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%.) Factor dosage adjustments 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.
