Goal
Prevent illness and disability related to blood disorders and the use of blood products.
Overview
Blood disorders include hemoglobinopathies and abnormal bleeding and clotting. Hemoglobinopathies are inherited, lifelong blood disorders that result in abnormal hemoglobin (protein in red blood cells that carries oxygen). Complications of bleeding and clotting disorders can be prevented if the conditions are promptly recognized and treated.
Blood transfusions are lifesaving for people with severe bleeding or disorders of decreased blood production and for people undergoing surgery or recovering from severe accidents. Despite efforts by blood banks, shortages of blood product persist.
Why Are Blood Disorders and Blood Safety Important?
Millions of Americans carry genes for bleeding and clotting disorders and hemoglobinopathies. Most will lead normal lives without experiencing any harm. However, some individuals may see health effects related to carrying a blood disorder gene.
Bleeding and Clotting Disorders
Yearly, about 1.2 million Americans develop blood clots in veins of the leg. This condition is called deep venous thrombosis (DVT). About 10 percent of people with DVT die from pulmonary embolism (PE), when a blood clot from a vein in the leg travels to the lungs, making DVT the most common preventable cause of death in hospitals.1 Non-symptomatic DVT is more common and can develop in nonhospitalized persons, such as long-haul air travelers.2
Hemophilia is a genetic disorder that affects males and causes a deficiency (shortage) of proteins needed for normal blood clotting. For a person with hemophilia, minor injuries causing repeated joint bleeds, can lead to chronic joint disease. Chronic joint disease reduces functional capacity and causes disability. Early preventive care decreases bleeding and lessens joint disease and disability, improving quality of life.
Von Willebrand disease (vWD) is the most common blood disorder, affecting 0.6 percent to 1.3 percent of the general population. It is a bleeding disorder that affects both men and women. Women experience more symptoms because vWD increases bleeding during menstrual periods, pregnancy, and childbirth.3
Hemoglobinopathies
Hemoglobinopathies include sickle cell disease and thalassemias. These are recessive genetic disorders. This means that the disease occurs when a person inherits an abnormal gene from both parents. If both parents carry a hemoglobinopathy gene, there is a 25 percent chance that their baby will be born with the disease.
Babies born in the United States are tested at birth for common forms of hemoglobinopathies. It is estimated that at least 2 million people in the United States carry one sickle cell gene. Hemoglobinopathies are more common in people of African, Southeast Asian, and Mediterranean descent.4 Increased public awareness of testing for hemoglobinopathy genes (or carrier status) may increase awareness of risks for carriers and their children, and affect health-related decisions.5
Blood Safety
Blood service providers are responsible for the collection, maintenance, and distribution of the Nation’s blood supply. Providers include:
- U.S. Department of Health and Human Services
- American Association of Blood Banks
- American Red Cross
- America’s Blood Centers
Certain blood types are more rare, causing limited supplies. Other blood products, like platelets, have a limited shelf life; on average, less than a 2-day supply is available.
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Understanding Blood Disorders and Blood Safety
Bleeding and Clotting Disorders
Bleeding and clotting disorders result from genetic, biological, and environmental risk factors. vWD affects more women, while hemophilia is a disease that affects only men. The risks of DVT and PE increase with age. Early recognition of signs and symptoms and prompt treatment can prevent most complications.
Hemoglobinopathies
Hemoglobinopathies are caused by mutations in the hemoglobin gene. Additional genetic modifiers influence disease severity and development of complications. Because these are complex, rare diseases that disproportionately affect population subgroups in the United States, lack of access to knowledgeable providers is a major barrier to appropriate health care. Economic and health disparities have contributed to premature mortality from hemoglobinopathies.
Blood Safety
A safe and adequate blood supply is vital for health services such as surgeries, cancer treatment, emergency management of trauma, and care of persons with hemoglobinopathies who are on chronic transfusion therapy. Outreach and education to inform the public about the importance of donating blood can help alleviate blood shortages.
References
1Alpert JS, Dalen JE. Epidemiology and natural history of venous thromboembolism. Prog Cardiovasc Dis. 1994 May-Jun;36(6):417-22.
2Scurr JH, Machin SJ, Bailey-King S, et al. Frequency and prevention of symptomless deep-vein thrombosis in long-haul flights: A randomized trial. Lancet. 2001 May 12;357(9267):1485-9.
3James AH. Von Willebrand Disease. Obstet Gynecol Surv. 2006 Feb;61(2):136-5.
4American College of Obstetricians and Gynecologists (ACOG). Hemoglobinopathies in pregnancy [ACOG practice bulletin No. 78]. Washington: ACOG; 2007 Jan.
5National Institutes of Health. Meeting summary: Population-based carrier screening for single-gene disorders: Lessons learned and new opportunities. 2008 Feb. Available from: http://www.genome.gov/27026048
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