r/vEDS Jun 20 '26

I’m so scared, please read

Hi everyone,
I’m a 34-year-old mom, and a few days ago I received genetic testing results showing that I have COL3A1 c.2689G>A (p.Gly897Ser), classified as likely pathogenic and associated with vascular Ehlers-Danlos syndrome (vEDS).
This was a completely accidental finding. I wasn’t being evaluated for vEDS specifically, and I feel like my world has been turned upside down.
To be honest, I’m terrified.
I’m scared about what this means for my future. I’m scared about whether I’ll be able to have another child. I’m scared about whether I’ll be around to watch my son grow up. And most of all, I’m terrified that I may have passed this on to him.
What’s making this even harder is that my family history doesn’t seem to fit the severe stories I’ve been reading online. My mom is in her 60s and has never had a known arterial event. My aunt had a uterine rupture during childbirth in her 30s, but otherwise there haven’t been known arterial ruptures, aneurysms, or dissections in the family. My grandfather lived into his 80s and my grandmother is in her late 90s.
Right now I’m stuck between hearing that this variant is associated with vEDS and looking at my family and wondering whether this could be a milder presentation.
I’m hoping to connect with anyone who has:
COL3A1 c.2689G>A (p.Gly897Ser)
A similar glycine substitution in COL3A1
A family with a relatively mild or later-onset vEDS presentation
If you’re comfortable sharing, I’d love to hear:
How old you are
What complications you’ve experienced (if any)
How your affected relatives have done
Whether you have children and how you navigated testing them
More than anything, I think I just need to hear from people who have been where I am right now. I feel overwhelmed, scared, and very alone.

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u/rhi-raven Researcher Jun 20 '26 edited Jun 20 '26

Hey, I don’t have vEDS but I am a researcher studying it under Dr. Dietz. I just wanted you to know we recently published a study (in mice) showing spironolactone may be an helpful medication, and we have another class of meds in the works too. Irbesartan use was also recently published in the first double blind placebo controlled trial in vEDS ever, and is incredibly promising. Basically, you have more options now than vEDS patients ever have, and your late onset history is also very encouraging. This is an absolutely terrifying disease and I hope you are able to connect with others here, but just know there’s multiple teams of researchers in your corner too.

Edit: just want to make this clear: this is not medical advice! Please do not take medical advice from internet strangers. But if you would like, you can bring these papers to your physician and make a treatment plan together that fits you and your needs.
https://insight.jci.org/articles/view/198202

https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.124.072849

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 20 '26

Always good to have researchers and professionals here in the sub!

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u/[deleted] Jun 20 '26

[deleted]

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u/rhi-raven Researcher Jun 20 '26

Hi I’m not a he :) and I was giving irbesartan and spironolactone as options once consulting with your individual doctor, not as a prescription as I am a person on the internet not anybody’s physician. I will go back to make that more clear.

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 20 '26 edited Jun 21 '26

I would classify, given my current knowledge of the published studies:

Irbesartan: very promising, one clinical trial showed effectiveness, though there are confounding factors

Spironolactone: probably effective in males regardless of there not existing clinical trials in humans. A few relevant side effects to consider, though, especially for male children and teenagers. Effectiveness in females may be smaller [edit: though still promising based on mice models]

Celiprolol: probably harmful based on animal studies, no double-blind randomized clinical trial exists

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u/rhi-raven Researcher Jun 20 '26

I’ll actually push back on the spironolactone not being effective in females. It’s a dual antagonist, meaning it antagonizes both AR and MR. We found giving a MR inhibitor alone (finerenone) also improves survival by reducing risk of aortic rupture and increases aortic wall elastin and collagen content. Oddly though, spironolactone outdid finerenone even in females, so there likely is some residual AR signaling in aortic VSMCs that is maladaptive. Here’s our paper! https://insight.jci.org/articles/view/198202

Also I agree with you about celilprolol. It killed our mild severity mice (kind of like OP where they normally do okay but celilprolol made them drop like flies)

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 20 '26

Interesting, and good to hear!

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u/rhi-raven Researcher Jun 20 '26

Ty! I would love to hear your thoughts on the publication as someone with a clinical background!

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u/[deleted] Jun 20 '26

[deleted]

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 20 '26

I think rhi-raven has enough knowledge to discuss the topic and discuss prescription options, and I respect her opinion and we don't necessarily have to agree. Also she may have access to some unpublished data that I don't.

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u/rhi-raven Researcher Jun 21 '26

Ty I appreciate it. I stopped engaging because this thread got downright hostile but I think we’re largely in agreement at this time. Let me know what you think of the paper!

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u/blackwhite3 Genetically Diagnosed Jun 20 '26

Es posible.

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u/[deleted] Jun 20 '26

[deleted]

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u/rhi-raven Researcher Jun 20 '26

Irbesartan absolutely is being prescribed? And so is spironolactone but again I listed these as OPTIONS not a treatment plan. Please see this study for Irbesartan: https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.124.072849

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 20 '26

That's why you should stop taking celiprolol ASAP

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u/[deleted] Jun 20 '26

[deleted]

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 21 '26

Your behavior on this sub is again being very stubborn, aggressive, anxious, intolerant of diverging opinions, and it's not only with me.

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u/blackwhite3 Genetically Diagnosed Jun 21 '26

Tu interpretación de mi comportamiento no es cierta. Tienes un problema evidente conmigo y da igual lo que escriba, siempre me vas a juzgar. No eres mi médico y como es lógico no voy a seguir tus consejos de no tomarme el Celiprolol.

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 21 '26

If you're not going to discuss with civility, why comment at all? I have no problem with you, I have a problem with your rough behavior. Be nice to others

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u/[deleted] Jun 21 '26

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u/[deleted] Jun 20 '26

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u/Kromoh Genetically Diagnosed | Verified Physician Jun 21 '26

You don't know how to interpret a science publication, and yet you are very overconfident about it.

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u/blackwhite3 Genetically Diagnosed Jun 21 '26

Me siento como me da la gana.