Interview: Living with hEDS and POTS

Hello everyone, this is an interview about ‘Living with hEDS and POTS’.

Our interviewee is xxParkerxx and they are happy to answer any questions you might like to post regarding their condition.

Thank you for taking the time to answer our questions so we may better learn and understand what life is like with hEDS and POTS. It is our pleasure to find out more.

1. What does hEDS stand for?

Hypermobile Ehlers-Danlos Syndrome

2. How did you find out you had hEDS?

So it’s actually kind of a funny story. In my 7th and 8th grade years, I had a lot of injuries. In my 8th grade year, I picked up gymnastics again and that’s probably when things got pretty bad. So my mom had posted a video of something I was doing for gymnastics to her ***, and she had been keeping family apprised of things and every injury just because they lived far away and wanted to know. One of her high school friends sent her a private message saying I looked hypermobile and asked if she had considered an hEDS evaluation. Her friend sent us the Beighton Scale and I scored incredibly high on it. So we talked to my doctor, who referred me to a geneticist. My geneticist was actually really surprised that I hadn’t been diagnosed before then, given my history. I walked away from that appointment with a diagnosis of hEDS and suggestions for specialists.

3. How does it affect you?

When I was first diagnosed it honestly wasn’t a huge impact. I was injured more often than others and rehab took longer, wounds took longer to heal, and things dislocated more frequently, but that wasn’t anything I wasn’t already used to. As time went on though, it’s had more of an impact on my life. I actually know how to fix a dislocation of every joint in the arms and legs (fingers and toes included), that can possibly dislocate. Every time we go in, they teach us how to fix whatever is going on at home because doctor appointments are expensive and time-consuming. At this point, I’m in pretty widespread pain on a daily basis, with multiple dislocations on a weekly basis, weak joints overall, muscle spasms, and needing to wear various braces for support of my joints on a daily basis. I also have a severe progressive astigmatism and lack of depth perception due to my hEDS. What this means is that my eyes are misshapen, and will continue to become more misshapen over time due to the tissue in my eyes being stretchy because of the hEDS. I wear hybrid contacts, and those help tremendously, but my vision will never be perfect, even with aids. They aren’t yet sure if they’ll be able to do LASIK due to my hEDS but I’m hoping by the time I’m old enough to get LASIK they’ll have more research and be able to give a definitive answer.

4. What is POTS?

POTS stands for Postural Orthostatic Tachycardia Syndrome. At its core, it is a condition of the autonomic nervous system, where the body struggles to regulate heart rate, which causes issues with regulating other bodily functions. When I change positions, from sitting to standing, lying to sitting or lying to standing, my heart rate increases by an abnormal amount, and takes a while to come back down. This causes things like dizziness, syncope, weakness, brain fog, and pre-syncope. POTS also causes issues like temperature regulation, dehydration, low sodium levels, low vitamin and mineral levels, and either excessive sweating or a lack of sweating. I specifically have secondary, hypovolemic POTS. This means that my POTS is caused by low blood volumes and my hEDS causing blood pooling, thus not allowing my heart to get the blood it needs. POTS can also cause a phenomenon known as POTS anxiety. Researchers aren’t totally sure why this occurs, but it has been found that medicating the POTS symptoms using beta blockers can be helpful in reducing anxiety in POTS patients.

5. How did you find out that you had POTS?

My 7th grade year started getting dizzy. I was a high-level soccer player at the time, so this caused lots of problems. I was dizzy pretty much whenever I was upright, and it was worse when I would change positions, even to the point that when I would stand up, my vision would go black for a few seconds. We went to the doctor. They did a brain MRI, thinking it was either a tumor or a Chiari Malformation, but it was neither. So then my neurologist thought maybe it was migraines. We tried every migraine medication that we could think of. The problem was that none of these touched my symptoms, and some even made them worse. I, needing answers desperately because I was sick of not knowing why things were happening to my body, went to Dr. Google. I put my symptoms into google and up came both POTS and Orthostatic Hypotension. I did what’s called a poor man’s tilt table test, or a poor man’s TTT. This involves taking your heart rate, blood pressure, and blood oxygen at various positions. The results of that showed that I most likely had POTS. I showed my mom, and she agreed. We asked my doctor to refer us to a cardiologist for the diagnosis, and she did. The cardiologist did a poor man’s TTT and an echocardiogram, and confirmed a POTS diagnosis. 

6. How do the two syndromes affect your life?

The combination of the syndromes actually makes some things really hard. I can’t hold my arms above my head for too long, because the blood will drain from them and my shoulders will dislocate. Typing or writing for long periods of time is difficult, because my fingers will dislocate. Running, jumping, or turning quickly are no-nos unless I have braces on to secure my joints and prevent them from dislocating. I have to sit when I shower because the blood pooling and dizziness and high heart rate can cause me to pass out and hit my head, which is dangerous. I have to drink a ridiculous amount of fluids and consume much more salt than the regular person. I have to do joint strengthening exercises at home. I also have a progressive astigmatism that they think is caused by my hEDS. They also have to test my hearing every year as hEDS and the syncope (passing out) from POTS can cause hearing loss. I’m limited on physical activity, and I’ll need mobility aids somewhat soon.

7. How do the two syndromes affect your school?

I actually have a 504 that is currently being transitioned to an IEP. A 504 is a disability plan through Section 504 of I believe the rehabilitation act. Basically it is meant to give baseline accommodations for the classroom. An IEP is what is known as an Individualized Education Plan, and it allows for more individualized instruction and more intensive supports in the classroom. I have some accommodations for school, mainly for gym class. Teachers in regular classes have to have something for me to prop my legs up on to mitigate blood pooling, and a stool for me to sit on at lab tables if lab work is necessary. In gym class, I am allowed to self-pace and modify if necessary. This means that if my knee is bothering me and I don’t feel like I can do gym, then I sit out. If I’m too dizzy to do gym, I sit out. I can also leave classes 2 minutes early to allow me to move a bit slower without being knocked into in the halls if necessary. I’m allowed an alternative testing site on big exams too for when I have bad brain fog or bad anxiety. I do find that I’m slightly ostracized in some classes, but I have great friends who support me and are willing to help me with whatever I need!

8. How can you support someone with these conditions?

That’s really a difficult question to answer, because both of these conditions are usually what are known as invisible disabilities, and they affect everyone differently. For myself, if you see me struggling with something, come up and see if you can help. If I look disoriented, walk up and ask if you can help. If I look injured, offer to call someone for me. If I am unconscious, for the love of all that is good and holy, don’t call an ambulance. If you know the person has POTS and they don’t appear to have injured themself by passing out, wait for them to come to before deciding what to do. People with POTS can have what is called convulsive syncope, where they twitch a bit while they are unconscious. Obviously, if they appear to be having a full seizure and they don’t have a history of this, please call an ambulance. But yea, how you can support someone will vary from person to person, but please don’t hesitate to ask someone if they need help, most of us living with invisible disabilities are super nice and would actually love for someone to ask if they can help. We frequently need help but are too scared to ask.

9. What else would you like to tell us?

While POTS and hEDS are somewhat rare, it is quite possible for someone to live a full and fulfilling life with these conditions. Finding the right friends, getting the right medical treatment, and doing things you enjoy can really improve one’s quality of life. Also, I’ve been in a pretty bad POTS flare recently, and it’s had me pretty fatigued. I will answer any questions that you may have, but it may take me a hot minute because I don’t have a real lot of energy. 

Some Resources:-

https://www.ehlers-danlos.com/ The Ehlers-Danlos Society is a great resource for those looking to know more about EDS in general and all the different types.

https://www.dysautonomiainternational.org/ Dysautonomia International is a great resource for those diagnosed with POTS, or those looking to learn more about it.

https://www.standinguptopots.org/ Standing Up To POTS is a great resource for those needing to learn about school accommodations or quality of life with POTS. They even have a podcast called The POTScast that is great!

Thank you for answering our questions, it has been really interesting and a pleasure to find out more about your life.

If anyone has any questions or wishes to offer support then please share in a post. We look forwards to hearing from you!