The challenges identifying and medically managing carrier women are, finally, “hot” topics  -- for health care providers and families alike.   We are increasingly aware of the variability in factor levels and symptoms.   As discussed in an earlier newsletter, our HTC will be offering carrier women, between the ages of 12 and 40, the opportunity to undergo physical therapy evaluations and joint ultrasound examinations, because we now recognize that women with hemophilia may have unique joint issues.  

But, women (including carriers) may have other bleeding disorders which can negatively impact their life and health. Since many of these are genetic, it is not uncommon for women to not seek medical attention because their heavy periods, or easy bruising, or nosebleeds “run in the family”, so that must be “normal”. 

How many of our patient’s sisters/mothers/daughters/wives have been diagnosed with iron deficiency anemia, or “low iron”?  It turns out that 15 – 25% of women with iron deficiency may actually have an undiagnosed bleeding disorder!  And, not surprisingly, many health care providers --- internists, pediatricians, family medicine and OB/GYN practitioners  -- are not aware of this implication of what is so often considered a “routine” female problem. 

Bleeding disorders in women almost always affect what is called “reproductive bleeding” – menstruation, miscarriage and childbirth. It is logical (and easy) to “blame” iron deficiency on heavy menstrual flow , prescribe iron supplementation and move on.   It is the responsibility of informed health care providers and patients/families to advocate for further investigations to determine whether or not an underlying bleeding tendency is more directly responsible. 

What are the bleeding disorders which can affect women?   Remember that the formation of a clot requires normal quantities of normally functioning platelets (the sticky pieces of cells which form the first fragile clot) AND a normal amount of functional clotting proteins.    There are many proteins involved in clot formation besides VIII and IX.  

The most common bleeding disorder in the world, affecting both men and women, is von Willebrand disease.  People with vWD either make a less-than-normal amount of the von Willebrand protein, OR they make defective von Willebrand protein.   People can be born unable to manufacture a normal amount of any clotting protein --- we have many patients who are deficient in clotting factor 1 (fibrinogen),  5, or 7, or  10, or 11. 

  • Carrier women may have lower-than-normal levels of factors 8 or 9. 
  • Some people have platelets which don’t stick well – the platelets are missing some critical proteins.
  • Some people don’t manufacture sufficient platelets to form that first delicate clot. 
  • Some people break down the clots they make too rapidly.  
  • Some people are sensitive to aspirin or Advil, which may actually interfere with how their platelets stick together. 

Rather than offer a textbook listing of symptoms, specific treatments, etc., it is important for women to recognize  the possible “warning signs” for excessive menstrual bleeding , so that the discussion for further workup can be had with her health care provider: 

  • periods routinely lasting for more than 7 days
  • “flooding” or bleeding through pads/tampons in 2 hours or less for several days of each period  (changing pad/tampon every 1- 2 hours because they are soaked)
  • history of iron deficiency anemia
  • family history of a bleeding disorder
  • personal or family history of excessive bleeding with surgery, trauma, dental extraction, childbirth
  • personal or family history of blood transfusion as management of heavy menstrual bleeding or             postpartum bleeding
  • family history of women who underwent early hysterectomy to control heavy menstrual bleeding or       postpartum hemorrhage

There are many possible explanations for women who are experiencing heavy periods, recurrent nosebleeds, easy bruising.   However, it is absolutely necessary to include a possible underlying bleeding disorder in the list of potential diagnoses, and to include the hematologist – preferably at an HTC – in the evaluation process!



For Women Only...Sort of

by Joanna A. Davis, MD