The Health Psychologist

Society for Health Psychology

Living with Ehlers Danlos Syndrome: An Interview with an AYA

2024 Fall, Rare diseases and disorders, The Health Psychologist

Dominique Legros, M.A., M.S.
Trainee Representative, SfHP Adolescent Young Adult Interest Group
Doctoral Student, Clinical Psychology (Health Emphasis)
Pace University

Elise McKelvey, PsyD
Health Psychology Fellow | Georgia Cancer Center
Past-Chair, Adolescent and Young Adult IG  | Society for Health Psychology (APA Div38)

An interview with a 22-year-old female diagnosed with Ehlers Danlos Syndrome at age 16 following an onset of symptoms at age 13.

  1. What was the process of seeking out and receiving a diagnosis?

It took multiple years before I actually had a formal Ehlers Danlos Syndrome (EDS) diagnosis. A lot of my earliest issues were put off as some sort of anxiety and it wasn’t until I was having undeniable—kind of devastating—physical effects that I was taken seriously. For the first couple of years that I had symptoms, it felt like only my parents, my primary care provider, and I were positive that I was not crazy, and others being like ‘no, but she is [crazy].’

Then a couple of years down the line I have these life-threatening diagnoses like superior mesenteric artery syndrome, which is a vascular obstruction that’s incredibly dangerous. It is physical and visible on tests and finally everyone was like “how could anyone possibly think you were ever crazy?”  It was my first piece of proof that I was not making anything up. I was validated. Yes, it was a horrendous experience that could have had an awful potential outcome, but I was finally being believed.

  1. That sounds like a very difficult thing at a young age—to not feel heard by doctors. Who did you turn to in those moments?

It was very hard to have friendships when I was in high school because I had stopped going to school pretty early in my sophomore year because I was so sick. And very quickly, I got out of contact with most of the people that I went to school with.

I spent a lot of time in the hospital growing up. It was a very isolating experience, and I was alone for the majority of those years. I had a few friends in the hospitals where I was frequently inpatient. We ran into each other a lot on our walks around the hallways, but there was very little in-person interaction with people.

  1. What has it been like to share this experience with friends, whether it was back then or now?

There’s an observation that you make as someone who had to take a forceful look at your mortality as a child when you see the way that people your own age who haven’t ever had to do that live their lives.

Things don’t mean the same thing to them, and they don’t make the same risk assessment that you make when someone says, ‘oh yeah, we should go skiing.’ I’ve fought so hard for my health for so long, why would I put my body in a position where I could risk any of that? It’s not that I’m ever judging people’s decisions. It’s more that I’m almost envious of the fact that people can go about their lives without these fears and anxieties.

If you have friends who are still sick, you can’t talk to them about feeling weird and lonely being healthy. But I’m also not going to say it to my friends who never experienced anything health wise because they’re never going to understand it and it’ll never mean anything to them in the same way that it means something to me.

  1. When you think of your own experiences throughout the years, what is something you wish healthcare providers knew about EDS and the things you’ve been through?

Just because I was used to things like hospitalizations, dismissal from providers, and medical tests, it doesn’t mean that I was emotionally used to it. These were things that were frequent in my life. They were constants, and they were more regular than anything else. But they were still really scary. There were so many times where providers would come in and say, “She is very well adjusted to what’s going on in her life!” That’s just because I was putting on this it is what it is, this is just my life [demeanor]. I think there were so many times where I could’ve used someone saying, “I know this has been your life for a while and this is your normal, but it’s still okay to be upset about it and have feelings about it.”

  1. Do you have anything you hope psychologists would keep in mind about experiences like those that you’ve had or about EDS?

I wish that psychology and medicine were a little bit more intertwined to be honest. There is such a distinct separation of physical and mental illnesses. And there’s so little recognition of the fact that you can’t have a body without a brain or a brain without a body. Anything going wrong in either of them is going to throw the other one out of whack. Why is there not automatically someone who’s checking in on how people are coping with the fact that they are in the hospital and may have been there for weeks?

  1. Is there anything I didn’t ask about that you think is important for people to know?

You know the statement of “you’re so strong” or “you’re so brave”? I hated that with such a passion. I really felt so often like I was not strong, and I was not brave. And I didn’t want to be, I just wanted to be typical.

I have gotten to this point, and I don’t want to associate my entire life with the worst things that I’ve ever been through. I’ve done all of these things not because of what I went through but because I’m smart and I work hard. 

Facts about EDS:

  • Ehlers Danlos Syndrome (EDS) refers to a group (13 types) of conditions that impact collagen formation and connective tissues which help provide support and flexibility to systems in the body such as joints, organs, and skin (Malfait et al., 2017).
    • Symptoms of EDS may include joint hypermobility and pain, slowed wound healing, and skin hyperextensibility or stretchiness (Miklovic & Sieg, 2024)
    • For some, cardiovascular concerns, gastrointestinal conditions, and autonomic dysfunction may occur alongside EDS (Song et al., 2021).
  • It is estimated that EDS affects 1 in 5,000 people worldwide, though rates for each type vary (Ehlers-Danlos Syndrome, n.d.).

Resource:

 References

Ehlers-Danlos syndrome. (n.d.). MedlinePlus. Retrieved July 3, 2024, from https://medlineplus.gov/genetics/condition/ehlers-danlos-syndrome/

Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., Bloom, L., Bowen, J. M., Brady, A. F., Burrows, N. P., Castori, M., Cohen, H., Colombi, M., Demirdas, S., De Backer, J., De Paepe, A., Fournel-Gigleux, S., Frank, M., Ghali, N., … Tinkle, B. (2017). The 2017 international classification of the Ehlers–Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 8–26. https://doi.org/10.1002/ajmg.c.31552

Miklovic, T., & Sieg, V. C. (2024). Ehlers-Danlos Syndrome. In StatPearls. StatPearls Publishing. http://www.ncbi.nlm.nih.gov/books/NBK549814/

Song, B., Yeh, P., & Harrell, J. (2021). Systemic manifestations of Ehlers-Danlos syndrome. Baylor University Medical Center Proceedings, 34(1), 49–53. https://doi.org/10.1080/08998280.2020.1805714