Ehlers-Danlos Syndrome

"Ehlers-Danlos syndrome is a group of disorders that affect the connective tissues that support the skin, bones, blood vessels, and many other organs and tissues. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos syndrome, which vary from mildly loose joints to life-threatening complications." —Genetic Home Reference

Kylee P. is an extremely close friend of mine who has an extreme case of EDS, and she agreed to an interview about her experience having EDS.

*** This interview is Kylee's personal experience, her last name will not be on here for privacy reasons. No two people have the same experiences or deal with anything the same way. This interview should not be used anywhere without my consent or Kylee's. ***

Bailey: Please state your name and age.

Kylee: Kylee P. and 19 and a half.

When were you properly diagnosed?

Kylee: Probably the summer of 2011.

Leading up to that what were your symptoms?

Kylee: From the age of 9 on I started having chronic spraining ankles, which turned to chronic dislocating ankles, and then because I was on crutches chronic dislocating shoulders.

What does, or did, EDS do to you specifically?

Kylee: EDS is a spectrum. I'm on the very high end of the spectrum. Almost every joint in my body dislocates from toes to bigger stuff like hips, my jaw has locked out of place before. I think the biggest problem is when it comes to my vertebra, they slide around and that creates problems with my spinal cord, my brain stem and things like that. But other illnesses can come along with it play a big part in MY EDS.

And do people with EDS typically have other illness like yours? Mast cell, Pots and Chiari?

Kylee: A lot of us do. When I see my neurosurgeon a lot of us are there at the same time. A lot of people suffer really bad from at least POTS and Mast cell but you see a lot of Chiari patients as well.

But they don't have to "go" with EDS?

Kylee: Not always.

How many surgeries have you had total? I know you've had more than 10.

Kylee: 14.

And if you can remember all of them, what were they for and did they work?

Kylee: Shoulder reconstruction, three of them. I'd say they worked pretty well. I think I've had six spinal fusions. And the other ones are similar to those.

How far does that go down your back?

Kylee: From my skull all the way down to T8, which is below my shoulder blades.

About how many medications are you on and generally what are they for?

Kylee: I take over 20 pills a day. They're mostly allergy pills, heart pills, pressure medications, acid reflex stuff.

Are you on any anti-depressants?

Kylee: Yes.

Did that start before or after your EDS?

Kylee: Before.

What's the longest you've been in the hospital?

Kylee: I think almost six months straight was the most.

Was that the most recent visit/stay?

Kylee: Yeah. Then we went straight to New York and stayed in the hospital in New York.

How many doctors have told you that you do not have EDS?

Kylee: It's mostly ER doctors and sometimes geneticists that know what they're talking about but probably over 15.

And how many doctors won't deal with you because EDS isn't well known, they think it's something else and try to treat that?

Kylee: Many. MANY. Many, many, many.

More than the 15 that have told you EDS isn't real?

Kylee: Yeah, and even if they do think that it is EDS, they realize that it's complicated and still don't want to touch it. It's really hard to find specialists.

So you're high on the spectrum, you have severe EDS right, to clarify?

Kylee: Yeah. Pretty severe. The only thing that could really be more severe is if I had vascular type, because that's like random organ rupture and skin rupture. So at least I don't have that. For hyper mobile type, as far as that goes, I've traveled across the country to see the two specialists. One said I was the worst case he's ever seen and the other one said "don't worry, you're only the second worst."

How has EDS, along with your other conditions, effected you emotionally?

Kylee: Emotionally... It's been difficult, maybe isolating, but overall it kind of makes me a stronger person. In the way that I know how to deal with situations even on my own because sometimes I'm by myself. I can't go see anyone, I can't go be with anybody. People still have life even when I can't. So I've learned to deal with things on my own but also with other people.

How has it effected your family?

Kylee: My family has had to give up a lot of time together, I think is the main thing. When we go to specialists in L.A. or across the country then we're split up a lot. Also financially it's a BIG thing.

Because of the surgeries and specialists?

Kylee: Yeah, and special flights. I mean, my insurance pays almost a million dollars for Angel Flights and I've had like five of them. I'm probably the most financially expensive patient that they have.

Is that another reason why doctors won't touch you?

Kylee: Probably. I have very good insurance but theyr'e still, like, wary of how much will rack up.

What about your friends? Have any of your friends left you because they don't quite understand?

Kylee: I think a lot of it is like people don't understand last minute changes. So, last minute I won't feel well enough to do something or last minute I end up in an ambulance going somewhere. People don't understand that. But also there are people who, not really just walked out, just over time distanced themselves because either it's just really hard to be there through that when your friend is really ill and you can't do anything to help. Also, like, people are in college in other states, full time work or school, just busy and if I can't go out and be with them out there, it's sometimes hard for certain people to come.

What has helped you with the hospital stays, the surgeries and day to day hardships over the years?

Kylee: Definitely my family, I have a really supportive family. All of my siblings have come visit me, we've had to sneak them into places they shouldn't be in, like ICUs, because they weren't old enough. We get everybody, my friends as well, I have a lot of friends that stay with me or bring me stuff or just talk with me if they can't physically be there. I have a lot of support that way. Also, my art has really helped, I've been able to develop that even from a bed (Kylee designed a tattoo on my forearm, it has the EDS zebra stripes and self harm support semi colon, from Cedar Sinai).

What is EDS to you, if you could describe it with one word?

Kylee: Complicated.

I know this is a tough question, I think I know the answer to it, who is the number one person who has been there through all of your issues that has supported you and has just been there for you?

Kylee: My mom. She's always there. She's been there through everything. She's stayed with me in the hospital. Also just my grandparents, both sets. Financially, physically, and emotionally, in every way they're there. I'm really lucky.

Because EDS is improperly diagnosed and almost unknown, I mean I've tried explaining your illness to my coworkers, family, and EMTs, they don't know what it is. My uncle who was an EMT can really only understand if he's reading about it. EDS isn't well known at all. What would you do to bring awareness, besides educating doctors more?

Kylee: I think that people spreading awareness and putting it into the simplest terms. (Kylee was having a small allergic reaction during this answer. Just FYI, symptoms can happen at any time, again the last minute problems.) Just spreading support, my family and friends really spread awareness and support. My mom started a support group and not just patients come, it's caretakers and other people. Being able to describe it in simple terms, general terms.

Do you think that people spreading awareness will make EDS well known and help/ make doctors understand that this is an issue, that it is real and that they need to know more about EDS and keep them from improperly diagnosing?

Kylee: Absolutely. Right now there's more information going into medical schools. Like, 10 years ago medical students were coming out with only half a page of knowledge about this condition that is spectrum. And now we find out that it's more common than autism. And that's huge, so I think that the more people talk about it and actually look at their symptoms, the more people are going to get accurately diagnosed and more doctors will care about accurately diagnose.

Also not all people with disability placards have obvious symptoms. Because I know Kylee so well, I know that sometimes she can't walk far distance all the time, having a closer space makes her life so much easier. And that goes for all illnesses or body issues. Do not judge people just based on a first glance. Has having a disability placard affected your life badly because you don't show obvious signs?

Kylee: We have been yelled at, especially in New York, people are so angry. I was walking, and it was fine, but it was hot outside. And if I'm walking and it's hot outside I'm probably going to drop. And people don't understand that. So yeah, I've gotten yelled at. One of my friends got their entire car plastered with sticky notes that said FAKER on them while she was in a doctors appointment in Florida. It's just that people are so quick to judge and you never know. When it happened to me in New York my grandfather said, "oh my god. I used to think that in my mind before you got sick the way that you did. I feel so bad that I even though that."

Tell me what you've been trying to do with the disability department and trying to get on disability.

Kylee: Social security/ Disability is one of the most dysfunctional programs of all time. People try to take advantage of it so much and I get that. But with a legitimate medical condition you have to go through hoops of fire to be able to prove your disability. I've given them the numbers of my specialists across the country, all of my surgical records and all of my hospital stays and I was still denied. I was told that although my disability is hindering it isn't technically disabling. And I was 80 days into my stay at Cedar Sinai at that time. Like yeah, I can really work. Right now I was offered a position for part time work and it would not be even enough to really do anything but I was advised not to even work that until I'm approved. Even if I'm working five hours a week, to disability, it's going to "prove" that I can work if I wanted to. It's really screwed up. So I have to wait until I get approved and log my hours and get deducted what I'm being paid. Which is fine, it's just so much time, I've been in this process for over a year and a half now.

So you can't even work from home?

Kylee: If I'm getting paid I could be working.

How would you really be able to find a job, having EDS and uncontrollable symptoms sometimes?

Kylee: It would be hard. It would have to be with someone I know. If all of a sudden I need to go to the hospital or I have to leave and medicine needs, they would understand. Otherwise I'd probably get fired or have to explain all my medical needs all the time.

*** Please remember that not everyone with a disability placard shows signs of their disability. Just because you can't see the problem doesn't mean it's not there. They could have someone in the car that you aren't aware of. Don't judge.***

Kylee is very high on the spectrum, but if you have any of the similar symptoms please go to your doctor and ask about it. Get all the tests done and make sure that you do or do not have it.

Thank you for reading! We need more awareness about EDS!