive bleeding, in particular;- Tetanus vaccinations, BCG, DPT, polio are normal;- Avoid prolonged immobilisation as leading to ankylosis;- Avoid circular plaster device;- Limiting hospitalization.Substitution treatment is essential to assessing the haemorrhagic accidents:- Light bleeding when factor VIII has a value of 20%;- Average bleeding when factor VIII has a value of 30%;- Severe bleeding when factor VIII has a value of 40-50%.- Severe bleeding when 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 clotting time value 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, lyophilized plasma has a concentration of 1UI/ml and EEC antihemofilica presents a concentration of factor VIII 2UI/ml; crioprecipitatul has 4UI/ml, vials containing human antihemofilica 25-50UI/ml with globulin, and the animal - 200UI/ml.
Half-life is necessary to know the 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.Izoimunizarii Prevention is the intra-venous infusion 15-30 minutes fast. Factor VIII transfusion requirements 1UI / kg increased by 2% concentration of factor VIII in severe environments - factor VIII is administered at 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 are 3-6 transfusion, retroperitoneal, 3-6 transfusions, retropharyngeal, 3-6 transfusions, intracranial, 7-14 transfusions.Adjuvant corticosteroid therapy 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, bleeding per-orally for gums, epistaxis, bleeding alveolo-dental (contraindicated in hematuria).It plans to avoid aspirin, indomethacin, a novocaine, morphine, papaverine has.
Sealant is made by: compression, administration of thrombin GELASPON and ice pack.
It is recommended inteventia surgical, orthopedic, functional rehabilitation and gym.Local epistaxis treated by performing compression wash with saline and ten minutes, mesa with thrombin.Cauterization substitution therapy is contraindicated, is used only in extreme cases.Post-extraction bleeding stops by: washing mouth, administration of thrombin GELASPON local performing transfusion of factor VIII 1-2, Epsilon aminocaproic ACID administration for 10-15 days (protects the clot) and corticoterpie with prednisone for a period 3-4 days. It is indicated for 5-9 days tracking (daily) because there is risk of late 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, school and professional training for professions without physical effort, mental health: limiting restrictions of school activities (tailored to their abilities) and expansion of home care.
Prophylactic treatment consists of substitution treatment at home in 1-3 16-32U/kgc/saptamana doses / week to make quality of life better, to avoid accidents repetitive bleeding, functional sequelae.This treatment provides a number of risks: izoimunizarea, increased costs in severe factor VIII less than 1% frequency of 3-4 accidents bleeding / month.Bear in mind that genetic counseling is radical.
Mortality is present in a 3%.Functionality influence prognosis:- Disabling chronic arthropathy haemophilia in leading to stiffness;- Muscle: motor invalidation;- Sensitive: deafness, blindness;- Hemiplegia, epilepsy.
Disease complications are:- Mechanical compression that causes paralysis, pathological fractures;- Iatrogenic - HIV, HBV, HCV antibodies circulating factor VIII (10-20%), transfusion.