What are the pregnancy complications for Hemophilia A?

Question

Hello doctor,

My younger sister, 29 years old, has been diagnosed as an asymptomatic carrier of hemophilia A. She experienced severe postpartum bleeding after the birth of her first child, which led to this diagnosis.

She is currently planning for her second pregnancy, but we are anxious about potential complications. Her menstrual periods have always been heavier than normal, and in recent months, she has also been experiencing painful cramps and irregular cycles.

We are especially concerned about the risk of passing the condition to her child.

Is it possible to do genetic screening or opt for in vitro fertilization (IVF) with preimplantation genetic testing and embryo selection?
Whether a planned Cesarean section would be safer, or if vaginal delivery poses less bleeding risk when managed properly with a hematology and obstetrics team?

Kindly help.

Answer 1

Hello,

Welcome to icliniq.com.

I have read your query and can understand your concern.

Given your sister’s diagnosis as an asymptomatic carrier of Hemophilia A, with a history of severe postpartum bleeding, it is very wise to plan ahead before pursuing a second pregnancy. Symptomatic carriers can have a deficiency of Factor VIII themselves, which increases the risk of excessive bleeding during pregnancy, delivery, and the postpartum period.

Before conception, she should undergo baseline Factor VIII level testing. These levels should also be checked again during each trimester, as they may fluctuate throughout pregnancy. Ideally, her care should be coordinated by a multidisciplinary team that includes a hematologist, a high-risk obstetrician, and an anesthesiologist.

Regarding the baby: preimplantation genetic testing (PGT) through in vitro fertilization (IVF) is a very viable option. PGT can screen embryos and assist in selecting those that are not affected by Hemophilia A, which can prevent transmission of the condition. If she conceives naturally, chorionic villus sampling (CVS) or amniocentesis can help diagnose the fetus’s status. However, both of these procedures are invasive and carry a slight risk of miscarriage.

As for delivery, a planned vaginal birth is generally considered safe if maternal Factor VIII levels are optimized and bleeding is well-managed. However, in certain situations, especially if the fetus is male and affected by Hemophilia A, a planned Cesarean section may be safer. The mode of delivery should be determined on a case-by-case basis, taking into account maternal clotting factor levels, fetal status, and bleeding risk.

To reduce bleeding risk, medications such as Tranexamic acid (an antifibrinolytic agent), desmopressin (DDAVP) (a synthetic antidiuretic hormone), or factor VIII replacement therapy may be required before and after delivery.

Lastly, regional anesthesia (such as an epidural) should be avoided unless factor VIII levels are confirmed to be within the normal range, due to the potential risk of spinal bleeding.

I hope this helps.