Labor and delivery in women with hemophilia
Planning for childbirth depends on the needs of the mother and the child affected by hemophilia. Levels of clotting factors should be measured in the last trimester of pregnancy, since it is difficult to measure during labor. If levels are low, treatment may be given during labor to reduce the risk of excess bleeding during and after childbirth. Clotting factor levels may also determine whether a woman can receive local anesthesia.
Male babes with hemophilia present a higher risk of head bleeding, especially if the delivery is prolonged or complicated. Carrier women may give birth vaginally, but prolonged labor should be avoided and delivery should occur in the least traumatic way possible. Such women should also avoid invasive monitoring techniques such as fetal scalp electrodes and fetal blood sampling, as well as delivery by vacuum extraction and forceps.
Soon after delivery, a blood sample from the umbilical cord should be collected to measure clotting factor levels. Doctors should avoid injections into the baby’s muscle tissue and surgical procedures such as circumcision until the results of blood tests are known.
Post-natal maternal care
Levels of blood clotting factors go back to their pre-pregnancy level after delivery, and the chance of bleeding is at its highest during this time. Excessive bleeding following delivery is called postpartum hemorrhage (PPH), which is a major cause of maternal death and disability in some parts of the world. Therefore, hemophilia carriers — particularly symptomatic carriers and women with hemophilia — should be cared for in an obstetric unit working closely with the hemophilia team.
Women should take certain precautions to reduce their risk of PPH. For example, doctors can give medications that keep the womb contracted, and can deliver the placenta by controlled traction of the umbilical cord. This is called “active management” for placenta delivery and has been shown to significantly reduce the risk of PPH.
Carriers are at risk of PPH for up to six weeks after childbirth and should see their doctor immediately if bleeding is excessive during this period. Treatment may be recommended as a preventive measure, especially in carriers with low levels of clotting factor.
Women who breastfeed maintain higher levels of clotting factors, meaning that carrier women are less likely to bleed if they breastfeed. However, women with mutations in genes encoding for clotting factors VII, IX, X, and XIII may have bleeding problems, whether or not they breastfeed.
Post-natal child care
Testing for Hemophilia
Some babies should be tested for hemophilia soon after birth, including:
- Babies born to families with a history of hemophilia
- Babies whose mothers are carriers of hemophilia
- Babies who have bleeding symptoms at birth
Cord blood may be used to test for clotting proteins. This should be repeated at six months of age to confirm a hemophilia diagnosis.
Some parents choose to have their baby boys circumcised, which involves removing the foreskin from the penis. Bleeding from circumcision is the most common cause of bleeding among babies with hemophilia. Bleeding may continue for days after the procedure is done; sometimes, this is how hemophilia is first diagnosed. Babies with hemophilia should not be circumcised. However, if circumcision is done, consult a pediatric hematologist — a doctor who specializes in blood disorders — before the procedure to ensure that the boy gets proper treatment to prevent excessive bleeding.
Bleeding of the head
The head is the second most common place of bleeding among babies with hemophilia, since it is squeezed when the baby goes through the birth canal. Increased bleeding from the head also occurs if a doctor applies forceps or a vacuum extractor to the baby’s head to assist with the delivery. Bleeding can be in the scalp or into the brain, which is very serious. The signs and symptoms of bleeding into the brain in a newborn baby are not specific and can be difficult to diagnose. Once it is diagnosed, bleeding in the head must be treated immediately with clotting factor concentrates. Without treatment, bleeding may result in long-term brain damage and could be life-threatening.
All newborns, including those with hemophilia, should get a vitamin K shot at birth, as well as other routine vaccines. Vitamin K may be given by mouth instead of an injection to prevent bleeding. Like all newborns, those with hemophilia should be vaccinated against hepatitis A and B. Pressure must be applied to the site of the shot, as well as to the site of heel pricks, to prevent bleeding.
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