The author of this story is an Australian bleeding disorders community member
I have lived my whole life surrounded by haemophilia. My father had haemophillia B, due to low levels of factor IX (9). Dad passed away in 1982 after suffering many years of pain, lengthy admission to hospital for months at a time and had severe joint damage due to regular bleeds.
When I was 11 I found out I also had the gene. I had always bled “a little more” than other girls when I fell over and when I became a young woman, I had very heavy periods but other than that my life was relatively normal. So I recollect with a smile when I decided to tell my friend at school about my bleeding disorder, her first question was, “how do you get it – do you catch it?” No, you don’t catch it; you are born with it, I said. My friend responded quickly with, “Well, I would prefer to have haemophilia then, because you don’t look sick at all, but when you catch a cold from someone you feel awful!”
When I decided to start a family we had genetic counselling and decided to have chorionic villus sampling (CVS) to determine the sex of the baby. Twelve years ago you could determine the sex, but not if the baby had haemophilia. Being an only child myself, if I had a girl or boy not affected by haemophilia, then that was the end of a long era in my family. I must say I was elated when the doctor rang 24 hours later and said that I was having a girl. In 2002 there was still so much unknown about girls having haemophilia, in my mind I thought – no problem. Worst case scenario, she will be just like me… My gorgeous girl was born via caesarean section, from which I bled and needed a blood transfusion. She had a large cephalhaematoma on her head, which is a blood clot. The doctors told me it was due to the delivery, it was a common occurrence and would take several months to disappear, but after three weeks it was completely gone. I was a little surprised but being a new first time mum and suffering those sleepless nights, I just went about getting on with life.
We had our daughter tested for haemophillia at the age of 2 and the results confirmed that she is a “symptomatic carrier” of haemophilia B. And classed as being mild to moderate with a factor level of 10%.
My daughter is now nearly 12 years old and over the years we have had falls, where she bruises for a few days, and then the bruising is gone. I have had various conversations with her school teachers, dancing instructors, sports teachers, family and friends to explain the condition. From the outside she does not look “different” to any other child, however when you mention bleeding disorder, their faces change.
My most important objective is making people understand that she will not bleed to death from a fall or a simple cut and haemophilia does not affect her academic achievement (what affects that is TV and not doing homework!) – and that provided they follow a simple set of guidelines for emergencies, believe it or not all will be OK. School teachers, family and friends now know the best thing to do is to stay calm, commence standard first aid treatment, as you would for any other child in regards to a nose bleed or a cut and that for more urgent matters call an ambulance and give them her MedicAlert™ details.
The first real incident we have had was last year when we had a dental appointment at the children’s hospital and she required a tooth extraction. A simple tooth out and the wound was still oozing a lot eight hours later, so we made the trip back to the hospital, where she was admitted and given intravenous BeneFIX™, a recombinant factor IX product, which stopped the bleeding. Everything was fine and we were discharged home the following day. We now know for future reference that any tooth extraction requires some prior planning and, much to my daughter’s delight, a day off school!
I have since discussed with my daughter and our haematologist issues that may arise with her becoming a young woman. She may experience heavier than normal bleeding during menstruation, and may require tranexamic acid tablets each month. We are yet to embark down that road.
I had a total hysterectomy in 2014 following years of painful, very heavy periods. The last five years were particularly bad with intermittent bleeding throughout the month. I could not enjoy simple pleasures like swimming and was constantly keeping a change of clothes at work in case. I discussed a hysterectomy with my haematologist and gynaecologist as I was nervous about bleeding during surgery, particularly following the experience after my caesarean. My fears were for nothing. My surgeon ensured that he was well prepared prior to surgery having held discussions with our haematologist and now looking back, I wish I had made the decision years ago. Life is wonderful and for the first time in many years I can wear white!
I have decided to tell you my story and share my experience not because I feel sorry for myself or for my daughter or for having watched my father live with haemophilia, but because for other mothers like me who have daughters there is hope, in fact there is so much hope. I have watched over the last 12 years the changes that have taken place. People, given the right information and informed about our condition, are no longer nervous. Resources are now available, there have been advances in medicine, you can now know at CVS if your child is a boy or a girl is a haemophiliac or not and education is the key “factor” not the number “nine” factor. Life for all women with a bleeding disorder, including my daughter, is becoming brighter each day.
In time, years down the track from now, I will have the discussion about family planning and all that it entails with my daughter. Who knows where we will be by then.
But for now I will continue to be amazed at the beautiful girl I am raising, who can be defiant, downright naughty, a pleasure to hang out with, someone who lifts my spirits high and just happens to have haemophilia….
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