The Beginning – Living with von Willebrand Disorder Part 1

Written by Aaron Dennis, November 2, 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.   

We were very lucky that Ella’s bleeding disorder was picked up at three weeks’ old while in hospital for an unrelated matter – they tried to perform a lumbar puncture on her and lots of the capillaries in her lower body burst, which rang alarm bells and needed looking in to.  

Obviously the term ‘lucky’ is relatively speaking. It’s heart-breaking to see your three-week-old baby going in to have a procedure done, and then coming out covered in a severe-looking rash over most of her body, and then hearing the next day that “something has come up in her bloods”. But I have heard stories about families who have come under suspicion and had their children removed from their care while investigations are carried out into their child’s unexplained bruising – I can only imagine how awful that must be for all involved. 

So, Ella has von Willebrand disease (VWD) – but what is it?

In simple terms it is a bleeding disorder where your blood doesn’t clot properly. There are three main types, and it is seen as a more common and less severe version of haemophilia.  

Well yes, in its milder forms it is more common and less severe than haemophilia, but our daughter Ella has type 3 (severe) VWD. This is extremely rare with only one in 500,000 people having it. People with type 3 have little or no von Willebrand factor (a clotting protein) and reduced Factor VIII levels in their plasma. Both of these are vital clotting proteins and without them the platelets aren’t able to bind together effectively to form a clot/plug to stop bleeding.  

In simple terms, when Ella bleeds she usually needs help to make it stop – whether that is by pressure, Vaseline, ice, oral or topical medication, a combination of all of these or for the more serious times an IV infusion of clotting factor. She also bruises very easily and an ice pack has become our best friend for bumps and bruises – she has basically been covered in bruises since she has been mobile.  

We have got used to the standard cuts and bruises usually caused by something in particular, but we know she is at risk of spontaneous and/or internal bleeds, in the gut, the limbs and the joints, and with a serious bang to the head, even the brain. So far we have been lucky not to have experienced these issues but live in fear of the day we do.  

We’ve recently had an appointment where we discussed prophylaxis – this would be three weekly infusions of clotting factor into a port-a-cath to help prevent bleeds. We are lucky that she is now one, and have been told that we don’t have to start this yet, as she hasn’t had an internal bleed and has only had 2 IV factor infusions. That news was such a relief but we know that it’s likely she will need it in the future. I can’t help at times but feel that we are living in limbo, trying to make the most of these times where life doesn’t revolve around IV infusions.