Factor VIII (FVIII) is a protein crucial for blood clotting. A deficiency or defect in this protein causes Hemophilia A, an inherited bleeding disorder characterized by prolonged or excessive bleeding. Treatment involves replacement therapy, where concentrated FVIII is administered directly into the bloodstream to restore the body’s ability to form a clot.
Home infusion of this factor concentrate is the standard of care for many patients, especially those on prophylactic regimens. This practice offers independence and the ability to treat bleeding episodes rapidly. This guide supplements the extensive, hands-on instruction provided by a hemophilia treatment center nurse and is not a substitute for medical instruction.
Preparing the Factor VIII Dose and Supplies
Preparation requires meticulous attention to hygiene and organization to maintain sterility and prevent infection. Start by thoroughly washing hands and ensuring the work surface is clean, dry, and uncluttered. All supplies must be gathered and checked.
Gathering Supplies
The necessary supplies include:
- The vial of lyophilized FVIII concentrate.
- The diluent (sterile water or saline).
- The administration syringe.
- A needleless transfer device or vial adapter.
- An approved sharps container.
Before use, inspect the FVIII vial and diluent syringe to confirm the correct dosage, check expiration dates, and look for any cracks or damage. Allow the FVIII powder and diluent to warm to room temperature, which aids dissolution and makes the infusion more comfortable. Most modern products use a needleless transfer system, like a vial adapter, to connect the diluent syringe to the factor vial, which minimizes the risk of contamination.
Once the diluent is transferred, reconstitution begins by gently mixing the powder and liquid. Avoid shaking the vial, as this can create foam or damage the delicate protein structure of the FVIII molecule. Instead, gently swirl or roll the vial between the palms until the powder is fully dissolved. Inspect the solution visually; it must be clear, colorless, and free of particulate matter. Discoloration indicates the product should not be used.
After reconstitution, draw the factor solution into the administration syringe, often by inverting the vial and slowly pulling back the plunger. Detach the syringe from the vial adapter and connect it to the infusion set (e.g., butterfly needle and tubing). Before venipuncture, prime the syringe by gently pushing the plunger to remove air bubbles and fill the tubing up to the needle hub.
Performing the Intravenous Infusion
The intravenous infusion requires careful technique for safe and effective delivery into the bloodstream. Select an appropriate vein, typically in the arm or hand, choosing a site that is straight, visible, and accessible. Applying a tourniquet four to six inches above the intended insertion site will help make the vein more prominent.
Cleanse the site thoroughly using an alcohol swab or antiseptic wipe, working outward from the center in a circular motion. Allow the antiseptic to air-dry completely before inserting the needle. Insert the butterfly needle into the vein at a shallow angle (10 to 30 degrees) with the bevel facing upward.
Successful venipuncture is confirmed by observing a “flashback” of dark red blood in the needle hub or tubing. Once blood return is confirmed, secure the needle and immediately release the tourniquet before starting the infusion. Infusing against the pressure of a tourniquet can cause discomfort or damage the vein.
The Factor VIII solution is then administered slowly and steadily by gently depressing the syringe plunger. A slow infusion rate is important to prevent adverse reactions, with many products recommending a duration of two to five minutes for the injection. If the patient experiences burning at the injection site, tingling in the lips or hands, or discomfort, immediately slow the rate of infusion. If swelling or pain occurs around the needle site, the needle may have slipped out of the vein (infiltration), and the infusion must be stopped immediately to prevent the factor from leaking into surrounding tissue.
Post-Infusion Care and Safety Monitoring
Upon completion of the infusion, the needle is carefully withdrawn from the vein while simultaneously applying gentle pressure to the injection site with a sterile gauze pad. Maintaining firm pressure on the site for a minimum of five minutes is necessary to ensure bleeding has completely stopped and to reduce the likelihood of bruising. Once the pressure is released and the site is dry, a small adhesive bandage can be applied.
Immediate and proper disposal of all used materials is a non-negotiable safety step to prevent accidental needle-stick injuries. The entire infusion set, including the butterfly needle, the syringe, the empty factor vial, and the vial adapter, must be placed directly into a designated, puncture-proof sharps container. Used gauze pads and other non-sharp waste can be discarded in regular trash.
Maintaining an accurate treatment log is an essential component of home therapy, providing a record for the patient and the healthcare team. The log must document:
- The date and time of the infusion.
- The dose administered.
- The specific lot number and expiration date of the Factor VIII product.
- The location of the injection site.
This documentation is necessary for tracking product usage and investigating any potential product-related issues or adverse events.
The patient must be monitored for signs of an immediate adverse reaction for a short period following the infusion. While rare, allergic reactions can occur and may present as symptoms such as hives, rash, itching, tightness in the chest, or difficulty breathing. Should any of these signs appear, the infusion must be stopped immediately if it is still running, and emergency medical help should be sought at once.