Treatments For Hemophilia
Hemophilia A occurs in every one in four to five thousand men, making it the most common type. Type B affects about one in twenty thousand males. Women can experience this condition, but it is rarer. This disorder is characterized by improper blood clotting, causing severe bleeding after surgery or injury, as well as spontaneous bleeding. Patients either have a deficiency in either factor VIII or IX, two proteins that work as clotting factors. This is almost always an inherited disorder, so patients are usually born with it. A gene mutation causes the clotting factor to either be completely missing or to work improperly. In general, this condition is more severe the more deficient the causative clotting factor is.
Clotting Replacement Therapy
Hemophilia A patients are often prescribed clotting replacement therapy with a concentrated VIII medicine referred to as a clotting factor. DNA technology is used to produce these products in a lab. This therapy is given intravenously, and patients may have a port in their chest, or it can be given in a vein in the arm. For hemophilia B, concentrated IX medicine is used, which is also administered intravenously into a chest port or arm vein. Patients may receive this therapy on a regular basis to prevent the risk of severe bleeding, or it can be used on demand when they have a bleeding episode. It is possible for patients to develop certain antibodies that can attack the clotting factor they receive, something most often seen in hemophilia A. When this happens, this therapy cannot be administered, and patients will receive other treatments to control the disorder.
Clotting Medication
A clotting medication called desmopressin, a manmade substance created to mimic the vasopressin hormone, might be considered for mild hemophilia A, but not for those who have hemophilia B. This medication works to stimulate the release of von Willebrand factor and stored factor VIII. Von Willebrand factor is a protein that binds to factor VIII and other proteins. This is important for the process of platelet adhesion when wounds occur, as it helps stop bleeding. Doctors may prescribe either the nasal spray or injection form of this drug. It is generally only given to reduce bleeding and not frequently because regular use may make it less effective. For example, patients might use it before dental work to minimize bleeding.
Other Medication
Certain other medications for hemophilia, such as anti-fibrinolytic drugs, may be beneficial for patients. These medicines work to aid in preventing the break down of clots to reduce the risk of bleeding. The most common anti-fibrinolytic drug is known as tranexamic acid, and it might be considered when there is a high risk of hemorrhage. These medications might be used in conjunction with desmopressin as part of a hemophilia treatment regimen. Tranexamic acid is an oral medication, but a liquid form might be given to children. The dosage and administration frequency are dependent on the severity of the patient’s condition. In some cases, it may only be taken short-term for a specific bleeding event or as a preventative measure for bleeding, such as before surgery.
Physical Therapy
Those with hemophilia are at risk for joint damage due to internal bleeding. When this is diagnosed, physical therapy may help to preserve joint function and minimize the risk of further damage. How often patients need sessions depends on the severity of their condition. It is often prescribed more than once with rest periods in between. The exact exercises used depend on the joints that require therapy, and patients might also be prescribed exercises to do at home.
In some cases, the joint damage becomes so severe that surgery is needed. For specific joints, such as the knees and hips, total joint replacement might be recommended. For others, other surgical repair methods may be utilized. Following surgery, patients will likely go through therapy to work their new or repaired joint.
Lifestyle Remedies
In addition to medications, there are lifestyle remedies patients and doctors can discuss. Patients should know basic first aid for bleeding. For example, applying pressure and ice to the bleeding site. Regular, low-contact exercise is recommended. Examples include bicycling, swimming, and walking, all of which can help to protect the joints by improving the strength of the surrounding muscles. Certain medications may increase bleeding risk and should be avoided, including nonsteroidal anti-inflammatory drugs and blood thinners. Dental hygiene is also critical since a simple tooth extraction puts patients at risk for severe bleeding. Regular dentist visits and attending to basic oral care at home should be a priority for all patients.