Hemophilia is a condition in which blood doesn't clot like it should. External bleeding is definitely a concern, but it's at least visible. Internal bleeding from hemophilia can be silent and dangerous. If it isn't treated properly, it can lead to bleeding in the joints, inside the skull, or even within the brain.
If you have a loved one with hemophilia, it's important to understand the condition, the therapy or infusions they may need to receive, and how you can help.
Many hemophilia treatments can be done safely by a home health aide, skilled nurse, or family caregiver with at-home infusion, which reduces the need for additional doctor appointments and travel time.
Let's take a closer look at hemophilia infusions and how in-home care can help.
What Are Hemophilia Infusions?
If you have hemophilia, you can experience heavy bleeding because your body is missing or deficient in proteins called clotting factors.
The main type of treatment for hemophilia is factor replacement therapy, an infusion directly into a vein that gives people the proteins, or clotting factors, they need. Infused factors temporarily replace missing clotting factors to prevent or stop bleeding.
“Factor replacement therapy by infusion is the cornerstone of hemophilia management and significantly reduces bleeding episodes and increases quality of life,” says Joshua Allen, RN, Senior Director of Infusion Services at BrightStar Care.
Emerging treatments for hemophilia include gene therapy and nonfactor replacement, which uses an antibody called emicizumab that mimics the factor's blood-clotting function.
How Does Hemophilia Type Affect Infusions?
Hemophilia is almost exclusively a genetic disorder that can be inherited. Males are affected more often because the hemophilia gene is on the X chromosome, but women can get hemophilia as well.
There are two main types of hemophilia that commonly respond to infusions. Less common forms of hemophilia usually have different treatment methods. Your doctor will diagnose the type of hemophilia before making a long-term hemophilia treatment plan.
Hemophilia A
About 1 in 400 people have what's known as "classic hemophilia." This type means you're missing a protein called clotting factor VIII. The treatment focuses on replacing the missing or deficient factor through transfusion. Plasma-derived factor VIII comes from human-donated plasma, so it carries a small risk of transmitting blood-borne pathogens. Factor VIII that's made in a lab is called recombinant factor VIII and is more commonly used.
Hemophilia B
This much less common type affects about 1 in 5,000 people. A different blood protein called factor IX is missing or deficient. As with hemophilia A, it's treated by replacing the missing or deficient factor through transfusion of either plasma-derived or lab-made recombinant factor IX.
Hemophilia C
This rare form of hemophilia affects only about 1 in 100,000 people. It involves low levels of clotting factor XI, but it may not require any treatment other than plasma infusion during surgery or other ways of preventing excessive bleeding.
Acquired Hemophilia
Even rarer than type C is acquired hemophilia, affecting about 1 in 1 million people. It's not caused by an inherited gene, but by disorders where the immune system mysteriously attacks healthy tissue. Many people with this type rely on anti-hemorrhaging medicines when needed rather than regular infusions.
Von Willebrand Disease (VWD)
Von Willebrand disease is a bleeding disorder similar to hemophilia A but with a different cause. It involves a defect in a protein called the von Willebrand factor, which carries and stabilizes factor VIII. The main treatment is an injection of synthetic hormone desmopressin before surgeries, but in severe cases, factor replacement therapy may be recommended.
Preventive vs. On-Demand Hemophilia Treatment
As the explanations of the different types show, hemophilia treatment is sometimes done on a regular basis and sometimes done as needed.
- Preventive or prophylactic home infusion therapy involves routinely injecting concentrated factors into a vein to keep the body at the ready in case any accidental bleeding event happens. These can be done at home by infusion nurses, trained caregivers, or by yourself on a prescribed schedule rather than going to a hospital or clinic every time.
- On-demand infusions or injections that aim to improve clotting are done before surgeries and other procedures. These are performed by healthcare professionals on-site as needed.
How Hemophilia Infusions Are Administered at Home
You can have a skilled nurse provide home infusion therapy, or you may have them teach you or your loved one how to do the infusion. After training and supervision, infusions can be done independently with a nurse checking in when needed.
Here is a step-by-step guide for how at-home hemophilia infusions are typically prepared and administered:
- Wash your hands thoroughly.
- Gather your supplies, including factor concentrate, diluent, syringes, needles, alcohol wipes, a tourniquet, and a sharps container.
- Double-check the expiration dates and the dosage.
- Reconstitute the factor by mixing the powder concentrate with diluent, often sterile water, according to the healthcare provider's instructions. Some vials come prefilled with diluent. Don't shake the mixture—instead, roll the syringe in your hands until the powder is dissolved.
- Wash your hands again and prepare the injection site. Draw enough factor from the syringe into the tubing so there's no air in the tube.
- If injecting directly into a vein, apply the tourniquet and feel for a pulse. Clean the injection site, and insert the needle into the vein. If using a port or central line, make sure the site is cleaned with alcohol and allowed to dry. Insert the needle or needleless device into the port.
- Remove the tourniquet, if using, and inject the factor at the prescribed rate. Infusions usually take 5-10 minutes.
- Flush the line as directed, and hold pressure on the site for several minutes. Bandage the site.
- Dispose of the waste, place needles in the sharps container, and wash your hands again.
- Monitor for about an hour for immediate adverse or allergic reactions, such as itching, rash, fast heartbeat, or dizziness. Continue to watch for delayed reactions up to 24 hours.
Keep in mind that these are only general instructions. Anyone performing an at-home hemophilia infusion should follow the specific training they received. If you're ever unsure about the procedure, contact your healthcare provider.
Hemophilia Infusion Best Practices and Guidelines
To ensure safe and effective in-home infusion therapy, it's important to follow these guidelines:
- Proper Storage and Handling: Store and handle factor concentrate according to the manufacturer's instructions. Most vials are refrigerated, but some can be stored at room temperature. Protect vials from light. Maintain a clean and sterile environment.
- Proper Dosing and Timing: The dosage depends on the type of hemophilia and if the infusion is being given preventatively or to treat a bleeding episode. Be sure you're clear on dosing and timing of treatment.
- Awareness of Complications: It's important to recognize the signs of allergic reactions, particularly anaphylaxis, during an infusion. Another possible complication is the development of inhibitors, antibodies made by the immune system that block the clotting factor from working. In some cases, inhibitors can also cause allergic reactions. Always be ready to contact your healthcare provider promptly if you notice unusual symptoms or call 911 for life-threatening concerns.
Hemophilia Treatment at Home: Are You Ready?
Before you start hemophilia treatment at home, be sure you're well-trained and comfortable with the procedure. A nurse or healthcare provider usually leads home training.
Children as young as 8 years old may be able to take care of their infusions with supervision or on their own, according to Allen.
Regardless of age, ask yourself these questions to determine if you're ready for at-home hemophilia infusions:
- Are you willing and able to take responsibility to learn the procedure, including safe handling and accurate record-keeping?
- Do you have access to a clean, well-lit space for preparation and administering the factor every time it's needed?
- Do you have a sharps disposal, proper storage for factor concentrate, and a place to keep extra supplies?
- Have you checked with your insurance for at-home infusion coverage or have another way to pay for home treatment?
How BrightStar Care Empowers Infusion Clients and Caregivers at Home
Hemophilia treatment at home can help save on the cost of emergency department visits and bring normalcy to your or your loved one's life by remaining in a familiar environment. Plus, if a bleed occurs, treatment can be started quickly. It's important for caregivers to receive ongoing education and support for hemophilia treatment options so they can safely and effectively deliver treatment.
BrightStar Care® caregivers and skilled nurses can provide home infusion services or help support families with hemophilia treatments. Whether you're looking for in-home care services or a reliable infusion services partner for your organization, our experienced local care team members are ready to help. Find a location near you, contact us online, or call (866) 618-7827 to learn more about how BrightStar Care offers A Higher Standard®.