Factor Replacement Therapy for Bleeding Disorders

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Learning to infuse is an important part of your child’s long-term health care and it takes some time to learn. At this stage your child will probably begin to learn to self-infuse either at their bleeding disorders camp or their Hemophilia Treatment Center (HTC). Your health care provider or HTC team will help you and your child learn how to infuse. This may sound a bit scary, but this guide will walk you through it step-by-step. You and your child will be on the way to infusing before you know it!

This section of Infusion Basics covers:

Setting the Stage for Safe Infusion

Information About Getting Into a Vein

Steps to Do an Infusion

Treatment Logs

Needle Fear and Phobia

Setting the Stage for Safe Infusion

You will practice the steps for safe infusion together with the HTC team before you infuse your child by yourself or before your child learns to self-infuse on their own. You can also contact your HTC team with questions as they come up. There are different ways to access a vein and your HTC team will work with you and your child to determine the best way to do this.

It is important to be organized and to have a clean workspace that is free of clutter. This is a great way to get your child involved in doing an infusion. Gather all the supplies and medications needed to do your child’s infusion. There are general safety guidelines that everyone who infuses should follow to prevent infection:

Clean your space before you begin the infusion. Clean means your workspace is free of dirt. Sterile means germ free, and you will need to use cleaning supplies like bleach or isopropyl alcohol to clean your work area.
Wash your hands and arms up to the elbow with soap and warm water.
If doing an infusion on someone else, it is good to wear gloves during and after finishing the infusion and while throwing away the used supplies.
Put used needles and bloody gauze in your sharps container.
If you spill blood in the workspace, use chlorine bleach to clean it.

Tips to Keep Supplies Sterile

Some of the supplies you will use to do the infusion are sterile and you will need to take care that they are kept sterile during the infusion.

Here are some tips to keep supplies sterile:

The infusion needle will come in a sterile package, and it must be kept sterile after you open it.
If the needle touches anything (even a table that has been cleaned), the needle is no longer sterile and needs to be thrown away.
Always use sterile alcohol pads.
Never reuse or share needles.

Information About Getting Into a Vein

There are different ways to access or get into a vein. Your HTC team will work with you and your child to determine the best way to do this. Two ways are directly into the vein or using a special device called a port that will help make the process a bit easier.

Using a Port (Central Venous Access Device) (CVAD)

When infusing into a vein is too difficult, some patients and their caregivers may decide to use a central venous access device (CVAD) also called a port. A port is a device that is surgically inserted under the skin and connected directly to a vein. This allows a person to inject the medication into the port rather than having to inject directly into the vein. It is used more often in children, but adults can also find it useful. There is more of a risk for infection using a port as compared to using a vein.^1,2 Talk to your health care provider to figure out what will work best for your child.

Tips to Prepare Your Child for an Infusion

Finding your child’s vein for infusion can be hard. These suggestions will help you and your child feel ready and confident to find a vein, whether it is your first time infusing or you’ve been infusing for years.

Make sure your child has had plenty of water to drink before you begin. This will help the veins to be seen easier.
Have your child do a little exercise first like going for a walk or some other way to move their body. This will help the veins to be seen easier.
A warm bath or shower may be relaxing and will help you to find a vein. Apply a warm washcloth to the desired infusion site (Remember to clean the area again before you infuse your child).

Steps To Do an Infusion

Prepare the Supplies and Work Area

1. Gather supplies.

2. Make sure the workspace is well lit.

3. Clean your work area before you begin using a sterile alcohol pad. If you would like, you can also place down a clean cloth or disposable pad to work on.

4. Wash your hands and arms up to the elbow with soap and warm water.

5. Put on gloves (wearing gloves is required if you have a port or for anyone helping with your child’s treatment).

6. Mix factor and pull the medication into the syringe which is explained in the guidelines that come with your child’s medication (the package insert). You can also find information on how quickly you will need to finish the infusion before the medication is no longer good to use.

Find a Vein

1. Find a comfortable position with your child’s arm resting on a firm surface.

2. Use a tourniquet to help you decide the best vein to use.

a. Using a tourniquet will allow the vein to become full and larger feeling. Place the tourniquet 3 to 6 inches above where you would like to do the infusion.

b. If looking at veins in the arms, ask your child to make a fist or dangle their arm which will allow the veins to become bigger.

3. Use your finger to feel for and trace the direction of the vein.

4. Gently slap the skin at the infusion area. This can help visualize the vein.

5. Finding a vein can be tricky. Follow the tips from above.

Clean the Area of the Vein You Want to Use

1. Clean the site by using a sterile alcohol pad and rubbing in a circular motion from inside to outside the area.

2. Allow the skin to dry. Do not touch the clean site before you insert the needle.

Do the Infusion

1. With the angled surface (known as the bevel) of the needle up insert the needle into the vein at an approximately 30-degree angle. This means not directly straight down on top of the vein and not flat into the vein but somewhere in between.

2. Once the needle has entered the skin you can adjust the needle to go a little deeper into the vein.

3. Pull back on the syringe slightly to check for a blood return. A blood return is when you pull back on the syringe slightly and blood enters the tubing connecting the needle to the syringe. A blood return lets you know you are in the vein. Sometimes you might get a blood return without pulling back on the syringe.

4. Open the tourniquet. This is a good step to practice with your child. As your child gets older and more comfortable, they can help you do this step.

5. Gently push the factor into the vein while watching the area where you inserted the needle for any issues like factor leaking out of the vein. Some things to watch for are swelling, redness, and pain at the infusion site. If you think you are having this problem, stop infusing and remove the needle.

6. If you need to begin again to finish infusing the factor, you will need to attach a new needle to the syringe and start back at step 5 by cleaning the site again.

7. Once all the factor is infused remove the needle and put it in the sharps container.

Finish and Clean Up

1. Apply pressure to the infusion site using a gauze pad once completed and keep pressure for a minimum of 5 minutes. Then apply a bandage.

2. If you spilled factor or if there is blood on your work area, use chlorine bleach to clean it.

3. Log your child’s infusion in their treatment log.

Here is a checklist to easily follow the step-by-step process:

Steps for Infusion

Treatment Logs

A treatment log is a record of all your child’s infusions and any bleeds they might have. Here are some of the reasons why keeping a well-organized treatment log might come in handy:

Treatment Changes

The treatment log gives information to your child’s HTC or other health care providers about how well your child’s medication and treatment plan are working. After reviewing your child’s treatment log, their health care provider may decide to change your child’s medication dose, change their treatment plan, and identify target joints.

Insurance Purposes

Insurance companies also use information from your child’s treatment log to watch how bleeding symptoms are managed, review the effectiveness of treatment, and for insurance reimbursement. Not all insurers require treatment logs, but some do.

Disability Benefits

A treatment log can be helpful if you are applying for disability or financial help from the U.S. Social Security Administration on behalf of your child. With documentation of bleeding episodes and factor infusions or other treatments, this information log helps describe how your child’s daily activities are affected by the bleeding disorder. The treatment log can be very useful in a disability application.

In Case of a Recall or Issue With Factor or Other Treatment

Sometimes a manufacturer may recall medication due to patient safety concerns. Keep a record of the name, lot number, and manufacturer of your medications so that you can figure out if your child could be affected by the recall.

What to Include in a Treatment Log

The treatment log gives your child’s health care provider and HTC a record of how you have managed your child’s bleeding disorder. A treatment log should track all factor infusions, treatment information, and all bleeds. As your child becomes older, they can record the information in their logs.

Each time you or your child infuse, you should record:

The date and time of the treatment
Information on the medication used (include the brand name, the expiration date, the lot number, and how much was given)
The reason for the treatment (for example: scheduled prophylaxis, pre-surgery, injury)

If your child has a bleed, you should also record:

The date and time of the bleed
Where the bleed is in your child’s body and information about the bleed (how long it lasted, level of pain)
What was the cause of the bleed (for example: fall, blowing nose, unknown)
The treatment plan for the bleed from your child’s HTC or health care provider
Additional steps taken to manage the bleed (for example: pain medication, ice pack, bandages with pressure)

Types of Treatment Logs

The treatment log gives your child’s health care provider or HTC a record of how you have managed your child’s bleeding disorder. A treatment log can be kept by writing down information in a notebook, a file on your computer, or you can keep an electronic log using a mobile app. Some apps allow you to share your records with your child’s healthcare provider or HTC. There are many treatment log apps. Keep in mind how the app will use your child’s information.

Your child’s treatment log should be kept in a safe place that you can get to easily, and you should bring it to all your child’s medical appointments. It’s also a good idea to let a loved one know where they can find your child’s treatment log in the event of an emergency. The best treatment log is one that you will use so find the best one for you.

Needle Fear and Phobia

Needle fear and phobia is a common issue in many children. Here is a video that will give you information and tips to tackle needle fear and phobia:

References

1. Santagostino, E., & Mancuso, M. E. (2008). Barriers to primary prophylaxis in hemophiliac children: the issue of the venous access. Blood transfusion = Trasfusione del sangue, 6 Suppl 2(Suppl 2), s12–s16. https://doi.org/10.2450/2008.0031-08

2. Titapiwatanakun R, Moir C, Pruthi RK, Stavlo PL, Schmidt KA, Rodriguez V. Central venous access devices for paediatric patients with haemophilia: a single-institution experience. Haemophilia. 2009 Jan;15(1):168-74. doi: 10.1111/j.1365-2516.2008.01906.x. PMID: 19149849

Learning to infuse is an important part of taking care of your health in the long term. It might take some time to learn how to do it properly, but that's completely normal. At this point, you'll likely start learning how to infuse on your own, either at a special camp for kids with bleeding disorders or at the place where you usually go to see your health care providers. Your health care provider or the team at your hemophilia treatment center (HTC) will help you learn how to do it step-by-step, so don't worry if it seems scary at first. They will give you a guide that will explain everything in detail, and before you know it, you'll be able to infuse by yourself like a pro!

This section of Infusions Basics covers:

Setting the Stage for Safe Infusion

Information About Getting Into a Vein

Steps to Do an Infusion

Treatment Logs

Setting the Stage for Safe Infusion

You will practice the steps for safe infusion together with the HTC team before you infuse on your own. You can also contact your HTC team with questions as they come up.
There are different ways to access a vein and your HTC team will work with you to determine the best way to do this.
It is important to keep all your supplies tidy and in a clean area.
Gather all the supplies and medications needed to do your infusion.
There are general safety guidelines that everyone who infuses should follow to prevent infection:
- Clean your space before you begin the infusion. Clean means your workspace is free of dirt. Sterile means germ free.
- Use cleaning supplies, like bleach or isopropyl alcohol to clean the surface where you will be infusing. These cleaning supplies are very strong and can be dangerous, so make sure to have supervision when using them.
- Wash your hands and arms up to the elbow with soap and warm water.
- Put used needles and bloody gauze in your sharps container.
- If you spill blood in the workspace, use chlorine bleach to clean it.

Tips to Keep Supplies Sterile

Some of the supplies you will use to do the infusion are sterile, and they will need to be kept sterile during the infusion. Here are some tips to keep supplies sterile:

The infusion needle will come in a sterile package. It must be kept sterile after you open it.
If the needle touches anything (even a table that has been cleaned), the needle is no longer sterile and needs to be thrown away.
Always use sterile alcohol pads.
Never reuse or share needles.

Information About Getting Into a Vein

There are different ways to get into a vein. Your HTC team will work with you to determine the best way to do this. The best two ways are infusing directly into the vein or using a special device called a port that will help make the process easier.

Using a Port ^1,2

Sometimes, when it's hard to find a vein to infuse medicine, patients and their caregivers might decide to use a special device called a port. A port is a small device that is put under the skin and is directly connected to a vein. This makes it easier to inject medicine without having to find a vein each time. It is usually used more often in children, but adults can also use it if they need to. However, it's important to remember that using a port does come with a higher risk of infection compared to infusing directly into a vein. So, it's important to talk to your health care provider to figure out what method is best for you and to always follow their instructions carefully to stay safe and healthy.

Tips to Prepare for an Infusion

Finding a vein for infusion can be hard. These suggestions will help you feel ready and confident to find a vein, whether it is your first time infusing or you’ve been infusing for years.

Make sure you drink plenty of water before you begin. This will help the veins to be seen easier.
Do a little exercise first like going for a walk or some other way to move your body. This will help the veins be seen easier.
A warm bath or shower may be relaxing and will help you to find a vein.

Steps to Do an Infusion

Prepare the Supplies and Work Area

Gather supplies
Make sure the workspace is well lit.
Clean your work area before you begin by using a sterile alcohol pad. If you would like, you can also place down a clean cloth or disposable pad to work on.
Wash your hands and arms up to the elbow with soap and warm water.
Mix factor and pull the medication into the syringe which is explained in the guidelines that come with your medication (the package insert). You can also find information on how quickly you will need to finish the infusion before the medication is no longer good to use.

Find a Vein

Find a comfortable position with your arm resting on a firm surface.
There is a special band called a tourniquet that you can use to find a vein. Place the tourniquet tightly above your infusion site to help you decide the best vein to use.
- Using a tourniquet will allow the vein to become full and larger feeling. Place the tourniquet 3 to 6 inches above where you would like to do the infusion.
- If looking at veins in the arms, make a fist or dangle your arm which will allow the veins to become bigger.
- A tourniquet should not stay tight on your body for a long time.
Use your finger to feel for and trace the direction of the vein.
Finding a vein can be tricky. Here are some tips for finding a vein:
- Gently slap the skin in the desired infusion site.
- Apply a warm washcloth to the desired infusion site (Remember to clean the area again before you infuse).
- Drink water leading up to the infusion.

Clean the Area of the Vein You Want to Use

Clean the site by using a sterile alcohol pad and rubbing in a circular motion from inside to outside the area.
Allow the skin to dry. Do not touch the clean site before you insert the needle.

Do the Infusion

With the angled surface of the needle pointing up insert the needle into the vein at an angle. This means not directly straight down on top of the vein and not flat into the vein but somewhere in between.
Once the needle has entered the skin you can adjust the needle to go a little deeper into the vein.
Pull back on the syringe slightly to check for a blood return. A blood return is when you pull back on the syringe slightly and blood enters the tubing connecting the needle to the syringe. A blood return lets you know you are in the vein. Sometimes you might get a blood return without pulling back on the syringe.
Release the tourniquet so it is no longer tight on your body.
Gently push the factor medication into the vein while watching the area where you inserted the needle for any issues like the medication leaking out of the vein. Some things to watch for are swelling, redness, and pain at the infusion site. If you think you are having this problem, stop infusing and remove the needle.
If you need to begin again to finish infusing the factor, you will need to attach a new needle to the syringe and start back at step 5 by cleaning the site again.
Once all the factor is infused remove the needle and put it in the sharps container.

Finish and Clean Up

Apply pressure to the infusion site using a gauze pad once completed and keep pressure for a minimum of 5 minutes. Then apply a bandage.
If you spilled factor or if there is blood on your work area, use chlorine bleach to clean it.
Log your infusion in your treatment log.

Here is a checklist to easily follow the step-by-step process:

Steps for Infusion

Treatment Logs

A treatment log is a special record of all your infusions and any bleeds you might have. It's important to keep a well-organized treatment log for a few different reasons. For example, your health care providers might look at your treatment log to see how well your medication and treatment plan are working, and they might use that information to decide if any changes need to be made. Insurance companies might also use information from your treatment log to see how well your bleeding symptoms are being managed and to decide if they will cover the cost of your treatment.

By keeping track of your bleeding episodes and treatments, you can show how your daily activities are affected by your bleeding disorder. Additionally, if there is ever a recall or issue with your medication, it can be helpful to have a record of the name, lot number, and manufacturer of your medications so you can figure out if you might be affected.

Overall, keeping a treatment log is a really important part of managing your bleeding disorder, and it can be very helpful for you and your health care team in making sure you get the best care possible.

What to Include in a Treatment Log

A treatment log is like a special notebook that helps you and your health care team keep track of your bleeding disorder. This way, you can make sure you're getting the right treatment and care, and your health care team can learn how your body reacts to different treatments.

In your treatment log, you should write down all your factor infusions and any bleeds you might have.

Each time you infuse, you should write down:

The date and time of the treatment
Information about the medication you used (like the brand name, expiration date, and how much you used)
The reason you needed the treatment (like if it was scheduled or because you got hurt)

If you have a bleed, you should also write down:

The date and time of the bleed
Where the bleed is in your body and how it feels (like if it hurts a lot or a little)
What your health care provider told you to do to treat the bleed
Any extra things you did to help, like using ice or taking pain medicine.
What might have caused the bleed, like if you fell or got hit.

Keeping a treatment log might seem like a lot of work, but it's really important! It helps you and your health care team make sure you're getting the best care possible.

Types of Treatment Logs

A treatment log is a record of all the treatments and bleeds related to your bleeding disorder. You can keep this record by writing down information in a notebook or using a mobile app. There are many apps available, but you should choose one that keeps your information safe and allows you to share it with your health care provider. Keep your treatment log in a safe place and bring it with you to all your medical appointments. It’s also a good idea to let a trusted person know where you keep your treatment log in case of an emergency. The most important thing is to use a treatment log that works best for you.

References