Delivering Integrated Care and Cost Management
The IHTC works collaboratively with payors to optimize care. We ensure that the patients and families we serve have access to care and therapies, thereby helping to contain costs and reduce both bleeding events and utilization of resources.
The IHTC Pharmacy’s ability to purchase clotting factor through the Public Health Service 340B discount program and our overall pricing structure benefit payors and patients by dispensing clotting factor at significantly reduced prices.
What is thrombophilia?
- Thrombophilia describes a group of conditions in which there is an increased tendency for excessive blood clotting.
- Thrombophilia can be due to inherited genetic abnormalities that are associated with a life-long increased tendency to clot.
- Thrombophilia may also be associated with an acquired condition such as lupus anticoagulant or an antiphospholipid antibody, which can occur in persons either with or without systemic lupus erythematosus.
- See IHTC’s webpages on Inherited Causes of Thrombosis and Acquired Causes of Thrombosis for further information on specific inherited and acquired thrombophilic conditions.
Who has thrombophilia?
- Thrombophilia affects a large number of people throughout the world.
- Persons who experience episodes of blood clots, either as an isolated event or as repeated events, may be affected with a thrombophilic disorder.
- Some people may have an identifiable disorder of clotting, such as factor V Leiden, yet not experience a blood clot.
- Both men and women can have thrombophilia.
- Both children and adults can have thrombophilia; thrombosis is more commonly diagnosed during the teen and adult years due to normal changes in the blood that occur with growth and aging.
- Women who have thrombophilia can have clotting problems during pregnancy or with the use of birth control pills or hormonal replacement therapy.
- Factor V Leiden affects approximately 5% to 7% of white Americans of European descent.
Are all clotting problems inherited?
- Not all clotting disorders are inherited. Clotting disorders can also be acquired, meaning they may develop at any time without an underlying genetic alteration.
- Some acquired clotting disorders are related to disease states that can be controlled or reversed, such as diabetes, high blood pressure, pancreatitis, inflammatory bowel disease, and obesity to name a few.
- Healthcare providers are able to determine the cause for excessive clotting in some patients and families, but not in all cases. Further research is required to understand why individuals without an identifiable cause experience clotting problems.
- For more information on acquired causes of thrombosis, click here.
What is thrombosis?
- Thrombosis is the formation of a blood clot (thrombus) inside a blood vessel that obstructs the normal flow of blood through the circulatory system.
- Thrombosis may occur in either veins (vessels that deliver blood from the tissues to the heart) or arteries (vessels that deliver blood from the heart to the tissues).
- A blood clot can reduce or completely shut off blood flow and oxygen to body tissues, resulting in pain, tissue damage and, in some cases, death.
What is an embolism?
- Embolism occurs when part of a blood clot breaks off and travels in the blood stream to another part of the body where it may block blood flow in the affected vein or artery.
Are there different types of venous thromboembolism?
- Yes. The most common types of venous thromboses are deep vein thrombosis (DVT) and pulmonary embolism (PE)
- DVT is the formation of a blood clot within one of the large deep veins of the body. It usually occurs in the deep veins of the legs or pelvis, although it can happen in other areas as well.
- A PE occurs when a DVT embolizes. In the lungs, the embolized blood clot can obstruct the blood vessels and reduce or prevent the flow of blood to the lungs.
- PE is the most serious complication of DVT.
How common is venous thromboembolism?
- Venous thromboembolism (DVT, PE) is a very common medical problem and significantly contributes to morbidity and mortality in the United States.
- According to the CDC, 300,000–600,000 people in the United States have DVT or PE.
- Of the people who have experienced a DVT, nearly one third develop postthrombotic syndrome, a chronic and potentially disabling condition characterized by swelling, pain, discoloration, and scaling of the affected limb.
- For some people, DVT may become a chronic illness. Approximately 30% of individuals who experience a DVT are at risk for a subsequent episode.
- It is important to note that DVT is preventable and treatable if diagnosed correctly and early.
- An estimated 100,000 to 200,000 people per year in the United States experience a PE, and in nearly one third of these (30,000–60,000) the PE is fatal.
What causes venous thrombosis?
- DVT is the result of three principal factors:
- Venous stasis* in the deep veins
- Vascular damage
- Hypercoagulability defined as an increase in the activity of the components in the blood that are part of the normal clotting process
- Several factors can help cause the conditions listed above, thus increasing the risk of DVT:
- Inherited genetic abnormalities that cause an increased tendency to clot
- A surgical procedure
- Immobilization that reduces circulation in the legs by 50%; for example, in a postsurgical state or sitting for long periods (more than 4 hours)
- Major trauma
- Increasing age
- Heart failure
- Use of hormone therapy such as birth control pills or hormone replacement therapy
- Having a history of DVT
- Other risk factors for DVT may include:
- Diabetes, which leads to damage of the blood vessels
- Obesity, where excess weight places pressure on veins and causes them to weaken
- Pregnancy and childbirth, where physical strain places pressure on deep veins, causing them to weaken
- Tobacco smoking, which is associated with damage to blood vessels and doubles the risk of thrombosis
Can thrombosis be prevented?
Some blood clots can be prevented through physical activity, a healthy lifestyle, and quality medical care. Some important tips for thrombosis prevention that you should share with your patients include the following:
- Take breaks and stretch legs when traveling long distances
- Perform heel-toe exercises when seated during prolonged travel and get up and walk as frequently as possible
- Drink fluids, especially during prolonged travel, preferably water
- Know the symptoms of DVT and PE and seek early medical attention if they occur
- Know the risk factors for blood clots and make lifestyle changes, such as smoking cessation, weight control, control of cholesterol and blood pressure
- Be aware of a family history of blood clots
- In case of major surgery, trauma, prolonged immobility, or when in a cast, patients should ask their physician whether they should receive DVT prophylaxis and, if so, for how long.
- In some cases, individuals at high-risk for blood clots or with a history of excessive clotting may be prescribed long-term DVT prophylaxis using oral medications
- For optimal clot prevention, it is important that patients adhere to their antithrombotic regimens.
What are the symptoms of a blood clot?
- The symptoms of a thrombotic episode relate to the location and size of the blood clot and whether the clot embolizes.
- Some of the symptoms of a clot in the lungs (PE) include chest pain, shortness of breath and hemoptysis/±.
- Symptoms of a deep vein thrombosis in the legs include swelling, pain, and sometimes erythema in the affected area.
- Individuals who develop an ischemic stroke (clot in the brain) may experience a sudden headache, facial and/or limb numbness, and weakness and/or paralysis, particularly on one side of their body. Difficulty with vision, walking or maintaining balance also may occur. An individual with a stroke may appear to be confused and sometimes may experience a seizure or lose consciousness.
- Symptoms of chronic swelling, swelling with standing, erythema, dilated blood vessels and pain are relatively common after a significant blood clot has occurred.
- These symptoms are collectively referred to as postthrombotic syndrome (PTS). This syndrome may be present for months after the development of a clot or may become chronic if the first clot was large or if repeated clots occur.
- Patients can manage PTS symptoms through the following interventions:
- Use of a compression garment, often prescribed by a physician to provide a specific amount of pressure
- Stand and move on a regular basis to encourage and maintain blood flow. Patients should talk to their healthcare provider regarding appropriate exercises
- Avoid long periods of standing still
- Drink plenty of fluid
- Elevate as much as possible the affected area to encourage venous drainage
- It may be difficult to know whether the symptoms of leg swelling are from a new clot or from PTS. Tests are available to assist in distinguishing old from new clots.
- Healthcare providers should be alerted by patients if their symptoms continue or change, or if they are concerned that a new clot may be present.
- Healthcare providers can reach the IHTC toll-free at 1-877-256-8837.
SERVICES AT A HEMOPHILIA TREATMENT CENTER
Why should my patients go to a hemophilia treatment center for a clotting disorder?
- Hemophilia treatment centers (HTCs) provide comprehensive care for people with bleeding and clotting disorders.
- HTCs are staffed by a range of healthcare providers, including hematologists, nurses, nutritionists, genetic counselors, physical therapists, and social workers, who work as a team to address the needs of persons with clotting disorders.
- HTC providers assist persons with clotting disorders to better understand and manage their medical condition.
Do my patients with thrombosis need to see a specialist?
- Many primary care providers manage patients with thrombosis. However, if a patient has or has had a blood clot, it is recommended that they also be evaluated by a hematologist who specializes in the area of hemostasis and thrombosis.
- Hematologists are an essential part of the HTC healthcare team. A hematologist accurately identifies and diagnoses clotting abnormalities that may contribute to the development of a clot. Accurate diagnosis is essential to the health of patients and families.
- Hematologists have extensive experience in the management of anticoagulation. Appropriate choice of an anticoagulant, and the level and duration of anticoagulation are provided based on each patient’s diagnosis and individual circumstances, including site and extent of thrombotic event, presence of precipitating factors, patient age, and family history.
If a patient has been diagnosed with factor V Leiden, should family members be tested, even if they have not personally experienced a blood clot?
- If a patient has been diagnosed with an inherited clotting disorder such as factor V Leiden, his or her other potentially affected family members should be tested. Testing of family members allows for appropriate preventive healthcare management and for individuals to make informed decisions about their healthcare. This information may be helpful in medical decision making in certain circumstances, such as pregnancy, type of birth control utilized, and surgery.
- Family members potentially at-risk for these conditions may visit a hematologist to have this testing performed.
- Many HTCs provide this testing and education for affected individuals. In addition, the IHTC also has a genetic counselor who can explain this testing and the test results to patients and their families.
What can the IHTC do for you?
- The IHTC’s anticoagulation clinic uses a multidisciplinary comprehensive approach to treating patients on anticoagulation therapy. Patients commonly are seen every 6 months by the anticoagulation clinic staff. The clinic is led by physician assistants experienced in the treatment of clotting disorders, with board-certified hematologists available for additional consultation. In addition, the IHTC’s physical therapist, registered dietitian, genetic counselor, and social worker provide support services on an as-needed basis. See our Meet the Staff page to read about how these healthcare professionals help providers and their patients who require anticoagulation therapy. The IHTC’s anticoagulation clinic is held weekly. For more information about the clinic, contact the IHTC at 317-871-0000 or toll free at 1-877-CLOTTER.
- The IHTC provides genetic counseling services for individuals with clotting disorders and their families. To contact our genetic counselor, please call the IHTC at 317-871-0000.
- The IHTC’s registered dietitian provides information and advice on diet-related issues that affect anticoagulant medications, particularly warfarin (Coumadin®). Visit the IHTC website (http://www.ihtc.org/medical-professionals/blood-disorders/clotting-disorders/nutritional-information) for useful nutritional information, important dietary tips, and educational resources for providers who have patients on anticoagulant therapies.
- Visit the IHTC’s Resources webpage (http://www.ihtc.org/medical-professionals/blood-disorders/clotting-disorders/resources) for useful educational websites and brochures on clotting disorders that you can provide to your patients. The Resources page also provides links to educational presentations for clinicians.
- The IHTC website provides education on clotting disorders for patients, their families and healthcare providers to assist in all aspects of thrombosis prevention and treatment.
WARFARIN (COUMADIN) QUESTIONS
When is Coumadin taken?
- Coumadin should be taken daily at approximately the same time every day. It is often best to take this medication in the late afternoon or evening as this allows for dose adjustment if required based on testing.
- If patients forget to take a dose, they should contact their medical provider for specific instructions.
What medications should be avoided with the use of Coumadin?
- There are many medications that may increase the anticoagulant effect of Coumadin.
- Unless specifically instructed by their healthcare provider, patients should avoid medications that affect the clotting system, such as aspirin, which has an antiplatelet effect.
- Some antibiotics can affect the level of anticoagulation while on Coumadin.
- Some people taking Coumadin who have a high risk of clotting also need to take antiplatelet agents. These drugs, like Coumadin, also interfere with the clotting mechanism. It is important to be aware of all medications a patient takes before initiating anticoagulation with Coumadin.
- Herbal medicines, although available without prescription, may also affect the level of anticoagulation. For example, supplements that may affect clotting include ginseng, ginko biloba, bromelain, flaxseed, fish oil, vitamin E in large doses, garlic, ginger, and bilberry fruit. Be aware of any herbal medications patients may be taking. See also IHTC’s webpage on Coumadin® interactions to learn about herbal products that may interact with Coumadin.
- When patients take medications that may interact with Coumadin and affect the level of anticoagulation, the level of anticoagulation¥ may need to be followed more closely to maintain the correct range.
Should patients avoid vitamin K foods while they are taking Coumadin?
- Although Coumadin works through interference with the vitamin K pathway (which is important in some of the body’s clotting factors), anticoagulation patients on Coumadin do not have to avoid all vitamin K-containing foods. These patients should, however, remember the following:
- Eating large amounts of foods with vitamin K can decrease the effectiveness of Coumadin.
- Patients should eat approximately the same number of servings of foods with vitamin K per day to maintain a consistent INR. For example, they should eat the same amount of green leafy vegetables every day.
- Drastic changes in patients’ diets, such as trying to lose weight by eating more green leafy vegetables or cutting out all green leafy vegetables, will affect their level of anticoagulation.
- Changes in diet may affect the level of anticoagulation. For example, eating more salad will decrease the INR (make clotting more likely) while cutting out vitamin K containing foods will increase the INR (make bleeding more likely).
- For more nutritional information for people taking Coumadin, please see the information on Coumadin® interactions with food on this website.
What is the PT/INR?
- The prothrombin time, also referred to as the PT, measures the time needed for a clot to form in a tube of the patient’s blood after reagents have been added to artificially activate the clotting reaction.
- The PT is obtained either by venipuncture or by an instrument that provides an INR result from a blood sample taken from a finger stick.
- Because different laboratories use different reagents to perform the PT, results can vary between laboratories. Therefore, the PT was standardized through development of the International Normalized Ratio (INR) system so that results from different laboratories could be compared and made interpretable. The INR is standardized only for the measurement of the effect of Coumadin on the PT to monitor therapy.
- The INR is calculated to adjust for the varying sensitivities of diverse reagents used in different laboratories.
How often should a patient’s anticoagulation level be monitored?
- The frequency of monitoring required for each patient depends on how well they respond to the Coumadin dose and other medical issues.
- Some patients require weekly monitoring, while others with stable responses who require long-term therapy may be monitored from intervals of every other week to once monthly.
What information should be provided to patients about their anticoagulation?
- Hematologist as well as primary care providers should provide educational materials about the use and monitoring of Coumadin.
- Patients should be aware of their target INR range and the results of their tests.
- Patients should be encouraged to maintain a record of their INRs values and Coumadin dose. This information will allow them to be a better partner in their healthcare.
When is a low molecular weight heparin (LMWH) more appropriate than Coumadin?
- Physicians may prescribe LMWH instead of Coumadin in a variety of situations. These situations include but are not limited to patients who:
- Experience clotting events while on Coumadin
- Are pregnant. Coumadin should NOT be used in pregnant women
- Have new or very large clot
- Are about to undergo surgery or dental work
- The use of LMWH requires intensive patient education before start of therapy. This should be coordinated through your office. The IHTC can help provide patient education. Healthcare providers can reach the IHTC toll-free at 1-877-256-8837.
Can a patient who is taking Coumadin still experience a thrombotic event?
- It is possible for patients to develop a thrombosis while taking Coumadin.
- If the INR falls to the sub-therapeutic range, a thrombosis may occur.
- A change in brand of warfarin may lead to fluctuations in the INR. If the INR becomes sub-therapeutic, a thrombosis may occur.
- Other variables, such as the presence of an underlying malignancy, may place patients at risk of development of thrombosis even if the Coumadin is taken as prescribed and the INR is therapeutic.
- Patients should contact their physician’s office or the IHTC (toll-free at 1-877-256-8837) with questions or concerns.