Hemophilia: Care and Treatment for Bleeding Disorders
Hemophilia is not just one condition but a group of conditions caused by decreased amounts of clotting proteins. This decrease results in excessive bleeding tendencies. There are two major forms: Hemophilia A which is factor VIII deficiency and Hemophilia B which is factor IX deficiency. Both are caused by a mutation change in a gene located on the X chromosome.
In most cases females carry hemophilia and males are affected by the condition. However some females also have increased bleeding risk. Hemophilia is rare with only about 20,000 cases in the United States. Care is complex and patients treated in a comprehensive hemophilia treatment center (HTC) have the best results. With good care, life expectancy is nearly normal. Unfortunately, about 75% of persons affected don’t have access to expert care.
Most people affected with the more severe forms of hemophilia are diagnosed early in life, either because of a known family history or due to bleeding symptoms. A known family history or severe bleeding, such as bleeding into joints or muscles should be evaluated by a hematologist. Knowing the diagnosis is important because prevention of bleeds is an important focus of care.
Avoiding certain medications and contact sports are easy ways to reduce bleeding risks. For children with more severe forms of hemophilia, the typical treatment is routine infusion of clotting factor concentrates to prevent bleeding.
If and when injuries do occur, clinicians recommend that patients talk to their HTC about appropriate factor dosage and frequency. Additional recommendations are Rest, Ice, Compression and Elevation (RICE) to reduce the complications of bleeds.
Rest– The patient should rest the site of a bleed especially if it is in a joint or muscle
Ice – Apply a cool pack or ice to the injured site
Compression – Apply pressure such as an ACE wrap to the affected area
Elevate – If an arm or leg is hurt place it on a pillow or folded blanket so it’s higher than the patient’s body
Finally – Don’t leave home without the factor!
Persons with hemophilia always need to plan ahead to have a supply of factor, since only certain specialty pharmacies carry factor products. Children should have factor easy to access anytime and anywhere and should always have a bleeding action plan in place with their HTC.
To learn more about Nationwide Children’s Hospital Factor Program and about the HTC at Nationwide Children’s Hospital visit www.NationwideChildrens.org/Homecare-Factor or www.NationwideChildrens.org/Hemophilia.