| Q: | What is a venous access device (port-a-cath)? |
| A: |
A port-a-cath, or
implantable venous access device (IVAD), is implanted under the skin, usually
in the upper chest but there are models which can be inserted into the arm. It
has a small metal reservoir with a rubber diaphragm which is connected to a
catheter which is then threaded into a large vein in the chest or arm.
The entire device is surgically implanted under the skin so there is no
catheter that hangs out of the body. The device provides ready access to a vein
for administering medications and fluids intravenously. It can also be used for
drawing blood samples. The device is accessed
by inserting a special needle through the skin and into the rubber diaphragm of
the reservoir. The medication or fluid is injected into the device and it flows
through the catheter into the vein. These devices have made
prophylaxis in hemophilia much easier for families because the problems of
"finding a vein" for infusion two to three times a week are
eliminated. However, there are risks involved with their use, the most
worrisome being that of infection. Studies differ but some show an infection
rate as high as 50 percent. These infections can usually be treated with
intravenous antibiotics but sometimes the device must be removed. Also, there
are other studies that show a risk of clots forming at the tip of the catheter.
Still, many families have chosen to use the device in spite of the risk because
of the benefits. Like any other procedure, one must weigh the risks and
benefits. |
| Q: | What is the life expectancy of a person with Hemophilia? |
| A: |
The life expectancy of someone with hemophilia varies depending on whether they receive proper treatment. Without adequate treatment, many people with hemophilia die before they reach adulthood. However, with proper treatment, life expectancy for
people with hemophilia is about 10 years less than that of males without
hemophilia, and children can look forward to a normal life expectancy. |
| Q: | Are there any precautions a carrier should take if she becomes pregnant? |
| A: |
It is important that a carriers hematologist is involved in the supervision of the pregnancy and that there is some liaison with the obstetrician before delivery. It is not necessary to perform prenatal diagnosis just for management of the pregnancy. This is only done if termination of a pregnancy is being considered. The factor VIII level (but not factor IX) tends to rise during pregnancy but it should bechecked sometime in the last couple of months of pregnancy. A normal vaginal delivery is perfectly acceptable even if the fetus is known to be male and at risk of hemophilia. Epidural anesthesia does not usually present a problem and is generally permissible if the patients factor level is 40 percent or more. A cord blood sample after delivery will be used to check if a male baby has hemophilia. |




