The need for good venous access is critical. Inhibitor patients may need frequent doses of bypassing agents to control bleeding. Those undergoing ITT need frequent needle sticks in order to deliver the factor. While peripheral vein infusion is the preferred method because it offers a potential reduction in infection risk and clots, it is often necessary for patients to look at other options because veins can be difficult to access or the frequency of dosing would be too difficult on peripheral veins. It is often necessary for patients to look at other venous access options.
Central Venous Access Devices (CVAD)
Central Venous Access Devices (CVADs) can be very helpful for patients (particularly children) who need repeated or urgent doses of factor. Many parents choose to have a CVAD placed in their child with a bleeding disorder simply for the freedom of being able to treat when needed at home rather than relying on a medical professional for each infusion. It does require a surgical procedure to place, and ultimately remove, a CVAD. There are some risks associated with CVADs: pneumothorax, hemothorax, bleeding, pain, infection.
Typically in hemophilia, a port is the preferred CVAD device. A port is a small disc shaped medical appliance inserted below the skin (most often in the chest area). It consists of a portal/reservoir compartment that is topped by a silicone bubble for needle insertion. This connects on the back side to a septum/plastic tubing. The septum in threaded surgically into a vein.
Patients and caregivers can be taught how to infuse and take care of a port at home. Ports can last for months to years. Patients can bathe and swim with a port.
Peripherally Inserted Central Catheter (PICC Line)
A peripherally inserted central catheter (PICC line) is typically inserted in the upper arm and is a long, slender, small and flexible tube. The tip of the catheter ends in a large vein in the upper chest. The end of the PICC line is external, meaning a small portion of the line hangs from the point of insertion on the arm. Patients and caregivers infuse through this point.
PICC lines are often a temporary solution for infusion necessity. They are helpful because each infusion does not require a needle stick, but bathing with a PICC line can be challenging and swimming is not allowed.
Tunneled Central Venous Access (Hickman or Broviac lines)
Hickman or Broviac lines are thin, long tubes made of silicone. A surgeon places or tunnels the line into one of the central veins that leads to the heart. The line hangs externally, (like a PICC line) and patients and/or caregivers can infuse through this point rather than a needle poke for each infusion. These lines can be more prone to infection because they are external and many parents report that bathing can be challenging.
Arterio-Venous Fistula (AVF)
A fistula is defined as an abnormal opening between body parts. In the case of an arterio-venous fistula (AVF), a surgeon creates a passageway or merge between an artery and vein, thereby allowing for an easier target vein to use for access. This most often is done in an arm or leg. This surgical procedure can allow for peripheral access of the AVF, but it takes time for the surgical site to heal and mature to a point of use. Advantages of an AVF include: can last for years, durable venous access, low risk of infection. Disadvantages include: visibility of AVF, maturation time after surgery, thrombosis and stenosis.
The physical toll on inhibitor patients can be very difficult due to the frequent bleeding episodes. Mobility can be limited and the use of assistive devices (wheelchairs, crutches, canes) may be needed. Many inhibitor patients develop joint damage. Pain management can be difficult. Surgical options do exist for inhibitor patients to relieve some orthopedic challenges. Inhibitor patients are encouraged to work closely with their HTC and physical therapist to determine what exercise and treatment options are best for them. Learn more about joint damage.
The costs associated with treating a person with inhibitors can be staggering because of the cost and amount of treatment needed to stop bleeding. In addition, people with inhibitors are more likely to be hospitalized due to a bleeding complication. Learn more about financial considerations.
The effects of living with an inhibitor go beyond the physical and financial challenges. It takes an inordinate amount of time and effort for patients and caregivers to treat bleeds and the inhibitor. Frequent visits to the HTC, as well as emergency room and in-patient hospitalizations, can mean significant absences for school age children, and can create hardships in terms of employment. Pain issues can lead to feelings of anger, frustration and helplessness. Depression is one of the most common complications of chronic illness, and having an inhibitor can magnify some of those feelings. Limitations to mobility and independence can undermine confidence. Missing out on some of the everyday activities that others enjoy can lead to feelings of isolation. Families often have to dedicate large amounts of time to the patient and family relationships (siblings and spousal) can suffer. Both patients and caregivers report than interpersonal relationships are the best coping mechanism for the psycho-social challenges faced by living with an inhibitor. Learn more about the emotional and psychological challenges one faces living with a bleeding disorder.
For more information on how to advocate for needs in the school setting, including an example of an Individual Education Plan (IEP) for an inhibitor student, please visit the HFA Back to School Toolkit. The HFA Be Prepared Toolkit provides resources that can help make trips to the emergency room a bit easier.
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