The Power of Your Choice
Your choice of the IHTC Pharmacy Program directly supports your IHTC team and patient services, and activities provided to the hemophilia community. The IHTC Pharmacy Program provides savings to you and your health insurance plan.
Every patient has the right to choose their clotting factor pharmacy provider. The IHTC supports your right of choice and will assist you in making an informed decision.
Bleeding Disorder Clinics
The Centers for Disease Control and Prevention (CDC) strongly recommends that persons with bleeding disorders receive an annual comprehensive evaluation at a federally recognized comprehensive hemophilia treatment center. An excellent cost/benefit ratio has been well documented for these multidisciplinary evaluations for individuals with hemophilia, as well as decreased patient morbidity and mortality rates. According to J. Michael Soucie, Ph.D., of the CDC:
“The finding that HTCs (hemophilia treatment centers) have a significant effect on reducing mortality in patients with hemophilia supports the effectiveness of such centers in providing specialized preventive care….”
“The 40% reduction in death that we observed among persons using HTCs is even more remarkable because HTCs provide health care services to a higher proportion of severely affected patients as well as to a disproportionate share of patients with severe liver disease, HIV infection, and AIDS – the primary risk factors for mortality in this population.” – Soucie JM, et al. Blood. 2000; 96: 437-442.”
The multidisciplinary evaluation in comprehensive clinic is provided without charge. The only costs of comprehensive clinic to the patient and insurers are for laboratory tests and radiological evaluations.
This in-depth comprehensive evaluation involves the entire multidisciplinary team and takes approximately three hours per patient. Members of the multidisciplinary team include specialists in pediatric hematology, adult hematology, nursing, genetics, physical therapy, dentistry, social work, nutrition and pharmacy. During the evaluation, these specialists identify and assess any problems or concerns and report them to the patients and their referring physicians. The comprehensive evaluation includes a physical examination, laboratory studies, and assessment of bleeding episodes and other medical conditions and their sequelae (associated pathological conditions). Comprehensive clinic evaluations also address the psychosocial, genetic and financial burden these disorders place on patients and their families, as well as any problems that may have been encountered in acquiring optimal care for their condition.
The IHTC’s multidisciplinary team informs patients about all aspects of dealing with disorders of coagulation. The program’s main objective is to assist people with disorders of coagulation in managing their healthcare and to help these patients and their families maintain a healthy normal lifestyle.
Report to Referring Staff
In the interest of facilitating the healthcare of the patient, a written summary of the IHTC’s findings and recommendations is sent to the referring physician following either the initial evaluation or comprehensive visit. Recommendations can be carried out locally, or alternatively, the patient may be referred to the IHTC.
To provide comprehensive care to all Hoosiers with bleeding disorders, the Indiana Hemophilia & Thrombosis Center Inc. (IHTC) conducts a series of outreach comprehensive clinics throughout Indiana each year. These clinics deliver multidisciplinary, comprehensive care to persons with blood disorders who may otherwise go without the benefits of treatment from a federally recognized HTC.1 The multidisciplinary outreach team includes adult and pediatric hematologists, treatment nurses, a research nurse, dietitian, dental hygienist, social worker, physical therapist, pharmacist and genetic counselor. In addition to providing routine comprehensive care to bleeding disorders patients throughout Indiana, several outreach clinics are conducted with a concentration on a specific patient population or disorder, such as Indiana Amish communities, or communities that share a rare genetic mutation. The IHTC’s outreach programs are state and federally funded; there is no charge to patients for the comprehensive multidisciplinary evaluation. However, patients or their insurers are responsible for costs of laboratory tests, radiologic evaluations, and other services outside the scope of the comprehensive evaluation.
For more information about attending an outreach comprehensive clinic, please call the IHTC general number at 317-871-0000 (toll free: 877-256-8837 [877-CLOTTER]) . See our Reading Room for dates of outreach.
Some individuals with hemophilia develop an inhibitor. Inhibitors are antibodies that some people develop against infused clotting factors, because their immune system recognizes the clotting factor as “foreign” and therefore produces antibodies to destroy the factor concentrates. Inhibitors prevent the clotting factor from working effectively to stop bleeding. This serious problem is discovered through either routine laboratory tests or when a patient has a bleeding episode and factor does not stop the bleed. The presence of an inhibitor requires a special infusion plan and close monitoring by a hematologist.
Once an inhibitor is detected, the patient may start immune tolerance therapy. The idea behind this is to infuse factor on a regular basis (sometimes as often as two times per day) so that the body will become familiar with the clotting factor and stop destroying it with antibodies. Depending on the level of the inhibitor, different treatment products and/or additional factor infusions may be needed to stop bleeding when it occurs.
The frequency of clinic visits for individuals using immune tolerance therapy varies according to each patient’s situation. Patients who bleed while on immune tolerance therapy may require more intense therapy and more frequent clinic visits to manage bleeding events.
During a routine immune tolerance therapy clinic visit, the patient will be asked about his recent bleeding history and whether the current therapy seems to be working. If a central venous access device is present, issues related to that device will be reviewed and addressed. Oftentimes blood is drawn during clinic visits to measure the presence and level of the inhibitor. A half-life study may be performed to measure the body’s response to infusion therapy. Half-life is the amount of time that clotting factor remains active while in the bloodstream. It is measured as the amount of time it takes for half of the factor to disappear. A half-life study involves administering clotting factor and then measuring factor levels at specific time intervals, such as one, two, four and six hours after infusion. Additionally, during the immune tolerance clinic visit, x–rays and other radiographic studies may be ordered depending on the patient’s particular history and problems.
Patients visiting the IHTC for immune tolerance clinic will always see a nurse and hematologist. Other members of the multidisciplinary team are available as needed during these clinic visits. The physical therapist plays a particularly active role in the treatment of inhibitor patients, as one goal of immune tolerance therapy is to minimize joint damage caused by chronic bleeding. For more information on the IHTC’s immune tolerance clinic, call 317-871-0000 (toll free:877-256-8837 [877-CLOTTER]).
The purpose of prophylaxis in hemophilia treatment is to replace missing clotting factor on a regular basis to prevent joint bleeding and joint disease. Prophylaxis is recommended as the standard of care for individuals with severe hemophilia.2
There are two types of prophylaxis: primary and secondary. Primary prophylaxis is the use of regularly scheduled clotting factor infusions initiated prior to bleeding problems with the purpose of preventing bleeding episodes and related joint damage for a prolonged or indefinite period. Secondary prophylaxis is the use of regularly scheduled clotting factor infusions for a defined period of time (e.g., one month) initiated after bleeding problems begin with the purpose of treating a chronic joint or soft tissue bleed, or allowing an affected or “bad” joint to rest and heal.
Ideal candidates for primary prophylaxis are young patients (as young as one year old) who have experienced very few, if any, bleeding episodes. Prophylaxis initiated after joint damage has occurred may prevent additional bleeding episodes, but joint disease will continue to progress and prophylactic treatment will be less effective.
Primary prophylaxis for factor VIII deficiency usually involves an infusion schedule of three times per week; however, two times per week or every other day infusion schedules are possible for some patients. In factor IX deficiency, primary prophylaxis generally requires twice weekly infusions. IHTC hematologists work with the patient to develop an infusion schedule that will work for the patient and his family.
Patients using prophylactic treatment should visit the HTC every six months so the hematologist can evaluate the patient’s treatment plan and verify that it is working effectively. During the prophylaxis clinic, trough levels (i.e., pre–dose clotting factor levels) may be checked to ensure that the level never falls below 1%. If the trough level is less than 1%, the hematologist may decide to change the patient’s treatment to increase clotting factor coverage. The treatment plan may also be changed based on changes in the patient’s weight and bleeding history.
Many young patients using prophylaxis require a central venous access device, known as a port. This simplifies the infusion process in children who may resist infusion and whose veins may be difficult to access. During prophylaxis clinic, patients’ ports will be examined and port problems will be addressed. However, patients should call IHTC staff immediately at 317-871-0000 (toll free:877-256-8837 [877-CLOTTER]) to report any port problems, rather than waiting for prophylaxis clinic. Problems with ports can be severe, such as infections and blood clots. Port problems may also include difficulties in accessing the port for infusions and swelling or bruising at the port site.
Peripheral (IV) infusion is the goal for any child with a port. IHTC nurses are experienced in teaching parents to infuse their children, and eventually teaching the patients to infuse themselves. By the age of ten, most children’s ports have been removed and their parents are skilled at peripheral infusion.
For more information on the IHTC’s prophylaxis clinics, call 317-871-0000 (toll free: 877-256-8837 [877-CLOTTER]) or visit our Reading Room.
- Soucie J, Nuss R, Evatt B, Abdelhak A, Cowan L, Hill H, et al. Mortality among males with hemophilia: relations with source of medical care. Blood 2000;96:437-42.
- Medical and Scientific Advisory Council (MASAC) recommendation #179: MASAC recommendation concerning prophylaxis (regular administration of clotting factor concentrate to prevent bleeding). 2007. (Accessed January 7, 2010, at http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=1007.)