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The bleeding tendency associated with Hermansky-Pudlak Syndrome is very variable. Due to this variability it is hard to predict the outcome of obstetric and gynaecological issues.


Menstruation


Some women experience very heavy and prolonged menstruation and may suffer from anaemia. Some women choose to control menstruation by using oral contraception.  However, others find IUC’s (Intra-Uterine Contraception) more helpful.  These slowly release a drug into the uterus over an extended time.  In the United Kingdom this type of IUC is called a IUS or Intra-Uterine System.  In the United States there are two types of intra-Uterine Contraceptive available: the copper Paragard and the hormonal Mirena.  Both of these contraceptives are called IUDs in the United States.


During labour and delivery


Some women with HPS experience excessive bleeding during delivery.  If you have HPS and are pregnant you make sure that all doctors involved in your care are fully aware of the bleeding disorder of HPS.  Your doctor may need to prepare Platelets, DDAVP, and medications like Amicar for your delivery.  Conscientious prenatal care is vital and it is advisable to have a haematologist involved during your pregnancy,  An individual's response to the platelet disorder (bleeding history, bleeding time) as well as other contributing factors, such as the possibility of platelet von Willebrand's deficiency might be indicators of possible bleeding during menstruation and delivery.


Women who have the Crohn’s like colitis of HPS


Some people with HPS develop a Crohn’s like colitis or Inflammatory Bowel Disease (IBD), around 15 percent.  In some cases the inflammation can be severe and the wall of the bowel may become perforated (develop a hole).  The perforated area may adhere to another organ such as the skin of the abdomen or wall of the vagina and an infection may set in.  In time the perforation or hole may spread through the skin or wall of the vagina to form an opening through which faecal matter may leak. These connections, or channels, are called ‘Fistulas’.  It is important that all possible care is taken to avoid a urinary tract infection.  It may be necessary to perform surgery to separate the bowel wall from the wall of the vagina and close the holes in each.  As with all surgery measures should be taken to control bleeding.  These may include a blood platelet transfusion prior to surgery and medicines such as DDAVP.  Read more about fistulas.

HPS, pregnancy, and gynaecological issues