A Life Inhabited
Written by Aaron Dennis, February 3, 2022
Thanks to advances in the treatments made available in the last few years, developing inhibitors isn’t as worrying as it might have been a few years ago. Member Josh Crombie tells us about his journey.
Josh, 24, lives in Middlesbrough, North Yorkshire, where he’s working for his local council. “I have severe haemophilia A, and when I was 7 months old, I developed inhibitors. Because I was growing up without access to all the different types of treatment that we thankfully have today, my bleeds became difficult to treat. There were only two options open to me – one didn’t work, and one only worked a bit!
“Limited treatment options meant I had to be very careful to do what I was told and sometimes I missed out, when the risks of activities didn’t outweigh the benefits.
“At 7, I went on to FEIBA treatment, using a Hickman line. This was a big change and much more effective. The downside was that it was pretty inconvenient as a kid, but for the most part, it did its job, which came as a huge relief to my family.
“Then, at 19, I was offered the treatment Emicizumab, which has been life-changing. My old annual bleed rate was 50, but overnight it went to zero and I haven’t had a bleed for three or four years. “I inject into my thigh, stomach or arm under the skin once a week, though some people only have to use it once a month. Emicizumab works differently to replacing factor. In a way, it fools my body into accepting treatment.
“Changing my treatment has allowed me to do so much more and it’s the best choice I have ever made with my treatment.
“Then, at 19, I was offered the treatment Emicizumab, which has been life-changing. My old annual bleed rate was 50, but overnight it went to zero and I haven’t had a bleed for three or four years.”
“It gave me the confidence to finish University, and made it possible for me to find work without the worrying I’d need to take lots of sick-leave.
“It’s also made a big difference to my mental health. I can be much more positive, knowing that I’m ok and that my treatment is ok.
“I know that some people find under the skin injections uncomfortable, and prefer a standard needle but I couldn’t be happier. I have a few scars, and some arthritis, but you’d barely know I have haemophilia.
“My mum still gets upset talking about my new treatment, as it’s been such a transformation and made her so happy! After everything that we were going through before; never knowing if I’d run out of options. But now I have so much more confidence for the future.
“There’s a phrase which says ‘If you have an inhibitor, it’s a life inhibited, not inhabited’ and as a family, we struggled when I was younger. What I was allowed to do, or not, seemed very black and white and inflexible. But now, with the right treatment in place, you can find work-arounds to ‘cheat’ haemophilia, and give yourself the life that you want to live.”
An inhibitor is a type of antibody that prevents factor replacement treatment from working, making bleeding more difficult to treat.
Inhibitors typically develop in the first 50 treatment days, with most developing in the first 20 days. This is why when people with severe haemophilia develop inhibitors it is usually during childhood, whilst people with mild or moderate conditions tend to develop them later in life.
Inhibitors affect around 30 per cent of people with severe haemophilia A, around 9 per cent of people with mild and moderate haemophilia A, about 3 per cent of people with haemophilia B, and only rarely people with other bleeding disorders.
Today, inhibitors are treated with immune tolerance induction (ITI) which involves regular exposure to factor treatment with the aim of the body becoming accustomed to the clotting factor. If this doesn’t work then the new treatment Emicizumab, which mimics the role of factor VIII, allows people with haemophilia A and inhibitors to live a much more normal life with far fewer bleeds.
For further information, you can read more about inhibitors here.
This article first appeared in Community Matters magazine, Winter 2022.