Patient story

Harry, age 3, Haemophilia A

My name is Emma, I am 26 years old and I live with my partner Simon and our 2 sons, Lewis aged 6 and Harry aged 3, in Gillingham, Kent, UK.

Both Lewis and Harry have severe haemophilia A with factor levels of less than 1%.

Up until Harry was 18 months old he was relatively problem free apart from the odd expected bleeds which were treated with recombinant factor VIII.

Then one Friday morning Harry woke up and was very unsteady on his feet, and within a short space of time he was unable to walk at all. I rang our centre and was told to bring him up for treatment. When we arrived the consultant examined him and confirmed he had had a bleed into his right ankle and he was given a dose of factor VIII.

Over the weekend Harry improved a little but was still unsteady and experiencing some pain which we just put down to the swelling. By the Monday morning Harry was again unable to walk and I believe he had re-bled. At the hospital he was given another dose of factor VIII and we were sent home.

Over the rest of that week Harry received 6 more treatments of factor VIII, but still he didn’t improve. On that sixth visit to the hospital I remembered once reading a leaflet in the centre that talked about inhibitors. So on seeing the consultant I mentioned if it was possible that Harry had an inhibitor and asked if they would run the tests needed, which they agreed to do.

We attended the centre 2 days later as Harry was still no better. We were then told that the test had confirmed that Harry did have an inhibitor. We were told that Harry had a high responding inhibitor which was harder to treat. We talked about what the options were and we left the centre feeling a bit numb.

We couldn’t really believe this was happening and were also trying to take in the journey we were about to start.

Harry has now had his inhibitor for 1 year and 7 months and during that time we have had many ups and downs. Harry’s consultant decided that Harry’s best chance of being inhibitor free was to commence with immune tolerance therapy (ITT). Before this could start Harry needed to be fitted with a port-acath and his inhibitor levels needed to drop.

Harry was fitted with a port-a-cath while being treated, and ITT was started. When Harry started ITT he was given high doses of factor VIII twice a day, by the end of a month Harry’s inhibitor levels came back as undetectable, which we were pleased about but at the same time the doctors were surprised that the levels had dropped so quickly. We were then made an appointment to have a Half Life Study done. This showed that Harry’s half life was shortened and we were advised to continue with the ITT until this had improved.

After many more studies Harry’s half life has not improved which is quite frustrating as it feels like after many hard months things are not going the way we would have liked, but we know we have to push on for Harry’s sake.

Since finding out about the inhibitor our lives have been affected in many ways. My partner was a self employed bricklayer but due to the amount of time he needed off work and because we had no other family around us he ended up being laid off many jobs which caused us great financial difficulties, which in turn affected our normal day to day lives.

I had just started a part time childcare course which I had to stop attending and Lewis also had to learn to spend even more time in and out of hospital. He also found it hard to see and to understand some of the things Harry had to go through.

APROM ID# 3072. May 2011.