To think my husband is gravely ill at the wrong time?

[MyOtherCarIsAPorsche]

Masses of confusion.

Husband spoke to GP yesterday morning and described his chest pain. He was advised to call an ambulance. My husband refused because he didn't think he needed one - thought he had chest infection or torn muscle.

He went to A&E - was sat in a corridor from 10.30am - he had ecg, x ray and blood test. He continued to sit on a chair in the corridor all day.

He was told he was waiting for blood test results. These came back at 6pm. Then he had to wait for a CT scan - then rushed to resus.

Dissected aorta from heart level to naval level.

I was called to be allowed to sit with him because I wasn't allowed to be in A&E. Was then told he was being blue lighted to another hospital an hour away for immediate surgery. I asked is this time critical? The answer was - yes life saving.

Paramedics arrived and told me to set off to this other hospital. There were three paramedics. One said 'I'm not taking him because I can't use that bit of kit, I'll lose my job if something goes wrong'. No advanced paramedics available and no doctor available to go in the transfer.

I was 15 min into the journey and then called back to the hospital.

No surgery.

Trying all day today to get him transferred. Nothing available.

He's critically ill.

I'm out of my mind with worry.

There's a saying about not being ill on the weekend. The standard of care is not the same. The 24hr cover appears to not exist.

I feel like we're being fobbed off with poor excuses big style.

It's vital that you do. Preferably battered.

@Lougle

i now know what's for lunch 🤣

i've decided to be a blatant chocoholic whilst I can and return to being a secret chocoholic later, now I feel more confident that my husband will be coming home.

I've not been hungry much lately and it's been concerning with regards to what I can be actually arsed to do this past week.

i don't like being on my own - it's strange because I don't usually see him throughout the day and we're like passing ships during the evening as we both like to do our own thing.

It's just that he's not here.

most of our conversation is when we wake up and after that life gets in the way

it's weird locking the house at night - I'm not saying I've never done it, I just usually don't have to remember it.

it feels pathetic to be so entwined with him as to sit and wonder what on earth I would do without him

have I been cowed by the patriarchy as some on here would point out - can't live without a man?

I've been scared witless this last month - I would be lost without him and of course I would carry on and manage because of my children but it didn't stop me from being afraid of a bleaker future.

it's a wake up call to appreciate our mortality

and thanks to the genius of others dedicated to our well-being (@olympicsrock ) we will make the rest of it count

my stream of consciousness runs away with me

Just to say we are also in North Yorkshire and my FIL had a very very similar thing BUT we calked an ambulance who after assessing him took him directly to the regional specialist hospital. If the doctor says call an ambulance, listen to them!

@MyOtherCarIsAPorsche There is no reliable genetic test for hEDS, which seems to be what your daughters are currently diagnosed with? But it is possible that the consultant who diagnosed them missed another subtype of EDS, or that in fact there is some other connective tissue disorder in the picture. So it would certainly be a good idea to seek a referral as olympicsrock suggested.

Generally speaking, genetics does not see hEDS patients as it is considered a condition that can be managed easily in the community. But with the family history they ought to be willing to see your children and screen them for vEDS and cEDS (etc).

I would still suggest that your oldest daughter meet with a private cardiologist as this could be arranged very quickly, faster than any NHS referral. Even if she does have some other subtype of EDS, it sounds like autonomic nervous system dysfunction is part of the picture for her too.

@MyOtherCarIsAPorsche - I think hitting the chocolate and/or cheesecake is the very least you can do to be kind to yourself at the moment. You need the energy and can’t imagine that you give a monkey’s about shopping and preparing nutritious meals atm.
I am pleased that your DD’s DH is pushing her to speak to her consultant. Carrying twins IS a much tougher load for both the babies and mum. An EDS dx makes it a much higher risk again.
Her cardiac symptoms that did not disappear post-partum are a bit concerning, especially with the added load of pregnancy and the reported breathlessness. I think you need to maybe explain everything re your DH’s current situation, what HIS consultant has said about having all of your kids checked out, and all of the symptoms you have observed in point form with your concerns re anaesthetics, etc…and hand them to your SIL to take to the consultant. I am sure he will have his own list too. (Doctors love lists.)

@Innocenta

Yes - I also think cEDS

I have seen a research paper which said that brachydactyly (sp?) is a feature of classic/al EDS. She has 'normal' big toes but virtually no other toes - you have to really look to see the stumps. She HATES her feet.

when she was born I was so upset when I saw her feet. I said 'how will she walk?'

the paediatrician said that if the 'stumps' bothered her with regards to being painful when walking she could have them snipped off. I was horrified.

her feet are unusually broad (remind me of hobbit feet but I've not said that) - they don't look like they belong on the end of her legs! Finding shoes to fit continues to be difficult.

can classic/al be detected by genetic testing?

so it's a case of ruling out the more severe types and coming back to the hypermobile type when all other types have been ruled out?

we paid privately to get the original diagnosis - it's absolutely possible we could pay for genetic testing.

I would like us all to be tested.

@innocenta While it is likely that OP’s family has h-EDS, it sounds like they also have v-EDS, or Type 4. There is a genetic test for this type. A lot of people straddle more than one type of EDS.

@Fraaahnces

🤣

oh yes! I've had plenty of eye rolling when I've gone in with lists. 🤣

@innocenta While it is likely that OP’s family has h-EDS, it sounds like they also have v-EDS, or Type 4. There is a genetic test for this type. A lot of people straddle more than one type of EDS.

@Fraaahnces

🤣

oh yes! I've had plenty of eye rolling when I've gone in with lists. 🤣

@Fraaahnces

🤣

oh yes! I've had plenty of eye rolling when I've gone in with lists. 🤣

@Fraaahnces If you look at my post, I said:

"There is no reliable genetic test for hEDS" and that genetics normally doesn't see hEDS patients, but likely would see OP's children to assess for the other subtypes. I'm not sure what you're objecting to.

Also, referring to vEDS as 'type 4' is outdated terminology. Not a huge issue, but it can confuse people!

OP - yes, there are genetic tests for all types except hEDS. You should be able to access genetic testing relatively easily. Vascular problems can certainly occur with cEDS and even sometimes with hEDS (though less common).

@fUNNYfACE36

Aye - he's not one for making a fuss. He remains confident that he's not as bad as everyone else knows he is.

He's still convinced he'll be fine tomorrow - despite his setbacks. He's already asking to go home.

He's got a confirmed chest infection this morning - waiting for physios at the moment.

he once made a right old mess of his thumb when he sawed almost through it. He wrapped it up for the best part of a fortnight before he was told he now needed a small procedure because he refused to acknowledge that he needed it stitched in the first place.

Convincing him that he needs urgent medical attention has always been a sticking point. Up until the dissection he'd never been to our GP surgery and we've lived here 30 yrs.

i wonder if this phenomena has a name? - refusing to believe what others are telling you in medical terms.

could be some sort of oppositional defiance disorder?

@MyOtherCarIsAPorsche - my dad was like that. He broke his arm and his knee slipping on our hill. (Carrying a puppy I had rescued from a horrible man down the road - he didn’t want to risk hurting the puppy!) Both we’re patently broken. He didn’t want to go to the hospital. He just wanted to “Sit for a bit and it’ll be alright…” When the guy had a cold, everyone had to head for the hills! Worst manflu ever! (Luckily rarely more than 24hrs in duration, but we’d all be threatening euthanasia.) He ended up with MND, and I wouldn’t have wished that on anyone.

@Fraaahnces

i'm very sorry to hear about your father.

my own father had vascular problems which affected his guts (needed a stoma) and resulted in him losing his leg just below the hip. He died 3 yrs ago.

my mother, who would perform the most amazing party tricks, because of how hypermobile she is/was, has vascular dementia and is confined to bed, close to death at the moment - she's 91. She would perform gymnastics, zip herself into a shopping bag, clasp her hands in front of her and step through, taking her arms up behind her back and over her head - back to their start point. My younger daughter does this - she can also apply her own suncream to her own back and also apply self tan to her own back without missing a spot.

I would appreciate more movement in my shoulders at the moment because I have two frozen shoulders and still have quite limited movement on the right - for the past three years. Just as I thought I was improving I feel as though I'm starting the 'freezing process' all over again. Sleep has been elusive these past three years as the main problem I find is it's impossible to sleep on your side.

we all need putting in a bag and shaking up - as my grandmother used to say.

Wow I have not much to add but just wanted to say I’m in awe of your strength. I know you think you’re not strong but you really are.

Hello OP glad to hear that things are slowly improving.
It’s reassuring to hear that your daughter has a consultant appointment in two weeks time. Given that this is not yet 20 weeks , this is time enough to talk them about the potential for her to have a connective tissue disorder .

I agree with @Innocenta - better for DH and kids to see a geneticist who can look for various different Connective tissue disorders as well as EDS given Mr Porsche’s history abs you family history. Things are rarely straightforward.

in terms of Mars bars for breakfast - I recommend Lion bars personally . ( that was my ‘second breakfast’ today) xxx

@MyOtherCarIsAPorsche - my grandmother would have been a great yoga teacher too. She used to love asking for two fingers of scotch and then dislocating her wonky, ancient fingers and asking for “These two, please” and they’d be further apart than the glass was tall. She wanted to make it to 100 so she could get a letter from the queen (they even send them here to Australia) and be interviewed on the news so she could tell them that she attributed her longevity to having smoked for over 80 years. (Bad advice, children. It didn’t work as planned. She only made it to 97.)
My son is very bendy and can do the tricks your grandmother can. He loves to do the Namaste yoga greeting, but with his hands behind his back, and touching his head. He did that as part of his diagnosis and the GP screwed up her face and said “Eeeeew! Please don’t do that again!”
I have unraveling fingers and thumbs like my grandmother and ganglions galore. (Sexy) My party tricks involve thumbs that dislocate at really opportune times like while performing CPR on a limbless dummy during an assessment, (I passed anyway), fingers that pop out of joint when I press elevator buttons. (My idea of hell is being that person standing closest to the buttons in a very crowded elevator in a really tall skyscraper, when people ask me to press “28, please”…. Who knew there was another positive from COVID?) and having toes that twist themselves upside down when weight-baring. (Cos feet aren’t hideous enough already.) I also have hips that dislocate at the drop of hat. (Poor DH is a patient man.) I had calipers until I was eight, so my ankles are crapulous (that is now a word.). They are hypermobile in every possible direction. Ballet and ice-skating were always absolutely off the menu. I taught myself to walk when I was three by hanging onto the world’s most patient German shepherd and dragging myself up.

No one with EDS should be doing party tricks. Even if it isn't painful to do them at the time, it often causes more pain later - please warn known EDS patients not to do them.

@Fraaahnces

i'm chuckling at your patient husband 🤣

my oldest daughter's trick is a full front flip which propels her right over onto her back. Of course she doesn't mean to do it. If she's done a fair bit of walking for one day, and for instance she is rushing/walking quickly her hip will pop and throw her forwards and flip her over if she's got up enough speed. Of course we know it's not funny but it never fails to bring on the hysterics. It once happened rushing toward an escalator (going down) and she eventually managed to land on her feet that time - a bit spectacular. Then there was the day she landed awkwardly on her head and her neck bent at a peculiar angle. It was being filmed at the time - the A&E consultant was fascinated and could not get over how bendy she was. She had a couple of bulgy discs which had gone back in by the time she was discharged 4 hours later.

I have a cousin who got quite far with her ballet career because she was so bendy - it can have some advantages. Her sister, was a successful gymnast - they both now teach their craft. I was forced to go to similar classes but didn't like any other children so I stopped 😬

I knew I liked you @MyOtherCarIsAPorsche!!! Not liking other kids makes us twins from other hemispheres. Your description of your poor DD’s “Escalator Escapades” made me both wince and think of Mr Bean. I wonder if Rowan Atkinson has EDS. Wouldn’t be at all surprised. Same as most of the Cirque du Soleil Troupe. If you and DH ever get a chance to see them, you MUST go!!! (You’ll never look at clowns with simple abject horror again!)

This is misinformation. You can't diagnose people with EDS because they are visibly hypermobile. Benign hypermobility exists, and where EDS 'begins' is an unresolved question.

@Innocenta is absolutely right, EDS is so much more than bendiness. I have hEDS and the bendiness is the least of my problems. In fact, for some of my joints, the hypermobility is masked by the extreme muscle tightness that has developed as over-compensation.