Women with hbd

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Women with hbd

  1. 1. Health Problems in Women withHereditary Bleeding Disorders Magdy El Ekiaby, MD Shabrawishi HTC, Egypt
  2. 2. Hereditary Bleeding Disorders in Women Hereditary platelet vWF vWF F II F VIII disorders TFPI F Xa F VIIIa F IIa F Xa TF F VIIa F Va F XI vWD (1%) Platelet F V TF bearing cell FV F Va F XIa Hemophilia A & B TF F VIIa F X carriers (<1/10,000) F IX F IXa F Xa F VIIIa F XIa F Va Coagulation factor Activated platelet F II F IIa deficiency I – XIII F IIa F IIa (1/0.5 – 2 million) F IIa F IIa F IIa F IIa F IIa F IIa F IIa
  3. 3. Inheritance Hereditary bleeding diseases are inherited as recessive characters Women with heterozygote bleeding disorders usually are asymptomatic, or experience mild bleeding problems In rare cases, there might be compounded heterozygote inheritance of bleeding disorders in which case bleeding symptoms may be severe Women with homozygote inheritance pattern suffer from severe bleeding symptoms, particularly those related to their reproductive system, eg menstrual
  4. 4. Reproductive Health Problems Menorrhagea Dysmenorrhea Hemorrhagic ovarian cyst Pregnancy and delivery Impact of bleeding disorders on women
  5. 5. Menstrual Cycle Normal menstrual cycle should be less than 7 days long during which blood loss does not exceed 80 ml* Women of modern societies may experience up to 500 cycles during their fertile age Chronic disturbance of menses may have its impact on the health, psychological and social life of the women Every effort should be done to identify menorrhagea, its probable causes, so that it can be properly managed. *ACOG, Management of anovulatory bleeding 2000, p.1-12
  6. 6. Menorrhagea It is defined as prolonged menstruation (>7 days) and/or excessive blood loss (>80 ml) Excessive passage of blood clots, pain and flooding are also common features of menorrhagea* Adolescent girls and perimenopausal women may suffer most, as the menstrual cycles are usually anovulatory *Kadir et al, Haemophilia 2009
  7. 7. Hemorrhagic Ovarian Cysts In normal women, minor bleeding may occur with rupture of the graafian follicle Women with HBD are more likely to have more significant bleeding at ovulation in the form of:  Hemorrhagic ovarian cyst  Broad ligament hematoma  Hemoperitoneum Prevalence of hemorrhagic ovarian cysts in women with HBD ranges from 2 – 25%* *James, Hemophilia 2005
  8. 8. Clinical Assessment of Menorrhagea Iron deficiency anemia in women complaining of menorrhagea is an indication of excessive blood loss during menstruation Iron deficiency anemia will not manifest for a long time, while menorrhagea may be existing Semi-quantitative assessment of menorrhagea can be done using Pectorial Blood Assessment Chart (PBAC)* *Higham et al, Br J Obstet Gynaecol 1990
  9. 9. Assessment of menstrual blood loss using the pictorial blood assessment chart (PBAC) Scoring System SCORE:>100 = >80 ml blood loss
  10. 10. Prevalence of Menorrhagea in Women with HBDDisease Prevalence ReferencevWD 74-92% Kouides et al, Hemophilia, 2000 Ragni et al, Hemophilia, 1999Berbnard-Soulier 51% Lopez et al, Blood 1998Glanzmann Thromb. 98% George et al, Blood 1990FXI deficiency 59% Kadir et al, American J of Hematology 1999Hemophilia Carrier 57% Kadir et al, Hemophilia 1999RBD 35 – 70% James, Hemophilia 2005
  11. 11. Prevalence of HBD in Women with Menorrhagea Disease Prevalence reference vWD 13% Shankar et al, Bjog 2004 Platelet dysfunction Limited data, up to 47% Philipp et al, J Thromb & Haemost 2003 RBD Limited dataMenorrhagea should alert clinicians about possibility of anexisting bleeding disorder
  12. 12. When do we suspect HBD as a cause of Menorrhagea? Menorrhagea since Menarch Recurrent midcycle pain due to ovulation bleeding Family History of a bleeding disorder Personal history of one or more of the bleeding symptoms such as epistaxis, notable bruisis, mucocutaneous bleeding, postoperative and postpartum bleeding Failure to respond to conventional management of menorrhagea
  13. 13. HEMATOLOGICAL WORK UP
  14. 14. Management of Menorrhagea
  15. 15. Hormonal Therapy Levonorgestrel IUS (Mirena®) is the most effective medical treatment of menorrhagea, and is useful in women with HBD as well as a reversable contraceptive tool It is implanted for 3-5 years It reduces endometrial proliferation and reduces blood loss during menstruation
  16. 16. Hormonal Therapy Combined hormonal therapy to reduce blood loss by thinning the endometrium and probably increasing FVIII & vWF levels, such as contraceptive pills, transdermal contraceptive patches & vaginal rings Oral progestogens Gonadotropin-releasing hormone (GnRH) analogues (stop ovulation)
  17. 17. Hemostatic Therapy Anti-fibrinolytics DDAVP Specific clotting factor concentrates Blood component transfusion
  18. 18. Anti-fibrinolytic Agents Tanexamic acid and Epsilon Amino Caproic Acid (EACA), are known to have hemostatic effect and may help to control menorrhagea Oral Tranexamic acid (1g, 3 – 4 times daily) during menstrual period is known to reduce menorrhagea Usually it is well tolerated but side effects include nausea, vomiting, headache and diarrhea
  19. 19. DDAVP DDAVP (1-desamino-8-D-vasopressin), a synthetic vasopressin that stimulates release of vWF and FVIII from their endothelial stores It has a formulation for treatment of nocturnal enuresis and a hemostatic form The hemostatic form comes as a nasal spray and ampoules which are administered subcutaneously or by iv infusion For management of menorrhagea a daily dose of 150 – 300 micro-gram for a maximum of 3-4 days during heaviest days of the cycle
  20. 20. DDAVP It is mainly effective in Hemophilia A carriers, vWD (except type 2B) and to some extent in Glanzmann thrombasthenia and Bernard-Soulier Syndrome Side effects include tachycardia, flushing, and headache Small risk of hyponatremia and water intoxication with repeated doses, so better advise water intake restriction during therapy
  21. 21. Acute Adolescent Menorrhagea Usually achieved by a combination of hemostatic agents and high doses of hormonal therapy In severe cases specific factor concentrates and intravenous anti-fibrinolytics may help rFVIIa is successful in patients with severe platelet dysfunction Platelet transfusion (HLA matched whenever possible) may be required in cases of severe thrombocytopenia and thrombasthenia
  22. 22. Counselling Issues of counselling include:  Marriage  Preconception
  23. 23. Marriage Women with bleeding disorders usually have social and psychological problems connected with marriage Couples & their relatives usually have questions about sexual life, hymen defloration, risks of pregnancy and delivery as well as disease inheritance
  24. 24. Preconception Advantages:  Provides adequate information on the genetic implications of their disorders, the available reproductive choices, and options for prenatal diagnosis  Allows planning for pregnancy and establishing how and where pregnancy can be best managed  Immunization against HBV & HAV for those likely to receive blood transfusion  DDAVP test dose to assess response  General hematenic supplmentation
  25. 25. Prenatal Diagnosis (PND) Particularly important in hemophilia carrier due to the severity of the disease in the male offspring It is also of importance in heterozygote carriers in cases of consanguineous marriage PND diagnostic methods include:  Chorion Villus Sampling (CVS), weeks 11-14 1% risk of  Amniocentesis, weeks 15-20 abortion  Cordocentesis, weeks 18-20  Fetal sex determination  Pre-implantation diagnosis
  26. 26. Antenatal management Increased levels of vWF, FVIII, Fibrinogen as well as plasminogen activator inhibitors, particularly during third trimester of pregnancy, reduces bleeding episodes and complications in women with HBDs (Bremme, Best Pract Res Clin Hematol, 2003) 20% of normal pregnancies may wetness a bleeding episode due to obstetric problems, which should not be overlooked in women with HBDs Women with FI & FXIII deficiency are particularly at risk of miscarriage, placental abruption and preterm delivery which may require factor replacement during pregnancy (Chi & Kadir,
  27. 27. Labour & delivery Third trimester coagulation factor levels should be known before delivery and factor replacement may be considered in those with low levels DDAVP may be used in hemophilia carriers and vWD during delivery with caution Matched packed RBCs should be reserved for emergency bleeding Cesarean section as well as other obstetrical procedures should be evaluated for least traumatic manipulation of the fetus Cord fetal blood sample to assess if the baby has inherited the bleeding disorder
  28. 28. Postpartum management Elevated coagulation and antifibrinolytic levels during pregnancy, slowly return to its low levels in the postpartum period It is recommended to use prophylaxis of deficient hemostatic agent immediately before delivery as well as for 3-4 days after normal vaginal delivery and 5-7 days after cesarean section 14-21 days postpartum a secondary hemorrhage can be expected
  29. 29. Hemostatic materials
  30. 30. Suggested hemostatic levels
  31. 31. Quality of life Women with HBD suffer many bleeding problems during their fertile life Many of these women experience anemia, fatigue which affects their marital, familial, social and practical life They are at a higher risk of postpartum hemorrhage, which may lead to severe morbidity and risk of mortality
  32. 32. Conclusion The prevalence of HBD is not insignificant Women suffering from menorrhagea, particularly since adolescence should be investigated to exclude HBD Women with HBD should receive care for their reproductive life in specialized centers, or at least under common supervision from an obstetrician and a hematologist Proper management of women with HBD can greatly improve the quality of their lives

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