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July 08, 2018 – 09:00
By:
Valeria Araque Yepes, special for El País
Recurrent internal or external haemorrhage, inflammation and pain are some of the symptoms that can occur if you have haemophilia. This condition, characterized by the persistence of haemorrhages difficult to control, is considered, according to Miguel Antonio Escobar, hematologist at the Universidad Libre and Medical Director of the University of Texas Hemophilia Center. , an orphan and hereditary disease, and therefore, non-transmissible, which affects blood clotting and can be fatal if it attacks vital organs such as the brain.
"What happens to hemophilia patients is that they lack or do not have enough factor VIII or IX, the ce are essential proteins responsible for blood clotting that are being made correctly, "adds César Garrido, board member of the World Federation of Hemophilia.
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These proteins play a crucial role in the process so that
the blood coagulates properly and controls the bleeding. These then work, that is to say by activating one by one, without interruption.
Generally women are the carriers and transmitters of this condition, because the defective gene is on the X chromosome, that is, the chromosome that is linked to the female bad. That's why men, in most cases, suffer from it (men have an X and Y chromosome), explained specialists at the Roche Press Day 2018, which was held this week in Cali
. of some of the factors, hemophilia can be clbadified into two types:
-Hemofilia A: caused by lack of factor VIII.
– Hemophilia B: caused by the absence of factor IX.
Both hemophiliacs A and B may be mild, moderate or severe.
Signs
Caesar Garrido and Dr. Escobar mention some of the manifestations:
– Internal or external bleeding: cause pain and inflammation.
– Joint bleeding: is characterized by the sensation of heat, inflammation
and pain in the area. Without prompt treatment, the remaining blood deposits damage the tissues and synovial membrane (responsible for lubricating the articular surface of the joint and providing nutrients to the articular cartilage), resulting in some cases in deformities or deformities. disabilities such as osteoarthritis
– Frequent bruising of the body causing pain.
– Extended bleeding after a cut or stroke
– Spontaneous bleeding
Treatment
While there is currently no cure or a means of preventing this disease, there is significant advances in treatment, which improve the quality of life of patients and allow them to access appropriate care such as:
– Home treatment: which refers to the application, by way of intravenous, missing factor after a stroke or injury. In many cases, the same patient applies it, because acting quickly in these situations is vital.
– Prophylaxis: which is applied preventively to raise the levels of protection and the individual can maintain his normal activities.
According to the hematologist Miguel Antonio Escobar, both treatments require frequent intravenous infusions, at least several times a week, in order to prevent bleeding.
On the other hand, César Garrido explains that these patients must learn to live with this disease and avoid sports that require a lot of contacts
Data key
According to Miguel Antonio Escobar, medical director of the Center de In hemophilia at the University of Texas, a large number of people with "severe hemophilia A" develop inhibitors (antibodies that attack and destroy factor VIII substitution), reducing Treatment efficacy or cancels it completely. What
implies the need for more frequent or alternative treatments:
– Immune tolerance induction (ITI): Frequent administration of factor VIII over a prolonged period to help the body tolerate the factor
– Agents bypbad: short-term dose, used in case of ITI failure.
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