In a story for Hemophilia News Today, author Shellye Horowitz has written a piece titled “An Open Letter to Providers Treating Women with Hemophilia.” Women still face discrimination and unequal treatment when they seek medical care, and for women with a long-term illness like hemophilia, this impact can be felt very acutely. Shellye has a multi-generational history of hemophilia A in her family. In this article, she lays out considerations that health professionals need to account for in order to ensure fair, quality treatment for female patients.

About Hemophilia

Hemophilia is a genetic disorder which affects the ability of the blood to form clots, a process that is vital for stopping bleeding after a wound is sustained. The severity of symptoms can vary widely. The disorder is caused by a mutation found on the X chromosome. Symptoms include bleeding for a long time after an injury, risk of bleeding in the brain and joints, and easy bruising. Bleeding in the joints can cause permanent damage and brain bleeding can lead to headaches, decreased consciousness, and seizures. There are multiple types of hemophilia, with the most common types being type A and type B, which are distinguished by having deficiencies in different clotting factors. Treatment involves replacing the missing clotting factor. Drugs that thin the blood should be avoided. To learn more about hemophilia, click here.

Appropriate Care for Women with Hemophilia

Hemophilia can affect women differently than men, as women can be asymptomatic carriers of the mutation as well as experience disease symptoms that range from barely noticeable to very severe. Shellye lists out several recommendations that doctors need to look out for.

1. All women born into families with a history of the disorder should have their factor levels tested at birth. She warns that lyonization, or the deactivation of one of the X chromosome copies, is a possibility for these patients. These tests are essential for determining if bleed symptoms are possible.
2. All women should get factor tests if a close male relative has been diagnosed with hemophilia. This is important for safety and it can also be important to know if a woman could be a carrier.
3. She recommends using the chromogenic factor assay, which studies indicate is the most accurate.
4. OB-GYNs and hematologists should be in close contact to address potential heavy bleeds associated with menstruation; some patients can bleed for as much as half the month.
5. Listen to female patients. Ask plenty of questions, ask for bleed photos, order ultrasounds and other tests to find bleeds, and get baseline imaging of joints for arthritis.
6. Avoid making blanket statements, as the disorder is unpredictable and each case varies considerably.
7. Believe women when they say the factor treatment helps them. Even patients with mild disease can still get joint bleeds and even some carriers can still have occasional symptoms.
8. Stay informed and research treatment resources for women with hemophilia.
9. Be patient with frustrated patients; the average time before diagnosis following symptom onset is still 16 years. Build a partnership with your patients by showing compassion and understanding; prior bad experiences can leave patients feeling bitter and skeptical.

What are your thoughts on Shellye’s guidelines for fair treatment of female hemophilia patients? Share your stories, thoughts, and hopes with the Patient Worthy community!