Study: 4 of 5 hemophilia centers in Italy lack pregnancy protocols
Findings lead to effort to draft guidelines for use at treatment centers
Special care is needed to manage pregnancy and childbirth for women who have or are carriers of hemophilia, yet many hemophilia treatment centers in Italy don’t have a specific protocol in place for this type of management.
Indeed, fewer than 1 in 5 centers in the European nation were found to have a treatment protocol in place for pregnant women with hemophilia or who are carriers of the bleeding disorder.
That’s according to a new study from researchers in the country that aimed to “share real-world clinical management in Italian haemophilia treatment centres.” Given these findings, its researchers are already working to draw up general guidelines that can be widely used at centers across Italy.
“Multidisciplinary pregnancy management is recommended,” the team wrote. “Delivery mode choice is primarily driven by ensuring the newborn’s maximum protection.”
The study, “Management of Pregnancy, Delivery, and Postpartum in Italian Carriers and Women With Haemophilia A and B,” was published in the journal Haemophilia.
Hemophilia A and B are genetic disorders caused by mutations in genes that are located on the X chromosome, one of the two sex-determining chromosomes. Because biological females have two X chromosomes, most women who carry a hemophilia-causing mutation are carriers — meaning they generally won’t experience symptoms, but may still pass the mutation to their biological children. There also are some women who have overt hemophilia.
Management of pregnancy and hemophilia is complicated
Management of pregnancy in women who have hemophilia or are carriers for the disease is complex, both because there is an elevated risk of bleeding from the mother and also because there’s a high probability that the baby will have hemophilia, especially if it’s a boy. Guidelines from the World Federation of Hemophilia (WFH) suggest that specialist care should be given to any pregnant woman with a family history of hemophilia.
Here, researchers conducted a survey of hemophilia treatment centers in Italy to evaluate how these pregnancies are managed in the real world. A total of 33 centers responded.
It is recommended that pregnancies in women who have or are carriers of hemophilia be managed with a multidisciplinary team of specialists. About half of the centers reported regularly using multidisciplinary care. More than half of centers said they rarely or never offer counseling for patients to discuss how bleeding during pregnancy or childbirth will be handled.
Additionally, at least 4 of every 5 centers lacked a specific protocol for handling pregnancies in women who have hemophilia or are carriers. Based on this finding, the researchers began working on general guidelines that could be implemented at hemophilia treatment centers across the country to use with pregnant women.
Most centers with guidelines recommend cesarean delivery
Most centers reported that decisions on how to deliver babies were made on a case-by-case basis after discussions between the pregnant woman and her care team.
Some centers specifically recommended either spontaneous labor or a planned cesarean section. Where there were specific recommendations in place, cesarean section was generally preferred if it was unknown whether the baby had hemophilia. If the baby’s health status was known, there was a more even split.
“All centres agree to avoid instrumental delivery in affected male [fetus] or when prenatal diagnosis has not been made,” the researchers wrote, noting that the question of how best to deliver babies that might have hemophilia is still an open debate in healthcare and subject to further studies.
All in all, this survey [study] emphasizes the importance of a protocol for the management of congenital [bleeding] diseases in pregnancy to share with all clinicians involved in childbirth.
Hemophilia is caused by a deficiency in clotting factor proteins, specifically factor VIII in hemophilia A and factor IX in hemophilia B. All centers said they ran tests to assess the levels of the relevant clotting factors at some point during pregnancy, and if treatment was needed to manage bleeding, the vast majority of centers recommended using recombinant, or man-made, versions of these proteins.
“All in all, this survey emphasizes the importance of a protocol for the management of congenital [bleeding] diseases in pregnancy to share with all clinicians involved in childbirth,” the researchers concluded.
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