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Hemophilia

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by Carson-DeWitt R

(Hemophilia A—Factor VIII Deficiency; Classic Hemophilia; Hemophilia B—Factor IX Deficiency; Christmas Disease)

 

Treatment

Talk with your doctor about the best treatment plan for you. Options include:

Blood Factor Concentrate

You'll be tested often to monitor your factor levels. When your factor levels drop too low, you may be given a dose. You may also be given a dose if you are bleeding or at high risk for bleeding, such as during surgery.
Blood factor concentrate may be given through an IV. The type of blood factor that you are given depends on the type of hemophilia you have.
People with hemophilia and their families can be taught to administer blood factor concentrate at home at the first signs of bleeding. This can help prevent a crisis. People with severe forms of the disease may need regular infusions.
Some patients develop an inhibitor to a particular factor. This may require treatment with other clotting factors.

Medication

Mild hemophilia A may be treated with an infusion of medication. This infusion causes the release of blood factor stored within the body on the lining of blood vessels.

Immunization

The hepatitis B and hepatitis A vaccines are important. There is an increased risk of exposure to hepatitis with frequent infusions of blood products.

Bleeding Prevention

The following steps may help prevent bleeding:
  • Good dental care is important. It may decrease the risk of major dental work.
  • Be aware of the effect of high impact activities:
    • Take extra care to protect the head from injuries.
    • People with severe hemophilia should avoid activities that include a high risk of collision.
  • Avoid drugs that can also aggravate bleeding problems such as:
    • Blood thinners, such as aspirin or heparin
    • Antiplatelets, such as warfarin
    • Certain analgesics, such as nonsteroidal anti-inflammatory drugs
 

RESOURCES

American Society of Hematology
http://www.hematology.org

National Hemophilia Foundation
http://www.hemophilia.org

 

CANADIAN RESOURCES

Caring for Kids
http://www.caringforkids.cps.ca

Network of Rare Blood Disorder Organizations
http://www.hemophilia.ca

 

References


Explore hemophilia. National Heart, Lung, and Blood Institute website. Available at: http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia. Updated July 1, 2011. Accessed June 25, 2013.


Hemophilia A. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated May 10, 2013. Accessed June 25, 2013.


Hemophilia B. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated May 10, 2013. Accessed June 25, 2013.


10/24/2012 DynaMed's Systematic Literature Surveillance. http://www.ebscohost.com/dynamed/what.php: Broderick CR, Herbert RD, et al. Association between physical activity and risk of bleeding in children with hemophilia. JAMA. 2012 Oct 10;308(14):1452-9.


4/24/2014 DynaMed's Systematic Literature Surveillance. http://www.ebscohost.com/dynamed: US Food and Drug Administration. FDA approves first long-acting recombinant coagulation Factor IX concentrate for patients with Hemophilia B. US Food and Drug Administration website. Available at: http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm391037.htm. Published March 28, 2014. Accessed April 24, 2014.

 

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