As #REDS4VEDS day, the global awareness day for vascular Ehlers-Danlos syndrome (vEDS) approaches, CTDNA would like to introduce our first Ambassadors. Brad and Holley, who live in Victoria Australia with their young sons, Hunter and Isaac.

Brad, Hunter and Isaac live with vEDS, and very generously are giving some of their time to help CTDNA in spreading awareness for vEDS and more generally, heritable connective tissue disorders (HCTD).

vEDS has an incidence of somewhere between 1 in 100,000-200,000 people. Meaning in Australia there are in the range of between 130-260 individuals living with vEDS, and in Victoria where Brad and his sons are, that number is even smaller at between just 35-70 people living with vEDS. These figures show clearly just how rare vEDS is.

vEDS like all types of EDS is a multisystem syndrome which causes a variety of complex chronic symptoms that can affect just about any organ system. Red flag signs and symptoms indicating an individual may have vEDS can include; a positive family history for vEDS, any early arterial rupture/s, bowel perforation or pregnancy complications such as uterine rupture. These can all be indicators that may warrant investigation and screening for vEDS.

Physical signs indicating a possible vEDS diagnosis when exploring HCTD include easy bruising, thin skin with very identifiable and visible veins, joint hypermobility affecting primarily small joints and some characteristic facial features such as; thin lips, narrow nose and large appearing eyes.

Early identification and diagnosis of vEDS is important to ensure timely and appropriate monitoring is undertaken. Implementation of lifestyle modifications, and the importance of these is emphasised to reduce the occurrence of injuries which can lead to greater negative impacts than they might otherwise, in people not living with vEDS, and as such worse outcomes.

CTDNA asked our ambassadors to share a little of their story so far, and here it is. Holley shares her experience as a mother, wife and carer to the immediate family who all are living with the chronic illness, and rare disease – vEDS.

Qn: Can you share a little about your family’s journey to being diagnosed with vEDS?

Prior to Bradley, Hunter and Isaac’s diagnosis of Vascular Ehlers Danlos Syndrome in 2018 Bradley was in and out of hospital with various medical emergencies. After returning to the ED around every six weeks via ambulance because of spontaneous dissections in his body, genetic testing was done. This discovered that Bradley had vEDS. This meant that our boys had a 50% chance of having inherited this syndrome which unfortunately they both did.

Qn: What vEDS issues most impact your day to day life?

vEDS is a rare and life threatening syndrome and affects all arteries, vessels and organs in the body. It also comes with fatiguing quickly and being at risk of spontaneous medical events. This means being alert 24/7 for what could potentially be fatal. Something as simple as a headache or tummy ache could potentially be fatal for someone with vEDS. The boys are not allowed to participate in contact sport because high impacts to their body comes with risks.

Qn: What issue/symptoms, if any, have you been surprised to discover are vEDS related?

Fatigue is a big one. We didn’t realise how quick people living with this syndrome are affected by fatigue.

Qn: What advice would you give to someone newly diagnosed with vEDS?

It’s a lot when you are first diagnosed with vEDS. It can fill you with lots of grief and uncertainty. Reach out to those around you for support.

Qn: What do you wish the general community understood about vEDS?

That, because it is so rare, medical professionals often haven’t heard of it or they have only met someone with Ehlers Danlos Syndrome (EDS). Vascular Ehlers Danlos syndrome is the most life threatening of all the subtypes of EDS and should be taken seriously when presenting to a hospital. It’s important to carry a medical information pack to give to the doctors or paramedics.

Qn: Can you share any ‘hacks’ you have found that assist your family in managing and living with vEDS?

Have and wear medical ID’s, put a medical card in your wallets/kids bags for school. Join the community groups on Facebook to make connections with other families or people going through the same challenges. Annabelle’s challenge is the only charity for Vascular Ehlers Danlos Syndrome and they work hard to fund research for vEDS in hope that one day there might be a cure.

_____

To hear more from Bradley and Holley on their vEDS journey so far; CTDNA recommend you take a listen to the two part podcast they did with Two Humerus Nurses; here and here.

Follow Bradley on his instagram account VEDS_Zebra where he graciously shares some of the behind the scenes of life with vEDS as a parent, carer and person living with the disease too.

Signing off until next time,

The CTDNA Board of Directors.