Hemophilia and other bleeding disorders are characterized by a permanent tendency towards spontaneous or traumatic hemorrhages, which are responsible for significant disease burden and negative impact on the quality of life. Hemophilia, an X-linked condition resulting in a decrease or lack of clotting factor VIII or IX, is marked by bleeding into joints and muscle, but it may also lead to bleeding that causes neurologic damage, damage to other organ systems, or death. Hemophilia occurs in about 1 in 5000 male births1 and affects all racial and ethnic groups. It is estimated there are about 20,000 males with hemophilia in the United States at this time.
Von Willebrand disease (VWD) results from a deficiency or defect in the body’s ability to make von Willebrand factor, a protein that helps blood clot. Although VWD occurs in men and women equally, women are more likely to experience the symptoms of excess or abnormal bleeding due to physiologic bleeding associated with menstrual periods and childbirth. Joint bleeds and muscle hematomas may occur in severe forms of the disease, but mucocutaneous bleeding is more common. Deficiencies of other clotting factor proteins and platelet function defects may cause rarer bleeding disorders.
Approximately 7 in 10 of all persons with hemophilia in the United States receive at least some of their care at a federally-funded hemophilia treatment center (HTC)2. Federally-funded HTCs developed a model of care delivery called comprehensive (integrated) care that includes specialized prevention, diagnostic, and treatment programs designed to provide family-centered education, state-of-the-art treatment, research, and support services for individuals and families living with bleeding disorders. These centers currently provide care to over 43,000 children and adults with bleeding disorders3. For years, the Division of Blood Disorders (DBD) at the CDC has provided support to HTCs for programs designed to monitor and prevent complications of these disorders. The Universal Data and Serum Specimen Collection (UDC) System for Hemophilia was a collaboration between the HTCs and CDC to establish a sensitive blood safety monitoring system among people with bleeding disorders and collect a uniform set of clinical outcomes information that can be used to monitor the occurrence of infectious diseases and joint complications and their potential risk factors. From 1998 to 2011, UDC captured information on approximately 27,000 participants, representing over 97,000 annual visits. People with bleeding disorders were enrolled in UDC by care providers in each of the nation’s approximately 140 federally funded HTCs. HTC staff collected a uniform set of clinical data and blood specimens (plasma or serum) each year during each participant’s annual comprehensive clinic visit. A portion of each specimen was used to perform free screening tests for hepatitis A, B, and C viruses and for human immunodeficiency virus (HIV). The remainder of the specimen was stored for use as needed in future blood safety investigations. Since 1998, no new infections of hepatitis A, hepatitis B, hepatitis C, or HIV have been linked to using blood products to treat bleeding disorders. The data from UDC have also been used to describe complications among patients receiving care at HTCs, including the impact of being overweight on joint mobility, the use of prophylaxis to treat intracranial hemorrhage, and bleeding complications among children under 2 years old.
Community Counts is the next generation of surveillance and builds on the information and experience gained through 12 years of UDC data collection. CDC funds the project through a cooperative agreement awarded to the American Thrombosis and Hemostasis Network (ATHN) in partnership with the network of federally-funded HTCs. The purpose of Community Counts is to collect and share information about health indicators and complications that affect people with hemophilia and other bleeding disorders receiving care at HTCs. The data will be used to measure rates of complications of bleeding disorders and monitor trends over time, identify high risk populations for programs to prevent complications, and identify issues that require further study. Community Counts consists of three components:
- HTC Population Profile (HTC PP) gathers basic information on all HTC patients with bleeding disorders or blood clots. The goal of the HTC PP is to learn basic information about the patients at HTCs, such as how many receive care at the centers.
- Registry for Bleeding Disorders Surveillance (the Registry) gathers detailed information over time on characteristics, diagnoses, risk factors, and complications of HTC patients with bleeding disorders.
- Mortality Reporting tracks the characteristics, diagnoses and causes of death of HTC patients with bleeding disorders who have died.
- Soucie JM, Evatt B, Jackson D, and the Hemophilia Surveillance System Project Investigators. Occurrence of Hemophilia in the United States. American Journal of Hematology. 1998; 59:288–294.
- Baker JR, Riske B, Drake JH, Forsberg AD, Atwood R, Voutsis M, Shearer R. US Hemophilia Treatment Center population trends 1990–2010: patient diagnoses, demographics, health services utilization. Haemophilia. 2013;19:21-6.
Pages in this Report
- Highlights & Acknowledgements
- Geographic Distribution of Registry Participants
- Registry Characteristics 2014
- Weight Status
- Health Insurance Coverage
- Health Insurance Coverage by Age
- Viral and Vaccination Status
- Use of Healthcare Services and Absenteeism
- Chronic Diseases, Conditions, or Surgical Procedures
- Technical Notes
- Participating HTCs