Hello lovely people, today we’re going to be answering that age old question: “what’s
wrong with you, Jessica?” If you’ve seen my ‘I was misdiagnosed’ video, you’ll
know that I now have a better idea of what that actually is!
Many, many years ago I made a video called ‘my disabilities’ that explained a bit
about my disabilities
- you can tell it was made a long time ago because both the production values and my
eyebrows are terrible.
Subscribe to support my much better (newly microbladed) eyebrows
[thumbs up, ding]
At least, that video told you more about the disabilities I had been diagnosed with at
that point: Hereditary Neuropathy with Liability to Pressure Palsies and Mixed Connective Tissue
Disorder. The first of these is diagnosed via a blood test and DNA sequencing- I’m
missing a gene, so even a computer can spit that result back at you- but the second one
was diagnosed by my rheumatologist. Who apparently was wrong.
- I drop the word ‘rheumatology’ into videos all the time like it’s as common
as the word ‘egg’
Rheumatology is the study of immune-mediated disorders of the musculoskeletal system, soft
tissues, autoimmune diseases and heritable connective tissue disorders. If you have one
of those and live in Manchester you may have been treated at the Kellgren Centre for Rheumatology,
which is named after my grandfather, who was Britain’s first professor of Rheumatology
and greatly advanced the study of the physiology of pain, which is something I deal with every
day, because irony knows no bounds.
- please feel free to argue in the comments about whether or not that is actually irony
and what irony even is, points for dissection of Alanis Morrisette’s song.
So my doctor thought I had Mixed Connective Tissue Disorder and I thought I had Mixed
Connective Tissue Disorder and the internet was like... ~meh~
People left me comments saying “I think you have EDS” and I made friends with EDS
who said “I think you have EDS” so I went to a new rheumatologist who said “you definitely
have EDS”
It was obviously a much longer and more dramatic tale than that but I talked about it in the
misdiagnosis video. THIS video is about my NEW diagnosis.
Aaand this seems like a great point to throw in that I’m deaf but no one knows why. Put
a pin in that one. But I do keep getting tagged in Twitter conversations about the high crossover
of people with hearing loss and EDS so…
- [sips drink] interesting…
Let’s start with my first and much easier to understand congenital disability: HNPP,
Hereditary Neuropathy with Liability to Pressure Palsies, otherwise known as ‘I’m missing
a gene so the protective coating around my nerves has holes in it which means my nerves
damage easily and I can temporarily paralyse parts of myself’. Basically, if I trap my
finger in a door, it paralyses. If I sit on my crossed legs for too long, they paralyse.
If I hold my unsupported arm in the air whilst painting… it paralyses.
It’s a highly irritating condition that has the incredible knack of popping up exactly
when I don’t want it to.
Got a party? Watch me paralyse something!
First day of a holiday? Watch me paralyse something!
Need to open a fun parcel with some scissors? Ha! That paralysed my hand.
HNPP doesn’t just lead to floppiness and numbness though, it also makes my nerves randomly
fire pain signals at me and read things like cold water or a splinter as “you are being
murdered!”
But at the same time it can work the other way and I won’t notice that the skin on
my arm has been flayed
(that actually happened)
or that I’m walking around on a broken toe.
A thing that happened.
So at least it’s interesting!
And because it hurts so much I take a really huge dosage of pain medication that I then
put into these adorable pill cases. They make a rainbow and they’re fun. I’ll leave
a link to them in the description.
- was it a reach to find that particular silver lining? Well, it’s an affiliate link so…
looks like HNPP is being helpful after all! [wink]
So that’s Condition 1.
Then we come to Condition 2, which is a little more complicated and, as you’ll know if
you watched my video on Tuesday, quite misunderstood...
EDS, which is short for Ehlers-Danlos Syndromes, is a group of thirteen individual genetic
conditions that affect the body’s connective tissue. Think about connective tissues as
being the glue that holds your body together. The human body is composed of four basic types
of tissue: nervous, muscular, epithelial and connective. Connective tissue is the most
abundant as it connects, supports, binds, or separates other tissues or organs. From
fibrous tissues, fat, bone, blood and bone marrow, this stuff is everywhere! The most
abundant fiber in connective tissues is a tough protein called collagen. Tendons, ligaments,
and the white stringy tissue seen in some cuts of meat are composed almost entirely
of collagen- it’s not just something adverts tell you your skin is missing. Collagen also
strengthens bone and cartilage.
- and having strong bones and teeth is much more important than not having smile lines
so please don’t spend extortionate amounts of money on skin care with flakes of gold
in, you deserve better than that
and you’re already gorgeous.
EDS is a gene mutation that causes a certain kind of connective tissue to be fragile and
stretchy. The kind of tissue it affects will depend on the type of EDS you have, but most
often it’s collagen. As collagen is present throughout the body, people with EDS tend
to experience a broad range of symptoms, although being hypermobile (ie. joints that stretch
further than is normal) or double-jointed and the effect it can have on your very stretchy
skin are the most obvious ones whilst tissue fragility in general can be hard to see from
the outside.
Because it is, inside your body.
I’ve recently become really annoyed at my inability to grab toilet paper properly.
Yes, there is a correct way to grab toilet paper. Let me demonstrate:
Most people would make a pincer motion with their thumb and the top of their finger but
for me that finger is just too ouch, wobbly.
So I have to make a pince motion between my thumb and the first joint of my fingers.
Like so.
And that would be fine… if it didn’t make me sick to my stomach every time I do it.
Helpful(!) Glad it’s not something I have to deal with every few hours (!)
- how dare you.
Other than being made nauseous by toilet paper, symptoms include long-term pain, chronic fatigue,
dizziness, heart palpitations and digestive disorders. Since, you know, there’s a lot
of connective tissue down there!
EDS can hit you in very strange ways, to the extent that most people have a range of really
weird symptoms that don’t seem to connect or make sense until… you find out you have
EDS. For instance I’ve always been allergic to random things: trees, wool, rabbits, grass,
cats, dogs, glitter, caterpillars… aloe vera… to name just a few. But my reactions
to them are all over the place! Sometimes cats make my eyes itch, sometimes they make
me really need the loo, sometimes they make me lightheaded and sometimes they make my
skin dry and itchy.
- I know, I’m a lesbian who doesn’t like cats, I’m surprised the community hasn’t
ostracised me yet!
Fun fact: EDS and allergies are related. [bell sfx] so leave Jameela Jamil and her peanuts alone.
Mast cells are a migrant cell of connective tissue that contains granules rich in histamine,
which is part of your immune response, and heparin, an anticoagulant. Mast cells play
an important role in your immune system BUT if you have connective tissue issues they
can become over-responsive and do weird things when you come across an allergen. These responses
include: itchy skin, pain in the abdomen, fainting, blood pressure instability, gynecological
bleeding, brain fog, shortness of breath, bone pain, nausea and… needing to pee. Because
you are allergic to something.
Mast cells interact with connective tissues in the extracellular matrix, which may be
why people with connective tissue disorders such as EDS experience mast cell activation
syndrome (MCAS).
And the ‘allergens’ don’t have to be obvious things like animals or insect stings-
exercise or high emotions can also trigger an immune response.
- I mean… used to tell my gym teacher I was allergic to exercise… turns out I wasn’t
that wrong...
Despite the grouping and their common name, each of the 13 types is a distinct condition
caused by a different gene mutation. Meaning that one type cannot later turn into another
and you can’t inherit a different type to the one your parent has. Funnily enough, since
they’re each so rare in their own special way, the likelihood of someone having more
than one type is extremely low!
The type I have is called Hypermobile Ehlers-Danlos Syndrome or hEDS (with a little h and a big
EDS)
hEDS is characterized by hypermobility that affects both large and small joints (I can’t
really show you, is just my arms)
along with soft, smooth, velvety skin that bruises easily, and chronic muscle and bone
pain. It creates faulty connective tissues throughout the body and musculoskeletal issues
which lead to frequent joint subluxations (that’s partial dislocations).
Fun thing, that happens when I open my mouth too wide. Which is every time.
Cool. Partially
dislocating my mouth when I eat.
And then actually dislocations when your arm just pops off. It happens to my right shoulder
a lot, and also my hip.
What's wrong with my right side?
It also created my scoliosis- the double sideways curves in my spine that mean I’m at least
three inches shorter than I should be…
And I’m already 5’9”! So thank goodness my spine is curved or else my wife would have
to stand on her tiptoes to kiss me!
I have the ‘Marfanoid Phenotype’, which describes the type of body I have. It means
my arm span is longer than my height.
And I can wrap my hand around my wrist and do this with my thumb (which I genuinely thought
everyone could do this but apparently not) and I have a small jaw with a high palate.
I’ve had six teeth taken out and braces twice but my teeth are still crowded. It also
apparently makes me ‘giddy’ (which is an actual medical term) and imbalanced- which
explains why I spent the majority of my childhood falling flat on my face.
- honestly, it’s a marvel my nose still sticks out this far from my face.
Oh, and flat feet! Which is why I actually find it much easier to walk in heels than
with no shoes on. Because my feet are floppy and flat they don’t know how to roll and
then lift from the ground.
So yeah plus sides include: an excuse to own an excellent shoe collection, being able to
see over other people in a crowd and my jaw popping out when I open it, which… really
just means I can fit more food in it so yay(!)
Negatives: may have an impact on my heart in the future. I laugh but by body really,
really hurts pretty much ALL of the time, just ALL of the time for no reason. My bones
hurt. Sometimes bones I didn’t even realise I had.
Soft tissue is really everywhere in your body so EDS and the faulty proteins in my tissue
affect every part of me. It’s why I have a side condition called
Postural Orthostatic Tachycardia Syndromme (which I’m going
to leave the link to an explainer video about in the card above) which is a malfunctioning
of the autonomic nervous system that also affects my heart and blood pressure, which
then has the knock on effect of making me quite… delicate.
- Yep, no getting away from it: I’m a delicate flower with a core of steel!
By which I mean being disabled just makes me quite a strong person metaphysically.
Definitely not literally.
[struggles to open a jar]
Other rebellious soft tissues in my body include my stomach and guts, which react very, very
badly to the majority of grains, quite a few root vegetables, definitely anything that
resembles a bean or pulses, absolutely hates the entire onion family, has a standing war
with the garlic family, is the nemesis of dairy and, oh, can’t be doing with sugar
either.
And I’ve probably forgotten some other things I can’t eat.
I used to struggle a lot with my stomach and it was really hard keeping weight on. It still
intermittently bleeds when I eat the wrong type of gluten free bread or makes me throw
up if I have a pad thai with too much sugar but I’m definitely more on top of it than
I was.
If you’re really struggling with stomach issues- especially if doctors put you in the
box of ‘teenage girl’- it can be difficult to get help. I struggled for a long time to
have my digestion issues taken seriously and be referred to the gastrointestinal ward rather
than the eating disorder one. The way I was treated has left a pretty big scar on my psyche
that I sometimes struggle to deal with… but you’ll find out more about that when
I eventually publish that book about my life. It’s something that’s much easier to write
down than say out loud without crying.
- I mean… I’ll still cry when I write it. You just won’t be able to see my snot!
Other than medical trauma, from going through a lot of near-death experiences and hospital
procedures, I’m pretty blessed when it comes to my mental health and I’m really grateful
for that. Those of you who struggle with mental ill health are absolute champions in my eyes
and I’m both awed and impressed by what you manage to get through.
The hardest thing to deal with for me are my 20 migraines a month, which my EDS doctor
thinks is caused by HNPP and my HNPP doctor thinks is caused by EDS and my primary care
doctor just throws his hands up and shrugs with a “that sounds bad”.
- yeah (!) it does (!)
My migraines are currently under investigation so I’ll get back to you when someone has
finally managed to work out what is going on with my brain. The main thing you need
to know is: it’s awful. According to one doctor my brain is having tiny little mini
strokes occasionally and that’s knocking out parts of it… like the bit I need to
process sounds, which might be why I’m deaf, and the part of the brain that processes whatever
my left eye sees, because currently its…
[left eye effect] This. Hi. Yep, this is what life looks like through my left eye.
Blugh! Always makes me feel sick looking at that.
Anyway, I had a lumbar puncture when I was 17 that went wrong and bled out my spinal
fluid so my brain got damaged and now I have problems with memory and cognitive functions
so oh well it was kind of a weird brain anyway.
I am really good at visual awareness though and rubix cubes so it’s not all bad.
Unfortunately my memory loss doesn’t let me forget that I have memory loss and I’m
not sure whether that’s a good or bad thing because being aware I’m extremely forgetful
is desperately annoying (and for my wife when I’m telling her the same story for the fifth
time) but also forgetting I’m forgetting would probably be desperately sad...
Okay, no, I’ll just keep annoying myself! It’s fine…
Bringing this full circle back to EDS, since it’s the first time I’ve really spoken
about it and explained more: it’s important to remember that each individual’s experience
with EDS is their own and likely won’t be the same as other people’s. If you have
EDS or a similar condition and you’re feeling pretty alone with it just know that there
are others who deal with overlapping symptoms even if they’re not exactly the same and
that you can reach out for help. I have a Discord board with a thread for EDS and other
connective tissue issue friends so join up there and have a chat. I’ll leave the link
in the description.
I hope you’ve enjoyed this video where I essentially list what is wrong with me. But
I also added some things that are right with me for balance. And I think we can all agree
that my hair is looking great today! If you’ve made it to the end of the video, leave me
a gold star and tell me one thing that is RIGHT with you and we’ll all boost you!
I’d also like to give a shoutout to these amazing creators who have EDS:
Youtubers- Annie Segarra & Stevie Boebi
Drag Queen- Yvie Oddly
Singer- Sia
Blogger- Sarah Lex
Youtuber- Martina from @eatyourkimchi
Creator- Aaron Linguini
Actress & Presenter- Jameela Jamil
writer & consulting mortician- Ace Tilton Ratcliff
Which is just an excellent name and job title.
and Model- Sara Geurts
See you in my next video!
[scratch record sfx]
So amusingly right after I filmed this video
I managed to smack the inside of my elbow and now I got this to deal with:
Yeah..
I can only move my two main fingers!
And I had to dig out
My good old fashioned
wrist splint which to be honest it's a little bit manky right now so
Yeah.
But do you like my pyjamas?
Aren't they lovely? I had them custom made from Etsy.
I'm going to leave a link in the description.
It's also an affiliate link because now I need to buy
A lot of new wrist splints...
[whispering] Why...?
[cheerful jazz music]