April 17 has been designated as World Hemophilia Day to raise awareness of hemophilia and use consumer characteristics, care and support care and increase public consumption in this area.
Hemophilia is a rare genetic disorder in which blood does not clot because there is lack of clotting proteins. In patients with hemophilia in the event of injury, surgeon or surgery is much higher than normal and can make the patient with serious problems. Also, one of the most important problems of the disease is internal in the joints, especially knees, elbows and ankles.
Some people have abnormal bleeding inside their bodies for no clear reason.
Blood contains proteins called Clotting factors that terminate. People with hemophilia have no factor 8 or 9 or the level of factor is less than normal. The severity of the disease is determined by the amount of clotting factors in the blood. The lower these factors are, the more likely they are to occur and cause problems.
This genetic disease is usually associated with the patient from birth. But in rare cases, the patient develops this complication during his life. It is more common in middle-aged or elderly people, or in women after pregnancy or in the last months of pregnancy. The disease in this case can be eliminated with appropriate treatments. Sometimes the disease is caused by a weakened immune system caused by cancer, MS and autoimmune diseases.
The Quid 19 pandemic has caused serious problems for these patients. In some parts of the world, for obvious reasons, there are no drugs available in this field.
Causes of hemophilia
Hemophilia results from a mutation or change in one of the genes that provides instructions for clotting factor proteins. This change or mutation can prevent the clotting protein from working properly or to be missing altogether. These genes are located on the X chromosome.
Men have one X chromosome and one Y chromosome, and women have two X chromosomes. Men inherit the X chromosome from their mother and the Y chromosome from their father. Women inherit the X chromosome from each parent.
The three main forms of Hemophilia include the following:
-Hemophilia A: Caused by a lack of the blood clotting factor VIII; approximately 85% of hemophiliacs have type A disease.
-Hemophilia B: Caused by a deficiency of factor IX.
-Hemophilia C: Some doctors use this term to refer to a lack of clotting factor XI
Symptoms of hemophilia:
-Bleeding into a joint or muscle, which causes pain and swelling.
-Bleeding that is not normal after an injury or surgery.
-Blood in the urine.
-Bleeding after dental work.
Some people with milder types of the disease may not have symptoms until later in life. But most of the time, hemophilia symptoms are noticed during infancy or childhood. Symptoms noticed in infants include:
-Bleeding into the muscle, which causes a deep bruise after the baby gets a routine vitamin K shot.
-Bleeding in the scalp or brain after a difficult delivery or after special devices (vacuum or forceps) are used to help deliver the baby.
-Bleeding that goes on for a long time after the infant’s heel is pricked to draw blood for newborn screening tests.
-Bleeding that goes on for a long time after a baby is circumcised.
Diagnosing HemophiliaIn addition to a complete medical history and physical examination, your doctor may perform numerous blood tests including clotting factor levels, a complete blood count (CBC), assessment of bleeding times, and/or DNA testing.
Your doctor may also request details about your family history.
Specific treatment for hemophilia will be determined by your doctor. It is based on:
Your age, overall health, and medical history,Extent of the disease,
Your tolerance for specific medications, procedures, or therapies and
Your opinion or preference.
Treatment depends on the type and severity of the hemophilia. Treatment for hemophilia is aimed at prevention of bleeding complications (primarily head and joint bleeds). Treatment may include:
Parents may want to purchase soft toys with rounded corners for young children. Padded clothing and helmets may be necessary for the child that is learning to walk or becoming more active. Contact sports in school should be evaluated for risks of injury to the child.
Immunizations may need to be given under the skin (subcutaneous) instead of in the muscle (intra muscular) to prevent deep muscle bleeds.
Joint hemorrhages may require surgery and/or immobilization. Rehabilitation of the affected joint may include physical therapy and exercise to strengthen the muscles around the area.
Before surgery, including dental work, your child’s doctor may recommend factor replacement infusions to increase the child’s clotting levels prior to the procedures. Your child may also receive the specific factor replacement infusions during and after the procedure to maintain the clotting factor levels and to improve healing and prevention of bleeding after the procedure.
Your child’s doctor may also recommend the discontinuation of aspirin, and aspirin-containing products, since these products have been linked to bleeding problems.
Blood transfusions may be necessary if significant blood loss has occurred.
Proper dental hygiene is a preventive measure.
Prophylactic self-infusion of factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.
The use of gene therapy to replace the defective genes that cause hemophilia is under study, and clinical trials are being conducted.
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