Hemophilia is a genetic blood disorder affecting the body’s ability to clot blood effectively. Those with the condition may experience prolonged and excessive bleeding following injuries, dental work, medical procedures, and vaccinations, as well as unwarranted bruising and bleeding (both internal and external). Complications resulting from prolonged bleeding (and especially internal bleeding) are potentially life-threatening
Although no cure currently exists for hemophilia, several symptom management strategies exist to help patients maintain a healthy lifestyle.
The reason hemophiliacs are unable to stop bleeding effectively is due to the lack of clotting factors in the blood. There are over 20 types of clotting factors (also called clotting proteins), of which hemophiliacs are missing one or more to varying degrees. Clotting factor replacement treatments seek to replenish those supplies.
Replacement treatments come in two main forms: plasma-derived and recombinant concentrate.
Plasma-derived factors are taken from human plasma samples. Thoroughly screened for impurities, specific clotting proteins are extracted from these samples and then sterilized.
Recombinant concentrates are produced entirely in the laboratory by injecting the clotting protein-producing human gene into animal cells and then replicating those cells. These are then screened for impurities and sterilized.
Clotting factor replacements are administered through intravenous injections, with the particular method depending on the severity of the hemophilia. Mild hemophilia will often be treated with injections on an as-necessary basis, such as during a prolonged bleeding spell following an injury. The more expensive option — geared more toward moderate and severe hemophiliacs — is prophylactic injections. This option normally entails injections on a weekly basis to prevent uncontrolled bleeding before it occurs. Regular prophylactic injections, although pricey ($1000-$2000 per injection), are the preferred method in the treatment of children, who are naturally more accident-prone. They also help to reduce blood build-up in joints (and subsequent joint damage).
Adults typically undergo injection via a butterfly needle, which is sometimes avoided with children as their smaller-sized veins can be more difficult to locate. Children often use a port and catheter, which involves implanting a small port underneath the chest skin, from which a catheter tube connects to a viable vein.
Self-injection of clotting factors is normally only undertaken after thorough and regular training by medical professionals. Many hemophiliacs are able to perform injections at home, provided certain age considerations and after a certain level experience. More severe forms of hemophilia may require the performing of injections at hemophilia treatment centers. Research shows a significant portion of those that use hemophilia treatment centers avoid hospitalization due to complications.
Hemophilia treatment centers also provide additional services beneficial to those suffering from the disorder, including mental health professional who can address the psychological effects commonly produced by hemophilia, as well physical therapists who can treat and prevent joint and muscle damage, and other bodily complications.
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