ow do you manage hemophilia A in a 16-year-old girl?

Question

Hi doctor,

My 16-year-old daughter was just diagnosed with hemophilia A after having really heavy periods that lasted 12 days and soaked through super tampons every hour. We never knew she had this condition because her father has it, but everyone said girls do not get hemophilia.

Her factor VIII level came back at only 8 percent, which the hematologist said is moderate hemophilia. She missed three weeks of school last month because the bleeding was so bad, and she became anemic with a hemoglobin of 7.2. She tried birth control pills to control her periods, but they made her gain 20 pounds and gave her terrible headaches.

The school nurse keeps calling because my daughter gets huge bruises just from gym class, and they think something's wrong at home. Her period started again yesterday, and it is already really heavy - she used six pads in four hours. The hematologist wants to give her factor VIII concentrate, but insurance is fighting coverage because they say hemophilia in women is rare. I am also worried about her future pregnancies because I read bleeding can be dangerous during delivery. What treatments are safest for teenage girls with hemophilia?

Thanks.

Answer 1

Hi,

Welcome to icliniq.com.

I can understand your concern.

I am deeply concerned about your worries. Hemophilia is caused by a genetic mutation on the X chromosome that leads to a deficiency in essential blood-clotting factors. Hemophilic mothers have a 50 percent chance of inheriting the condition from their children. If the baby is a boy, the baby has X and Y chromosomes, and hemophilia affects the X chromosome, the chance is high. If the baby is a girl, she has XX chromosomes; however, if one X is affected, she will have one surviving X as soon as she becomes positive.

Management:

Start taking tablet Norethisterone 5 mg three times a day continuously for three months.
Tablet Tranexamic acid 5 mg till you have your menstrual cycle.
She has to start the Folic acid 5 mg preconception period till completion of the first trimester.
Do an obstetric ultrasound at seven to nine weeks.
Nuchal translucency anomaly scan.
A growth scan should be done after 28 weeks, every four weeks; she has to do a baseline investigation.
Blood group and cross-matching.
Complete blood count.
Urine complete examination.
Random blood sugar.
Viral markers levels of factor 8 and 9.

Delivery should be done at a tertiary care hospital where MDTA (multi-disciplinary team approach) should be present, senior anaesthesia, senior gynecologist, senior hematologist, and blood bank should be arranged. Postnatal or post-operative PPH (postpartum hemorrhage) management should be prepared, because there is a risk of heavy bleeding during and after delivery.

I hope this information will help you.

Thanks.