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Bleeding Disorders

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Bleeding Disorder Clinics

Comprehensive Care
Outreach Program
Immune Tolerance Clinic
Phrophylaxis Clinic

Comprehensive Care

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% reath 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.” – Blood 15 July 2000; Vol. 96, No. 2: 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 sequellae (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.

Outreach Program

In order to provide comprehensive care to the entire population of Hoosiers with bleeding disorders, the Indiana Hemophilia & Thrombosis Center Inc. (IHTC) annually offers a series of outreach comprehensive clinics throughout the state. The IHTC’s multidisciplinary medical team travels to Fort Wayne, South Bend, Merrillville, Evansville, Middlebury and Bloomington to bring comprehensive healthcare services to patients living in these areas. The team includes adult and pediatric hematologists, treatment nurses, a research nurse, dietitian, dental hygienist, social worker, physical therapist, pharmacist and genetic counselor. In some locations, the outreach clinic might concentrate on a specific patient population or disorder. For example, the Middlebury clinic primarily serves people with hemophilia from the northern Indiana Amish communities. In Bloomington, a large population of patients with von Willebrand disease is served by the outreach clinic. The IHTC’s outreach programs are state and federally funded; there is no charge to patients for the multidisciplinary care team’s evaluation. However, patients or their insurers are responsible for costs associated with laboratory tests and radiological evaluations.

Immune Tolerance Clinic

Some hemophilia patients develop an inhibitor. Inhibitors are antibodies that the body makes which are directed against the missing or abnormal clotting factor. This inhibitor prevents the factor from doing its job during bleeding episodes. This serious problem is discovered either through routine laboratory tests or when a patient has a bleeding episode and factor does not seem to 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 much as two times per day) so that the body will get used to the factor and stop making an antibody. Dependent upon the level of the inhibitor, additional infusion therapy may be required to stop bleeding when it happens.

The frequency of clinic visits for a patient on immune tolerance therapy is based upon each patient’s situation. Patients who are repeatedly bleeding while on immune tolerance therapy may require more frequent infusions and visits in order to manage these bleeding events.

During a routine clinic visit for a patient on immune tolerance therapy, a patient history will be obtained regarding bleeding episodes experienced and how well current therapy is 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 determine if the inhibitor is still present and its level. At times, response to infusion therapy through a half-life study may also be obtained (half-life is the amount of time that factor survives in the body or circulation; it is measured in the amount of time it takes for half of the factor to disappear). This is accomplished by giving factor and then measuring factor levels at specific times such as one, two, four and six hours, etc., after infusion. Additionally, during the immune tolerance clinic visit, x–rays and other radiographic studies may be ordered based upon the patient’s particular history and problems.

A physician and nurse staff the IHTC immune tolerance clinic. A physical therapist is available, as are other members of the IHTC’s multidisciplinary team for any identified problems. Notably, the physical therapist tends to be fairly involved with patients with inhibitors.
For more information about the IHTC’s immune tolerance clinic, call 317-871-0000.

Prophylaxis Clinic

In a medical sense, and from a hemophilia treatment standpoint, the word “prophylaxis” simply means to prevent joint disease.

There are two types of prophylaxis, primary and secondary. Primary prophylaxis is the initiation of clotting factor infusion therapy on a regular basis with the purpose of preventing hemorrhagic (bleeding) episodes for a prolonged if not indefinite period of time. Secondary prophylaxis is the initiation of infusion therapy on a regular basis for a defined period of time (e.g., one month) with the purpose of treating a joint that continues to re-bleed, or providing clotting factor coverage in order to rest an affected or “bad” joint.

The ideal candidate for primary prophylaxis is a young patient (as young as one year old) who has experienced very few bleeding episodes. Prophylaxis initiated after joint damage has occurred is not as effective. In this case, joint bleeding may stop, but joint disease will continue to progress.

Primary prophylaxis for factor VIII patients is usually an infusion schedule of three times per week; for factor IX patients, it is twice weekly. The actual days will vary based on each patient.

While on prophylaxis, patients should visit their local comprehensive hemophilia treatment center every six months, minimally, so their hematologist can obtain their bleeding history to be sure that the infusion plan is working. During the clinic visit, trough levels (i.e., pre–dose factor levels) may be checked. (For example, most patients with factor VIII infuse Monday, Wednesday and Friday. The IHTC monitors the patient’s factor VIII level on those days before the scheduled infusion to make sure the level never falls below 1%.) will be made based upon patient weight changes and bleeding events.

Most patients on prophylaxis require a central venous access device, known as a port. For patients with ports, during each clinic visit port problems will be addressed. Specific problems may include a history of fever, a problem with accessing the port or infusion of factor, or swelling or bruising at the port site.

A small numbers of patients with hemophilia may develop inhibitors, which are antibodies to infused factor. Inhibitor titers (strength of the inhibitor measured in volume) may be obtained if clinically indicated, such as the patient experiencing increased bruising or problems controlling bleeding. Additionally, the patient examination will include an evaluation of the musculoskeletal system (joints and muscles).

In general, the IHTC’s prophylaxis clinic is problem solving oriented and tailored to each of its patients’ prophylaxis issues. A physician and nurse staff the prophylaxis clinic. A physical therapist is available, as are other members of the multidisciplinary team for any identified problems.
For more information about the IHTC’s prophylaxis clinic, call 317-871-0000.