Pregnancy in carriers of haemophilia

Abstract

The aim of the study was to review the complications, management and outcome of pregnancy in carriers of haemophilia over a 10-year period following the introduction of a multidisciplinary management guideline. Comparison was made to a 10-year cohort prior to implementation of the guidelines. A retrospective review of case notes of carriers of haemophilia (41 haemophilia A, 12 haemophilia B) who had received obstetric care at the Royal Free Hospital between 1995 and 2005 was conducted. There were 90 pregnancies (65 live births, 13 miscarriages, 12 terminations). Prenatal testing was taken up in 97% (63/65) of pregnancies where the mother was known to be a carrier of haemophilia. The majority (71%; 46/65) chose only to have non-invasive fetal sex determination. Seventeen (26%) had invasive testing (13 primarily for haemophilia and four primarily for chromosomal abnormalities). Termination of pregnancy was opted for in 67% (6/9) of pregnancies affected with haemophilia. Pregnancy was accompanied by a marked rise in factor VIII levels compared to only a small rise in factor IX levels. Invasive intrapartum monitoring techniques and instrumental deliveries were avoided in all pregnancies known to be at risk of haemophilia. Regional block was performed in 25 pregnancies for labour/delivery with no complications. The caesarean section rate was 47%. The incidence of primary and secondary postpartum haemorrhage was 19% and 2%, respectively. There were two neonatal head bleeding complications associated with prolonged labour or instrumental delivery. Availability of management guideline and care provided in a multidisciplinary approach can help to minimize bleeding complications in carriers of haemophilia and their newborns.

Nacionalna priporočila za obravnavo bolnikov s hemofilijo

“…Teh posegov se, če vemo, da ima plod hemofilijo ali pri sumu na hemofilijo pri plodu, med porodom izogibamo. Če pričakuje-mo zaplete med porodom, če porod traja dolgo ali če pride do nenapredovanja poroda, se odločimo za porod s carskim rezom (270)(271)(272).…”

Management of Inherited Bleeding Disorders in Pregnancy

“…8 The risk of spontaneous miscarriage is not increased in haemophilia carriers, but if it occurs, bleeding is more likely to be heavy. 11 Although coagulation factors may normalise during pregnancy, they do not usually reach the levels seen in the general pregnant population and it may be for this reason, or an early fall in levels postnatally, that carriers of haemophilia have an increased risk of postpartum haemorrhage (PPH).…”

“…Factor IX levels are relatively unaltered. 11 For carriers of haemophilia A, the pregnancy-induced rise in factor VIII level reduces the risk of bleeding although they remain vulnerable in early pregnancy and those with low baseline levels (for instance, less than 0.15 iu/ml) may not achieve normal levels by delivery. Women with low factor IX levels remain at risk of bleeding throughout pregnancy.…”

How I treat von Willebrand disease

“…The risk of bleeding is very small when FVIII:C and VWF:RCo levels are more than or equal to 50 IU/dL, which also is the minimum level that should be achieved for a safe spinal or epidural anesthesia. 66,67 In type 1 VWD pregnant women with FVIII:C levels lower than 50 IU/dL, desmopressin on the day of parturition and for a couple of days thereafter is advisable. To prevent late bleeding, VWF:RCo and FVIII:C levels should be tested, and the women should be monitored clinically for at least 2 weeks postpartum.…”