What is hemophilia and how is it treated?

Posted on April 17, 2019 by kerri
Hemophilia is a rare disorder that primarily affects males, which causes blood not to clot properly—it is very rare for true hemophilia to affect females. [1] Like many rare disorders, hemophilia is caused by genetics. Women are more likely to be carriers of the genes that cause hemophilia, but it was not until fairly recently it was realized that even though these women do not have hemophilia, that they can still experience bleeding related symptoms. [1]
What causes hemophilia symptoms?
Hemophilia symptoms are caused by lacking a certain component in blood. In Hemophilia A and B, the blood components that are deficient are called Factor VIII or Factor IX, respectively. The amount of factor that is present, as a percentage of normal, determines if a person’s hemophilia is mild (5 to 30% of normal factor levels), moderate (1 to 5% of normal) or severe (less than 1% of normal). A severe hemophiliac will experience severe bleeding (hemmorrhages) “several times a month”—these can be caused by “a minor bump or twist” and “sometimes there is no apparent cause for the bleeding.” [1] Moderate hemophiliacs will have bleeds less often, “often as the result of minor trauma, such as a sports injury.” [1] As one would predict, milder hemophiliacs have even fewer episodes of bleeding—they may become aware of their hemophilia “only in the case of surgery, a tooth extraction, or a serious injury.” [1]
Women may also bleed more than is usual during menstrual periods. [1]
Another type of bleeding disorder is called Von Willebrand Disease. Symptoms of VWD are milder than those of Hemophilia A and B. People often do not know they have VWD if it is mild, and it often does not require treatment—in some cases, VWD can be more severe and require treatment. [1.1Other factor deficiencies, as well as platelet dysfunctions, can also be the cause of bleeding disorders.
Treating hemophilia
Most often hemophilia is treated with “replacement therapy”, which provides concentrate of the deficient clotting factor by infusion (IV) or injection. [2] These can be made from either donated human blood or “recombinant” clotting factors that are manufactured. [2]
Depending on the person’s needs and severity of hemophilia, clotting factor may be given prophylactically (preventatively) to prevent bleeding, or as needed to stop bleeding that has already begun. [2] This treatment can be done at home or in the hospital depending on a person’s circumstances. [2]
In some cases, medicines can be used. DDAVP is a medicine that helps with clotting in milder cases of hemophilia A or in Von Willebrand Disease. Antifibrinolytic medicines may be used with factor replacement, and help prevent blood clots from breaking down, allowing bleeding to slow or stop. Often these medicines are used before dental work or to treat mild bleeding.
Lifestyle and hemophilia
Following your doctor’s instructions is the most important thing to managing hemophilia. Depending on the severity of hemophilia, there may be restrictions to what activities can be done safely—sometimes a physical therapist will provide counselling on which activities can be done safely and what precautions should be taken. [2] Some over the counter medicines that can increase bleeding will also need to be avoided, including aspirin and salicylate drugs, as well as ibuprofen and naproxen and some other anti-inflammatory medicines for pain. Your doctor will advise what is safe to use. [2]
It is also important to wear a medical ID bracelet or necklace alerting others that you have hemophilia. Engraving your hemophilia medical ID with the type of hemophilia and factor deficiency can help expedite treatment in an emergency situation. View our styles of custom medical ID jewelry, including styles for children with hemophilia and other medical conditions.

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