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”.