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Transcription

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Please note:
• Opinions and materials presented by the nurses are theirs alone and do not necessarily reflect those of the sponsor.
• The information provided is not intended to be a substitute for professional medical advice. A licensed healthcare professional should be consulted for diagnosis and treatment of any and all medical conditions.
Made possible through an educational grant from GRIFOLS
EDL35-1-US-10
Acknowledgment
The information contained in this flash drive was prepared by:
Becki Berkowitz RN; Hemophilia Treatment Center of Las Vegas
Sue Geraghty RN, MBA; UCD Hemophilia and Thrombosis Center
Judy Kauffman RN, MS, CPNP; Kansas City Regional Hemophilia Center
Jennifer La Franco RN; Mary M. Gooley Hemophilia Center
Jennifer Maahs MSN, PNP; Indiana Hemophilia and Thrombosis Center
Susan M. Peterson MSN, ARNP; University of Kentucky HTC
Susan Zappa RN, CPN; Fort Worth Bleeding Disorders Program
Common issues for different age groups
Hemophilia and your child’s development
Toddlers - age 1 to 2½ years old
Relationships
Toddlers begin to explore the world and begin to get a little further away from parents sometimes, though
they always come back to Mom and Dad for comfort and assurance. The task for parents is to allow
exploration while keeping the child safe.
The World
Your toddler will learn about the world by exploring it actively! He will
use all of his senses but not a lot of “common sense” during this
stage! He will climb, run, jump as well as touch, taste, look at and smell
everything he can find! You have to allow this, and at the same time
protect him from being harmed. His bleeding disorder can make this
more challenging.
Bleeds
As your toddler begins to explore the world he may start having more bleeding episodes. Among the most
frequent are:
Head bumps
If your toddler experiences a significant “head bump” (if he rolls off the bed, hitting his head for instance)
he may need a factor infusion and head CT. Call your hematology team for instructions.
A few parents choose to use a soft helmet for their child to protect against head bumps. Ask your
hematology team for their input on this.
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FlashLINK to Hemophilia
Mouth bleeds
Mouth bleeds are common and sometimes difficult to control. They may occur when your child gets a new
tooth, or when he falls with something in his mouth. A factor infusion may be necessary and Amicar® can be
added to help keep the clot in place.
Muscle bleeds
As your child becomes more active, he may experience a muscle bleed. Generally there is no or minimal
bruising with this type of bleed, although it can be quite painful. The muscle involved will be swollen and
“tense” or “tight” to the touch. It will be fairly tender, and it will hurt to move or use the body part. Sometimes
a buttock bleed will occur when your toddler falls on his bottom while learning to walk. These bleeds can be
quite painful, and will usually require factor treatment. Most muscle bleeds will require a factor infusion or two
before they resolve. Call the HTC as soon as you are aware of a muscle bleed.
Joint bleeds
Joint bleeds may begin to occur more frequently once your toddler begins climbing and walking. The joint
involved will be warm to the touch, swollen and quite painful. Your toddler may not want to walk or to use the
body part that is affected. Factor will be used to control this type of bleed. Apply ice and call the hematology
team.
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Pre-school age - 2½ to 5 years old
Relationships
Your preschooler learns by doing things. His new favorite phrase will be “I can do it!” He will enjoy having
jobs to do within the home. Helping Mom and Dad, along with contributing to the family responsibilities are
very important for a pre-schooler. He is becoming more independent but still relies on having parents there to
support, guide and comfort him.
When your child needs a factor infusion or blood draw, give him choices where possible. For instance, you can
ask, “Would you rather sit on the chair or lay down on the couch for your infusion?” This is a good time to give
him tasks to do for the infusion, such as taking the factor out of the refrigerator, wiping down the table before
mixing, wiping bottle tops with alcohol wipes. When he needs an infusion remind him that factor helps him feel
better. Your love and support will help him through the experience.
The World
Your pre-schooler learns about the world by trying new things. This means he will do the same thing over and
over until he masters the task! You have to allow this, and at the same time
protect him from being harmed. His bleeding disorder can make this more
challenging.
Make your child’s environment as safe as possible! He should wear
appropriate protective gear for whatever activity he is enjoying. A bike
helmet while riding his tricycle or big wheel will protect him if he falls and
help him become accustomed to wearing the helmet. Knee and elbow
pads may help protect the joints with outdoor play activities.
However, accidents and bleeds may happen. Having an emergency bag
ready to go will be helpful for when you do have to seek care. Fill the bag
with contact information for your hematology team, include your flash drive, emergency care information and a
few things that will keep your child entertained while awaiting care. Having a bag ready will allow you to add
a dose of factor to the bag and take off quickly if baby is injured.
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Common injuries during this age include:
Bruising
By now you know that a child with a bleeding disorder will bruise more easily than other children. He may
develop bruises when he falls, or from injuries that you know nothing about. Bruises are unsightly, but not too
concerning unless they grow larger and cause pain to your child. It may be helpful to draw a line around a
concerning bruise so that you can tell if it is growing. Ice or cold packs can be applied to help treat a bruise. If
a bruise causes your child to stop using his arm or leg, call the HTC for advice.
Head bumps
If your child experiences a “head bump” (if falls and hits his head for instance) he may need a factor infusion
and head CT. Call your hematology team for instructions as soon as possible after the injury.
Mouth bleeds
Mouth bleeds are common and sometimes difficult to control. They may occur when your child falls with
something in his mouth, or bites his lip while playing. It is difficult, if not impossible to apply pressure or ice
to an injury in the mouth. It may help to give your child cold liquids to drink. Amicar® can often be alone used
to control bleeding in the mouth. Occasionally a factor infusion may be necessary and Amicar® added to help
keep the clot in place. Call your hematology team for instructions on treating mouth bleeds.
Nosebleeds
Nosebleeds may occur when the air is dry, or during “allergy season”. Efforts to prevent nose bleeding include:
using a humidifier to moisten the air, saline nose spray to keep the inside of the nose from cracking and
encouraging your child not to nose pick his nose. A persistent nose bleed may require factor and/or Amicar®
treatment to control
Muscle bleeds
As your child becomes more active, he may experience a muscle bleed. Generally there is no or minimal bruising
with this type of bleed, although it can be quite painful. The muscle involved will be swollen and “tense” or
“tight” to the touch. It will be fairly tender, and it will hurt to move or use the body part. Factor infusions are
usually required to treat this type of bleed. Call the HTC as soon as you are aware of a muscle bleed.
Joint bleeds
Joint bleeds may begin to occur as your child runs, jumps and climbs. The joint involved will be warm to the
touch, swollen and quite painful. Your child may not want to walk or to use the body part that is affected.
Factor will be used to control this type of bleed. Apply ice and call the hematology team.
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Infants – 6 months to 1 year old
Relationships
Your baby continues to learn and grow through strong, supportive relationships with his family. Parents
provide the foundation through which your baby can explore and learn about the world. He is becoming more
independent but still relies on having parents there to support, guide and comfort him.
Continue to use the daily bath as a time to check out your baby- looking for
bruising, tender spots, swelling or anything unusual. Call your hematology
team with concerning findings. When baby needs a factor infusion or blood
draw, be prepared to comfort him. When he needs an infusion he may cry
loudly! Much of the crying will be because he has to be held tightly for the
infusion. He won’t like to be restrained! Your love and support will help him
through the experience.
The World
Your baby learns about the world by exploring it with his senses. This means he needs to touch, taste, look at
and smell everything he can find! You have to allow this, and at the same time protect him from being harmed.
His bleeding disorder can make this more challenging.
Baby will begin to roll, crawl, creep and pull up to stand during this time. As he masters new skills he may fall
or become injured. He may begin to need factor infusions with these injuries.
Baby-proofing the house is a must at this stage of your baby’s life! It can help to have carpeting to pad the floor
and to put “bumpers” on the crib, coffee table and hearth. Protective gates are a must near stairways and other
places where injuries might occur.
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However, accidents and bleeds may happen. Having an emergency bag ready to go will be helpful for when
you do have to seek care. Fill the bag with contact information for the hematology team, your flash drive,
emergency care information and a few things that will keep baby entertained while awaiting care. Having a bag
ready will allow you to add a dose of factor to the bag and take off quickly if baby is injured.
Bruising
A baby with a bleeding disorder will bruise more easily than other children. He may develop bruises where
he is picked up, or where his car seat strap crosses his shoulder, or on his knees with crawling. Bruises are
unsightly, but not too concerning unless they grow larger and cause pain to the baby. It may be helpful to draw
a line around a concerning bruise so that you can tell if it is growing. Ice or cold packs can be applied to help
treat a bruise. If a bruise causes your baby to stop using his arm, or to stop crawling, call the HTC for advice.
Head bumps
If your baby experiences a “head bump” (if he falls while pulling up to stand, hitting his head for instance) he
may need a factor infusion and head CT. Call your hematology team for instructions as soon as possible after
the injury.
Mouth bleeds
Mouth bleeds are common and sometimes difficult to control. They may occur when your baby falls with
something in his mouth, or bites his lip with his new teeth. It is difficult, if not impossible, to apply pressure or
ice to an injury in the mouth. It may help to give the baby cold liquids to drink. Amicar® can often be alone used
to control bleeding in the mouth. Occasionally a factor infusion may be necessary and Amicar® added to help
keep the clot in place. Call your hematology team for instructions on treating mouth bleeds.
Joint bleeds
Joint bleeds may begin to occur as baby starts crawling and walking. The joint involved will be warm to the
touch, swollen and quite painful. Your baby may not want to crawl or to use the body part that is affected.
Factor will be used to control this type of bleed. Apply ice and call the hematology team. Many parents find that
using knee pads protects their baby’s knees while crawling, and may prevent a bleed in the knee joint.
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Bleeding with immunizations
Immunizations are good for your baby’s health! Ask your pediatrician to use the smallest gauge needle possible
when giving shots. After the shot is given apply pressure to the site for at least 10 minutes. Cold packs applied
to the site for 10 minutes on, then 10 minutes off, through out the day will also help with pain and swelling.
Some children with hemophilia will develop a muscle bleed after getting immunizations. If this occurs, the
baby’s leg will be warm, swollen and tender to the touch. Baby will be unhappy, because this hurts. Apply ice,
and call your pediatrician or hematologist. A factor infusion may be needed.
Bleeding with venipuncture
It’s sad to say that sometimes a baby will develop a bleed after having blood drawn in the doctor’s office!
Drawing blood from an infant can be challenging for the best nurse. Always apply pressure to the site of a
venipuncture for at least 10 minutes after the blood draw. Later, if you notice swelling at the site, apply ice
and pressure (if this doesn’t cause pain to the baby), then call the HTC for advice.
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School age - 6 to 10 years old
Relationships
Your school aged child will begin to have relationships outside of the family. His friends become very important
to him at this age. He will really enjoy and benefit from being a member of a club or team. Scouting, youth
sports and other “belonging” opportunities will give him a chance to be part of a group and will benefit his
development.
His school teacher, school nurse, coaches and others who spend time with him will need to learn about
hemophilia. The HTC staff or home health team may be able to supply
educational materials and in-service to those involved with your
child to insure that he will be well cared for when away from you.
Hemophilia care and factor infusion
Children at this age may begin to “hide” their bleeds, in order to avoid having a factor infusion or to not have to
miss an activity. Regular reminders of how factor helps him feel better will help him learn that earlier treatment
produces earlier response. You may have to continue to observe him closely for signs of bleeding episodes.
Teach your child to tell you when he is hurt, be able to describe where the pain is, what happened, etc. A
matter of fact attitude toward injuries/bleeds will allow the child to feel more comfortable telling you what has
happened
These years are an especially important time to start teaching your child to become involved in his care.
Parents need to start taking time to talk about the bleeding disorder in a language the child understands and
making sure the child can begin to talk about their bleeding disorder. The child should be able to say the name
and important facts about their disorder and even to be able to simply describe it to the others.
Your HTC will assist in this teaching, but they only see your child occasionally, so parents/caregivers are the
ones who must keep up with repetitive education.
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To help with an active role in treatment, allow the child to:
• Assist in inventory of medical supplies
• Help order and unpack supplies as they arrive
• Talk to medical personnel/home care suppliers, etc to make appointments, order supplies
• Fill out treatment logs- very important and a job the child can start doing at an early age
• Learn self-infusion (if applicable)- this is done at hemophilia camp or at your HTC.
The World
Your school-aged child learns about the world by trying new things and in relationships with family and friends.
Children with hemophilia should go to school as often as possible and should participate in normal childhood
activities. Your HTC providers can help you determine which activities are appropriate for your child.
Bruising
You have seen by this age that a boy with a bleeding disorder will bruise more easily than other children. He
may develop bruises when he falls, or from injuries that you know nothing about. Bruises are unsightly, but not
too concerning unless they grow larger and cause pain to your child. It may be helpful to draw a line around a
concerning bruise so that you can tell if it is growing. Ice or cold packs can be applied to help treat a bruise. If
a bruise causes your child to stop using his arm or leg, call the HTC for advice.
Head bumps
If your child experiences a “head bump” (if he falls and hits his head for instance), he will need a factor infusion
and head CT. Call your hematology team for instructions as soon as possible after the injury.
Muscle bleeds
As your child becomes more active, he may experience a muscle bleed. Generally there is no or minimal bruising
with this type of bleed, although it can be quite painful. The muscle involved will be swollen and “tense” or
“tight” to the touch. It will be fairly tender, and it will hurt to move or use the body part. Factor infusions are
usually required to treat this type of bleed. Call the HTC as soon as you are aware of a muscle bleed.
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Joint bleeds
Joint bleeds may occur more frequently as your child begins to be involved in sports and other activities. If he
is not already on prophylaxis, your HTC team may recommend using factor prior to sports activities to prevent
bleeding. If he gets a joint bleed, the joint involved will be warm to the touch, swollen and quite painful. Your
child may not want to walk or to use the body part that is affected. Factor will be used to control this type of
bleed. Apply ice and call the hematology team.
Mouth bleeds
Mouth bleeds may occur when your child begins to lose primary teeth, or when he falls, injuring his lip or gums.
These bleeds may respond to Amicar® alone or may require a factor infusion to control them. It is important
to have routine dental care. Good teeth brushing and flossing every day are good behaviors to learn at an
early age, and healthy gums and teeth will bleed less than those that are not well cared for. Call the HTC for
treatment advice when having any dental procedures.
Nosebleeds
Nosebleeds may occur when the air is dry, or during “allergy season”. Efforts to prevent nose bleeding
include: using a humidifier to moisten the air, saline nose spray to keep the inside of the nose from cracking
and encouraging your child not to pick his nose. A persistent nose bleed may require factor and/or Amicar®
treatment to control.
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School age - 11 to 15 years old
Each developmental stage brings new challenges and the early teen years are no different. Challenges
that will be faced as the young man starts to mature include the following items.
Joint bleeds
Target joints that have developed previously may continue to cause problems and
may present with arthritic changes. Arthritis pain is very similar to joint bleed pain
and even adults have trouble telling the difference. Arthritis will cause pain in the
morning, when the joint is stressed, or when the weather changes. If after walking
or moving the joint for 15-20 minutes, it feels better, this is arthritis. Treatment of
arthritis and a joint bleed is completely opposite, so contact your HTC for directions.
Occasional bleeds in other joints need to be treated as soon as possible and often
pre-teens and teens will not confess to having a bleed until the joint is really
compromised. This is standard for this age so continue the education on treating
early and watch your child for limping, not using a limb, etc.
Muscle bleeds
Iliopsoas
An iliopsoas bleed is a relatively common, but serious, bleed into the low back, hip or groin. This type of
bleed can cause back pain, stomach pain, hip pain, etc. This
bleed needs to be treated aggressively, under a physician’s
supervision, to avoid nerve damage. Contact your HTC
immediately.
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Calf and Forearm
Calf and forearm bleeds are also serious due to possible compartment syndrome, excessive bleeding in
these areas can compress the nerves running through this area. These bleeds are also treated aggressively
and under a physician’s supervision. Contact your HTC immediately.
Dental Care Orthodontia
Teeth extractions
A treatment plan is needed for any dental procedure. Your child may need both factor and aminocaproic acid
to keep him from bleeding. Contact your HTC for the plan.
Braces
Braces are fine for bleeding disorder patients. They just need to stay in contact with their HTC to have a plan
in place in case bleeding occurs.
Blood in the urine (hematuria)
Seeing blood in the urine is very scary and people forget that a little blood looks like a lot when mixed with
urine. This can happen without any obvious reason. Increasing clear fluid intake and bed rest will help and
then call your HTC for further directions.
Gastrointestinal bleeds
During this age the young men need to learn to be aware of what blood in stool looks like and notify their
parent if they see this and /or have abdominal pain. Bleeding ulcers can occur (although not common at this
age) and need immediate treatment.
Head bumps
Any blow to the head needs to be reported to their parents for assessment and immediate treatment if necessary.
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School
The young man may stop doing homework, skip classes, or receive poor grades. Watch for this and intervene
quickly with tutors, mentors, etc.
He may want to tell others about his hemophilia, or he may not. This is individualized and needs to be respected.
As long as the teacher, principal and school nurse know, there is no reason the child can’t choose who else to
tell.
Activities
Team sports are encouraged, see Sports section.
Playground sports are encouraged, see Sports section.
Time away from family, on his own
This is when sleepovers, camp, etc. will be more enjoyable for your child. Knowing how to self-infuse (if
applicable) is a major part of independent behavior and is encouraged. Be sure the adult in charge of the event
is aware of your child’s bleeding disorder.
Independence
Taking over parts of his health care
Now is the time to have your child start to order his own factor supplies, self-infuse, and call his nurse (all
under supervision).
Interacting more with caregivers
Make sure your child interacts with his doctor/nurse/PT/SW, etc. Have your child answer questions themselves
and have time to ask questions. Expect to be asked to leave the room during the HTC visit to allow your teen
some personal time with the HTC staff.
Should learn peripheral intravenous access by age 12
Peripheral IV access (if applicable) should be taught at age 7-9 for the best results, but should be learned for
sure by age 12. Your HTC can assist you with this plan. 3
Adolescents - 16 to 19 years old
Challenges
Teens need to start to take control of their lives, especially their medical treatment. Under the supervision
(always!) of their parents they need to be the person who orders factor, infuses themselves (as applicable),
makes appointments with medical personnel, calls the nurse when they are injured and does the most talking
to the doctor and nurse at comprehensive visits.
In order to make this possible, parents have to start to give up some of their role. This is
hard, especially for mothers, but it is the only way your child will become an independent
adult, capable of taking care of themselves and advocating for themselves.
Subjects that should be discussed:
Life after High School
The time for teens to begin to planning their future is during the high school years.
Topics that parents should address with their teens include:
•Further Education (college or tech school)
•Scholarships are available from many sources for persons with bleeding disorders
•Job choices: avoid choosing jobs that might cause bleeding
•Insurance availability
Responsibility
By the late teens, your child should be doing almost all of his own medical care and
follow-up with minimal supervision. There are more areas for responsibility though, like driving a car, staying
in school, keeping a job, saving money, etc.
Insurance
Know your coverage and how long your child will be covered under your policy or by the government program
he is on. Do not wait until the near end of the coverage to find other coverage. Talk about this as a family. Again,
check with your HTC about options.
Dating/partner in life
As teens start to date, they may not want to tell everyone immediately about their health. This is normal and
expected. Helping them to know when and who to tell as relationships develop is where parents and HTCs can
really be supportive. Learn about the genetics of hemophilia from your HTC team so that you can make future
decisions. See section on Genetics.
HTCs can really be supportive.
Complications of hemophilia
Access for infusions
Good vein access is important for factor administration, especially if large doses need to be infused frequently
or daily. Very young children often do not have good veins for injecting medications and may not be cooperative
during needle sticks. If daily infusion into the vein is necessary, your physician may recommend a central line
(which goes into a big vein) which requires surgical placement and removal.
Two different types of central lines are:
Hickman or Broviac Catheters
Implantable Port.
Please see the section on Venous access options.
If your physician recommends a Hickman or Broviac catheter, the factor syringe is attached to the adaptor for
factor administration, then removed without causing pain to the child. The tubing exit site requires regular dressing
changes to prevent infection.
If your child needs an implantable port, proper technique must be used to infuse medications, such as factor, into
this port. A special needle is inserted through the skin and into the device to administer factor. Once the infusion
is complete, the needle can be removed. Many like the ease of caring for this device when not in use. Proper care
must be taken to prevent infection, bleeding or bruising at the site, mechanical problems, and clotting. Ports are
temporary solutions and will eventually need to be removed.
The decision about what type of venous access is best for your child should be made after a full discussion of the
pros and cons of each type of device with your treatment center. Many children on regular infusions require the
placement of a central line as a young child, but then transition to a temporary device as they become older.
Medications
Amicar® (aminocaproic acid)
Amicar® is a medicine that helps keep bleeding in the mouth from starting up again, once it has stopped. It is NOT
a substitute for factor. When Amicar® is used correctly, it allows the injured area or tooth socket in the mouth to
heal from the inside to the surface.
•Amicar® syrup is not readily available at most pharmacies, so, like factor, you should keep an emergency supply at home and take it along when you travel on vacation.
•Amicar® is usually given every 6 hours and it is important to keep on schedule so that it will keep working until the area has healed, usually around 3-5 days.
•Amicar® syrup will come in a brown bottle that should be stored away from bright light. It should NOT be refrigerated.
•Always call the Hemophilia Treatment Center before starting Amicar® to be sure that it is safe for your child to take it at the time, and also so that the provider can calculate the proper dose based on your child’s current weight.
•NEVER give your child Amicar® if he has blood in the urine, like from an infection, injury or surgery. Tell the Treatment Center provider if your child has had blood in the urine recently. Amicar® helps to prevent the breakdown of blood clots. It is most useful with mucous membrane bleeding, (such
as nose, mouth and gastrointestinal tract bleeding) and is not usually helpful with joint bleeding or other internal
bleeding.
How to give:
Liquid/syrup: Use an oral syringe to measure the amount of medication needed. Take the medicine as directed by your doctor. Be sure to swallow the medicine.
Tips for taking Amicar®:
Fill plastic popsicle mold with Kool-Aid®, then squirt in the Amicar® syrup dose. Freeze. Most children will eat a popsicle even if they won’t swallow the syrup from a teaspoon.
Another way to “disguise” the taste of Amicar® syrup would be to squirt the Amicar® in an “Otter Pop” and stand upright to freeze.
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Tablets: Swallow as directed. Tablets can be crushed and mixed with a liquid or soft food.
Intravenous (IV): If you (your child) are unable to swallow, Amicar® can be given IV.
Special instructions:
Amicar® can be taken with or without food. Give with food if an upset stomach occurs after taking the medication.
It is important to take the medication as directed by your doctor
Amicar® is dosed by weight. The dose will change as a child grows. It should be taken every 6 hours
(6am, noon, 6pm and midnight for example), not 4 times daily.
If you miss a dose of Amicar®, take it as soon as you remember and do not try to “make up” a missed dose.
Do not take Amicar® for kidney or bladder bleeding (blood in the urine) unless directed by your doctor
Amicar® should be used with caution by people who have kidney or liver disease.
Side effects: (that do not need to be reported)
Mild upset stomach
Mild diarrhea
Mild headache
Side effects that must be reported:
Skin rash
Chest pain
Difficulty breathing
Slurred speech
Vomiting
Special considerations:
Tell your doctor of other medications, vitamins, herbal products that you (your child) are taking
Keep this medication out of the reach of children
Keep the medication in its original container and store as instructed
Call 911 or go to the Emergency Room if you (your child) has:
Chest pain
Slurred speech
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Medications
Common preparations containing aspirin
Why can’t I take aspirin?
Persons who have bleeding disorders are told not to take aspirin for fever or pain and are usually given a list of
over-the-counter medications that contain aspirin. This does not mean that you have an allergy to aspirin (unless
you have taken aspirin and had a bad reaction such as hives or difficulty breathing, etc).
Aspirin causes platelets to lose the ability to stick together and form a plug so that a blood clot can build up on it.
Persons with VWD have platelets that function normally. This is why bleeding from just a scratch or scrape or a
nose bleed may stop without taking a dose of factor. If you have VWD and then take aspirin too….you will lose any
help from platelets when you have a bleeding episode.
See below to review the common preparations containing aspirin to avoid.
Please note that this is a partial list. ALWAYS check the ingredients in both over-the-counter and prescription
medications for aspirin, also listed as acetylsalicylic acid or ASA. In addition, always check with your physician,
treatment center, and/or pharmacist before starting any new medication for interactions with your bleeding disorder.
Medications obtained outside the United States are not subjected to the same US regulatory/ labeling processes
and may contain aspirin. Thus, if truly needed, use with caution.
Common preparations containing aspirin
Alka Seltzer Plus Cold (Bayer)
B-C Tablets (Block)
Alka Seltzer w/Aspirin (Miles)
Bufferin (Bristol-Myers) - all types
Anacin ( Whitehall) - all types
Buffets II Tabs (JMI)
Arthritis Pain Formula (Whitehall)
Buffex (Hauck)
Ascriptin (Rhone-Poulenc Rorer)
Cama (Sandoz)
Aspergum (Schering-Plough)
Carisoprodol Compound (Various)
Axotal (Adria)
Cope Tablets (Mentholatum)
Azdone (Central)
Damason-P (Mason)
B-A-C (Mayrand)
Dolobid (MSD)
Bayer (Glenbrook) - all types
Easprin (Parke Davis)
Bayer Childrens (Glenbrook)
Ecotrin (Smithkline Beecham)
B-C Cold-Sinus-Allergy Powder (Block)
Empirin (Burroughs Wellcome)
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Empirin w/Codeine (Burroughs Wellcome)
Norgesic Forte (3M)
Equagesic (Wyeth)
Norwich Aspirin (Chattem)
Excedrin (Bristol-Myers) - all types
Oxycodone w/Aspirin (Various) - all types
Fiorinal (Sandoz)
Pain Reliever Tabs (Rugby)
Fiorinal w/Codeine (Sandoz)
Panasal 5/500 (Robins)
Genprin (Alra)
Percodan (Dupont)
Genprin (Goldline)
Percodan Demi (Dupont)
Goody’s (Goody)
Robaxisal (Robins)
Halfprin (Kramer)
Roxiprin (Roxane)
Isollyl Improved (Rugby)
Saleto (Mallard)
Lortab ASA (Whitby)
Sodol Compound (Major)
Magnaprin (Rugby)
Soma Compound (Wallace)
Menoplex Tabs (Fiske)
Soma Compound w/Codeine (Wallace)
Methocarbamol w/ASA (Various)
Synalgos-DC (Wyeth Ayerst)
Momentum (Whitehall)
Vanquish (Sterling)
Night-Time Effervescent Cold Tabs (Goldline)
Wesprin Buffered (Wesley)
Norgesic (3M)
ZORprin (Boots)
There are other medications that also cause platelets to lose their clumping ability. Among these are
common medications that are listed under the category of NSAIDs or non-steroidal anti-inflammatory drugs.
Over-the-counter NSAIDs include ibuprofen, (Advil®, Motrin®, etc) and naproxen (Aleve®, etc). You should not
take these medications without checking with your VWD care provider or treatment center.
There are two NSAIDs that do not cause platelets to lose their clumping ability. These are salsalate (Disalcid®)
and celecoxib (Celebrex®). You will need a prescription from your provider for any of these medications for
joint pain relief.
All NSAIDs have the potential of causing stomach irritation. They should be used with caution by persons with
bleeding disorders and discontinued at the first sign of stomach pain or burning.
2
Inhibitors
What is an inhibitor?
Inhibitors are antibodies produced by the immune system that mistakenly target replacement factor concentrate
as a foreign substance, rather than a therapy. These antibodies then launch an attack on the FVIII molecules
infused into the bloodstream during a bleeding event. The antibodies interfere with or completely block the
ability of factor to stop a bleeding episode. A simple analogy may make this concept easier to understand.
Imagine the FVIII molecules contained in replacement concentrate as keys that fit into the lock of the clotting
cascade (Figure 1). When the key is inserted into the lock and turned to the locked position, a clot forms, and
bleeding stops (Figure 2). When an inhibitor attaches to the key, it acts like gum. The key becomes sticky and
no longer slides easily into the lock (Figure 3). As a result, the lock remains open, making it difficult for a clot
to form and allowing bleeding to continue. (figure 3)
figure 2
figure 1
figure 3
An inhibitor can’t develop until after a patient has been exposed to FVIII concentrate. Most inhibitors occur
within the first 50 days of exposure to factor replacement therapy, typically between 9-12 days.1
What is the impact of an inhibitor?
Some inhibitors are transient, cause no problems, and disappear without treatment. Other inhibitors are
persistent, and their development is considered the most serious adverse event associated with the management
of hemophilia A. Patients with inhibitors don’t bleed more frequently than persons without inhibitors. However,
their bleeds are more difficult to control because they don’t respond to standard treatment with FVIII replacement
concentrate.2, 6 Consequently, patients with inhibitors are at higher risk for developing complications, particularly
arthropathy and, eventually, disability that may require the use of crutches, wheelchairs, and other mobility
assist devices.4 Inhibitors also increase the cost of hemophilia care by about 400% because high doses of more
expensive therapeutics are needed.5
1
Who develops inhibitors?
Up to 30% of individuals with severe hemophilia A develop inhibitors, usually during infancy or early childhood.6
The reason for this is that people with severe hemophilia tend to bleed more frequently than those with mild
or moderate hemophilia and require factor replacement therapy multiple times during the first few months to
years of life. And as mentioned earlier, inhibitors generally occur within the first 50 exposure days to FVIII.1
An estimated 3% to 13% of persons with mild or moderate hemophilia also develop inhibitors.5 But as they
are less likely to bleed and need FVIII infusions, their inhibitors may not appear until later childhood or even
adulthood, when serious bleeding (eg, from injury or surgery) requires treatment.
What are the risk factors linked to the development of inhibitors?
A variety of genetic factors may be linked to the development of inhibitors, including large mutations in
the FVIII gene;7 a family history of inhibitors (which triples the risk of developing an inhibitor),8 and African
heritage (which doubles the risk of developing an inhibitor) (Table 2).9 Inhibitors also occur more frequently in
hemophiliacs of Latino descent.10 Certain environmental factors may also increase the likelihood of inhibitor
formation, although the evidence is not conclusive. These include intensive FVIII exposure (numerous doses
over several days),11 and vaccination, surgery, and bacterial or viral infections—all of which stimulate the
immune system and may promote inhibitor formation.12
Genetic and Environmental Factors Associated with the Development of Inhibitors
Genetic Factors
Environmental Factors
Severity of hemophilia
Intensive FVIII treatment
Large mutation of the FVIII gene
Immunologic/inflammatory/infectious events
(e.g., vaccination, surgery, illness)
Family history of inhibitors (eg, sibling, cousin)
Use of rFVIII products*
African or Latino descent
Table 2
*May increase risk, although further research is needed for confirmation.
2
Accumulating evidence suggests that the choice of replacement factor concentrate may also contribute to the
development of inhibitors.13 Many parents request that their children be treated exclusively with recombinant
FVIII (rFVIII) concentrates because they believe that these products are safer than plasma-derived FVIII (pdFVIII)
concentrates. However, the safety of plasma-derived products has improved dramatically over the last 20
years due to enhanced donor screening and manufacturing techniques that have been proven to detect and
kill viruses.14 Consequently, not a single incident of disease transmission has resulted from pdFVIII infusions
since 1985 in the United States and since 1987 in Canada.15 Many experts now believe that rFVIII and pdFVIII
products are equally safe from a disease-transmission standpoint.14 On the other hand, there is growing
concern that patients treated with rFVIII may be at higher risk for inhibitor development than those given pdFVIII
concentrates containing von Willebrand factor (VWF).15
VWF is a large protein found in blood plasma and platelets as well as in the cells lining blood vessels, and
it’s a critical component of the clotting process. VWF also plays a key role in the functioning of FVIII,16-19 and
it may protect FVIII molecules from attack by inhibitors.20 Remember the “lock and key” analogy discussed
earlier? Some (but not all) keys contain both FVIII and VWF molecules. Think of VWF as a protective armor for
the FVIII that may prevent the gum (inhibitor) from forming on the key—or may assist in eliminating the gum
altogether (Figures 4 and 5). Thus, the inclusion of VWF along with FVIII helps the factor concentrate stop
bleeding episodes.
figure 4
figure 5
A recently completed study analyzed data from 148 hemophilia A patients treated exclusively with either rFVIII
products or pdFVIII concentrates that contained VWF.13 The researchers found that the relative risk of inhibitor
development was about 2.5 to 3 times higher in patients given rFVIII, regardless of their other risk factors,
such as race or family history.13
3
How are inhibitors diagnosed?
FVIII replacement concentrates reliably stop bleeding in patients with hemophilia A who don’t have inhibitors.
So, if a bleeding episode isn’t promptly controlled with the usual dose of FVIII, or if a patient on FVIII prophylaxis
experiences increased breakthrough bleeding, an inhibitor may be to blame. Two laboratory tests can detect
inhibitors, even in patients without symptoms: an activated partial thromboplastin time, or aPTT, and a FVIII
recovery assay. When an inhibitor is present, the aPTT is prolonged and cannot be corrected by mixing the
patient’s plasma with normal plasma, termed a mixing study.21 In addition, FVIII levels are very low when
measured 1-hour after an infusion of FVIII concentrate. The Bethesda assay is used to assign a numerical value
to the inhibitor. This number, which is measured in Bethesda units (BU), can range from less than 1 BU to well
over 1000 BU. It is called the inhibitor titer.
How are inhibitors classified?
Inhibitors are categorized as low-titer (5 BU or less) or high-titer (greater than 5 BU).22 Inhibitors also are
classified as low-responding or high-responding. The inhibiter titer in a patient with a low-responding inhibitor
never rises above 5 BU no matter how much factor he receives or how often it is given. In contrast, the inhibitor
titer in a patient with a high-responding inhibitor increases significantly—sometimes 50 BU or more—within a
few days of his receiving FVIII. This marked increase in the inhibitor titer is termed anamnesis or an anamnestic
response (Figure 6). If a patient has not received factor for an extended period of time, his previously high-titer
inhibitor may drop to 5 BU or less, thus becoming a low-titer inhibitor. His inhibitor will still be high-responding,
however, and the next time he is given factor, his titer will once again surpass 5 BU.
Low- vs high-responding inhibitors 23
Figure 1
When repeated doses of FVIII are given to a patient with a low-responding inhibitor (solid line), anamnesis does not occur. In contrast,
the first dose of FVIII given to a patient with a high-responding inhibitor whose inhibitor titer is low (dotted line) does provoke an
anamnestic response. Subsequent doses given while the inhibitor level is high cause no further rise in the inhibitor titer.
Adapted with permission.
4
What can be done about inhibitors?
How are inhibitors treated?
The treatment of bleeding events in a patient with an inhibitor depends on his current inhibitor titer. Higher than
normal doses of factor are usually effective in overcoming inhibitors and controlling bleeding in a patient whose
inhibitor titer is 5 BU or less.3 Once the inhibitor titer exceeds 5 BU, though, FVIII replacement is ineffective,
and bypassing therapy is needed.
What is bypassing therapy?
Bypassing therapy, as the name implies, circumvents the need for FVIII by using agents that contain other
clotting factors to stop bleeding episodes. Two bypassing products are available:
• Activated prothrombin complex concentrate (aPCC)
• Recombinant activated factor VII (rFVIIa)
Both bypassing agents successfully control at least 80% of bleeding episodes in patients with high-titer
inhibitors, including bleeding associated with surgery or invasive procedures, such as placement of a central
line.24-29 However, aPCC and rFVIIa have several drawbacks. As mentioned, these products are very costly—
about 4 times that of FVIII concentrates.30 In addition, while factor replacement can be monitored using the FVIII
recovery assay, thus enabling your physician to adjust dosage, no test is available for monitoring bypassing
therapy.3 That means a patient may receive a higher dose than is needed to stop a bleeding event, thereby
increasing both the cost of care and the risk of thrombosis, (the formation of clots within blood vessels).
Nonetheless, the likelihood of thrombosis is low with either aPCC’s or rFVIIa,31 and this serious complication
may also occur with FVIII replacement concentrates.
By far the biggest disadvantage to bypassing therapy is that it’s simply not as good at controlling bleeding
in patients with high-titer inhibitors as FVIII concentrates are in patients without inhibitors or those with lowtiter inhibitors.3 Bypassing agents are also intended to manage a specific bleeding episode and do not work
toward the elimination of the inhibitor. For this reason, your physician may recommend trying to eradicate
the inhibitor.
What is immune tolerance induction?
Immune tolerance induction (ITI) is a process that attempts to permanently eradicate an inhibitor. Several
different regimens, termed protocols, are used for ITI. All involve regular (daily or several times weekly)
infusions of FVIII concentrate. Over a period of time—from several months to 4 years—a patient’s immune
5
system may get used to and accept the presence of FVIII, and the inhibitor antibodies will gradually disappear.
This process is called tolerization.
When ITI is successful at eradicating an inhibitor, bleeding episodes can once again be treated with FVIII
concentrates, which work better than bypassing agents in stopping bleeds.3 Without inhibitors, patients have a
lower risk of developing arthropathy or other bleeding complications, and they may have longer lives and more
active lifestyles.32
How effective is ITI?
ITI is successful in about 50% to 80% of patients.33-36 Certain risk factors increase the likelihood of success.
These include: 33-38
• An inhibitor titer less than 10 BU at the start of ITI
• Peak (maximum) inhibitor titer during ITI less than 500 BU
• No interruptions in ITI
• No infections during ITI
The type of FVIII concentrate used may also influence the success of ITI. VWF appears to protect FVIII molecules
from inhibitors (recall the key and lock analogy discussed earlier).39 Patients whose inhibitors can’t be eradicated
using rFVIII products are often switched to ITI protocols that use VWF-containing pdFVIII concentrates.40-45 After
making this switch, ITI is frequently successful, even in patients with poor risk factors.
What should families consider before starting ITI?
ITI is a demanding undertaking for patients and families. The schedule of FVIII infusions, whether daily or
several times each week, can be disruptive and can interfere with school, work, family and social activities.
In addition, venous access often is difficult in youngsters, and your child may need to have a central venous
access device (CVAD) inserted to simplify the administration of factor. Surgery is needed to insert a CVAD under
the skin, usually in the chest . Infection and thrombosis are well known complications associated with these
devices.46 Frequent infections reduce the likelihood that ITI will be successful.47
Before making a decision about whether to start ITI, you should be fully aware of the time commitment and
effort involved as well as the potential problems that may occur. Cost is another consideration, as ITI is very
expensive in the short-term.32 However, when it is successful, ITI actually can reduce the long-term cost of care
by allowing FVIII concentrates to be used to treat bleeding events, in place of more costly bypassing agents,
and possibly reducing complications associated with inhibitors, including arthropathy.
6
Summing Up
Before we close, let’s review the key points discussed.
The development of inhibitors to FVIII:
• Makes the treatment of bleeding episodes more difficult
• Increases the risk of uncontrollable bleeding and serious catastrophic complications
• Increases risk of arthropathy
• Increases the cost of hemophilia care
• Interferes with quality of life with missed days from school and work
Inhibitors are categorized as:
• Low-titer (≤5 BU) or high-titer (>5 BU)
• Low-responding (nonanamnestic) or high-responding (anamnestic)
High doses of FVIII concentrates can be used to treat bleeding in patients with low-titer inhibitors.
Bypassing agents are usually needed to control bleeding in patients with high-titer inhibitors.
ITI is used to eliminate high-titer inhibitors and once again allow the use of FVIII replacement concentrates to
treat bleeding events because these products:
• Work better than bypassing agents
• Are less expensive than bypassing agents
ITI is successful in most patients, but it may take months to years of intensive treatment to eradicate an
inhibitor. Several factors, including the choice of FVIII product used in the ITI regimen, may increase the
likelihood that ITI will be a success. The FVIII that is used to tolerize should continue to be used for ongoing
treatment.
An inhibitor log is included under the Medical Information section to help assess and compare your inhibitor
titers over time.
We hope this information has helped you better understand the complicated subject of inhibitors in
hemophilia A. Thank you for reading this section—and good luck with your hemophilia care.
7
References:
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Lusher JM. Inhibitor antibodies to factor VIII and factor IX: management. Semin Thromb Hemost. 2000;26(2):179-188.
Leissinger CA. Prevention of bleeds in hemophilia patients with inhibitors: emerging data and clinical direction. Am J Hematol.
2004;77(2):187-193.
Leissinger CA. Use of prothrombin complex concentrates and activated prothrombin complex concentrates as prophylactic
therapy in haemophilia patients with inhibitors. Haemophilia. 1999;5(suppl 3):25-32.
5. Hay CR. Factor VIII inhibitors in mild and moderate-severity haemophilia A. Haemophilia. 1998;4(4):558-563.
6. Brackmann HH, Wallny T. Immune tolerance: high-dose regimen. In: Rodriguez-Merchan EC, Lee CA, eds. Inhibitors in Patients
With Hemophilia. Oxford, England: Blackwell Science, Ltd.; 2002:45-48.
7. Oldenburg J, Pavlova A. Genetic risk factors for inhibitors to factors VIII and IX. Haemophilia. 2006;12 Suppl 6:15-22.
8. Astermark J, Berntorp E, White GC, Kroner BL. The Malmo International Brother Study (MIBS): further support for genetic pre
disposition to inhibitor development in hemophilia patients. Haemophilia. 2001;7(3):267-272.
9. Lusher J, Abildgaard C, Arkin S, et al. Human recombinant DNA-derived antihemophilic factor in the treatment of previously
untreated patients with hemophilia A: final report on a hallmark clinical investigation. J Thromb Haemost. 2004;2(4):574-583.
10. Aledort LM, DiMichele DM. Inhibitors occur more frequently in African-American and Latino haemophiliacs.
Haemophilia. 1998;4(1):68.
11. Sharathkumar A, Lillicrap D, Blanchette VS, et al. Intensive exposure to factor VIII is a risk factor for inhibitor development in mild
hemophilia A. J Thromb Haemost. 2003;1(6):1228-1236.
12. Astermark J. Overview of inhibitors. Semin Hematol. 2006;43(2 Suppl 4):S3-7.
13. Goudemand J, Rothschild C, Demiguel V, et al. Influence of the type of factor VIII concentrate on the incidence of factor VIII
inhibitors in previously untreated patients with severe hemophilia A. Blood. 2006;107(1):46-51.
14. Mannucci PM. The safety of plasma-derived versus recombinant concentrates. World Federation of Hemophilia. Occasional
Papers. September 2004; no. 5.
15. Allen G, Aledort L. Therapeutic decision-making in inhibitor patients. Am J Hematol. 2006;81(1):71-72.
16. Mannucci PM, Chediak J, Hanna W, et al. Treatment of von Willebrand disease with a high-purity factor VIII/von Willebrand factor
concentrate: a prospective, multicenter study. Blood. 2002;99(2):450-456.
17. Rodeghiero F. Introduction. Haematologica Reports. 2005;1:1-2.
18. Federici AB. The factor VIII/von Willebrand factor complex: basic and clinical issues. Haematologica. 2003;88(6):EREP02.
19. Lin Y, Yang X, Chevrier MC, et al. Relationships between factor VIII:Ag and factor VIII in recombinant and plasma-derived factor
VIII concentrates. Haemophilia. 2004;10(5):459-469.
20. Dasgupta S, Repesse Y, Bayry J, et al. VWF protects FVIII from endocytosis by dendritic cells and subsequent presentation to
immune effectors. Blood. 2007;109(2):610-612.
21. Kasper CK, Aledort L, Aronson D, et al. Proceedings: A more uniform measurement of factor VIII inhibitors. Thromb Diath
Haemorrh. 1975;34(2):612.
22. White GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Definitions in hemophilia. Recommendation of the
scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society
on Thrombosis and Haemostasis. Thromb Haemost. 2001;85(3):560.
23. Srivastava A, Giangrande P, Poon MC, M. C, McCraw A, Wiedel J. Guidelines for the management of hemophilia. Available at:
http://www.wfh.org/index.asp?lang=EN. Accessed March 28, 2008.
24. Arkin S, Blei F, Fetten J, et al. Human coagulation factor FVIIa (recombinant) in the management of limb-threatening bleed
unresponsive to alternative therapies: results from the NovoSeven emergency-use programme in patients with severe
haemophilia or with acquired inhibitors. Blood Coagul Fibrinolysis. 2000;11(3):255-259.
25. Hilgartner M, Aledort L, Andes A, Gill J. Efficacy and safety of vapor-heated anti-inhibitor coagulant complex in hemophilia
patients. FEIBA Study Group. Transfusion. 1990;30(7):626-630.
8
26. Key NS, Aledort LM, Beardsley D, et al. Home treatment of mild to moderate bleeding episodes using recombinant factor VIIa
(Novoseven) in haemophiliacs with inhibitors. Thromb Haemost. 1998; 80(6): 912-918
27. Negrier C, Goudemand J, Sultan Y, Bertrand M, Rothschild C, Lauroua P. Multicenter retrospective study on the utilization of FEI
BA in France in patients with factor VIII and factor IX inhibitors. French FEIBA Study Group. Factor Eight Bypassing Activity.
Thromb Haemost. 1997;77(6):1113-1119.
28. Shapiro AD, Gilchrist GS, Hoots WK, Cooper HA, Gastineau DA. Prospective, randomised trial of two doses of rFVIIa (NovoSeven)
in haemophilia patients with inhibitors undergoing surgery. Thromb Haemost. 1998;80(5):773-778.
29. Tjonnfjord GE. Activated prothrombin complex concentrate (FEIBA) treatment during surgery in patients with inhibitors to FVIII/
IX: the updated Norwegian experience. Haemophilia. 2004;10(suppl 2):41-45.
30. Gringeri A, Mantovani LG, Scalone L, Mannucci PM. Cost of care and quality of life for patients with hemophilia complicated by
inhibitors: the COCIS Study Group. Blood. 2003;102(7):2358-2363.
31. Aledort LM. Comparative thrombotic event incidence after infusion of recombinant factor VIIa versus factor VIII inhibitor bypass
activity. J Thromb Haemost. 2004;2(10):1700-1708.
32. Colowick AB, Bohn RL, Avorn J, Ewenstein BM. Immune tolerance induction in hemophilia patients with inhibitors: costly can
be cheaper. Blood. 2000;96(5):1698-1702.
33. DiMichele DM, Kroner BL. The North American Immune Tolerance Registry: practices, outcomes, outcome predictors. Thromb
Haemost. 2002;87(1):52-57.
34. Haya S, Lopez MF, Aznar JA, Batlle J. Immune tolerance treatment in haemophilia patients with inhibitors: the Spanish
Registry. Haemophilia. 2001;7(2):154-159.
35. Lenk H. The German Registr y of immune tolerance treatment in hemophilia--1999 update. Haematologica. 2000;85(10Suppl):45-47.
36. Mariani G, Kroner B. International immune tolerance registry, 1997 update. Vox Sang. 1999;77 Suppl 1:25-27.
37. Kroner BL. Comparison of the international immune tolerance registry and the North American immune tolerance registry. Vox
Sang. 1999;77 Suppl 1:33-37.
38. Hay CRM. Inhibitors to factor VIII/IX: treatment of inhibitors—immune tolerance induction. In: Lee CA, Berntorp EE, Hoots WK,
eds. Textbook of Hemophilia. Malden, Mass: Blackwell Publishing Ltd; 2005:74-79.
39. Berntorp E. VWF/FVIII complex and the management of patient with inhibitors: from laboratory to clinical practice.
Haemophilia. 2007;13 Suppl 5:69-72.
40. Auerswald G, Spranger T, Brackmann HH. The role of plasma-derived factor VIII/von Willebrand factor concentrates in the
treatment of hemophilia A patients. Haematologica. 2003;88(6):EREP05.
41. Gringeri A, Musso R, Mazzucconi MG, et al. Immune tolerance induction with a high purity von Willebrand factor/VIII complex
concentrate in haemophilia A patients with inhibitors at high risk of a poor response. Haemophilia. 2007;13:373-379.
42. Kurth MAH, DiMichele D, Sexauer C, et al. Immune tolerance therapy utilizing factor VIII/von Willebrand factor concentrate in
haemophilia A patients with high titre factor VIII inhibitors. Haemophilia. 2008;14(50-55).
43. Ettingshausen CE, Kreuz W. Role of von Willebrand factor in immune tolerance induction. Blood Coagul Fibrinolysis.
2005;16 Suppl 1:S27-31.
44. Kreuz W, Escuriola-Ettingshausen C, Auerswald G. Immune tolerance induction in haemophilia A patients with inhibitors: the
choice of concentrate affecting success. Haematologica. 2001;86(suppl 4):16-20.
45. Orsini F, Rothschild C, Beurrier P, Faradji A, Goudemand J, Polack B. Immune tolerance induction with highly purified plasmaderived
factor VIII containing von Willebrand factor in hemophilia A patients with high-responding inhibitors. Haematologica.
2005;90(9):1288-1290.
46. Valentino LA, Ewenstein B, Navickis RJ, Wilkes MM. Central venous access devices in haemophilia. Haemophilia.
2004;10(2):134-146.
47. DiMichele DM. Immune tolerance: critical issues of factor dose, purity and treatment complications. Haemophilia.
2006;12 Suppl 6:81-86.
9
Contact information / resources
Contact information for regional Hemophilia Treatment Centers
Center for Disease Control (CDC) supports a network of specialized healthcare centers to prevent and reduce
complications experienced by persons with certain blood disorders. These centers provide comprehensive health
management and prevention services to persons with bleeding disorders. The centers use multi-disciplinary teams
of health-care specialists, state-of-the art clinical research programs, and outreach and education programs.
Currently, the network consists of approximately 140 hemophilia treatment centers (HTCs).
To locate a regional HTC, please see the following website: https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
The National Hemophilia Foundation is dedicated to finding better treatments and cures for bleeding and clotting
disorders and to preventing the complications of these disorders through education, advocacy and research.
Established in 1948, the National Hemophilia Foundation has chapters throughout the country. Its programs and
initiatives are made possible through the generosity of individuals, corporations and foundations as well as through
a cooperative agreement with the Centers for Disease Control and Prevention (CDC).
To find your local chapter, please see:
National Hemophilia Foundation (NHF) Chapters
http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=51&contentid=193
The World Federation of Hemophilia (WFH) is an international not-for-profit organization dedicated to improving
the lives of people with hemophilia and related bleeding disorders. Since it was established in 1963, the WFH has
grown into a truly global network with member organizations in more than 113 countries and official recognition from
the World Health Organization. WFH works in partnership with healthcare professionals and people with hemophilia,
governments and regulators, industry and foundations, to improve hemophilia care throughout the world.
Passport from World Federation of Hemophilia
The Passport, is a directory of hemophilia treatment centers (HTCs) and hemophilia organizations worldwide. The
Electronic Passport is updated daily (as changes are received). You can use the various search features to get a list
of all the centers in a country you will be visiting or find an address of an organization or a person. Please refer to
this website.
http://www.wfh.org/2/8/8_1_Passport_Directory.htm
Medications
DDAVP®/ Stimate®
DDAVP® causes the release of Factor VIII and von Willebrand factor from the cells lining the blood vessels into
the blood stream. This prevents, or helps stop bleeding. DDAVP®is used to treat people with bleeding disorders
such as von Willebrand disease or mild hemophilia A.
How to give:
Intravenous (IV): DDAVP® can be given into a vein.
Intranasal (IN): Stimate® is given as a nose spray.
Subcutaneous (Sub Q or SQ): DDAVP® can also be given subcutaneously.
Stimate is a name brand medicine; there is no generic form of the medicine. Concentration is 1.5 mg/mL.
Generic forms (used for the prevention of bed wetting) of the medication have lower concentration and will not
help stop bleeding.
Special instructions:
DDAVP®/Stimate® may not work for all people with VWD or Hemophilia A. Your doctor may recommend a DDAVP®/Stimate® challenge test to see if this medicine works for you. This is done by giving a dose of the medication and testing the blood before and afterward to see if the circulating factor levels rise. Not all hospitals or pharmacies stock Stimate. You will have to keep the medication for your own use. Order from the pharmacy, in advance of running out, to insure a supply.
After each dose of DDAVP®/Stimate® is given, you should take just enough fluids to satisfy your thirst for
about 24 hours. Ask your hematology doctor or nurse for more specific instructions. There are no
restrictions for foods taken with the medicines.
DDAVP®/Stimate® is dosed by weight. Dosing will change as a child grows. Be sure to speak with your
hematology team regarding dosing instructions.
When using Stimate blow your nose to remove any discharge or blood clots. Then use the nasal spray
(1 or 2 sprays) depending on weight.
1
Side effects (that do not need to be reported):
Redness and warmth of the face and neck
Mild fluid retention
Mild headache
Side effects that must be reported immediately:
Very rapid heart rate
Fever of 101.5 ºF (38.6 ºC) or greater
Chills
Dizziness
Seizures
Report use of other medications, vitamins, and herbal products to your doctor.
Keep this medication in its original container and store as instructed
Call 911 or go to the Emergency Room if you (your child) have:
A seizure
2
Dental care and hemophilia
People with Hemophilia have the same types of dental problems that other people have. However, if you need
dental work such as fillings, extractions, root canals or other procedures, your hematology team should be
involved in planning for the procedure so that you don’t have bleeding.
Good tooth and mouth care will help prevent mouth bleeding. You can also take steps to prevent mouth injuries
when possible. Even with the best dental care, mouth bleeds may still occur. You should learn how to recognize
and treat mouth bleeds quickly so that you will have fewer complications from the bleeding.
Prevention
•Oral hygiene- Brush your teeth twice a day. Daily flossing removes food and bacteria that your toothbrush can’t reach. Regular brushing and flossing will help your gums remain healthy- and healthy gums bleed less!
•Healthy diet- Eat a variety of foods every day. A balanced diet includes meat, vegetables, fruit, grains and dairy products. Calcium-containing foods like cheese, yogurt, and milk help to keep teeth and bones strong.
•Regular dental care- Visit the dentist every 6 months. Regular check-ups and cleaning helps to keep your teeth and gums healthy.
•Don’t use tobacco- Don’t smoke or chew tobacco. People who use tobacco are more likely to have gum disease and plaque on their teeth that causes cavities. If you don’t currently smoke- don’t start! If you already smoke, consider a smoking cessation program.
1
Dental Issues for People with Hemophilia
•Routine dental care- most of the time, people with hemophilia don’t have bleeding with routine dental procedures like prophylactic cleaning or small fillings. It can be helpful to have Amicar® available if you have oozing from the gums after dental work.
•Tooth eruption- sometimes when new teeth come in, bleeding might occur. If the bleeding is prolonged, call your hematology team. Amicar® or Cyklokapron® may help control this bleeding.
•Tooth loss- sometimes bleeding occurs with the loss of “baby teeth”. Again, if the bleeding is prolonged, call the hematology team.
•Tooth extraction- when primary or “baby teeth” are taken out, bleeding is often minimal because
the roots of these teeth are not very deep. As a person ages and gets permanent teeth, the roots go much deeper. Sometimes it is necessary to extract teeth due to crowding in the mouth,
as part of orthodontic treatment, or if the tooth is diseased. A treatment plan to prevent bleeding
is usually necessary for this type of procedure. Call your hematology team before having any teeth pulled out.
•Orthodontic treatment- People with hemophilia are able to wear braces. Discuss with your Hematology team.
•Mouth injury- Injury to the lip, gum or tongue can produce an injury that bleeds. Try to remember
that a little blood looks like a lot when mixed with saliva. Drinking cold liquids may help control
oral bleeding. If you can apply pressure to the site of bleeding with a piece of gauze or moist tea
bag, this may be helpful. If the bleed keeps recurring, Amicar may be helpful in controlling it. Call your hematology office for advice.
Bleeding to the tongue or throat can be very serious if it produces swelling that blocks the airway.
Call 911 if you have difficulty breathing or swallowing with mouth or throat injuries.
2
Disaster preparedness
Now that you have received this flash drive it is important to keep it in a safe, but readily accessible (easy to
locate) space. If you choose to have your health information on this drive, it can then be used during a bleeding
emergency (at the ED) or for another emergency or disaster situation.
The best thing you can do related to a disaster or an emergency is to be prepared.
Preparation
Designate a clean, safe place to keep a duffel bag, filled with important items, such as important local and out
of state phone numbers (including HTC’s), a water bottle, flash light, first aid kit, flash drive (or other material)
with important health information, a dose or two of factor concentrate and supplies for infusion (if you treat
with factor), coban, activating ice pack, and other identifying information. Think of this as your “To GO” bag!
*If you do set aside a dose of factor, make sure to monitor the expiration date and rotate as necessary.
Ideas from the CDC website regarding other items to prepare in the event of an emergency:
http://emergency.cdc.gov/
Water
Store 3 gallons of water per person and 4 additional gallons per person if you are confined to the home
during the emergency.
Food
Stock up on non perishables; a three day supply with an additional 4 day supply per person for use at
home.
Other items
Prescription and non-prescription meds, kitchen accessories, battery powered radio and or TV,
several flashlights, batteries, change of clothing and shoes, blankets, sleeping bags, hygiene items,
entertainment, paper, pencil, medicine dropper, spare keys, cash, credit card, matches in waterproof
container, compass, photo id and passport.
**As with the factor, be sure to rotate the emergency supplies and replace expired items. A 6 month time frame
for replacement of these items should be sufficient.
CDC LINK of HTCs in the United States: https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
Emergency identification
Wearing a MedicAlert® bracelet or necklace is the best way to identify that you or your child has a bleeding
disorder. Young children can wear a bracelet either around their wrist or ankle. Older children and adults can
wear a bracelet or necklace. Sports wrist bands are available for children who are very active. Your HTC can
assist with helping you get one of these with accurate information.
If your HTC or homecare company provides you with an Emergency or Bleeding Alert card, please be sure, as
parents, that you carry the card for your child. Teens and adults should always keep their card in their wallet.
Check out this website for more information:
http://www.medicalert.org/home/Homegradient.aspx
Medications
Factor facts
By Susan C. Zappa RN CPN
Cook Children’s Medical Center
You have been given a very important job.
You are storing an intravenous replacement factor product in your home. This is a privilege.
Learning about this factor product will assist you and your Bleeding Disorders Center to make this a successful
combined effort to enhance the care of yourself or your child.
Factor products are very expensive, the cost can be approximately $300 per dose for the smallest baby,
up to $4000-$6000 per dose for an adolescent or a child with an inhibitor to factor VIII or factor IX.
Due to the expense and because we do not want to waste a precious resource, we treat factor products like they were ‘gold’ and never throw away factor without first talking to your nurse
coordinator. No matter what you think, or what has happened, call your nurse first! On the factor box and the bottle with the powder you will find these four important items:
•Factor name: important to remember this and the company
•Amount of international units (IU): this is the actual dose - written 287 IU or IU 287 (examples). Factor is measured in international units, abbreviated IU on the box,
but called ‘units’ by your doctor and nurse. Your factor may be measured in Factor VIII, FVIII or
AHF IU, and ristocetin co-factor or VWF:RCo IU or Factor IX IU.
•Lot number: important to record so you can track the factor given.
•Expiration date: best used by, but if expired, again, call your nurse before throwing away.
Sample box label:
Antihemophilic Factor (Recombinant) or Antihemophilic Factor (Human) or Antihemophilic Factor/ von Willebrand
Factor Complex (Human)
Brand Name
Lot 27NO5R1
Exp. 12 Oct 06
IU
605
1
Factor is calculated by body weight and the amount of percent increase the doctor wishes to accomplish
with the factor level.
Calculations are different for Hemophilia A, Hemophilia B and von Willebrand patients, so check with your
nurse.
Factor comes in vials that have different amounts of IU in them, and they are not usually the exact dose
calculated.
Ex: your doctor orders your child to have 500 IU. The factor bottle has 555 IU in it. We
give the entire amount.
If the bottle has 478 IU in it, that’s okay, too, the amount is an approximate amount, not exact.
Doses are usually 10% above or 10% below the actual calculated dose.
Storage of factor products:
Place in refrigerator in a plastic bag to keep the box intact and clean.
Keeping factor in the refrigerator helps to make sure it will stay at the exact potency on the box until the
expiration date.
Factor can be stored at room temperature, though, for different lengths of time (months to years). Each
product is different, so read the package insert or check with your nurse.
Factor does not need to be continually refrigerated, just kept out of extreme hot temperatures
(temperatures that may happen in a car in the summer).
People have thought they needed to keep it cold for the trip to the hospital - not true. People
have thought they needed to keep it cold for a day of travel in an airplane (see also ‘Travel’ section) - not true.
People have thrown factor away that was in the refrigerator, but the power went off for several days - it is still
good.
As you can see, you need to talk to your nurse before you think that factor has “spoiled”
or gone bad. Factor may lose a little of it’s potency, but it
does not spoil or go bad as
long as the container is intact and the rubber stopper is not
punctured.
When we say you might lose a little potency, maybe the
calculated 50% dose will only
2
make the factor
are working with
VIII level go to 45%. This is not a difference that needs to be considered when you
factor products.
You may have enormous amounts of factor to keep refrigerated:
You can take the bottles with the powder in them out of the box and just refrigerate those bottles. You need to
know how to read the units (IU) on the bottle, so you can mix up the correct dose, and you need to keep the
box and bottle with water in a clean, dry place.
Mixing the factor:
Mix the factor using the directions given by the manufacturer. Use the mixing tool or needle-less system
provided. Each brand of factor has its own system to mix. Each brand also has different water amounts (2.5
mL, 4 mL, 5mL or 10 mL). You must use all of the water provided. You may have to mix two or more vials of
different strengths to reach your child’s dose. You can draw up all of the factor in one syringe, after you have
mixed all of the vials.
Once you mix the factor it should be given within the manufacturer’s recommended time frame (usually 3
hrs.). Again, the factor can still be okay to use, especially if refrigerated after being mixed, for longer periods
of time, so check with your nurse.
Traveling with factor:
Keep factor with you at all times.
Pack it in your carry-on luggage only; keep it in the passenger part of the car (not the trunk).
Do not allow it to become really hot.
It is best to have a letter from the doctor explaining the factor and syringes
when you travel through airports.
Copyright 2009
Printed with permission from Susan Zappa, RN. CPN
3
Medications
Factor products
Hemophilia is treated by replacing the missing clotting factor in the blood. This is done by injecting a product
that contains the needed factor into a vein. Bleeding stops when enough clotting factor reaches the affected
area. With proper treatment, people with hemophilia live relatively normal lives. Without treatment, most
children with severe hemophilia can die young.
There are two main types of hemophilia factor concentrates. Those made from human plasma and those
made using recombinant technology. No seroconversions to HIV, HBV, or HCV have been reported with any of
the currently available products, plasma derived or recombinant, marketed in the United States.
See list of list factor concentrates from the following resources:
•National Hemophilia Foundation Website, MASAC, list of current MASAC documents, MASAC Docu ment #190
http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=138
•World Federation of Hemophilia, Publications, Printed Materials, Treatment Products, Registry of Clotting Factor Concentrates. Eighth edition, 2008; Facts and Figures monograph no. 6 [English], select English
http://www.wfh.org/2/docs/Publications/Treatment_Products/Monographs/FF6_Registry_8th_2008.pdf
Genetics
How is hemophilia inherited?
Hemophilia is passed on in families to boys through women (Moms) who are carriers.
The odds are 100% that all daughters of men (Dads) with hemophilia are carriers. The sons of a man with
hemophilia will NOT have hemophilia and these sons cannot carry hemophilia or pass it on.
Every time that a carrier has a baby, if the baby is a boy, he has a 50/50 chance of having hemophilia. Or if
the baby is a girl, she has a 50/50 chance of being a carrier.
Within the same family, the type of hemophilia (factor 8 or factor 9 deficiency) and whether it is mild,
moderate or severe is also passed on.
X-Linked recessive, carrier mother
Unaffected
father
Carrier
mother
Unaffected
Affected
Carrier
Unaffected
son
Unaffected
daughter
Carrier
daughter
Affected
son
U.S. National Library of Medicine
1
How did my baby get hemophilia if I have no family history?
1. About 30% of all babies who are born with hemophilia have no known family history.
2. Some of these babies have a new mutation (change) in the gene that usually makes factor VIII and their mother is not a carrier at all.
3. Some babies who have hemophilia come from a long line of carriers who gave birth to only other carriers and so no one remembers a man with hemophilia.
4. Still another explanation is that a new mutation happened in the sperm of the mother’s father. Her father did not have hemophilia, but at least some of his sperm were coded such that factor VIII production was missing.
Only genetic counseling can help figure this situation out. Blood samples can be taken from the baby who
has hemophilia and the mutation or change can be identified. Then, the mother can be tested to see if she
is a carrier. If she is a carrier, then any of her sisters should be tested for the carrier state as well. Also, any
daughters would have a 50/50 chance of being carriers and would need testing to determine whether any of
their children might be affected by hemophilia.
Ask the provider at the Hemophilia Treatment Center to refer you to a genetic counselor or geneticist so that
this can be sorted out in your family.
2
Emergency Care for
Patients with Hemophilia
An instructional manual for Medical Professionals
Determine if the patient has:
Hemophilia A or factor VIII (8) deficiency
Hemophilia B or factor IX (9) deficiency
Head injury (pg. 5)
Always treat immediately with a
major factor dose
Mucous membrane bleeds (pg. 12)
Treat with a routine factor dose
and anti-fibrinolytics
Slings, splints, immobilizers
p.r.n. for joint bleeds
Joint bleeds (pg. 6)
Treat an early onset bleed with a
routine factor dose. Treat an
advanced joint bleed with
a major factor dose.
Abdominal bleeds (pg. 10),
Trauma (pg. 23)
Treat with a major factor dose
Avoid intramuscular
injections due to the
possibility of causing
a muscle bleed
Crutches
hip, knee, ankle bleeds
Minor cuts / bruises
no treatment
Ice pack / Ace wrap
joint bleeds
Written by the Nursing Group of Hemophilia Region VI
Editors: Karen Wulff, R.N.; Susan Zappa R.N., C.P.N., C.P.O.N.; Mack Womack R.N.
Emergency Care for Patients with Hemophilia
Table of Contents
Factor Replacement Guidelines ........................................ 1
Introduction ......................................................................... 2
Hemophilia basics .............................................................. 3
Head injury .......................................................................... 5
Joint bleeding....................................................................... 6
Signs & symptoms, Aspiration, Dislocated joints
Muscle /soft tissue bleeding ............................................. 8
Compartment syndrome, Iliopsoas
Gastrointestinal / urinary tract bleeding ........................ 10
Abdominal muscle wall, Hematuria
Mucous membrane bleeding............................................. 12
Anti-fibrinolytics, Epistaxis, Retropharyngeal bleeds
Factor administration ......................................................... 16
Mixing, Dosage formula
Venous access ...................................................................... 18
Site for administration, Prophylaxis, Venous access devices
Invasive procedures / labs / x-ray .................................. 20
Fractures, Lacerations, LP, Blood gases
Other medications .............................................................. 22
Pain medications, Immunizations
Trauma / emergencies ....................................................... 23
Bites, Burns, Falls, Fractures, Gunshot wounds, MVA, MI, Ocular
injuries, Puncture wounds
Inhibitors .............................................................................. 24
Definition, Treatment
Acknowledgements ............................................................ 25
Copyright © 1999 The Nursing Group of Hemophilia Region VI.
Updated March, 2012
All rights reserved. Permission granted to photocopy for educational purposes only.
All brand names and product names used in this publication are trade names, service marks, trademarks, or
registered trademarks of their respecitve owners. These terms are used in this publication only in an
editorial fashion and should not be regarded as affecting their validity.
Factor Replacement Guidelines
Each hemophilia treatment center throughout the United States sets its own dosage
guidelines. The guidelines below are specifically for patients of Hemophilia
t• •
n
a
t
Region VI. Guidelines for patients from other treatment centers throughout
or
Imp
the United States and Canada can be found at the respective treatment center.
Hemophilia A or Factor VIII (8) deﬁciency
Hemophilia B or Factor IX (9) deﬁciency
Routine dose = 20 - 25 units x kg.
Routine dose = 40 - 50 units x kg.
Major dose = 40 - 50 units x kg.
Major dose = 80 - 100 units x kg.
Examples: sutures, early onset joint
bleeds, muscle and mouth bleeds.
Examples: head, GI, prior to invasive
procedure (ex. LP), and advanced joint
bleeds.
Examples: sutures, early onset joint
bleeds, muscle and mouth bleeds.
Examples: head, GI, prior to invasive
procedure (ex. LP), and advanced joint
bleeds.
NOTE: Please contact the hematologist before any infusion. For patients with Factor 8 or 9 inhibitors (see page 24) it is imperative that you contact the patient’s hemophilia physician before any
infusion. Have all patients contact the Hemophilia Center the next day to report their status.
1
Introduction
Purpose
This manual contributes to hemophilia care by enhancing the emergency department staff's
understanding of hemophilia and its treatment. The goals of this manual are to:
-
promote understanding of the complexities of hemophilia treatment
-
provide a reference for the emergency center staff
-
promote a consultative dialogue with the emergency department, hemophilia
treatment center, and patient/family
Use
This manual provides a standardized format for evaluation and treatment of hemophilia emergencies.
The content is segmented by systems and complications of hemophilia. Turn to an area of interest. The
illustrations provide information points for quick review. The text gives further detail of bleeding
presentations, their possible complications, and treatment.
It is suggested that the patient's hemophilia treatment center or hematologist be consulted for anything
other than routine bleeding episodes.
To The Attending Medical Staff:
This manual is a guide for medical personnel who
may be less familiar with hemophilia treatment. The
content consists of guidelines, recommendations
and suggestions only. The attending physician has
the final responsibility for appropriate diagnosis
and treatment.
2
Basics
Hemophilia is a genetic disorder characterized by a deficiency or absence of one of the clotting proteins in
plasma. The result is delayed clotting. Deficiencies of factor VIII (8) [Hemophilia A or Classic Hemophilia]
and factor IX (9) [Hemophilia B or Christmas Disease] are the most common and referred to as hemophilia.
Hemophilia mostly affects males due to the X-linked inheritance pattern.
Effects of hemophilia
Hemophilia prevents the formation of a firm, fibrin clot and results in a soft, unstable clot. Persons
with hemophilia do not bleed faster than others; rather the bleeding is continuous. Significant blood
loss can occur if treatment is delayed.
Incidence
The incidence worldwide is estimated to occur in 1:7,500 live male births; all races and ethnic groups
are affected. Factor VIII (8) deficiency is four times more common than factor IX (9) deficiency but the
clinical presentations and inheritance patterns are the same.
Severity
The amount of bleeding expected in an individual with hemophilia depends upon the severity of the
deficiency. Normal plasma levels of factor VIII (8) and IX (9) range from 50-150%.
Those with less than 1% factor VIII (8) and IX (9) are considered to have severe hemophilia. Frequent
bleeding episodes are common, particularly into joints. Bleeding can occur spontaneously or from
trauma.
Persons with factor levels of 1-5% are considered to have moderate hemophilia. These persons may
experience bleeding after minor trauma but should not bleed spontaneously. After repeated bleeding
into the same joint, persons with moderate hemophilia may experience spontaneous bleeding in that
joint.
Persons with more than 5% factor activity are considered to have mild hemophilia and bleed only after
significant trauma or with surgery. Some carrier girls and women (called symptomatic carriers) can
have lower than normal plasma levels of factor VIII (8) or IX (9) and thus may exhibit symptoms of
mild hemophilia.
3
Head injury
Treat all head injuries, with or without swelling,
by infusing a major factor dose. * Then perform
diagnostic studies, such as CT scan, neuro exam.
Discharge Instructions
Call the hemophilia treatment center or the
patient’s hematologist for follow-up factor doses.*
Report any signs or symptoms to the hemophilia
treatment center or to the patient’s hematologist.
Head injury instructions for a two week
period (instead of the usual instructions for 24- 48
hour period.)
Intracranial hemorrhage (ICH) is the leading cause of death from bleeding in all age groups. Without
early recognition and treatment, death or severe neurologic impairment can occur. ICH may be
spontaneous, without history of injury. Early neurologic symptoms may not be evident due to the slow,
oozing nature of hemophilia bleeding.
Treatment
All significant head trauma, with or without hematoma, must be treated promptly with the major
dose of factor replacement* before any diagnostic tests.
Diagnostic imaging
Obtain an emergency CT scan to rule out ICH after the major factor dose* has been given. Notify the
patient's hematologist or hemophilia treatment center of the ED admission and the diagnostic
findings.
Possible admission
The patient should be admitted to the hospital for observation if he suffered a severe blow to the
head or if he exhibits any neurologic symptoms such as headache with increased severity,
irritability, vomiting, seizures, vision problems, focal neurologic deficits, stiff neck, or changes in
level of consciousness. Patients with a past history of ICH are at increased risk of repeated head
bleeds.
Instructions
If the patient is discharged home, instruct the family to monitor the patient for signs and symptoms
of neurologic deterioration and report any abnormalities to the hematologist. Consult the
hematologist for follow-up factor replacement doses if the patient is discharged home from the
emergency department.
* See page 1 for factor dosage guidelines or refer to your patient's Individual
Medical Emergency Information on FlashLINK to Hemophilia flash drive.
5
Joint bleeding
Crutches for weight
bearing joints and
crutch instructions.
Advanced joint bleed
- heat
- pain
- swelling
- decreased range
of motion
Treatment: a major factor dose. *
Early onset joint bleed
- tingling
- pain
- limited range
of motion
Treatment: a routine factor dose. *
Remember, toes and
fingers are joints too.
For the next 24 hours:
- RICE (rest, ice, compression [ace wraps],elevation)
- sling or splinting if support is needed (i.e. Aircast for ankles)
Follow-up with the hemophilia treatment center or with the patient’s hematologist
The hallmark of hemophilia is joint and muscle bleeding. Spontaneous joint and muscle bleeding can
occur without a definite history of trauma. The patient may not be able to identify a specific event that
resulted in bleeding.
While persons with hemophilia may bleed into any joint space, the joints which most frequently bleed are
the elbows, knees, and ankles. Other possible bleeding joint sites include the shoulders and hips. As
repeated bleeding occurs, the synovial tissue thickens and develops even more friable blood vessels. A
vicious cycle of bleeding and rebleeding may set in and the affected joint is referred to as a "target joint."
Eventually, repeated bleeding into joints leads to a form of chronic arthritis with destruction of cartilage
and the eventual destruction of bone resulting in decreased joint mobility and function.
6
Joint bleeding
Signs and symptoms
Outward signs of joint bleeding include restriction of movement, swelling, heat, and erythema on and
around the joint. The patient may report symptoms of a bubbling or tingling sensation with no physical
signs. Later symptoms include a feeling of fullness within the joint and moderate to severe pain as the
bleed worsens.
Treatment
Advanced or Early onset joint bleed?
Some patients may present for treatment with no
other outward signs of bleeding than decreased
range of motion and a complaint of pain or
tingling. This is indicative of an early onset joint
bleed and is the optimal time to treat. The patient
should be infused as quickly as possible with a
routine dose of factor* in order to minimize pain
and joint destruction.
Extreme pain, swelling, heat, and immobility are
signs and symptoms of an advanced joint bleed
which occurs only after blood has filled the joint
space. Symptoms suggestive of an advanced joint
bleed require a major factor dose.*
Swelling and a major
decrease in range of
motion are signs of an
advanced joint bleed.
A 'tingling' sensation and
a limited or small decrease
in range of motion are
signs of an early onset
joint bleed.
Infuse before any diagnostic procedures such as xray. If a joint bleed is treated early before obvious
outward signs occur, then the need for expensive
follow-up infusions may be lessened or avoided
altogether. Before dislocated joints are reduced, infuse with a major factor dose.*
Joint aspiration: Caution!
Joint bleeds in hemophilia are generally not treated by aspirating the joint. This procedure can make
matters worse by creating another site from which the patient may bleed. If joint aspiration is deemed
necessary, such as in suspected joint sepsis, then the joint should be aspirated by an orthopedic surgeon
associated with a hemophilia treatment center. The patient's hematologist must be involved in order to
arrange for factor coverage before and after the procedure.
Discharge and follow-up care
Upon discharge, the hemophilia patient with a joint bleed should be instructed to keep the affected
joint at rest, elevate the affected limb, and apply ice packs. Additional support to the affected joint may
be applied by wrapping with an ace bandage. For ankle bleeds, an Aircast may be a useful splinting
device. Crutches are useful to help individuals when they have lower extremity joint bleeds and need
to be non-weight bearing. Follow-up should be made to the local hemophilia treatment center or to the
patient's hematologist as soon as possible.
7
Muscle / soft tissue bleeding
Deltoid / forearm bleed
- routine factor dose *
- major factor dose* if a
compartment syndrome
is suspected
Neck swelling: EMERGENCY
- potential airway
compromise
- major dose of factor *
Soft tissue bleeds
and bruising
- no functional
impairment
- tenderness, but
no severe pain
- no factor needed
Iliopsoas bleeds
- flexed hip
- pain/inability to extend
the leg on the affected
side
- major factor dose*
Buttock bleeds
- pain
- with/without swelling
- routine dose of factor *
- major dose of factor * if
the leg on the affected
side exhibits tingling or
swelling
Thigh/calf bleed
- pain
- with/without swelling
- impaired mobility
- routine factor dose *
- major factor dose* if a
compartment syndrome
is suspected
-
rest
ice
non-weight bearing
Follow-up with the hemophilia treatment center or with the patient’s hematologist
8
Muscle / soft tissue bleeding
Signs, symptoms, and sites
Muscle bleeding is common in persons with hemophilia. Any muscle group may be subject to
bleeding. Muscles that exhibit warmth, pain, and swelling should be treated with a routine dose of
factor.* Common bleeding sites include the upper arm, forearm, thigh, and calf muscles. Toddlers
frequently have buttock and groin bleeds. Abdominal wall muscles and iliopsoas muscles are also
common bleeding sites. These abdominal muscles generally do not have observable swelling, yet
they may hold a large amount of blood. Patients who complain of low abdomen or groin pain,
especially with signs of nerve compression, are probably experiencing an iliopsoas bleed. These
patients should receive an emergency hematology consult and possible admission for observation
and several major doses of factor coverage.* The hallmark sign of iliopsoas bleeding is
spontaneous flexion of the leg on the affected side with an inability to extend the leg without pain.
Consequences of muscle bleeds
Muscle bleeds can result in serious consequences if not treated promptly. Extensive blood loss may
occur in large muscle groups. Muscle bleeding places pressure on nerves and blood vessels and, if
left untreated, these bleeds may result in permanent disabilities such as foot drop and wrist
contracture. It is important that the patient's hematologist be consulted before any surgical
consults. Most muscle bleeds respond well to medical management and do not require fasciotomy.
Such an extreme measure will usually generate problems for the patient and require a tremendous
amount of post-surgical factor infusions, more so than if the patient was treated medically.
Treatment and follow-up care
Most muscle bleeds are treated with a routine dose of factor.* Large abdominal muscle groups and
iliopsoas bleeds should be treated with a major dose of factor.* Rest and ice packs are also helpful.
The patient should be referred back to his hemophilia treatment center or to his hematologist for
follow-up as soon as possible. If any suspicion of compartment syndrome and nerve
compression exists, then the patient should have an emergency hematology consult and should
be admitted to the hospital.
Soft tissue and superficial bleeds
Soft tissue bleeds usually do not require aggressive treatment. Superficial hematomas and bruises
may appear anywhere on the body and if they do not threaten function and mobility, they do not
need to be treated.
9
Gastrointestinal / urinary tract bleeding
Nausea and vomiting may indicate intracranial hemorrhage as well as gastrointestinal
problems.
Abdominal pain
Treat immediately with a
major dose of factor * for:
- flank pain
- melena
- vomiting blood
Iliopsoas bleeding
- flexed hip
- pain on extension
- major dose of factor *
Hematuria
- bed rest for 24 hours
- force fluids
- consult the hemophilia
treatment center or
the patient's hematologist
-
force fluids for hematuria
rest
no weight bearing
report any symptoms
follow-up with the hemophilia treatment center or with the patient’s hematologist
10
Gastrointestinal / urinary tract bleeding
Initial presentation
Acute abdominal pain in a patient with hemophilia may have many origins, such as GI tract
hematomas (both spontaneous or trauma induced), pseudotumors, iliopsoas or retroperitoneal
bleeding. Bleeding may also occur with hemorrhoids or the passage of kidney stones. Notify the
hemophilia treatment center or the patient's hematologist.
Patients who present to the emergency department with abdominal or flank pain, melena or
hematemesis should be triaged for immediate examination and given factor replacement therapy
at the major dosage.* After factor therapy, then diagnostic x-rays, scans and endoscopy procedures
can be carried out.
Abdominal trauma and benign events such as forceful coughing or vomiting can precipitate an
abdominal bleed. Blood loss can be significant before outward signs and symptoms appear. Infants
can have bleeds with gastroenteritis, intussusception or Meckel's Diverticulum.
A history of lifting heavy objects, weight lifting, falling on bicycle handlebars or stretching the
groin can precipitate abdominal wall, iliopsoas (see pages 8 and 9), or retroperitoneal bleeding.
Symptoms
Symptoms of abdominal muscle bleeding (rectus, pectorals, latissimus, obliques) are a palpable
mass, rigidity, and pain. Concurrent bleeding in the abdominal cavity may be present and go
unnoticed for days with a steadily dropping hemoglobin. Rupture of the liver, spleen, or pancreas
should be considered when the hemoglobin falls dramatically following trauma.
For nausea and vomiting without an obvious cause, consider that these may be symptoms of
intracranial bleeding. Inquire about head injury, mental status changes, and other neurologic signs
and symptoms, and consider CT scan of the head.
Genitourinary bleeding
Hematuria is often frightening to the patient but not a serious event. Instruct the patient to remain
at bed rest and force fluids the next 24 hours. Protracted hematuria may require a routine dose of
factor coverage.* Anti-fibrinolytics are contraindicated with hematuria. Contact the hematologist.
Scrotal bleeding may occur after trauma, especially in toddlers. Infuse with a routine factor dose*
and have the family contact the patient's hematologist for follow-up care.
11
Mucous membrane bleeding
A Dental or E.N.T. consult may be needed.
Nose bleeds may respond to
other measures. Refer to
"Controlling Epistaxis" section on page 14.
Mouth bleeds (gum, tooth, frenulum or
tongue laceration) need factor * and antifibrinolytics. Refer to the "Anti-fibrinolytics" section
on page 15.
Anti-fibrinolytics are not routinely available from all
pharmacies. Pharmacies associated with the
hemophilia treatment centers will be able to fill a
prescription.
Patients should follow-up with their hemophilia treatment center or hematologist
the next day.
Instruct the patient on: Controlling Epistaxis, Anti-fibrinolytics, Diet Modification
(pages 14 -15) as needed.
12
Mucous membrane bleeding may require medical care in the emergency department. Patients should
receive factor who:
- are experiencing profuse and/or prolonged bleeding
- have sustained a known injury to the mouth, tongue, or nose
- have severe swelling in the mouth or throat area
- are experiencing respiratory distress
- have difficulty swallowing
The patient may not know the reason for the symptom or bleeding. It may have been caused by
trauma, infection, or the bleed may be spontaneous. If airway blockage is suspected, the patient
should immediately receive a major dose of factor.* After the factor level has been raised, further
interventions including invasive procedures may be done.
Do not make a person with hemophilia wait for factor replacement. The longer he waits, the more
bleeding takes place. If the bleed is in a closed space, the accumulation of blood will cause
surrounding tissue damage, airway obstruction, and enhance pain.
Epistaxis
A person with epistaxis who is unable to control the bleed himself may need a routine dose of
factor* and anti-fibrinolytic treatment. Be sure the person knows how to control and stop
the bleeding. (See section on page 14.)
Oral Cavity
Bleeding in the mouth can be hard to control. The patient will probably need factor. A single
infusion of a routine dose of factor* may temporarily stop the bleeding, but clots break down
normally on days 3-5 and bleeding may start again at that time. An anti-fibrinolytic may be
indicated to maintain hemostasis. (See section on page 15.) Anti-fibrinolytics may be available
through the patient's home health company for next day delivery. A modified diet should be
started at the same time as factor therapy. (See section on page 15.)
Bleeding may occur with erupting or exfoliating teeth. It is more common with exfoliating teeth,
especially a tooth that is very loose. A dental consult may be needed to extract the tooth since it
will continue to lacerate the tooth socket as long as it is in place. A routine dose of factor* should be
given prior to extraction. A frenulum or tongue laceration will require a routine dose of factor.*
Retropharyngeal
After the major dose of factor* is given, further observation, x-rays and admission may be
required.
13
Controlling Epistaxis
Instruct the patient:
1. To gently blow his nose to remove mucous and unstable
clots which will interfere with hemostasis.
2. Tilt his head forward so any blood will come out the nares and not down the back of
the throat.
3.
Apply firm pressure to the entire side of the nose that is bleeding for 15 minutes.
4. Release the pressure to see if bleeding has stopped, blow out any soft clots.
5. If the bleeding continues, reapply pressure for another five minutes.
6. Factor replacement* at a routine dose and/or anti-fibrinolytic agents (see next page)
may be needed.
7. During active bleeding, or when the bleeding has stopped, spray or apply two drops
of oxymetazoline (ex. NeoSynephrine, Dristan, or Afrin) nasal spray/drops to the
side that was bleeding. These can be used at home PRN for epistaxis.
8. Instruct the patient to use Vaseline in the nares to keep the membranes soft and moist,
and prevent the formation of hard crusts which might crack and restart bleeding.
9. An ENT consult may be required for possible cauterization of a vessel.
14
Anti-Fibrinolytics
Anti-fibrinolytics may also be indicated in nasal or oral bleeding. Amicar and
Cyklokapron are both anti-fibrinolytic agents. Either may be prescribed for
mucous membrane bleeding to promote clot adhesion in conjunction with factor replacement
at a routine dose.* In some cases they may be prescribed without factor replacement.
Amicar - aminocaproic acid
Recommended dosage:
child: oral dose 50-100 mg/kg (not to exceed 4 Gms) every 6 hours for 3 - 10 days
adult: oral dose 3-5 Gms every 6 hours for 3 -10 days
Cyklokapron - tranexamic acid (may not be available in the USA)
Recommended dosage:
child and adult: oral dose 25 mg/kg every 8 hours for 3 - 8 days
These medications must be given around the clock to keep blood levels constant.
These medications may be available through the family's home health company, the
hemophilia treatment center, or the family may have a supply at home. They are difficult to
obtain from most pharmacies.
Follow-up through the hemophilia treatment center or patient's hematologist.
Topical agents such as Topical Thrombin and Gelfoam may also be used to help control
mucous membrane bleeding.
Antibiotics and pain medications may also be indicated.
Diet Modifications
Directions for the patient:
1. Diet should be restricted to soft, cool, or lukewarm foods until the area
is fully healed. Suggested foods: Jello, noncarbonated drinks, sherbert, lukewarm
soups (not cream soups), baby foods, blenderized or pureed foods, spaghetti.
2. Milk products may cause nausea and vomiting if the patient has swallowed blood.
3. Avoid using a straw. Negative pressure from the sucking action can dislodge the clot
and aggravate the bleeding site.
4. Avoid hard foods like chips, popcorn, tacos, etc.
15
Factor Administration
Factor Reconstitution
Note : This illustration shows one way to reconstitute factor. Each product
has its own transfer system. Please follow directions on the drug insert.
Filter needle
Transfer needle
attach
syringe
Note : Draw the
reconstituted factor into a
syringe. Discard the filter
needle after withdrawing
the factor.
The volume will
vary by product.
Examples: Antihemophilic factor (AHF), Antihemophilic Factor/von
Willebrand Factor Complex (AHF/VWF) or factor VIII (8)*
Coagulation factor IX (9)*
Mixing instructions and the rate of administration are found on the drug
insert. Document the lot number(s), expiration date(s), factor concentrate trade name and
total number of units infused.
What if the patient brings his own factor?
Some patients are instructed to bring
unmixed factor concentrate with them
to the ED to minimize treatment delay
and cost. Occasionally, patients will
bring prepared factor concentrate after
unsuccessful home venipuncture
attempts. Please assist with
venipuncture and allow the patient or
family to infuse the prepared factor
concentrate if less than 3 hours have elapsed since
reconstitution.
Note: Patients may request
the box top for their records.
16
Factor Administration
Dosage
Each bottle of factor concentrate is labeled with the activity expressed as International Units (IU,
example: 287 IU). The dosage to be administered is based on the patient's body weight in kilograms
(kg)*.
IMPORTANT!
These are examples.
Please consult the
patient's hemophilia
treatment center for
specific dose ranges.
Factor VIII (8) is calculated using the formula:
1 IU/kg = 2% rise in Factor VIII (8) activity
50 IU/kg = 100% correction (example)
Factor IX (9) is calculated using the formula:
1 IU/kg = 1% rise in Factor IX (9) activity
80 IU/kg = 80% correction (example)
The ENTIRE contents of all vials reconstituted for an infusion should be used, even if it exceeds the
calculated dosage ( ± 10% of prescribed dose ).
The half-life of factor VIII (8) is 8-12 hours; the half-life of factor IX (9) is 18-24 hours.
17
Venous access
Do not use the affected or injured limb for venous access.
No jugular or femoral sticks except in life-saving situations.
Use 25g or 23g "winged" needles. After the needlestick, apply
pressure and bandage.
See factor administration (pages 16 - 17) for further information.
Scalp vein (infants only)
Hickman, Broviac or
Groshong catheter
Port
(use Huber needle only)
Antecubital fossa
(caution in infants)
Percutaneous intravenous
central catheter (P.I.C.C.) line
Dorsum of hand
Dorsum of feet
(infants/children)
18
Venous access
Intravenous access for a person with hemophilia is basically the same as for any patient. The use of 23
gauge or 25 gauge "winged" needles is preferable, especially for children.
Sites for access
Sites to consider for a peripheral IV include:
-
dorsum of the hands
antecubital fossa (caution in infants
due to the risk of compartment syndrome)
dorsum of feet (infants and children)
scalp veins (infants only)
NOTE: Groin and neck
veins are contraindicated
except in life-threatening
situations.
The patient's IV should not be started in the affected limb. The injured area should be
minimally manipulated, if at all.
Venous access device
Some venous access devices currently used in hemophilia care include:
Port or peripheral port - access with non-coring needle as per your institution's procedure
External central catheter - Hickman, Broviac, or Groshong; access per your institution's
procedure
P.I.C.C. line - access per your institution's procedure
Heparin flush
It is recommended to do a final flush with heparin for any venous access device (except for
Groshong's which are only flushed with normal saline). Check with the parent or the patient's
institution for the amount of heparin flush to use. If these options are not available, use your
institution's procedure for the amount and concentration of heparin. This small amount of heparin
will not harm the patient (remember - you've just given him factor). The access device needs to
stay patent; this is accomplished with the heparin.
The patient on "prophylaxis"
Some patients with hemophilia now receive "prophylactic" doses of factor replacement on an
every-other-day or 2-3 times a week routine schedule. This should be asked of the patient. These
patients may have venous access devices for infusions. Always check to see if the patient has a
venous access device before starting a peripheral infusion.
19
Invasive procedures, labs, x-rays
Head injury
(see page 5)
First give a major
dose of factor * . . .
. . . then perform
a CT scan.
Fracture
First give a major
dose of factor * . . .
. . . then apply
the cast.
Patient should follow-up with the hemophilia treatment center or his hematologist the
next day.
If the patient received sutures, remind the patient he will need factor for suture removal.
If the patient received a head injury, discharge with routine post head injury instructions
(patient to follow-up for two weeks instead of 48 hours - see page 5).
In any situation, the infusion of factor should never be delayed if any bleeding is suspected. Delaying
the infusion simply increases bleeding that will result in greater morbidity. Diagnostic work-ups are
not indicated for patients with an established hemophilia diagnosis. In general, patients with
hemophilia who are experiencing an acute bleeding episode first need a factor infusion. Other
procedures should not be done unless there is another clinical indication for the study.
20
Invasive procedures, labs, x-rays
Laboratory studies
If the only complaint is an acute joint or muscle bleed, no laboratory studies are necessary. If GI,
abdominal, large muscle, or oral cavity bleeding is suspected and has potentially been extensive, a
CBC may be indicated to determine if the individual is anemic. Factor levels and inhibitor levels
are not necessary for treatment in an acute emergency setting. Factor should not be delayed for
laboratory studies to be drawn or completed.
X-rays and other radiological studies
Give factor first, then decide if a radiological study is indicated. Remember that a swollen joint or
extremity is usually the result of internal bleeding, not a fracture. X-rays of the joint can be used to
document a joint bleed, but are generally not useful in detecting early onset bleeds (and that is when
treatment is optimal). The patient will be aware of joint bleeding before radiological changes are
evident.
A CT of the head (see page 5) is necessary when dealing with a potential head bleed. It can document
location and extent of bleeding and help direct further treatment. First give a major factor dose.*
Fractures
Give a major dose of factor* replacement, then x-ray and set the bone.
Lacerations and sutures
Sutures and staples should be used as on any other patient. If the laceration is significant enough to
require sutures, the patient should first receive a routine dose of factor* then the procedure. Contact the
patient's hematologist for follow-up factor infusion instructions. For removal of sutures, a routine dose
of factor* is usually needed.
Invasive procedures
Invasive procedures should be performed as clinically indicated, i.e., lumbar puncture with symptoms
of meningitis. However, a major dose of factor* should be given before the procedure begins.
Arterial sticks and venipunctures
Do not do arterial sticks unless no other option is available. If an arterial stick must be done, then a
major factor dose* must be given first.
Venipuncture (see page 19) may be done at any location; hands are generally excellent and no factor
treatment is necessary. Avoid "digging" for deep veins. Apply pressure for several minutes after the
puncture.
21
Other medications
Pressure and ice pack
after injections
Routine medications
Patients with hemophilia can receive routine medications (e.g. pain medications, antibiotics, etc.) that
do not interfere with clotting function. Avoid non-steroidal anti-inflammatories (NSAIDS), ASA and
any product with aspirin-related ingredients (e.g. Pepto-Bismol, Excedrin, Percodan).
Medications for fever or pain
Acetaminophen can be given for fever or pain. Narcotics/opioids are often recommended to control the
pain experienced by a patient with hemophilia. Avoid giving intramuscular injections of antibiotics,
pain medications, or immunizations because of the possibility of causing a muscle bleed.
Routes of administration
Medications which can be given PO, SC, or IV are preferred. Routine immunizations and tetanus toxoid
may be given subcutaneously. If the rabies vaccination series is needed, an experienced hematologist
(preferably the patient's) should be contacted to arrange factor infusions prior to and after the injections
in order to prevent internal bleeding.
For any needle stick, pressure and an ice pack afterward will minimize soft tissue or muscle bleeding.
Caution
Some patients with hemophilia may have liver disease from hepatitis. Use caution when prescribing
drugs that may cause liver toxicity. Other patients may be on other therapies for hemophilia-related
complications such as HIV or hepatitis. Be aware of potential serious drug interactions.
22
Trauma / Emergencies
Trauma / Emergencies
Many different emergencies/traumas may occur to persons with hemophilia, just as to others. The
more common are:
-
-
Animal bites
Burns
Falls
Fractures (see page 21)
Gunshot wounds
-
-
-
Motor vehicle accidents
Myocardial infarctions
Ocular injuries
Puncture wounds
Dislocated joints
Treatment
A major dose of factor* should be infused as soon as possible (before any test, x-rays, debriding,
sutures, etc.).
23
Inhibitors
An inhibitor is an antibody that some individuals
with hemophilia develop against factor VIII (8) or IX
(9). These antibodies neutralize the factor
procoagulant activity, thus counteracting the
desired effect of an infusion of factor concentrate.
Contact the hemophilia treatment center or
the patient’s hematologist due to the
complexity of managing inhibitors.
How inhibitors are measured
Labs express the presence of an inhibitor in terms of Bethesda units (BU). One Bethesda unit is the
amount of antibody that destroys half of the factor VIII in an equal mixture of normal and patient
plasma in two hours.
Low responding inhibitor measures less then 10 BU. High responding inhibitor measures greater than
10 BU. An infusion of factor concentrate further stimulates the inhibitor antibodies, causing a rise in
BUs.
When to suspect an inhibitor
Suspect an inhibitor if bleeding doesn't stop after several infusions of factor concentrates.
Call the patient's hematologist if the inhibitor is known or suspected before attempting treatment. Be
sure to also ask the patient and family if they have been told the patient has an inhibitor.
Treatment of inhibitors
Inhibitor management is difficult for the experienced hematologist. Contact the patient's
hematologist or hemophilia treatment center when these patients present in the emergency
department for treatment. Bleeding in an inhibitor patient can quickly lead to serious life- or limbthreatening complications without expert management.
24
Acknowledgements
Emergency Care for Patients with Hemophilia was written and prepared by the Nursing Group of
Hemophilia Region VI (AR, LA, OK, TX) in order to help facilitate prompt care of patients through their
emergency departments. The group wishes to thank the following for their contributions:
Nurses from Region VI Hemophilia Treatment Centers:
Cook Children's Medical Center, Ft. Worth, TX
Gulf States Hemophilia and Thrombophilia Center, Houston, TX
Hemophilia Center of Arkansas, Little Rock, AR
Louisiana Comprehensive Hemophilia Care Center, New Orleans, LA
North Texas Comprehensive Hemophilia Center,
Adult Program, Dallas, TX
North Texas Comprehensive Hemophilia Center,
Pediatric Program, Dallas, TX
The Oklahoma Center for Bleeding Disorders, Oklahoma City, OK
South Texas Comprehensive Hemophilia Center, San Antonio, TX
Reviewers:
Howard Britton, M.D.
Medical Director, South Texas Comprehensive
Hemophilia Center, San Antonio, TX
Cindy Leissinger, M.D.
Medical Director, Louisiana Comprehensive
Hemophilia Care Center, New Orleans, LA
Arlo Weltge, M.D.
Associate Professor of Surgery/ER Medicine,
The University of Texas Medical School, Houston, TX
Rita Gonzales
consumer parent and past President, Lone Star
Hemophilia Chapter, Houston, TX
Production Team - Project Manager / Layout: Art Gardner, Illustrations: Mark Gilmore and Heather Swaim
25
Hemophilia terms
Abbreviations Used
aPTT CVAD FIX FVIII ITI pd FVIII rFVIII VWF activated partial thromboplastin time (aPTT)
central venous access device
factor IX
factor VIII
immune tolerance induction
plasma-derived factor VIII
recombinant factor VIII
von Willebrand factor
Terms
activated partial thromboplastin time
(aPTT)
laboratory test used to detect an inhibitor
activated prothrombin complex concentrate
(aPCC)
a bypassing agent used in patients with high-titer inhibitors
anamnesis/anamnestic response
marked increase in inhibitory titer after factor VIII is administered
antibodies molecules produced by the body’s immune system; hemophilia A inhibitor antibodies block the function of factor VIII replacement
concentrate
arthropathy permanent joint disease caused by repeated bleeding into a joint
Bethesda assay laboratory test that assigns a numerical value to an inhibitor
Bethesda unit (BU) unit of measurement used to quantify an inhibitor
bypassing therapy agents that bypass the need for factor VIII by using other clotting factors to stop bleeding episodes in patients with high-titer inhibitors
1
central venous access device (CVAD) a device implanted under the skin that simplifies the administration of
factor in young children
factor VIII recovery assay laboratory test used to detect an inhibitor and to monitor factor VIII
replacement therapy
factor replacement concentrate used to control bleeding episodes in patients with hemophilia; factor
VIII is used for patients with hemophilia A, and factor IX is used for patients
with hemophilia B; often referred to simply as “factor”
hemarthrosis bleeding inside a joint
hemophilia A a hereditary bleeding disorder caused by a deficiency or absence of clotting factor VIII
hemophilia B
a hereditary bleeding disorder caused by a deficiency or absence of clotting factor IX
high-responding inhibitor inhibitor that markedly increases after factor VIII is administered;
anamnestic
high-titer inhibitor inhibitor titer >5 BU
historical peak titer the highest inhibitor titer recorded for a patient prior to starting immune
tolerance induction
immune tolerance induction (ITI) procedure that attempts to permanently eradicate an inhibitor by regularly
exposing the patient to factor VIII concentrate
inhibitor antibodies produced by the immune system that partially or totally block the
ability of factor VIII replacement concentrate to control bleeding
inhibitor titer the quantity of an inhibitor; expressed in Bethesda units
low-responding inhibitor inhibitor that never rises above 5 BU after factor VIII is administered;
nonanamnestic
low-titer inhibitor inhibitor titer ≤5 BU
mild hemophilia A factor VIII levels that are 5-25% of normal values
mixing study mixing of normal plasma with plasma from a patient with a suspected
inhibitor; used to confirm the presence of an inhibitor
2
moderate hemophilia A factor VIII levels that are 2-5% of normal values
plasma-derived factor VIII factor VIII replacement concentrate derived from human donors; some of
(pdFVIII) these concentrates contain von Willebrand factor
prophylaxis regularly administered doses of factor VIII to prevent bleeding in patients
with severe hemophilia A
protocol
a treatment regimen
recombinant activated factor VII (rFVIIa)
a bypassing agent used in patients with high-titer inhibitors
recombinant factor VIII (rFVIII) factor VIII replacement concentrate formulated without human plasma
using recombinant DNA techniques
severe hemophilia A factor VIII levels that are <1% of normal values
target joint a joint that has become damaged because of repeated bleeding
thrombosis formation of clots within blood vessels; an undesirable complication of
factor replacement or bypassing therapy
tolerization the disappearance of inhibitor antibodies in a patient who has undergone
immune tolerance induction
von Willebrand factor (VWF) large protein found in blood plasma and platelets and in the cells lining
blood vessels; critical component of the clotting process; plays key role in
factor VIII functioning and may protect factor VIII molecules from inhibitors
3
Hemophilia
Hemophilia is a hereditary bleeding disorder caused by a deficiency or absence of clotting factor VIII (hemophilia A)
or clotting factor IX (hemophilia B). Hemophilia A, the more common form of hemophilia, affects between 1 in 5000
to 10,000 males in the United States, or approximately 400,000 people worldwide.1 It causes prolonged bleeding
that often occurs inside a joint. The joints usually affected are the knees, hips, wrists, and ankles. Joint bleeding is
called hemarthrosis, and it can be quite painful. Repeated bleeding into a joint may cause permanent joint disease,
termed arthropathy. Arthropathy results in a loss of normal joint function and, often, deformity.
Unaffected knee
Knee affected by arthropathy
Hemophilia is classified not only by the type of deficient clotting factor—FVIII for persons with hemophilia A, factor
IX (FIX) for those with hemophilia B—but also by the amount of clotting factor present in a person’s blood. As Table
1 shows, FVIII levels in people without hemophilia range from 50% to 150% of normal values.2 People with mild
hemophilia A have FVIII levels that are 5% to 25% of normal, those with moderate hemophilia A have FVIII levels that
are 1% to 5% of normal, and FVIII levels in individuals with severe hemophilia A are less than 1% of normal values.
FVIII levels in patients with hemophilia A
Normal factor VIII levels are 50-150% of normal values
Mild hemophilia
>5% - <40% of normal values
Moderate hemophilia
1% - 5% of normal values
Severe hemophilia
<1% of normal values
Table 1
Intravenous infusions of factor VIII (FVIII) replacement concentrate are used to control bleeding episodes in patients
with hemophilia A.
References:
1. Srivastava A, Giangrande P, Poon MC, M. C, McCraw A, Wiedel J. Guidelines for the management of hemophilia. Available at: http://www.wfh.org/index.asp?lang=EN. Accessed March 28, 2008.
2. Knight C. Health economics of treating haemophilia A with inhibitors. Haemophilia. 2005;11 Suppl 1:11-17.
How to backup your flash drive
The data on your flash drive may be safe and secure now, but take a moment to think of all the potential
disasters that can strike at any time: like losing your flash drive, theft, operator error, fire, or damage due to
water or other accidents. Backing up your flash drive is a necessary safeguard against potential threats.
To back up Important Medical Information that you have entered into your interactive forms, simply following
these steps:
1. After you have entered personal information into one of the three interactive forms (Individual
emergency medical information sheet, Clinic follow up sheet, or your Log Sheet), you must save your
edited file. To save your file, select or click on the “Save” button in the lower left corner of the
page. Make sure you have seen the “Save successfully” message pop up to confirm that you have
successfully saved your information.
2. Select or click on the “Printer Friendly” button in the upper right corner. You will now see a preview
of your printed page.
3. Select or click on the “Backup” button in the upper left corner of this page.
4. Save your file to your desired/preferred location (e.g. a designated folder in your personal drive)
on your personal computer (PC), not to your flash drive if you wish to keep a copy of this file in a
separate secure place. You may keep the given name of this file or rename it at this time. Save your
file as an html file by selecting or clicking on the “Save” button in the Window.
5. Select the “Back” button on the upper right corner of your preview page to return to the Table of Contents.
6. Your file will be saved on your computer, following your directions as to your preferred location and drive.
Regular back up of files is encouraged especially if your medical information has changed or is changing. You’ll
also want to keep your clinic follow up sheets and infusion records up to date.
Immunizations
One of the first challenges that your baby with hemophilia will face is the routine immunizations or “baby
shots”. These may begin while he is still in the hospital and then at 2 months of age, 4 months and so on
according to the recommended schedule that the doctor follows.
Babies with hemophilia should have immunizations the same way as other babies to protect them from
getting serious diseases.
Recommendations to prevent a muscle bleed:
•The nurse should use a 25 or 26 gauge needle and proceed
as needed for an immunization into the muscle or into the subcutaneous tissue (SC)- if that is the preferred route from
the manufacturer.
•You can help to reduce the chances of a muscle bleed by holding
firm but not applying hard pressure on the area afterwards with
a pressure bandage.
•Provide a small ice pack or ask for one that you can wrap in a wash cloth and hold over the area for 10-15 minutes -be sure and do not hold the unwrapped ice pack directly on a baby’s skin! •You may choose to limit immunizations to one shot per thigh per visit.
How do I know if there is bleeding in the muscle?
Baby shots can cause a little red area around the injection site. Some hurt worse than others and you should
give your baby acetaminophen drops as directed by the provider.
If the baby is crying several hours after the shots, will not move his leg, and/or you notice the red area is getting
larger and harder and is warm to touch, call the Hemophilia Treatment Center doctor ‘on-call’. DO NOT WAIT – especially if you have given a dose of acetaminophen and that has brought no relief. Your child may need a dose of factor to stop the bleeding in the muscle and you may have to take him to the
HTC, Emergency Room or office to be infused.
If you have factor at home -TAKE YOUR FACTOR WITH YOU!
Infancy – the first 6 months
Relationships
Your baby learns and grows through strong, supportive relationships with his family.
Parents provide the foundation through which your baby can explore and learn about the
world. Get to know him, and learn to tell the difference between his “hungry cry” and
“pain cry”. This will help you as you learn to respond to injuries and bleeding.
Use the bath time to check out your baby- looking for bruising, tender spots, swelling
or anything unusual. Call your hematology team with concerning findings. When baby
needs a factor infusion or blood draw, be prepared to comfort him. Your love and support
will help him through the experience.
The World
Your baby learns about the world by exploring it with his senses. This means he needs to touch, taste, look at
and smell everything he can find! You have to allow this, and at the same time protect him from being harmed.
His bleeding disorder can make this more challenging.
Bleeds are not common in the first few months, because he isn’t moving very much yet. Among the most
frequent bleeds are:
Bleeding with circumcision
This may be how the bleeding disorder is discovered. There should be no more than a few drops of blood with
circumcision. If your child has bleeding that continues over several days, or that seems to be more than a very
small amount- call the pediatrician.
Bleeding with immunizations
Immunizations are good for your baby’s health! Ask your pediatrician to use the smallest gauge needle possible
when giving shots. After the shot is given, apply pressure to the site for at least 10 minutes. Cold packs applied
to the site for 10 minutes on, then 10 minutes off, through out the day will also help with pain and swelling.
1
Some children with hemophilia will develop a muscle bleed after getting immunizations. If this occurs, the
baby’s leg will be warm, swollen and tender to the touch. Baby will be unhappy, because this hurts. Apply ice,
and call your pediatrician or hematologist. A factor infusion may be needed.
Bleeding with venipuncture
It’s sad to say that sometimes a baby will develop a bleed after having blood drawn in the doctor’s office!
Drawing blood from an infant can be challenging for the best nurse. Always apply pressure to the site of a
venipuncture for at least 10 minutes after the blood draw. Later, if you notice swelling at the site, apply ice and
pressure (if this doesn’t cause pain to the baby), then call the HTC for advice.
Bruising
A baby with a bleeding disorder will bruise more easily than other children. He may develop bruises where
he is picked up, or where his car seat strap crosses his shoulder, or on his knees with crawling. Bruises are
unsightly, but not too concerning unless they grow larger and cause pain to the baby. It may be helpful to draw
a line around a concerning bruise so that you can tell if it is growing. Ice or cold packs can be applied to help
treat a bruise. If a bruise causes your baby to stop using his arm, or to stop crawling, call the HTC for advice.
Head bumps
If your baby experiences a “head bump” (if he rolls off the bed, hitting his head for instance) he may need a
factor infusion and head CT. Call your hematology team for instructions as soon as possible after the injury.
Some parents choose to use a soft helmet for their child to protect against head bumps. Ask your hematology
team for their input on this.
Mouth bleeds
Mouth bleeds are common and sometimes difficult to control. They may occur when your baby gets a new
tooth, or when he falls with something in his mouth. It is difficult, if not impossible, to apply pressure or ice to
an injury in the mouth. It may help to give the baby cold liquids to drink. Amicar® can often be used by itself
to treat a mouth bleed. A factor infusion may be necessary, along with Amicar®, used to help keep the clot in
place. Call your hematology team for directions on how to treat mouth bleeds.
Joint bleeds
Joint bleeds are rare during the newborn period but can occur after baby starts crawling and walking. The joint
involved will be warm to the touch, swollen and quite painful. Your baby may not want to crawl or to use the
body part that is affected. Factor will be used to control this type of bleed. Apply ice and call the hematology
team.
2
Infections
The safety of blood products is important for all people, especially those with hemophilia. The introduction of
clotting factor concentrates in the 1970s greatly improved the treatment for hemophilia patients. While the risk of
hepatitis from these products was recognized early on, the large number of persons with hemophilia who became
infected with human immunodeficiency virus (HIV) in the early 1980s brought new questions to the safety of the
blood supply. Prevention measures, including more sensitive donor screening methods, viral inactivation (viral
killing) techniques, and the development of genetically engineered (recombinant) factor, were rapidly introduced to
avoid future contamination. The use of these strategies has substantially eliminated the spread of viral diseases,
including HIV and hepatitis, through the use of blood products.
The nation’s blood supply is safer now than ever before. However, monitoring is important to make sure that
it remains safe and to detect the appearance of any new infectious agent that may pose a threat to its safety.
Members of the bleeding community can help protect themselves in two important ways:
1. GET VACCINATED.
Vaccination is a safe and effective way of protecting oneself against infection from HAV and HBV. CDC’s Advisory
Committee on Immunization Practices recommends HBV vaccination for all children and HAV vaccination for
persons greater than 2 years of age who have hemophilia or other bleeding disorders. No vaccine is currently
available against HCV. (See Immunizations)
2. PARTICIPATE IN Universal Data Collection or UDC.
Early identification of potential blood borne diseases in the blood supply can help prevent further spread to others.
Blood samples collected as part of the UDC program are stored in a national serum bank, and if a new disease is
discovered, these stored blood samples can be tested to identify any potential threats to the safety of the blood
supply. (See Universal Data Collection or UDC)
Information for coaches and physical education teachers
What is hemophilia?
Hemophilia is a genetic bleeding disorder in which a protein (Factor VIII or Factor IX) in the blood, needed for
blood to clot, is missing or deficient.
Activity choices
Students have no restriction in activities at school with the exception of contact sports. Sports such as
wrestling, football or ice hockey, where head butting or spinal injuries may occur are discouraged.
Safe sport choices would be, but are not limited to, swimming, tennis, golf, basketball or baseball. (See Sports
rating by risk)
Exercise should be encouraged with all hemophilia patients. Exercise encourages:
• Strong muscles which help protect joints against bleeding episodes
• General feeling of well being
• Group participation and team work
Emergencies / Injuries
If an injury should occur, the parent should be notified, and a plan of care should be established. This would
include a discussion as to how long to avoid the sport to avoid re-injury, and a treatment plan such as RICE
(Rest, Ice, Compression, Elevation), or potential additional protective gear for the future.
Always report all injuries to the parent. If the parent is unavailable, the Hemophilia Treatment Center should
be notified.
Injuries to the head, throat, neck, abdomen or eye are considered life threatening and the parent and emergency
services should be contacted immediately.
Tips in caring for a student with hemophilia
Students will not bleed any faster than anyone else who may cut themselves or have a nosebleed, they will just
bleed longer. This will require holding pressure for a longer period of time to the area that is bleeding.
Do not give aspirin or products containing aspirin to hemophilia students
Listen to the student. They often know they are having a bleed before anyone else would suspect it.
Information for teachers and school nurses
Teachers
What is hemophilia?
Treatment
If a student has a bleeding episode in class:
• Wear protective gloves
• Apply pressure to the area that is bleeding
• Send them to the school nurse for further evaluation
Always report all injuries to the parent.
Injuries to the head, throat, neck, abdomen or eye are considered life threatening and the parent should be
contacted immediately. If the parent is unavailable, the Hemophilia Treatment Center should be notified.
Students will not bleed any faster than anyone else who may cut themselves or have a nosebleed, they will just
bleed longer. This will require holding pressure for a longer period of time to the area that is bleeding.
Do not give aspirin or products containing aspirin to hemophilia students.
A student with hemophilia may not show signs of bleeding such as a nosebleed or cut, but may be bleeding in
a joint or muscle. If he is limping or favoring a limb, or if you notice swelling or redness in an area, send him
to the school nurse for evaluation.
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School Nurses
What is hemophilia?
Treatment options
Many students are on a prophylaxis regimen at home in which the student or parent gives them replacement
factor on a routine basis. This will keep their factor level high enough to prevent spontaneous bleeding. They
can, however, still bleed with an injury.
Amicar®, an antifibrinolytic, is a medication given in a liquid or pill form. It helps hold a clot together once it is
made. Amicar® is used with factor replacement or conventional methods such as ice, pressure and elevation to
stop the bleeding. It is usually given every 6-8 hours for several days to prevent the bleeding from reoccurring.
Always report all injuries to the parent. If the parent is unavailable, the Hemophilia Treatment Center should
be notified.
Injuries to the head, throat, neck, abdomen or eye are considered life threatening and the parent and emergency
services should be contacted immediately. Factor replacement will need to be provided.
For minor bleeding problems, such as a nosebleed or scrapes or cuts, use basic first aid procedures and RICE
(Rest, Ice, Compression and Elevation). The parent should be called if the bleeding has not stopped within 20
minutes.
Students will not bleed any faster than anyone else who may cut themselves or have a nosebleed, they will
just bleed longer. This will require holding pressure for a longer period of time to the area that is bleeding.
Do not give aspirin or products containing aspirin to hemophilia students.
If the parents feel the student needs factor replacement therapy for the injury they have been called for, they
may be able to bring the medication to the student, infuse them and then allow the student to return to class. The student may not always need to go home after a minor bleeding episode.
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Infusion record
Why keep records of your child’s infusions?
As you know, hemophilia is one of the most costly disorders to treat and factor concentrates are expensive!
When factor 8 or 9 is given to a patient in the hospital or clinic, a record of the number of units and the lot
number used is recorded in the patient’s chart right along with the reason it was given.
When the Hemophilia Team decides that your family is ready to have factor at home and someone learns to
infuse factor without a nurse present, it is important to continue the record keeping part of home therapy.
How are these records used?
1.The record of infusions lets the Team know if your child is at risk of developing a target joint. The earlier that a target joint is treated, the less likely it is to have permanent damage. Bring in your infusion records every clinic visit!
2.If your child is on prophylaxis and you say that the infusions were the same for the past 6 months,
a written record allows the Team to know if there was any breakthrough bleeding or procedure that
required even more infusions. Maybe the dose needs to be increased or the days of the week need to
be changed….OR…..maybe you can decrease the number of days of prophylaxis each week.
3.If there is ever a need to see if your child had an infusion from a particular lot number, you and
the Treatment Center will have that information. These records were critical for people to have during
the days before the factor products were free from viruses.
4.Insurance companies/Social Security Disability base their payment on the record of factor used.
This is the only way that the Treatment Center has of proving that your child has bleeding episodes and
is using factor!
5.It is possible to have your home therapy privilege taken away if you do not keep accurate records of
infusions.
*Discuss any number of infusion record keeping methods with the nurse; including web based programs!
Instructions for use
From a Windows PC, double click on the file called Open me first Home Page.hta from your USB flash drive.
Open me first - Home page.hta
Once open, the user can view or hide the Table of Contents by clicking on the FlashLINK to Hemophilia link.
Under Contents, the user can then click on topics relating to hemophilia via the links provided.
Contents
All forms under Medical Information are interactive and can be edited.
•Specific information can be saved by clicking on the Save button (bottom left corner of the page). Make sure you see “Save Successfully!” pop up to confirm that your information has been saved.
•Forms can be printed by clicking the “printer friendly” icon twice. Select the back button to return to your document and Contents.
Medical Information Form
All other links in the Contents provide read-only documents for educational purposes which can also be printed.
Enjoy using this resource!
You need Adobe® Reader to view and print each page. Our printable page uses the Portable Document Format
(PDF) created by Adobe®. Most likely, you already have it installed. But if you don’t you can get a free copy
directly from Adobe®.
To install - click on this logo
Joint bleeding or hemarthrosis treatment options
Introduction
Joint bleeding or hemarthrosis is the most common type of bleeding that persons with hemophilia have. If it
is not recognized and treated early, it can lead to lifelong problems. The most common joints that persons with
hemophilia bleed into are the ankles, knees, and elbows, but joint bleeding is not limited to just these joints.
A bleed into a joint is usually the result of some type of injury, but not always. Persons with severe hemophilia
bleed more frequently into joints than persons with moderate or mild hemophilia.
What happens during a joint bleed?
It does not take much of an injury to cause a joint bleed in a person with severe hemophilia and may even be
spontaneous. For those with mild or moderate hemophilia, an injury or trauma usually occurs to cause the
bleeding episode. Keep in mind for those with mild hemophilia, the injury could have been several hours or
even a day before the symptoms show up. With severe hemophilia, the symptoms usually occur within an hour
or two of the injury.
Tiny blood vessels in the lining of the joint break as a result of the injury. If you have hemophilia, the body is
unable to stop the bleeding from these vessels, resulting in a joint bleed. When there is no room for the blood
in the joint, the joint will begin to swell. Persons with hemophilia often can feel a joint bleed before there are
any outward signs of swelling. If the bleed occurs with an infant or toddler, parents may note that the child is
not using an arm or leg. THIS IS THE TIME TO SEEK TREATMENT. If a bleed is not treated at this stage, the
joint will become more swollen. The person with hemophilia will no longer be able to move the joint through
its normal range of motion and the joint may even become warm and red. One bleeding episode that goes
untreated or is treated late can lead to a chronic problem.
Treatment
The number one treatment for a joint bleed is factor replacement. This should be instituted as soon as the
person/child notes a problem. The optimal time for treatment is 2 hours or less from the onset of symptoms
to the infusion of factor. This is not always possible, if the family needs to access the Emergency Room, but
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every effort should be made to try. Most joint bleeds will require follow up treatment (this will depend on the
joint involved, the type and severity of hemophilia and your treatment centers policy/protocols).
In addition to factor replacement RICE is an important adjuvant treatment for a joint bleed. RICE stands for
Rest, Ice, Compression and Elevation.
Rest
This means staying off the joint if it is a weight bearing joint (ankle, knee) or simply not using a joint if the
bleeding is in the upper extremity. This can be accomplished in a number of ways: using crutches, staying
home on the couch, using a sling etc. In young children it is often difficult to have them rest so having a new
video or movie on hand to introduce at the time of the bleed may be helpful.
Ice
Ice should be used intermittently for the first 24 hours after a bleed occurs. It should never be left on for longer
than 20 minutes at a time. Do not apply ice directly to the skin; make sure that there is a towel or other cloth
(like a sock) in between the skin and the ice.
Compression
Compression can be applied using an elastic bandage or support. It should not be so tight as to cut off
circulation.
Elevation
The joint should be elevated higher that the heart. This will help to reduce swelling. Keep in mind that you
really cannot adequately elevate a knee or ankle from a sitting position, you must be lying down.
Joint bleeds can be very painful. It is important to address this along with other treatment aspects. Tylenol
can be given to most people to control their pain. It is important to remember to give it to young children as
well because they do not always verbalize the pain they are having. NSAIDs can be used safely in persons
with hemophilia on the advice of your treatment center. Not all treatment centers allow the use of these
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medications. A person having a joint bleed might require a narcotic for pain relief. These must be prescribed
by your physician.
Chronic hemarthrosis
Over time the body will break down and absorb the blood left in a joint following a hemarthrosis. The blood that
is absorbed and iron left behind are irritants and can cause the synovial lining of the joint to become inflamed
and swollen. This swollen synovium bleeds easier so the person with hemophilia may note frequent bleeds
into this joint. This is called a “Target Joint”. This can cause a loss in motion of the joint and can eventually
lead to chronic joint disease or arthritic changes. Early treatment of joint bleeds is the best way to prevent
these complications.
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Joint damage
Joint bleeding or (hemarthrosis) is the most common type of bleeding that persons with hemophilia have. If it
is not recognized and treated early, it can lead to life long problems. The most common joints that persons with
hemophilia bleed into are the ankles, knees, and elbows, but joint bleeding is not limited to just these joints. A bleed
into a joint is usually the result of some type of injury but not always. Persons with severe hemophilia bleed more
frequently into joints than persons with moderate or mild hemophilia.
Bleeding into knees, elbows, ankles, shoulders, and hips can lead to chronic swelling and later joint deformity. Many
people with severe hemophilia can suffer from painful, debilitating joint bleeds and associated mobility issues that
severely impede their quality of life.
Links to other resources
The National Hemophilia Foundation (NHF)
NHF seeks to promote and enhance the quality of life for all people with coagulation disorders through education,
advocacy and research. Towards these ends, NHF engages in a number of program initiatives along with its
48 chapters nationwide. NHF supports important clinical research, funds educational outreach, issues medical
advisories, convenes educational meetings and symposia, advocates for improved treatments, makes policy
recommendations, and promotes communication and cooperation among all members of the community.
NHF also offers a wide range of educational services and publications through HANDI, its information resource
center.
NHF Member Chapter Organizations
NHF’s 48 state and local chapters are wonderful resources for networking with people affected by bleeding disorders.
Many chapters provide valuable information, materials, advocacy and support to area families. Some chapters assist
individuals with accessing appropriate healthcare, offer support groups, become involved in state-based advocacy
issues, and provide financial assistance and other special services. While many people with bleeding disorders have
found the services provided by their chapters indispensable, others have built upon their chapter experiences to
take on leadership roles within the community.
For more information about NHF, go to: http://www.hemophilia.org.
Hemophilia Treatment Centers (HTCs)
There are approximately 140 hemophilia treatment centers in the United States that offer comprehensive medical
care to people with coagulation disorders. Each of these federally-funded hospitals is staffed by at least a
hematologist, nurse, social worker and physical therapist. These medical professionals work together as a team to
provide quality treatment, education and psychosocial support services for patients and their families.
To find the HTC located closest to you, click on the U.S. Centers for Disease Control and Prevention link below:
https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
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Listed below are direct links to several websites related to hemophilia.
American Thrombosis & Hemostasis Network (ATHN)
http://athn.org/
Bloodline
www.bloodline.net
Centers for Disease Control and Prevention (CDC), Hematologic Diseases Branch
http://www.cdc.gov/ncbddd/hbd/default.htm
The Coalition for Hemophilia B
www.coalitionforhemophiliab.org
Committee of Ten Thousand
www.cott1.org
Factor Foundation of America
www.factorfoundation.org
Hemophilia Alliance
www.hemoalliance.org
Hemophilia Emergency Care
www.hemophiliaemergencycare.com
Hemophilia Federation of America
www.hemophiliafed.org
Hemophilia Forum
www.hemophilia-forum.org
Hemophilia Region VI
www.hemophiliaregion6.org
Hemophilia Region IX
www.hemophiliaregion9.org
Inalex Communications
www.inalex.com
LA Kelley Communications
www.kelleycom.com
National Heart, Lung, and Blood Institute (NHLBI)
www.nhlbi.nih.gov
Patient Services Incorporated
www.uneedpsi.org
Plasma Protein Therapeutic Association
www.pptaglobal.org
www.donatingplasma.org
World Federation of Hemophilia
www.wfh.org
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Nosebleed basic information
Nosebleeds can be very common for children and adults who have a bleeding disorder. Often a person is diagnosed with their bleeding disorder because they are having
frequent, hard to stop nosebleeds.
Nosebleeds occur for different reasons. One reason is that an irritated area can occur
inside the nose. This area then has a hard time healing, especially with breathing dry
air, nose picking, etc. Trauma to the nose can also cause nosebleeds. Or a blood vessel can be close to the
surface and bleed easily from irritation.
There are many risk factors that will cause increased nosebleeds such as allergies, smoking, and infections,
in addition to dry air and trauma as mentioned earlier. Trying to eliminate any irritation to the lining of the nose
is your best plan.
Your Treatment Center will give you instructions on when to call them for nosebleeds. Generally, if the nosebleed
lasts for 20-30 minutes (despite applying continuous pressure), or if it occurs frequently (like 2-3 times per
day for several days in a row), you need to contact your hematologist or nurse.
Always have the child sit up and lean slightly forward during a nosebleed. This
keeps them from swallowing the blood. They should apply continuous pressure by
squeezing just below the bridge of the nose. Cold cloths can be put on the back
of the neck or bridge of the nose. If the person can hold ice in their mouth, this
will help (but the pressure needs to be continued on the outside of the nose at the
same time).
Some over-the-counter treatments are:
•
Afrin® or Neo-Synephrine® nasal spray - one spray into the nostril that’s bleeding
•
QR Powder- apply as directed
•
Use of an allergy medicine- e.g. Claritin®
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For prescription treatment options check with your Treatment Center.
Some preventative measures are:
•Apply olive oil to the openings of the nose each night and in the morning (use olive oil only, dip a QTip into the olive oil and apply)
•Keep air cool at night with a cool mist humidifier
•Increase fluid intake
•Sleep in a cool room
•Use a saline nasal spray 3-4 times a day
•Cover the nose when outside in the winter
•Do not take hot showers right after a nosebleed or after swimming
For more information, contact handi@hemophilia.org and ask for the Nosebleed Booklet available in English
and Spanish.
Reference: ‘Nosebleeds’, Gue D, Munn J, Purcell S, Zappa S, Copyright 2007, National Hemophilia Foundation 2
Orthopedic treatment options
Introduction
Orthopedic surgeons can offer a wide variety of treatment options for persons with hemophilia who have
degenerative joint disease. These treatment options can be everything from supportive bracing to total joint
replacement. These procedures should never be entered into without full discussion with your hemophilia care
provider.
Physical Therapy
The physical therapist is an important team member. This is the person on the team who measures you/your
child’s joints during clinic. They help to track any changes in range of motion (ROM) of the joints over time. They
are often able to pick up on early changes in muscles and joint function
and they help patients prepare for and recover following surgery.
Many joint bleeding episodes will need physical therapy treatment to
fully recover.
Bracing
Bracing can encompass a wide variety of things for the person with hemophilia. Braces can offer support,
decrease pain and sometimes improve range of motion in a joint. Bracing sometimes is used as an intermediate
option to put off surgery for a period of time. The orthopedist and physical therapist is familiar with what is
available and what might be of assistance.
Surgery
There are many potential benefits for orthopedic surgery in a person with hemophilia. Surgery can, but does
not always, decrease pain, increase range of motion and mobility, and improve quality of life. Hopefully the
surgical procedure will decrease the number of bleeding episodes into that joint.
Correcting the problem sometimes leads to less bleeding in other joints, also. For example, if a person with
hemophilia uses crutches frequently because of lower extremity bleeding they may start to have elbow bleeds.
So getting them off the crutches can save their elbows.
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Radionuclide Synovectomy:
This procedure is done as an outpatient and should not be done on just anyone with a target joint. It is
done at a limited number of hemophilia centers. A needle is inserted into the joint under local anesthesia,
any blood in the joint is removed and then a radioactive material, P-32 chromic phosphate, is injected.
This procedure is not very invasive and requires minimal factor replacement. The ideal candidate may be
someone who has failed conservative treatment with prophylaxis and physical therapy, a person with a
high titer inhibitor and who has recurrent joint bleeding with very little arthritic changes on X-ray.
Arthroscopic Synovectomy:
This procedure is done in the operating room. It may, but does not always, require overnight hospitalization.
Multiple small incisions are made in and around the joint and a tube called an arthroscope is inserted
through these incisions. The swollen synovium (lining of the joint) is then removed through the arthroscope.
This procedure is designed to decrease the frequency of joint bleeding. Each person is different, but most
patients benefit from a physical therapy program along with this surgery. Intense factor replacement is
required for a minimum of two weeks and often longer to support physical therapy.
Open Synovectomy:
This procedure is similar to the arthroscopic synovectomy but here the joint is opened and the synovium
is removed. This procedure allows the surgeon better visualization of the joint. It does, however, require
a larger incision. A person undergoing this type of procedure should expect at least one night in the
hospital and physical therapy is essential for the person to regain range of motion in the joint.
Fusion/Arthrodesis:
Fusion is most common in the ankle joints. It is done when there is “bone on bone” arthritic changes
that are causing the person with hemophilia enough pain to alter their life. This is still the gold standard
of treatment for advanced hemophilic arthropathy of the ankle. The goal of this procedure is pain relief.
Many patients are afraid that a fusion will decrease their activity, but in actuality the person is more
active because they are in less pain. This requires at least 1 night of hospitalization and at least 14 days
of factor replacement to assure adequate healing. The patient having this procedure is usually in a cast
for 6 weeks to 3 months and weight bearing on the ankle is limited for a period of time.
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Total Joint Replacement:
This is the treatment of choice for persons with hemophilia who have significant arthritis in a joint.
Total joint replacement is primarily done for the knee and hip in persons with hemophilia, but can be
done in the shoulder, elbow and ankle. The ends of the damaged bones are removed and replaced with
metal, ceramic and plastic parts. These parts do wear out and the average life of a total joint is about 15
years. This procedure can result in decreased pain and bleeding in the affected joint. It does not always
result in increased range of motion and physical therapy is essential for a good outcome. This requires
a hospitalization for several days and factor replacement for up to six weeks depending on therapy and
your HTC’s protocol.
Persons with hemophilia who do not have physical therapy in conjunction with this procedure often
experience a loss of motion in the joint.
Surgery in persons with hemophilia should never be taken lightly. There are multiple
risks of surgery including but not limited to:
•Bleeding
•Infection
•Pain
•Blood clots
A full preoperative evaluation needs to be done prior to any surgical procedure. Not only
is the person with hemophilia evaluated, but also the entire family and caregivers. This
is essential to a good outcome.
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Patient Notification System
The Patient Notification System is a free, confidential, 24 hour communication system providing information on
plasma-derived and recombinant analog therapy withdrawals and recalls.
The system was created to provide consumers with a single, convenient, and confidential source for up-todate withdrawal and recall information. Led by the Plasma Protein Therapeutics Association (PPTA), the Patient
Notification System was developed by the manufacturers of plasma therapies with direct input from consumers.
Everyone should register with the Patient Notification System so they can be directly notified when adverse
conditions occur with blood products.
The system is being administered by the National Notification Center. The identity of those individuals who have
registered is completely confidential.
Each person who registers with the Patient Notification System will select how they wish to be notified—
telephone, express delivery letter, fax or e-mail. All participants will then receive a follow-up first-class letter
back-up. Participants can also select which products they wish to be notified about.
The system is available by telephone for updates and news at 800-UPDATE-U. There is also a Web site at
www.patientnotificationsystem.org
Register now!
• By phone: 888-873-2838 (888-UPDATE-U)
• Online Enrollment: www.patientnotificationsystem.org
• Fax registration forms to: 1-800-442-2906
• Mail registration forms to:
National Notification Center
20 N. Meridian Street, Suite 300
Indianapolis, IN 46204.
Medications
Plasma safety
Because hemophilia treatment products sourced from human blood have been responsible for transmitting
blood-borne infectious agents (such as HIV and hepatitis) in the past, it is very important to ensure that
products being considered for use are safe and free from viral infection. Since the 1980s, manufacturers and
the agencies regulating the manufacture of fractionated plasma products have responded to concerns about
transmission of blood-borne viruses by developing a comprehensive set of measures designed to reduce, if not
eliminate, infectious risk. These measures are based on the following principles:
1) Selection of appropriate blood and plasma donors
2) Screening of the plasma raw material with laboratory tests
3) Elimination of any potential contaminating viruses through the manufacturing process
Of these three principles, the elimination of potential viruses through the manufacturing process has enhanced
the safety of hemophilia treatment products the most.
Measures to enhance the viral safety of plasma products include:
• Selection procedures which ensure that donors with high-risk behavior are excluded
• Mandatory serological testing on all plasma donations for HIV, hepatitis B, and hepatitis C
• Plasma inventory hold and exclusion based on post-donation information
• Nucleic acid testing (NAT) of minipools for HCV-RNA (and increasingly for other viruses • Testing starting-manufacturing plasma pool samples for viral markers and viral genomic material
• Inclusion of one or more validated specific viral inactivation and/or removal steps in the including HIV, HBV, B19, and HAV) and exclusion of reactive donations
manufacturing process
• Full traceability of plasma from donors to end products
In addition, some agencies and manufacturers also test finished products for viral markers and genomic
material.
The combination of appropriate donor selection procedures, screening with the current generation of standard
serological tests, and, in particular, the inclusion of measures to inactivate or remove potential viruses has
made fractionated plasma products free from serious known blood-borne viruses such as HIV, HBV and HCV. FlashLINK to Hemophilia
Role of Hemophilia Treatment Center (HTC)
Having a chronic disease means spending a lot of time and energy negotiating the healthcare system. The
hematologists, nurses, psychosocial professionals and physical therapists not only help consumers with their
medical care issues, but also lend tremendous emotional support. Many people with bleeding disorders use
the resources of their hemophilia treatment center (HTC) for many years because the staff understands their
unique needs.
Individuals who go to hemophilia treatment centers will not only find state-of-the art medical care, but also
benefit from an experienced, caring staff that takes time to develop comprehensive treatment care plans for
patients and families.
Hemophilia treatment centers are located in cities across the United States.
To find the one closest to you, go to the CDC website at https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
The treatment center not only provides specialty care but can also act as a resource to your regular family
physician or dentist.
Members of the care team at HTCs include:
• Hematologists—specialists in blood disorders.
• Pediatricians—specialists in caring for infants, young children, and teenagers.
• Nurses—medical specialists in hemophilia care. The nurse is probably the person you will see most frequently.
• Social Workers—specialists who assist you with the issues of daily living, such as adjusting to hemophilia
and locating resources (e.g., insurance, transportation,
housing, etc.).
• Physical therapists—specialists in activity, exercise, and rehabilitation.
• Orthopedists—specialists in disorders of the bones and joints.
• Dentists—specialists in disorders of the teeth and gums. The dentists at HTCs are experts in
treating children with oral bleeding problems.
• You are also an important member of the treatment team. The staff needs your input to develop a plan of care that will ensure you remain healthy, active, and able to live successfully with the added challenge of hemophilia.
Sample travel letter
(On HTC letterhead)
Please notify your HTC at least 2 weeks before travel to get your customized Travel Letter.
D
I
O
Patient Name:_____________________________________________________________________
DOB:____________________________________________________________________________
Factor Deficiency:______________________ Baseline level: ________________________________
____________________________ is a patient at _________________________________________
V
__________________________________________________ Hemophilia Treatment Center. He needs to carry his factor and infusion supplies with him in his carry-on bag in the event of injury or bleeding.
His current treatment plan is __________________________________________________ units/ kg of
_____________________________________________________________________ for prophylaxis.
Breakthrough bleeding should be treated as below:
He is treated on demand with ____________________________________ units/kg for minor bleeds and
________________________________ units/kg for major bleeds.
Please contact the HTC at ______________________________________ with any questions or concerns.
Self or home infusion
When your child is first diagnosed with a bleeding disorder, the idea of self/home infusion seems intimidating.
Mastering the skills required for home or self infusion will allow you to take control of treatment and have
better outcomes for bleeding episodes. When your child is young, successful home infusion may require
the placement of a central line (see Venous access section). As your child grows and learns more about his
bleeding disorder, the idea of self-infusion becomes achievable. This
process can not be learned overnight. Patience is necessary during
this process. Everyone has good days and bad days. Your treatment
center will guide you in this process and provide the resources and
support to ensure your success.
The first step in a child learning self infusion starts with the parents/caregiver mastering home infusion. It
is important to approach this with a confident attitude. Your child looks to you to help develop his view of the
world. If your child sees you confidently approach infusion, he will feel in control and will better handle the
situation. Infusion is a necessary part of treatment. It should never be portrayed as a punishment or an item
of negotiation.
If your child has severe hemophilia, home infusion may begin at about 1 year of age. Your child may need a
central line to begin this process. Whether your child requires a central line or you are trained on peripheral
infusion, this process may take several training sessions in order for you to become completely independent.
Your independence in this process is the first step in preparing your child for self-infusion.
Begin involving your child in the process of infusion as soon as he is developmentally ready. You may start
by having your child hold items until you are ready to use them. Another idea is to have your child verbalize
the steps and put him in charge of telling you the next step. As your child gets older, he can be taught to mix
the factor. These steps may add time to the infusion process but are a good investment toward your child’s
independence.
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If your child has a central line, you should begin learning how to infuse him using his peripheral veins at about
4-5 years of age. Around this age, children tend to get taller and thinner and it becomes easier to identify veins
in the arms and hands. The time required to learn this type of infusion varies greatly. Families that are most
successful know this is a process and there will be successes and failures along the way.
As your child enters grade school or middle school, he is mastering numerous developmental tasks necessary
to be an independent adult. Self-infusion is one of these necessary tasks. Hopefully, you have educated your
child along the way so that he can verbalize the steps required for infusion and mix his factor. At this point,
the only remaining step is learning to put the needle in the vein. If a child is fearful, numbing cream is often
helpful to make the poke painless. Allowing your child to practice on you may help make this a family event.
The confidence of a child, who can independently self-infuse, is amazing.
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Spontaneous bleeds
Persons with hemophilia may experience uncontrolled, often “spontaneous bleeding” or bleeding episodes without
obvious cause. Internal bleeding into the joints can result in pain, swelling and, if left untreated, can cause permanent
damage.
Sports ratings by risk
1
Safe
1.5
Safe to Moderate
2
Moderate
2.5
Mod.-Dangerous
3
Dangerous
Archery
Aquatics
Elliptical mach.
Stationery bike
Fishing
Frisbee
Golf
Hiking
Snorkeling
Swimming
Tai Chi
Walking
Bicycling
Rowing mach.
Treadmill
Circuit training
Body sculpting
Physio-ball
Spinning
Frisbee golf
Pilates
Wt. Lifting/
resist. training
Ski mach.
Aerobics
Bowling
Stepper
Dance
Diving
Cardio kick-boxing
Ultimate Frisbee
Jumping rope
Rock climbing (indoor)
Roller-skating
Rowing/crew
Running/jogging
Skiing-Cross Country
T-ball
Tennis
Yoga
Baseball
Basketball
Canoeing
Cheerleading
Gymnastics/
track & field
Horseback riding
Ice skating/
In-Line skating
Water skiing
Jet skiing
Kayaking
Karate/
Tae Kwon Do
Mountain biking
Racquetball
River rafting
Scooter (non-motorized)
Scuba diving
Skate-boarding
Skiing/downhill/
telemark
Surfing
Snowboarding
Soccer
Softball
Volleyball
BMX racing
Boxing
Diving/comp.
Football
Hockey-all
Lacrosse
Rodeo
Rugby
Motorcycling/motor
cross racing
Power Lifting
Rock climbing-natural
Scooter-motorized
Wrestling
**Trampoline is NOT recommended for ANY child ever.
References: Anderson, A, Forsyth, A Playing it Safe, NHF 2005 and the American Academy of Pediatrics.
Medications
Tranexamic acid
Tranexamic acid is an antifibrinolytic agent (that is, a medicine that slows the breakdown of blood clots). It can
help keep bleeding in the mouth and mucous membranes from starting up again, once it has stopped. It is often
used along with factor or DDAVP to treat bleeding with surgeries in the mouth and nose, dental procedures
and/or mouth injury.
• Cyklokapron® (tranexamic acid) tablets are currently not available for sale in the United States. They may be available through Canadian Pharmacy websites. Speak with your hematology team about how to obtain this medication.
• Lysteda® (tranexamic acid) tablets (650 mg) were approved by the FDA for sale in the United States in December 2009 to treat menorrhagia. This is the same agent, tranexamic acid, as contained in Cyklokapron® tablets.
• Cyklokapron® is available for use in the IV form. Your doctor might want to use this form of the medication when you are in the hospital.
• Bulk powder of the generic form of Cyklokapron® (tranexamic acid) is available in the US. Your specialty pharmacy may be able to obtain it for compounding into an oral liquid medicine for you.
• Cyklokapron® is usually given every 8 hours. It is important to take the medicine on a scheduled basis around the clock, as the doctor prescribes it.
• NEVER take Cyklokapron® (or give it to your child) for blood in the urine. Report any blood in the urine to your hematology team.
Special Instructions:
• Cyklokapron® can be taken with or without food. Give with food if you have an upset stomach after taking the medication.
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• Cyklokapron® is dosed by weight. The dose will change with weight gain or loss.
• If you miss a dose of the medication, take it as soon as you remember. Do not try to “make up” a missed dose.
• Do not use Cyklokapron® for kidney or bladder bleeding (blood in urine) unless directed by your doctor.
• Cyklokapron® should be used with caution by people with kidney or liver disease.
Side Effects (do not need to be reported):
Mild upset stomach
Mild diarrhea
Mild headache
Side effects that must be reported:
Skin rash
Chest pain
Slurred speech
Vomiting
Visual changes
Tell your doctor of other medications, vitamins, herbal products that you (your child) are taking
Keep the medication in its original container and store as instructed
Call 911 or go to the Emergency Room if you (your child) has:
Chest pain
Slurred speech
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Universal Data Collection or UDC
Today, clotting factor concentrates are considered safe from contamination with known viruses. However, continued
monitoring of blood products provides an additional measure to ensure their safety. With this in mind, the Centers
for Disease Control and Prevention (CDC), in cooperation with federally funded hemophilia treatment centers (HTCs),
established the Universal Data Collection Project (UDC) or “Study”.
Hemophilia Treatment Centers (HTCs) across the US are helping the Centers for Disease Control and Prevention (CDC)
to learn about how to prevent joint damage from hemophilia and also they are studying possible viral contamination
of factor products. Because hemophilia only affects a small number of people, by everyone sending the information
to the same place, this study will help doctors and nurses to find out what treatments are best.
How the UDC study works:
• Every year, at the Comprehensive Visit, you will be asked if you want to participate in the UDC
“study”. Someone who can answer your questions will ask you to read and sign a paper called an “Informed Consent”. That will give the HTC permission to ask the questions needed, collect the lab specimens needed and send all of it to the CDC in Atlanta
• The information that you give will go to the CDC with a number in place of your child’s name - the only thing that the CDC will know is what HTC you are attending
• You can refuse if you want- it is your right and nothing negative will happen
• The information is used to help the treatment and care of those with bleeding disorders
• When your child is less than 2 years old, only very basic information is asked
• When your child is more than two years old
o the physical therapist will measure his joints to see if there has been any change in how well he can bend or straighten each joint- this will take some time, but it doesn’t hurt at all
o questions will be asked about treatment and how many bleeds your child has had in 6 months
o blood will be drawn, the specimens are sent to the CDC for testing- Hepatitis A, B, C and
HIV- this is done for free
o some of the blood specimen is stored at the CDC, in case further testing is needed in the future
Your HTC will answer all your questions.
If you give permission for your child to be in the “study”, you will be given a copy of the informed consent that you
signed. This will give you more information and who to call if you ever have a question or concern about the “study”.
This paper will also help remind you of what we mean by the “UDC study” or the “CDC study”.
Venous access options
Venous Access Devices
All factor concentrates currently utilized to treat bleeding episodes require intravenous (in the vein) infusion.
When a bleeding episode occurs, an infusion of factor concentrate should be given within 2-4 hours. Your
hemophilia treatment center has a full understanding of the options available and you should contact them to
discuss which is best suited for your particular infusion needs. This section will provide an overview of current
options to prepare you for discussion with your HTC.
Metal (Butterfly) Needle
The Metal (Butterfly) Needle is a metal needle attached to tubing with an adapter for your factor syringe at the
end. There are two plastic wings that allow gripping the needle for insertion. These wings make the needle
resemble a butterfly. Typically butterfly needles used for
insertion range for 21 gauge (larger) to 27 gauge (smaller).
Once the needle is inserted and confirmed in the vein, factor
is pushed through the needle and when completed, the
needle is removed. This method is most often used by people
who self-infuse independently and is the easiest method for
venous access.
Temporary Infusion Devices
Temporary Infusion Devices allow access of a vein either for a single infusion or are placed for a few days
during a bleeding episode. These devices come in different sizes. It is important to know that the smaller the
diameter of the needle, the higher the size number.
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Plastic Needle
The Plastic Needle device may be used if venous access is required for a few days to treat a bleeding episode.
This device has a needle that is covered by a plastic tube. The needle/plastic tube is inserted into the vein
together and once placement in the vein is confirmed, the needle is removed,
leaving the plastic tube in the vein. This plastic tube has tubing attached and
an adaptor for the factor syringe. It is taped in place and can stay in the vein for
several days. This type of device has many different names and styles. Typically
this device is placed by a nurse, as it is more difficult to place. Removal is simple
and may be safely done at home.
PICC line (Peripherally Inserted Central Catheter)
The PICC is a hybrid device which is inserted in the vein in the arm like a temporary device but is long
enough to extend into the central circulation like a central line (see below). Because it extends to the central
circulation, it can last for several weeks. It is placed by a medical provider who is specially trained in this
insertion technique. Since this device extends to the central circulation system and is in place for an extended
period of time, the risk of complications is higher than temporary devices. This device requires special training
on infusion technique and dressing changes.
Central Lines
A central line is a device which requires surgical placement and removal. It can be in place for several
years. Like the PICC, the central line also extends to the central circulation system releasing the factor into
the large vein which enters the heart. As the heart pumps, it pushes factor through the body. Since the
tubing sits in the central circulation, the risk of complications is higher than temporary devices. The central
line requires special training on infusion technique and dressing changes if needed. The advantage of this
type of device is guaranteed venous access. The disadvantage is the risk of infection or other complications
related to the devices. The following are two different types of central lines:
2
Hickman or Broviac
Hickman and Broviac are common brand names for a particular type of central line that is partially
tunneled under the skin. One end of the tubing sits in the central circulation by the heart and the rest
of the tubing is tunneled under the skin then exits the chest wall. There are several inches of tubing
extending from the chest wall. The end of the tubing has an
adaptor for the factor syringe. Many people like this type of device because once in place, the factor syringe is attached to the adaptor for factor administration then removed without
causing pain to the child. The tubing exit site requires regular
dressing changes. The insertion and removal of this device is
also a little easier than the next device called an implantable
port.
Implantable Port
An implantable port (e.g. Port-a-cath®) is a reservoir with connected tubing. The tubing is threaded
into the central circulation and sites close to the heart. The reservoir is placed in a pocket between
the skin and a bone in the chest. When infusion is
needed, a special needle is inserted through the skin
and into the device to administer factor. Once the
infusion is completed, the needle can be removed.
Many like the ease of caring for
this device when not in use. The
only thing visible on the child is a
small bump under the skin.
The decision about what type of venous access is best for your child should be made after a full discussion
of the pros and cons of each type of device with your treatment center. Many children on regular infusions
require the placement of a central line as a young child, but then transition to a temporary device as they
become older.
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