Living with von Willebrand disorder, part 5

Written by Aaron Dennis, December 8, 2022

Carly, 32, lives with her husband Nick in Devon. They have a busy house; with daughters Leah (6) and Ella (4), who has type 3 von Willebrand disorder (VWD), as well as an excitable boxer dog.  

Since Ella was diagnosed, Carly has found it helpful to write down her experiences about coming to terms with her daughter’s bleeding disorder, and how they grow to manage it as a family. Carly has now decided to share some of her journaling with us, in the hope that sharing her experiences is helpful for other families.   

Having to Take the Next Big Step (warning, this contains very scary stuff!)  – March 2022

Well, we had a good phase that lasted five weeks after treatment finished. Then Ella had a nosebleed overnight. She came into our room, and we gave oral and topical medication and kept her in our bed. It didn’t seem a bad nosebleed so we were fairly relaxed. Around an hour and a half later all four of us were laying in the bed and suddenly Ella did a couple of coughs then projectile vomited a lot of blood across the bed.  

Ella’s big sister Leah flew out of the room terrified and we called an ambulance. Both my husband Nick and I realised, in that moment, that our life changes from now. We couldn’t go on like this; it wasn’t fair on Ella. At one point in PAU she collapsed and was taken to resuscitation. Seeing her on oxygen and fluids barely responsive was terrifying, I thought we were losing her. 

Another week of treatment and by this stage we’d made arrangements for her to go to Bristol Children’s Hospital – exactly a month short of her 4th birthday – to have her port fitted and start prophylaxis 3.5 weeks after treatment was due to finish (basically she will be moving on to preventative treatment rather than treatment on demand). This time I was taught to do the infusions at home, too. As someone who used to have a fear of veins, and as a teenager often went dizzy at the thought of them, this felt like a big step but one my daughter needed me to take. 

However, less than two weeks after treatment finished for February’s bleed, it happened again. Ella had a nosebleed in her sleep which went to the stomach and resulted in her vomiting blood. Much less this time but enough to require intervention. She had a few days of treatment and was started on iron medicine as she was a little anaemic by now, but not long to go…  

Treatment finished on Sunday morning, but by 4:30am on Monday Ella’s nose was bleeding again. A couple of hours later the vomiting started again. When it happened again, in triage in A&E, Ella’s colour was fading so fast that she was moved to resuscitation again. She was dangerously anaemic and required a transfusion. Two days later, surgery was performed to cauterise her nose and also insert a mid-line to enable treatment from home to continue via the mid-line until a port was to be fitted the following week. 

My brain is currently a bit of a blur, but I’m pretty sure the IV factor dose count is somewhere between 30 and 40… for the year so far! Luckily, as my husband and I have been taught to infuse through a cannula at home, we aren’t quite living at the hospital (it does feel like it lately).  

We really hoped to go as long as we could without the need for prophylaxis but are in no doubt that this is the right time. Our poor girl has been through so much in such a short space of time. This feels a massive step because life turns so clinical but it hopefully means that she can have a normal life instead of dealing with these awful bleeds and spending so much time in and out of hospital. I can also go to sleep at night hopefully a little more relaxed instead of holding the fear that there is an invisible bleed going on inside her that we might miss. 

We are lucky that although the hospital where the procedure is being undertaken is a couple of hours from home, Nick’s parents live near it and Leah is going to have a lovely week with Grandma & Grandad to keep her busy while Nick and I manage the hospital time between us. We’ve been told we can both be around in the daytimes, which is more than we are allowed locally, but only one parent in overnight. This works out well as one of us can get back to Leah each evening. 

I have gone from a happy state of denial about the whole condition to suddenly doing IV infusions via a cannula or mid-line in a matter of months. Ella has been a little warrior, Leah and Ella have both been amazing and Nick has helped hold me together when I have been falling apart. We’ve had amazing support from family, friends and teachers, not forgetting the amazing NHS. It has been tough, I’ve aged 50 years in the last 4 months but I know we will get through this next step and make this new normal work.  

For now I will be listening to Ella Henderson’s song Brave on repeat to get me through each day. The lyrics are so fitting for where we are now. 

If Carly’s story sounds familiar, why not join our VWD Live event, in Southampton on Saturday, 28 January 2023 and meet others who share similar experiences?

For the first time, a whole day where time is dedicated to von Willebrand disorder (also known as von Willebrand disease or VWD). Specialist speakers will be on hand to talk through the latest news and views.

Tickets are free to members.