I was trying to wait until we had more definite information and a firm plan of action until I posted an update but so many of you have been asking how I am (thank you for caring!) that an update is needed.
In case you don’t follow my twitter or facebook feeds, the following was posted on them on Friday 19th October:
As of yesterday, Jenny is in hospital again. It’s the worst place for her but after over 9 months of only being able to put her head down on her pillow for 2 hours per 24 hours because any pressure/touching of her head causes increasingly deep numbness and blood reduction to the head, causing her to start to black out unless she takes her head off the pillow, she couldn’t carry on anymore and is completely broken. That level of sleep deprivation is unliveable. She’s been superhuman to carry on as long as she has. The trouble is no doctor has heard of these particular symptoms before and can’t understand what is causing them; she is a rare and unusual case so they’re a bit at a loss. They’re going to try a cervical-thoracic back brace to see if that helps, as it’s a less drastic option than the surgery that the specialist doctor in Spain (one of the only doctors in the world who specialises in these problems in patients with Ehlers-Danlos Syndrome) thinks she needs (but that surgery means she would lose 100% movement in her neck for the rest of her life), but the brace will take a while to be ordered. Jenny doesn’t have time as she’s struggling to hold her neck up to keep her head off the pilllow so that she doesn’t become severely mentally incapacitated or braindead from blood being cut off when her head touches the pillow. The Spanish doctor and neurosurgeon here also want another upright MRI to see if the cervicocranial instability, reversal of lordotic curve and Chiari malformation have deteriorated since the MRI in February. They also want to see further down her spine to see if there are additional problems there. But an upright MRI is specialist equipment and booked up months in advance so we’re waiting to see if it’s possible to get an emergency appointment or not. It’s a dire situation and Jenny is in a bad way. The ambulance journey and loud hospital environment is too much for her but she had no choice. She has never been able to sleep at all while in hospital so she’s not even getting the two hours per 24 hours. She’s really struggling to keep going on and keep her head off the pillow while on no sleep at all and is not very with it. She no longer can put in the work of holding her spine and neck and head in certain positions which she had to do for 22 hours a day for the last 9 months, which returned just about enough blood to her head to safely go down on her pilllow for two hours. But she has no strength left to do all that anymore, which is why she had to go to hospital, although there’s no help with this yet. Medicine just doesn’t have the answers to this yet. She is a very rare case and there are no doctors in this country who specialise in it. It’s hard to get the head around that the chances are far far more likely of winning the lottery than of having what Jenny has.
We were able to get an “emergency” appointment on the Monday for the upright MRI and from there I went straight home as hospital wasn’t an option because not only could they not help me, I got zero sleep there and couldn’t cope with that, plus the level of noise of being in hospital and also the staff constantly coming in and out of the room. There was no point being there, even though I’m in such a bad way. They couldn’t save me.
I was in the upright MRI machine for three hours and after just over two hours of it touching my head, I blacked out and fell forward out of the seat. Thankfully, the radiographer was standing right next to me throughout so he managed to catch me. At least I have proof that I do black out when something is touching my head for about two hours for all the doctors that disbelieved me.
The report from the upright MRI has come and in addition to the craniocervical instability diagnosed from the previous scan, atlanto-axial subluxation and instability (AAI) were also found, which is serious (click here for more information on AAI). There are only 3 doctors in the world (one in Spain and two in New York) who specialise in this in EDS patients. The one in Spain we were already a bit in contact with for the craniocervical instability and when he saw the scans he sent a one sentence email confirming the atlanto-axial instability and subluxation, saying that he wants to operate. The next step would be a Skype call with him for a proper consultation, although I cannot speak so Mum would have to do the talking. The first available appointment for this Skype call isn’t until 27th November so I somehow have to hang on until then. He’s very in demand. In the meantime, we have a lead on one surgeon in the NHS who used to do this particular surgery for EDS patients but we’re not sure if he still operates or has stopped. There is literally nobody else in the UK. It’s so hard to get information and doctors are so difficult to get hold of and it always takes so long for them to get back to us or take action. There is a multi-disciplinary team slowly being set up in this area of expertise in the UK but it will be too late for me and the surgeon has never done this operation before; he’ll just be starting out when/if it does get up and running. I’ve been on the waiting list since January.
It all seems a bit impossible. The journey to London nearly killed me, let alone going to Spain and I would have to raise a ridiculously huge amount of money (probably through GoFundMe) if I were to go ahead with the operation for both the operation and Medevac air transport and hospital stay. It would cost extra as well because the vEDS means the surgeon would need extra insurance because vEDS patients often die during surgery.
Obviously if the surgeon in Spain says that I’ll die without the operation then I’ll do everything I can to raise the money to get it done.
The thing is, even in a previously healthy patient, I think the operation has a 10% chance of complications/death so goodness knows what such a weakened patient from M.E. and the dangers of vEDS would add to that. Another problem with the operation is that usually for atlanto-axial instability, the surgeon would put screws in the head/neck and put metal in to fuse the upper bits of the spine and anchor it to the vertebrae below but because I have craniocervical instability too (right below it) and degenerated discs, he would have to fuse it to quite a long way down (C0 to T1 for those who know about the spine), otherwise it would be too unstable. So I’d lose all motion in my neck forever, which is too permanent for my brain to take in at the moment. I wouldn’t be able to look up or down, left or right. And even then, we don’t know for sure that it’s this atlanto-axial stuff that’s causing the numbness upon touching/pressure on my head (the latest theory is that it’s a severe case of Cerebrospinal Fluid Stasis (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2842089/) when my head touches anything) so if I have the operation and it doesn’t fix that problem, I’ll be in big trouble. And I’ll be much more disabled after the operation and my care needs will be more than my parents can handle by themselves. According to the surgeon, the pain after the operation would be so great that it will feel like you’re in a dark tunnel for weeks, unaware of much else or other people. Which makes sense, given what is being done to the body. It’s likely that I’d be on a ventilator and life support for a while.
We’ve found the most knowledgeable doctor in the UK on this condition (he’s not a surgeon though) and he says that the operation would be necessary in my case to prevent death but did suggest a chiropractor (there’s only one chiropractor in the UK who he would trust to do this dangerous a thing) to try first before having surgery but that any adjustment the chiropractor made was unlikely to stick and stay in place, given the EDS (even though I have vEDS, all these symptoms means that I have some overlap with other types of EDS). We have since been advised though by various experts that with atlanto-axial instability and subluxation, chiropractic adjustment is extremely dangerous (as in dying type of dangerous) and should be avoided at all costs. So the experts are contradicting each other a bit. We have contacted the chiropractor but he hasn’t agreed to take me on yet and wants to see all my scans and all my other medical information first.
So I have a big decision to make on whether to try the chiropractor or go straight for the surgery. I really don’t want the surgery or the chiropractor but I can’t carry on like this anymore (I don’t know even if I can last until 27th November but I’ll try my best) and something needs to be done.
Sorry for this badly-written, muddled explanation of an update. I’ve struggled to find the words with all the head problems I have. I thought it was better to have a badly-written update than no update at all. Thank you so much for all your love and kind messages. Sorry that I’m too ill to reply to you individually.
Jenny xxx
P.S. One of the radiographers liked my t-shirt this time round. Here it is:
He also thought I was 25 and one of the ambulance crew thought I was 22 and refused to believe I was 32, which was quite funny. Maybe it was just the t-shirt or maybe good lighting. It’s weird because I think that my face has aged a LOT this year.