Precautions for pregnant women

It is very important that a woman with a bleeding disorder have confidence in a team of physicians to help her through her pregnancy. This team should include:

• an obstetrician (who specializes in caring for a woman during pregnancy and at delivery)
• a hematologist (who can make sure the woman's bleeding problem is under control)
• an anesthesiologist (who will be present at the birth and needs to know the special needs of a woman with a bleeding disorder).

Women with certain types of bleeding disorders may have more frequent miscarriages (spontaneous abortions), especially during the first trimester. These disorders are:

• Type 3 von Willebrand disease
• Factor I deficiency (afibrinogenemia)
• Factor X deficiency
• Factor XIII deficiency.

The risk of miscarriage can be lowered with factor replacement therapy.

Yes, plasma-derived factor concentrates can transmit parvovirus. This common virus is not normally dangerous to people. However, it can cause miscarriage (spontaneous abortion). Therefore, pregnant women, or women who might become pregnant, should avoid factor concentrates made from plasma, if possible. Alternatives are:

• desmopressin (although this makes the body retain water and, during pregnancy, could affect the cardiovascular system)
• anti-fibrinolytic agents (Cyklokapron and Amicar))
• recombinant (artificial) factor concentrates for deficiencies in Factor VII, VIII and IX.

No. In fact, for many women the opposite is true. They have less bleeding than they normally do. This is because high hormone levels during pregnancy stimulate the production of blood clotting proteins. As a result, levels of von Willebrand factor, factor VIII and other blood coagulation factors, except factor IX, rise closer to normal in women with bleeding disorders. Most women have few bleeding problems during pregnancy, or during childbirth.

Nevertheless, clotting levels should be monitored, especially as the date of delivery approaches. This way, doctors will know whether to prepare treatments. Some of the clotting factors needed by women with bleeding disorders are rarely used. The hospital blood bank will have to be alerted so that the factor concentrates are on hand when needed.

Desmopressin can also be prescribed to raise VWF and factor VIII levels during delivery.

If tests have shown that a woman is likely to suffer from bleeding during or after delivery, preventive treatments should be taken. These include:

• desmopressin
• anti-fibrinolytics (Cyklokapron and Amicar)
• VWF concentrates
• specific factor concentrates.

It should be assumed, unless prenatal testing has shown the opposite, that the fetus is also affected by a bleeding disorder. As a result, delivery should be as gentle as possible for both the woman and the baby. Natural delivery without the use of instruments is the goal for a woman with a bleeding disorder.

In order to prevent bleeding, the following should be avoided, when possible:

Women who breast feed keep the high hormone levels they had during pregnancy. This protects them from bleeding in the weeks following delivery (post partum). Women who do not breast feed see their hormone levels fall. This can lower the levels of clotting factors. They can have bleeding problems in the weeks after giving birth.

Women with certain types of bleeding disorders can have bleeding problems post partum whether or not they breast feed. These disorders are:

• Types 2 and 3 von Willebrand disease (the VWF does not work properly so larger amounts change little)
• Factor VII deficiency
• Hemophilia B (factor IX deficiency)
• Factor X deficiency
• Factor XIII deficiency.

All women should be watched carefully for bleeding in the hours, days and weeks following delivery. The following blood tests need to be done:

• bleeding time
• VWF and Factor VIII levels for VWD (or other factor levels for other factor deficiencies) hemoglobin (iron level in the blood).

Transfusions of factor concentrates, and even red blood cells, may be necessary. Babies with hemophilia A and B, von Willebrand disease and other factor deficiencies rarely bleed at birth.

Exceptions are:

It is recommended that the birth be as gentle as possible for the baby. In addition, circumcision is not recommended unless the baby is known not to have a bleeding disorder.

If parents suspect their baby might have a bleeding disorder, they can: