The main treatment for hemophilia today is called ReplacementTherapy. It is an IV drip or injection of the missing proteins in a plasma called Clotting Factor Concentrate. Most of these concentrates are derived from human blood, which sometimes, but very rarely, carry diseases. It is also possible to get non-human blood derived clotting factor concentrates, they are called recombant clotting factors.
Both of these treatments are commonly taken at home as this is faster, more convenient and much cheaper. Factor concentrates are all easy to store, mixe and use, having only a 15 minute injection time. Factors are normally stored in a refrigerator but are also stable at room temperature for long periods of time. Injections are usually done on the back of the hand or at the crook of the elbow.
Demand and Preventative
These are the two types of replacement therapies available.
Demand Therapy is a factor concentrate that is only taken on demand/as needed. This cuts down on the number of injections and the cost. Problems may arise though, when concentrates cannot be administered fast enough before damage is done.
Preventative or Prophylactic Therapy is taken on a regular basis. This ensures that blood clotting will take place more quickly in the event of an injury. Vein access devices called Broviac or Hickman lines can be surgically implanted into a vein, making regular injections easier. There is always the slight possibility that an infection will occur with the device, they must be used with care.
It is possible to acquire an infectious disease through human derived concentrates. HIV AIDS and Hepatitis could be carried in the concentrate, but cases of this have not occurred for at least 10 years. Doctors are very careful with blood donations and prevent infections through careful blood screening, intensive testing, treatment of the blood and vaccinations of the hemophilia patients.
Aspirin, ibuprofen or acetylsalicylic acid should be avoided for they interfere with blood clotting.
A patient may develop antibodies that act against the foreign clotting factors. These antibodies are called Inhibitors and develope in 25% of severe hemophilia A cases, but only 1% in hemophiliac B. In response to this, doctors use larger doses or different clotting factors and over time antibodies tend to subside. Less than 10% of patients have long-term inhibitor problems.
Desmopressin (DDAVP) is a man-made hormone meant to treat mild to moderate hemophilia. It stimulates the release of factor VIII and increases blood clotting protein production. It is taken through an injection or nasal spray but if taken too often will lose effect. It is normally taken before dental work.
Antifibrinolytic medicines (including tranexamic acid and aminocaproic acid) are sometimes taken in addition to replacement Therapy. It is taken as a pill and prevents blood clots from breaking down. It is used before dental work but also for treating bleeding from the nose or intestines.
RICE is an acronym for rest, ice, compression and elevation. This is the standard procedure for dealing with cuts and scrapes as well as joint bleeds. It reduces the damage and the swelling and gives time for the clotting factors to take effect.
Gene Therapy is a potential future cure and prevention for this disease, but research in this field is still ongoing.