Even though hemophilia mostly affects men, women who are carriers of the faulty gene causing hemophilia A and hemophilia B may also experience mild symptoms. They are rarely diagnosed with the disease, however, because the condition is not severe, allowing them to lead full lives. (In hemophilia C, women and men are equally affected).
Women who are carriers have one healthy and one mutated copy of the hemophilia gene on each X chromosome. The disease only truly develops if both copies of the hemophilia gene are mutated, and this is rare occurrence. In most carriers, one X chromosome gene is healthy and produces enough clotting factor to protect carriers from more severe forms of hemophilia, although clotting factor levels can vary.
Clotting factor levels in carriers generally range from 30 to 70 percent of normal levels, enough to protect from severe bleeding. One common symptom in hemophilia carriers, however, is heavy menstrual bleeding.
Women who have less than 30 percent of normal clotting factor levels are usually said to have mild hemophilia, and up to 60 percent of carriers are thought to have some degree of bleeding problems.
Abnormal bleeding: When to see a doctor
Because women are not usually warned about the possibility of having hemophilia, they should be on the lookout for these symptoms:
- Menorrhagia, heavy or prolonged menstrual bleeding
- Abnormal bleeding after childbirth
- Bruising easily
- Frequent or prolonged nosebleeds
- Bleeding abnormally after dental procedures
- Bleeding abnormally after surgery or trauma
Many women do not think of heavy menstrual bleeding as a sign of a bleeding disorder, and consequently don’t speak with a doctor about it. It’s common to think of it as a life-long personal characteristic.
However, menorrhagia can have significant effects on a woman’s life, including chronic fatigue, lost work hours, abnormally severe pain during menstruation (dysmenorrhea), and mood swings.
Unusual menstrual bleeding can also lead a doctor to recommend a hysterectomy, rather than checking for possible hemophilia.
If you suspect you might have a bleeding disorder, ask your doctor to rule out hemophilia first by measuring the level of two clotting factors (factor VIII for hemophilia A and factor IX for hemophilia B) in your blood. A genetic test may also be undertaken to determine the nature of your mutation.
In adolescents, the start of a girl’s menstrual cycle is often accompanied by heavier bleeding. If there is a known family history of hemophilia or any other bleeding disorder, girls should be closely followed through puberty by a gynecologist, a hematologist specialized in bleeding disorders, or a family physician or pediatrician.
Pregnancy and childbirth for women with hemophilia
Pregnancy, as well as childbirth, can also be challenging for women with bleeding disorders. It is critical that a woman’s healthcare team is aware of a carrier (or hemophilia) status so plans can be made for a safe delivery. This includes ensuring for safety measures at the time of birth, and regular monitoring of clotting factors to prevent major bleeding, especially as the date of delivery approaches.
Delivery should be as gentle as possible, for both the mother and the baby. Natural delivery without the use of instruments (forceps, or a suction extraction) is recommended. Use of an epidural or a deep intramuscular injection also are not advised.
According to the Canadian Hemophilia Society, women with hemophilia should talk to their doctors about factor replacement therapy during the first three months of pregnancy to lower the risk of miscarriage.
Plasma-derived factor concentrates can transmit a common virus known as parvovirus B19, which may cause a miscarriage. Pregnant women or those who might become pregnant are advised to avoid factor concentrates made from plasma, if possible. Possible alternatives that might be considered under a physician’s guidance include desmopressin, antifibrinolytic agents, and recombinant (artificial) factor concentrates for deficiencies in factor VII, VIII and IX.
Women with bleeding disorders, however, are often protected from bleeding episodes while pregnant, because of higher-than-usual hormone levels.
After birth, women can maintain these higher hormone levels by breastfeeding – helping to protect them from bleeding in the weeks following delivery.
If a bleeding disorder is suspected in a newborn, parents can request that a sample of cord blood be tested for the disease, and can request that the vitamin K (normally given to the baby shortly after birth) be given by mouth, and not by injection, to prevent bleeding at the site of the injection.
General precautions for women with hemophilia
Women with a bleeding disorder may need to follow some general precautions to prevent excessive bleeding, like avoiding medications that affect the platelets, clotting proteins in the blood. Of note, these drugs are often taken to ease menstrual cramps.
Common examples are aspirin, drugs containing aspirin (Midol or Alka-Seltzer), and non-steroidal anti-inflammatory drugs (Advil or Aleve). These drugs are often prescribed to ease menstrual cramps but can actually make bleeding worse.
Ask your doctor or pharmacist for advice, and for information about a drug you are planning to take.