Living with von Willebrand disorder, part 4

Written by Aaron Dennis, November 30, 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.   

A Very Bumpy Ride – February 2022

It was just a few months ago I was writing from my happy little bubble of denial where I was starting to believe that Ella, 3, was outgrowing VWD (note: I know you don’t outgrow it but it was a nice little dream while it lasted). 

So, in mid-November she had her first infusion of 2021. The medication took a little longer to work than it used to, I wasn’t sure why. Then a couple of days after Christmas she had her second (again, a nosebleed), but this time the first dose of IV medication didn’t stop it. Eventually they gave a second dose and it stopped. It made me start to doubt the medication slightly – I used to be amazed at just how quickly it could stop a bleed before, why isn’t it working its magic so quickly now? Still, 3 infusions in 2021 – I can’t complain at that can I?! Strap in, it’s a long bumpy ride from here… 

Well, that escalated quickly! Sunday 9th January 2022 was a day like any other and yet, little did we know that it wasn’t. Little did we know that during that day Ella had a bleed on one of her tonsils which rather than coming out of the mouth and making itself known was slowly dripping down her throat into her stomach.  

Ella was bouncing around, her usual lively self, and we were completely oblivious until early evening her stool turned dark and then 20 mins later another one was black. After a bit of hesitation and asking Dr. Google (or Dougal as it is known in our family!) we realised that this can only point to an internal bleed. It was bedtime and somehow we had to explain to our girls that Ella (who did not seem unwell and had been happily playing with her sister) needed to go and see the doctors.  

She cried, her sister cried…we nearly cried! As it was an evening and this was her first ‘different’ bleed I packed an overnight bag for us and went in. Luckily, fairly early on a registrar spotted that it wasn’t a stomach bleed as we suspected it was, but was in fact a bleed on a tonsil. Initially I felt relief as it was a mucosal bleed not a GI bleed, that wasn’t as scary… but it turns out this had been going on a long time.  Ella had lost and swallowed a lot of blood.  

While they were trying to put a cannula in her she got worked up and projectile vomited over us both.. but it wasn’t vomit, it was dark blood and clots and it was terrifying. The nurse said well done for how calm I was – I didn’t feel calm, I’m pretty sure I just froze on the inside but somehow comforted my girl and told her everything was going to be alright.  

Once again a single dose of factor wasn’t working and by this stage she was becoming anaemic. The rest was a blur – Ella continued to bleed even after more medication, her usually lovely olive skin colour was gradually fading before my eyes and within a matter of hours she was laying there extremely pale, her lips had lost colour completely. She ended up having a blood transfusion and having her throat cauterised under general anaesthetic. This was scary but her consultants were amazing and after a few days in hospital they arranged for us to be trained to infuse at home.  

My brain was too foggy to feel confident in retaining any information after the previous few days, so my husband Nick went in for that. Six days after this incident Ella had her first toddler ballet show and to everybody’s surprise (but approved by her consultant) she made it, and despite everything, she shone like a star. Nick had done the infusion that morning and Ella’s big sister Leah performed in 2 shows that afternoon. My heart is full of pride for my wonderful little family.  

Leah’s princess-themed birthday party was the next day and Cinder-Ella wasn’t going to let a cannula spoil her fun. After one week of treatment the cannula came out and life returned to normal.  

We had a video call with her specialist team a few weeks later and a plan was put in place to treat future bleeds, but it was agreed that we would hope this was a one-off and to hold off on prophylaxis for now, unless another big one happens. That’s fine, we’ve gone a long time without bleeds before, let’s hope she has another good phase…  

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.