Home Infusion Treatment for Hemophilia
When you are ready to administer a home infusion or factor replacement therapy for treatment of hemophilia, please follow this 10-step procedure.
Step 1 – Find a Responsible Assistant
Never infuse alone. Make sure someone is with you if you are infusing yourself. If complications arise, it is critical that someone be there to assist you or to call for help.
Step 2 – Calculating Dosage
The amount of factor concentrate suggested for a particular bleeding episode is determined by levels that stop bleeding for most people, most of the time; however, adjustments may be necessary. If in doubt, treat with the suggested amount and check with the treatment center for more assistance.
Calculating the number of units of factor to use is easy. The dosage is specified in units of factor per kilogram of body weight (u/kg). To convert the patient's weight in pounds to weight in kilograms, just divide the pounds by 2.2. To calculate the number of units needed for a single dose, multiply the weight in kilograms by the number of units in a single dose, as described in the following tables. A double dose, of course, requires twice as many units of factor.
Vials vary in the number of units each one contains. To decide how many vials to use, check the number of units in each vial and use enough vials to add up to the total number of units required. Call the hemophilia nurse if you have any questions.
Dosage instructions are specified as either single or double. The number of units required varies according to the following three criteria:
- The type of hemophilia, A or B
- The factor product selected
- The type and site of the bleed
The two tables below list dosages for hemophilia types A and B. The tables divide specific episodes into categories of routine, complicated and serious. Locate the specific bleed that you will treat in these tables to determine whether to administer a single or double dose. Use the instructions above and the number of units / kg of body weight listed in the tables to determine the number of vials of concentrate to prepare. Remember that any unusual pain or discomfort may signify a bleed. Do not delay treating. If you have any questions or concerns contact the Hemophilia Center staff immediately.
Treating Hemophilia A
| Type of Bleed | Treatment Action |
| Routine Bleeds | |
| Knee, ankle, elbow, shoulder, other joints except hip | Administer single dose* |
| Soft tissue | Administer single dose* |
| Early muscle bleed | Administer single dose* |
| Mouth bleed: gum, tongue, or lip | Administer single dose* |
| Note: If a routine bleed does not resolve within 3 days, call the center. | |
| Complicated Bleeds: Call immediately. The patient could require additional treatment. | |
| Hip, groin, buttock, or upper thigh | Call Center and administer double dose** |
| Calf or forearm | Call Center and administer double dose** |
| Gastrointestinal | Call Center and administer double dose** |
| Serious Bleeds: Go immediately to the emergency room. If time allows, prepare double dose to take with you. | |
| Head, Intracranial | Call or go to the emergency room immediately |
| Neck, throat, floor of mouth | Call center and administer double dose** |
| *Single dose denotes 25 units/kilogram | **Double dose denotes 50 units/kilogram |
| | |
Treating Hemophilia B
| Type of Bleed | Treatment Action |
| Routine Bleeds | |
| Knee, ankle, elbow, shoulder, other joints except hip | Administer single dose* |
| Soft tissue | Administer single dose* |
| Early muscle bleed | Administer single dose* |
| Mouth bleed – gum, tongue, or lip | Administer single dose* |
| Note: If a routine bleed does not resolve within 3 days, call the center. | |
| | |
| Complicated Bleeds: Call immediately. The patient could require additional treatment. | |
| Hip, groin, buttock, or upper thigh | Call center and administer double dose** |
| Calf or forearm | Call center and administer double dose** |
| Gastrointestinal | Call center and administer double dose** |
| | |
| Serious Bleeds: Go immediately to the emergency room. If time allows, prepare double dose to take with you. | |
| Head, Intracranial | Call or go to the emergency room immediately. |
| Neck, throat, floor of mouth | Call center and administer double dose** |
*Single dose denotes 40 units/kilogram of plasma-derived concentrate or 50 units/kilogram of BeneFIX.
| **Double dose denotes 80 units/kilogram of plasma-derived concentrate or 100 units/kilogram of BeneFIX. |
Step 3 – Washing Hands
Hand washing is the single most effective tool in combating disease. Hands should be washed with soap and warm water for at least 15 seconds before infusing and after removing gloves. Immediately after washing your hands, put on a new pair of gloves.
Step 4 – Reconstituting Factor
Check the expiration date on the box before using factor concentrate.
Do not expose factor concentrate to extreme temperatures, rather, store the vials in the refrigerator to assure a constant safe temperature. Bring the bottles of concentrate and water to room temperature before using them by rolling them between your hands. When the bottles are room temperature, remove the plastic caps from both bottles and cleanse the stoppers with alcohol swabs.
Remove the protective covering from the short end of a double-ended needle (also called a transfer needle) or use a needless transfer device. Be careful not to touch the exposed needle or cleansed stopper. Insert the exposed needle through the center of the stopper in the water bottle.
Next, remove the cover from the other end of the double-ended needle. Taking care not to touch the exposed needle, turn the water bottle upside down over the concentrate bottle and insert the free end of the needle through the concentrate stopper. A vacuum in the concentrate bottle will draw in the water. Aim the flow of the water toward the side of the vial to avoid foaming.
When the water bottle is empty, disconnect the two bottles by removing the needle from the concentrate bottle stopper. Place the water bottle and attached double-ended needle in a puncture resistant (sharps) container.
Gently swirl the bottle of concentrate and water mixture until the powder is dissolved – usually two minutes or less. You must infuse within three hours of mixing the solution. Next, attach a filter needle to a syringe, pull back on the plunger to place air in the syringe and insert the needle into the upright bottle of concentrate. Now, with the bottle inverted above the needle, push the plunger at the bottom of the syringe. Pull back on the plunger to withdraw all of the solution into the syringe. Remove the filter needle, dispose of the empty bottle and filter needle into the sharps container, and set aside the syringe so that you can prepare an infusion site.
Step 5 – Preparing the Site
You can use one of the following three areas on each arm to infuse factor:
- The back of the hand
- The forearm, or
- The inside of the elbow
Do not use the veins on the inside of the wrist. Even though they look adequate, they are small and unsuitable for infusing. Do not use the same vein each time you infuse. After many infusions, scar tissue thickens the walls of the vein making venipuncture difficult.
Place a tourniquet (rubber tube) just above the area where you are trying to locate a vein. The tourniquet backs up blood in the arm and distends the vein. This action makes the vein large and easy to find. If veins do not show up well, you may need to tighten the tourniquet, but do not leave the tourniquet in place for longer than a few minutes. If you have difficulty finding a vein, remove the tourniquet and try the other arm.
If a vein still does not become prominent, remove the tourniquet and try hanging the arm down while opening and closing the fist slowly. While the arm is in the down position, apply the tourniquet and then return the forearm to a normal horizontal position. The veins should be more prominent. If you need additional help making the veins more prominent, soak the arm in a basin of very warm water for five minutes or encourage fluid intake for a child who has not had much to drink prior to treatment.
Step 6 – Infusing the Factor
Select a straight length of vein for infusion. If you use your elbow, make sure your arm is as straight as possible. To prevent a vein from rolling, stabilize it by either applying a second tourniquet below the site of venipuncture or pulling the skin back over the vein with a thumb or other free finger. Now, with your materials ready, clean the skin with an alcohol swab.
Hold the tabs of the needle with the tip pointing up the limb towards the body. With the tip of the needle down against the skin, guide the needle at about a 30 to 45 degree angle through the skin and into the vein. If you are in the vein, you will see blood coming back into the tubing. Allow it to flow to the end of the tubing, then hook up your syringe to the tubing, being careful not to pull the needle out. Release the tourniquet. If necessary, put a small piece of tape over the needle to hold it in place. Gently push on the syringe and infuse the concentrate slowly over several minutes, pulling back gently once or twice to confirm placement.
If you are unsuccessful when you first insert the needle, do not take off the tourniquet and do not remove the needle. Feel again for the vein and move the needle toward it. You may be able to enter the vein without piercing the skin a second time. If you are unsuccessful after one or two attempts, remove the needle and tourniquet and start again with a new needle.
If any pain or swelling occurs at the site of the needle, it may indicate that the needle has not entered the vein, has slipped out of the vein, or the concentrate is leaking out of the vein. Check by pulling back on the syringe to see if there is any blood return, but do not pull so far back that blood enters the concentrate in the syringe. If there is no blood return, remove the needle and try again at another site with a new needle.
If you see a good blood return in the tubing at first but the blood then stops, you have probably gone through a vein and are underneath it. If you continue with an infusion, you will cause bruising and swelling. Again the needle must be removed and a new infusion attempted.
When the needle is properly inserted, completely infuse the factor by pushing slowly on the plunger with a smooth motion. When finished, use the tabs to remove the needle and apply pressure firmly over the puncture until bleeding stops. To maintain the stability of the vein and prevent bruising, avoid hanging the arm or hand down at the side for 5 minutes following infusion.
Step 7 – Disposing of Waste, Cleaning Up, and Re-washing Hands
Safe waste disposal and sanitation are an important step in preventing the spread of infection by contaminated needles and syringes. To prevent accidental needle-sticks, do not re-cap, bend, break, or remove needles from syringes. Dispose all needles and syringes in a puncture-resistant, sharps container. Also place empty concentrate vials, band-aids and any bloodstained items into the container. Securely seal the filled containers and dispose of them at your next visit to the clinic or through your home delivery provider. Remember to always keep the containers out of the reach of children.
Disinfect all work surfaces and any area where spills occur using either of the following products:
- A commercial germicide such as PDI SaniCloth or Medaphene Scrub
- A fresh solution of chlorine bleach and water (Note: dilute ¼ cup of household chlorine bleach with 1 gallon of water.)
Wear disposable latex or vinyl gloves to clean up surfaces soiled with body fluids. Do not use gloves with tears or cracks. Use the gloves only one time and discard them.
Soak up any spills with disposable paper towels. Place these towels in a plastic garbage bag and seal the bag before putting it into your normal garbage receptacle. Always discard these garbage bags along with the waste, rather than saving them for reuse.
Using paper towels or a disposable cloth, clean the work area with a solution of household detergent and water. Follow by disinfecting the surface with one of the disinfectants discussed above. If you used a mop, dust pan, broom, bucket, or other such tool to clean up a spill, also clean it immediately with disinfectant.
If you soiled any clothing or used cloths that are not disposable, do not wash them with other laundry. Launder them separately using detergent and chlorine bleach.
After you have disposed of all waste and cleaned up, rewash your hands, even though you were wearing gloves. This step is critical in protecting you and your family against the possible spread of blood-borne diseases.
Step 8 – Recording Treatment
If you treated a specific bleed, download and complete a Hemophilia Home Treatment Record (PDF).
You must have Adobe Acrobat" Reader installed on your computer to read this file. You can download Adobe Acrobat" Reader at Adobe's Web site by selecting the version appropriate for your type of computer.
If you have completed a prophylactic treatment, download and complete a Hemophilia Intensive Treatment Log (PDF). Submit the forms to the Hemophilia and Thrombosis Center each month using the self-addressed envelopes provided by the center. Each form is checked and recorded by the center, creating a permanent record of the types of bleeds, dosages, and number of treatments necessary to resolve bleeding episodes.
The Home Treatment Record monitors the use of factor concentrate for specific bleeding episodes. To make the records useful to the center, the information should include the date and time symptoms began, the time and type of concentrate administered, the location of the bleed, any absences from work or school, any pain medications taken, and identification of the person administering the infusion. Space is also provided for families to supply specific comments and feedback about treatments.
The Intensive Treatment Log monitors hemophilia treatment. Space is provided for date, time, type of concentrate and units of concentrate administered. Again, space is provided for relevant comments and helpful feedback. Remember that Home Treatment Logs are a vital component of the center's home treatment program. A condition for participating in the program is compliance in filling out these forms at the time of the infusion and submitting them to the center on a monthly basis.
Step 9 – Watching for Allergic Reactions
Some people develop an allergic reaction to transfusion when they receive blood products such as plasma, cryoprecipitate, or clotting factor. With a mild allergic reaction, signs and symptoms may include:
- Itching
- Hives or rash
- Fever
- Stuffy or runny nose
- Coughing
If a mild reaction develops, use Benadryl™ to reduce symptoms. Benadryl™ can be taken by mouth as a pill or liquid. Check with your child's pediatrician for dosage and schedule. Be sure to contact the Hemophilia Treatment Center as soon as possible if a reaction occurs and remember to record the symptoms in your bleeding log.
While it is rare to have a serious allergic reaction following an infusion, the possibility makes it important to have someone nearby whenever infusing. The signs and symptoms of a severe reaction include:
- Wheezing
- A tight, constricted feeling in the throat
- Shortness of breath
- Back pain
- Swelling in the face
If any signs of a severe reaction occur, take the following immediate actions:
- Give Benadryl™.
- Go immediately to the nearest hospital.
- Have someone call the Hemophilia Treatment Center, 513-636-4200.
Non-allergic complications can also occur following intravenous injection of clotting factor. These include:
- Skin infection at the site of injection
- Blood infection from contamination of needles or syringes
- Local bruising or bleeding as a result of the venipuncture technique
Accidental needle sticks are a complication of home treatments. If anyone other than the patient is accidentally stuck with a used needle, notify the center immediately.
Step 10 – Managing Pain
When treating pain from a bleeding episode, it is important to use only acetaminophen-based products such as Tylenol™. You can give Tylenol immediately before or following a treatment with clotting factor.
Hemophilia patients should never take aspirin or any aspirin-containing product. Aspirin inhibits platelet stickiness and may increase the risk of bleeding. Aspirin also irritates the lining of the stomach and can cause gastrointestinal bleeds. You may download a list of products that contain aspirin in portable document format (.pdf).
You must have Adobe Acrobat" Reader installed on your computer to read this file. You can download Adobe Acrobat" Reader at Adobe's Web site by selecting the version appropriate for your type of computer.
Avoid ibuprofen products as well, because they too impair the action of platelets. Hemophilia patients should have all of their medicines, including over-the counter preparations, cleared by their doctor or pharmacist.
EMLA" is a topical anesthetic cream that contains lidocaine and prilocaine, and it effectively prevents painful sensations at the site of infusions. EMLA prevents the pain of venipuncture and enables children to receive treatment without fear. However, this product must be applied about an hour before an infusion and covered with an airtight bandage. Therefore, while EMLA is very helpful for prophylactic treatments and less serious injuries, the time it takes to become effective makes it unsuitable for use following injuries that require immediate treatment.
Written 4/06
