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Von Willebrand's Disease

Von Willebrand's Disease Posted by Nibedita R.
A 30 year old woman with von willebrand?s disease is referred for antenatal care at 12 weeks gestation. Justify your antenatal, intrapartum and postnatal care.

Von willebrand?s disease is the most common inherited bleeding disorder. Severity of bleeding symptoms depends on the subtype of the disease. Type 1 is the predominant type (70%) and mild form with autosomal dominant pattern of inheritance. As she is affected, chances would be that 50% male and 50% female fetus would be affected.
The disease does not adversely affect pregnancy and disease symptoms generally improve during pregnancy due to increase vwf and factor 8 concentration. Greatest risk of haemorrhage is during puerperium when these factors falls rapidly.

Assessment of the severity of symptoms from history such as epistaxis, gum bleeding, past history of menorrhagia, GI bleeding, bleeding tendency to minimum trauma (tooth extraction) and history of heavy bleeding after childbirth.
Depending on the severity of the symptoms, pregnancy should be managed by multidisciplinary team consisting of senior obstetrician, consultant haematologist, neonatologist, experienced midwife and health care professionals. Her symptoms and psychosocial problems should be dealt with by shared care between haemophilia centre and comprehensive care centre.

She should be provided adequate information and formally educated regarding the pattern of inheritance. Prenatal diagnosis should be offered by CVS / amniocentesis for DNA testing by PCR. CVS can provide early result within 48 hours of an affected fetus. She should be counselled that the procedure carries a risk of miscarriage of about 1-2% and also risk of haemorrhage from the puncture site.
Parental attitude towards an affected fetus should be explored and termination of pregnancy of pregnancy would be offered, but should be informed that the procedure carries risk of haemorrhage.

Investigations including FBC, vwf concentration, factor 8 levels and bleeding time should be checked at booking visit and should be repeated as and when required.

If she gives a history of tranfusion of plasma derived products before the year 1986 she should be offered serological testing of Hepatitis B, Hepatitis C and HIV.

Pregnancy should be monitored carefully with factor 8 and vwf concentrations and bleeding time and additional risk factors like multiple pregnancy, polyhydramnios, placenta previa should be identified beforehand which would increase her risk of haemorrhage.
Timing and mode of delivery should be determined by discussions between obstetric team, haematologist, neonatologist, haemophilia centre and comprehensive care centre. Parents should be involved in this decision making.
If elective caesarean section is indicated for obstetric reason and vwf and factor 8 concentrations are low, they should be raised by transfusion of recombinant factor 8 and vwf concentrate to cope with bleeding.

Vaginal route would be preferred for delivery and should be least traumatic. A labour ward must have a protocol for management of bleeding disorders, which should be followed. Labour and delivery should be managed by senior obstetrician and experienced midwife. Consultant obstetrician should be informed. Epidural analgesia can be instituted following discussion with anaesthetist, provided vow concentration is within normal range and care exercised before removal of catheter. Invasive procedures like FSE and FBS should be avoided and delivery by venous is contraindicated..

Following delivery, cord blood should be collected for neonatal diagnosis. Intramuscular injection, example vitamin k is avoided until neonatal status is determined to minimize painful haematoma formation. Risk of primary as well as secondary PPH, should be monitored with factor 8 and vwf concentration. Acute episode of bleeding should be managed with DDAVP or cryoprecipitate and should continue for 3-5 days.

Couple should be provided adequate information and counselling and should put in touch with helpline/haemophilia support group regarding bringing up children with bleeding disorder.

Posted by narmin B.
Von willeband disease (VWD) is caused by partial or total lack or defective Von willebrand factor. This factor is necessary for platelet function in the formation of initial blood clot. It is the most common inherited bleeding disorder in pregnancy. The inheritance pattern varies depending on the type of VWD. Risks of VWD during pregnancy are antenatal, intrapartum and postpartum bleeding and the risk of inheritance of the condition.

In order to assess the severity of the VWD a history should be taken. Previous deliveries and complications such as bleeding during pregnancy and labour and their treatment should be asked. This can provide a prognosis for the present pregnancy. Current symptoms such as gum bleeding, epitacxis and vaginal bleeding should be enquired to assess the severity of the condition. History of receiving blood products is important, because it necessitates testing for hepatitis B and HIV infection.

Early pregnancy scan is required to accurately determine the gestational age as with regard to her bleeding disorder, the date of last menstrual period is unreliable.

The other issue in the antenatal period is testing maternal blood for hepatitis B, C and HIV infection especially where the mother has received blood products.

Regular measurement of maternal haemoglobin is required and iron deficiency anaemia should be treated as it deteriorates the condition if bleeding happens due to VWD.

Because of her bleeding disorder multidisciplinary care by an obstetrician and a haematologist with special interest in this area is mandatory.
A careful counselling of the parents is necessary. Although patients are usually aware of the nature of their condition but a summary of VWD should be explained. It should be told that usually VW factor increases in pregnancy but in spite of this fact, still there is risk of antenatal and postpartum bleeding. It should be explained that VWD is an inherited disease and the baby can be affected. Although chorionic villous sampling (CVS), amniocentesis and cordocentesis can be performed to determine the fetal status, but this is not recommended in light of the mild clinical manifestations of von Willebrand\'s disease and the fact that invasive testing have potential risk of fetal loss and bleeding.

Management of severe bleeding in VWD are the administration of DDAVP, and cryoprecipitate. Also these agents can be used prophylactically before surgery.

The mother should be reviewed by an anaesthetist in the antenatal period. Regional anaesthesia is acceptable where the patient?s condition is stable with mild form of disease.

Normal delivery is preferred and caesarean section should be reserved for obstetric indication. Invasive procedures such as fetal blood sampling, fetal scalp electrodes and instrumental delivery should be avoided as they may increase the risk of maternal and fetal bleeding. Active management of the third stage of labour reduces the risk of postpartum bleeding.

The neonate should be tested for the VWD and follow ?up appointment may be required for repeat testing.

Severe late onset post partum bleeding (usually in second week after delivery) can occur. Patient should be warned about the presence of this risk. The management of this condition is administration of DDAVP, and cryoprecipitate and whole blood transfusion. Procedures such as evacuation of the uterus should be avoided if possible because it may worsen the bleeding.

Mother should be put in touch with support groups for VWD for advice and information.

Posted by Sonali G.
Von Willebrands disease is one of the commonest inherited bleeding disorders in pregnancy. At her first visit, it is important to enquire about the type of vwdisease as type 1 is milder and most common form while type III is most severe. History regarding severity of disease in the form of any spontaneous bleeding or history of bleeding after trauma can help in future management. Affliction in previous pregnancy, if any should be asked for.

She should be made aware that in a pregnancy usually VW Disease improves because pregnancy is associated with increase in factor VIII and Von Willebrands factor. Risk is increased in puerperium when these levels fall. Usually VW disease doesn?t have any adverse effect on the pregnancy. Spontaneous fetal or neonatal bleeding is unlikely. But her view regarding transfusion of factor VIII on VWFactor should be discussed, in case it is needed during further management. Advantages and associated risks should be discussed. VW disease type I & II are autosomal dominant. Chances of fetus carrying the disease should be discussed after offering paternal karyotype. If father is normal, baby still carries 1:2 chance of having VW disease. Prenatal diagonosis in the form of CVS or aminocentres is offered but she should understand that it is associated with increased risk of bleeding and 1% increased risk of miscarriage over background risk. Serum Hep B & Hep C & HIV antibodies should be checked in view of history of Blood transfusion.
Antenatally, she should be managed in a centre with close liason with haemophilia centre. Multidisciplinary input in the form of involvement of haematologists, obstetricians, midwives and genetists is needed. She will be monitored regularly with factor VIII level and VWF and coagulation profile. Bleeding time & APTT are prolonged in VW disease. If she opts for invasive prenatal diagnosis and factor VIII is <50 IUml or hemostatic defects are present, she should be given factor VIII concentrate rich in VW factor before the procedure.
Caesarean section is done for obstetric indication. But the aim is to avoid any maternal or fetal trauma. Paediatrican should be informed and should be aware of the history. Ventouse is contra indicated and forceps only indicated if it is a pull out procedure with minimal trauma. Scalp electrode or Fetal blood sampling should be avoided. Difficult vaginal delivery leading to extensive maternal trauma should also be avoided. Continuous monitoring is needed to detect any asphyxia, epidural anaesthesia can be used if coagulation defects are corrected. If she is for caesarean section and hemostatic defects fails to improve them DDAVP / VWconc. can be given preoperatively.
Post partum breast feeding is encouraged. Cord blood is checked for factor VIII . DDAVP or factor concentrate should be give portpartum if needed. Supportive therapy to maintain haemostasis should be continued for 3-4 days post partum. Epidural catheter is removed after ensuring all haemostatic parameters are normal. Intramuscular vit k is avoided in neonate.
Effective contraception is offered. Information regarding support groups should be given to the couple.
Posted by SWATI M.
Von Willebrand?s disease is associated with increased maternal morbidity and mortality due to increased postpartum haemorrhage .Perinatal morbidity and mortality is increased in affected babies but does not cause spontaneous bleeding .
During antenatal management,subtype of disease should be taken into account as severity of the disease varies and mode of inheritance is different.Type 1 is most common.Type 1&2 are of mild variety and improve during pregnancy and transmitted by autosomal dominant trait implying transmission to 50% babies but not all babies will be affected because of variable expression of the abnormal gene..Type 3 is a serious type and does not improve during pregnancy and transmitted by autosomal recessive trait.Woman should be counseled according to the type she has.She should be reffered to the clinical geneticist for genetic counseling and advice regarding prenatal diagnosis.Woman needs to be managed by specialist care in close liaison with haemophilia centre to optimize the outcome.
She should be offered prenatal diagnosis if have type 3 disease, by CVS or amniocentesis after 14 weeks and informed risks involved due to the procedures of increased miscarriage,1-2% and 1% respectively above the background risk.There is a risk of maternal bleeding with CVS.Hence level of factor should be checked,corrected if required and ensure availability of factor during procedure. Parents atitude towards termination /continuation of pregnancy in affected pregnancy is obtained.Woman should be counseled regarding increased risk of having blood borne infections such as HIV,HepB infection due to use of blood products(factor concentrate) and risk to transmit those to the fetus.Anaesthetic consultation should be arranged to discuss labour analgesia.Factor levels should be checked at regular interval particularly towards term as prophylactic treatment arrangements can be made to minimize risk of bleeding during peripartum period.
Intrapartum management-Check FBC,clotting profile,factor level at admission and cross match and save serum ,will be useful in event of bleeding.Factor level should be more than 50 IU/ml.Senior anaethetist should be involved for managing labour analgesia and factor level should be checked,corrected before removal of epidural catheter.Avoid use of intramuscular injections to minimize haematoma formation. Avoid invasive procedures such as FSE,FBS in affected pregnancies.Avoid difficult operative vaginal delivery,vaccume delivery is contraindicated but simple lift out forceps delivery can be undertaken.Try to minimize perineal trauma at delivery and repair perfomed with particular attension to haemastasis.Cord blood should be collected to confirm/establish the diagnosis.Vigilance for post partum haemorrhage is important.Use DDAVP prophylactic if clotting defect or bleeding ,in type 1 and 2a to increase vWF, not used in 2b as causes thrombocytopenia.Type 3 will not respond to DDAVP,need factorVIII or vWF plasma concentrate. Use is recommended for 3-4 days if vaginal delivery or 4-5 days if caesarean delivery.
Vitamin K should be administered orally to neonate,avoid intramuscular injections till bleeding disorder is ruled out.Affected babies should be registered with haemophilia centre.Inform GP as immunization in affected babies should be done by intradermal or subcutaneous route.
Posted by Rani M.
Von Willibrand disease is the most common inherited bleeding disorder, incidence being about 1%.
Care of this woman requires multidisciplinary input comprising of hematologist,physician and an obstetician experienced in care of such mothers.
History is taken regarding affected family members,history of disease in any previous babies and any h/o of PPH in past.
Mild to moderate disease tends to improve during pregnancy due to physiological rise in levels of factor 8 and von willibrand factor.this may also create diagnostic difficulty.
Levels of VWF antigen and factor 8 are checked and regulrly monitored throught out the pregnancy Platelet counts and bleeding time are also checked as bleeding time may be prolonged and platelets may be low in type 3 VWD.Genetic counselling is offered to the couple if not already done prepregnancy to calculate the risk of inheritance to the fetus. type 1 & 2 being inherited as autosomal dominant and type 3 as autosomal recessive.
If antenatal treatment is required a recombinant factor 8 concentrate which also provide vWF should be chosen. Alternatively one very high purity vWF concentrate can be given.
ddAVP should not be used as its safety is not established in antenatal period.
If hemostatic defect fails to improve, the treatment is required for delivery by factor 8 or vWF concentrates to cover the labour and delivery.Full laboratory monitoring of hemostatic replacement therapy is necessary.
Senior anaesthetist should be involved &.Epidural analgesia can be given if coagulation defects have been fully corrected.
Cesarean section should only be done if indicated for obstetric indications.
Maternal perineal trauma should be avoided. Any wound including episiotomy should be promptly sutured
.Fetal scalp electrode and fetal scalp sampling should be avoided if the fetal status if unknown.
The risk of bleeding is maximum in postpartum periods due to decline in the levels of coagulation factors.ddAVP can be used now in type 1 disease and continued for 3-4 days after vaginal delivery and for 4-5 days after ceasrean. but it is contraindicated in type 2 disease and not beneficial in type 3 disease.
It should be ensured that the hemostatic parameters are normal before epidural catheters are removed.
Cord blood for neonatal evaluation can be taken but it is difficult and unnecessary to attempt to diagnose vWD disease in neonate except if there is risk of type 3 disease.
Neonatal I/M injections including vit. K should be avoided if the fetal status is not known
Hormonal contraceptives are preferred over IUCD when she asks for contraception.Mirena can be used as will reduce the menstrual blood loss.