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Pregnancy in Women with Inherited Bleeding Disorders

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Pregnancy in Women with Inherited Bleeding Disorders TREATMENT OF HEMOPHILIA FEBRUARY 2003 • NO 29 PREGNANCY IN WOMEN WITH INHERITED BLEEDING DISORDERS Paul L.F. Giangrande Oxford Haemophilia Centre and Thrombosis Unit Churchill Hospital Oxford, U.K. Published by the World Federation of Hemophilia (WFH). © Copyright World Federation of Hemophilia, 2003 The WFH encourages redistribution of its publications for educational purposes by not-for-profit hemophilia organizations. In order to obtain permission to reprint, redistribute, or translate this publication, please contact the Communications Department at the address below. This publication is accessible from the World Federation of Hemophilia’s web site at www.wfh.org. Additional copies are also available from the WFH at: World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA Tel. : (514) 875-7944 Fax : (514) 875-8916 E-mail: [email protected] Internet: www.wfh.org The Treatment of Hemophilia series is intended to provide general information on the treatment and management of hemophilia. The World Federation of Hemophilia does not engage in the practice of medicine and under no circumstances recommends particular treatment for specific individuals. Dose schedules and other treatment regimes are continually revised and new side-effects recognized. WFH makes no representation, express or implied, that drug doses or other treatment recommendations in this publication are correct. For these reasons it is strongly recommended that individuals seek the advice of a medical adviser and/or to consult printed instructions provided by the pharmaceutical company before administering any of the drugs referred to in this monograph. Statements and opinions expressed here do not necessarily represent the opinions, policies, or recommendations of the World Federation of Hemophilia, its Executive Committee, or its staff. Treatment of Hemophilia Monographs Series Editor Dr. Sam Schulman Table of Contents Haemophilia ................................................................................................................................ 1 Carriers of haemophilia ............................................................................................................... 1 Carrier testing ................................................................................................................ 1 Antenatal diagnosis of haemophilia ............................................................................... 2 Management of pregnancy ............................................................................................. 3 Delivery ......................................................................................................................... 4 Summary ........................................................................................................................ 5 Von Willebrand disease .............................................................................................................. 5 Management of pregnancy ............................................................................................. 5 Delivery ......................................................................................................................... 6 Summary ........................................................................................................................ 6 Other congenital bleeding disorders ........................................................................................... 7 Factor I deficiency ......................................................................................................... 7 Factor XI deficiency ...................................................................................................... 7 Factor XIII deficiency .................................................................................................... 7 About the Author ........................................................................................................................ 7 References ................................................................................................................................... 8 Glossary ...................................................................................................................................... 9 Pregnancy in Women with Inherited Bleeding Disorders Paul L.F. Giangrande Haemophilia chance that a daughter will be a carrier. However, one third of cases arise in families Haemophilia A is a congenital disorder of with no previous family history, reflecting new coagulation, characterised by deficiency of mutations. The most famous example of this factor VIII in the blood. Deficiency of factor IX phenomenon was the British Queen Victoria results in an identical clinical condition known (1819-1901) who gave birth to a haemophilic as haemophilia B (also known as Christmas son, Leopold, in 1853. disease). Haemophilia is encountered in all racial groups, with an incidence of The severity of haemophilia within a given approximately 1 in 10,000 of the population. family remains constant. If a woman has The clinical picture is dependent upon the relatives with only very mild haemophilia, then degree of deficiency of the coagulation factor in she may be reassured that there is no risk of the blood: severe haemophilia is associated with passing on a severe form of the disease to her a level of less than 1% of normal. The hallmark children. of severe haemophilia is recurrent and spontaneous bleeding into joints, principally the Most female carriers of haemophilia have levels knees, elbows, and ankles. Repeated bleeding of factor VIII (or IX) within the normal range into joints can, in the absence of treatment, but a significant proportion have a modest result in disabling arthritis at an early age. reduction in the baseline level. The baseline Bleeding into muscles and soft tissues is also level is seldom lower than 20% of the normal seen frequently. Advances in the treatment of level and should therefore be enough to protect haemophilia have led to improvements in both against significant bleeding problems in day-to- the longevity and quality of life of patients with day life. However, female carriers with these even severe haemophilia. It is anticipated that low levels of factor VIII (or IX) are at risk of the number of patients with severe haemophilia excessive bleeding from surgery or other will increase significantly in developed invasive procedures, such as dental extractions, countries, because as people with haemophilia biopsies, etc. In such circumstances, haemostatic live longer and integrate fully into society, it is support with desmopressin (DDAVP) or more likely that they will raise families and pass coagulation factor concentrates may be required the haemophilia gene on to their daughters, who and the choice of product will depend on both will be carriers. Obstetricians will thus be faced the factor level and the nature of the procedure. more frequently with managing pregnancies in Recombinant coagulation factor concentrates known or possible carriers of haemophilia. should be considered to be the products of choice in such cases because of the potential for transmission of parvovirus B19 with plasma- Carriers of haemophilia derived concentrates. The genes for both factor VIII and IX are located on the X chromosome and thus the Carrier testing inheritance of haemophilia is sex-linked and There is no need to carry out special genetic recessive, like colour blindness. The daughters tests in daughters of men with haemophilia to of men with haemophilia are obligate carriers of determine their carrier status as they are obligate the condition, and carriers have a 50:50 chance carriers. However, genotypic analysis to of passing on the condition to a son and a 50:50 determine the underlying genetic defect should 2 Pregnancy in Women with Inherited Bleeding Disorders be offered as this will facilitate antenatal testing may reflect the fact that many women with (see below) in due course if required. affected relatives recognise the tremendous advances in treatment in recent years, including The carrier status for other women in an the wider adoption of prophylaxis and the extended family may not be so clear. For introduction of recombinant products, which example, a woman who has an affected uncle have resulted in an essentially normal life for the may or may not be a carrier. A pregnant woman younger generation of people with haemophilia. with a vague history of a bleeding disorder in a distant relative presents a difficult and all too Chorion villus sampling (CVS), or biopsy, is common problem for obstetricians. Carrier the principal method used for antenatal testing can take some months to perform and it diagnosis of haemophilia. It offers the major may therefore seem logical to start carrier testing advantage over amniocentesis of permitting as soon as possible in girls with a family history diagnosis during the first trimester, although it of haemophilia, as this would help the should not be carried out before 11 weeks of management of pregnancy in the case of an early gestation as earlier biopsy may be associated and unexpected pregnancy. However, in with a risk of subsequent fetal limb countries where the ethical and/or legal rights of abnormalities (Firth HV et al., 1991 and 1994). children are protected, testing young children is A sample of chorionic villus is obtained by considered a breach of their rights as they are either the transabdominal or transvaginal
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