Always look on the bright side of life.

I felt it time for an update (AKA ramble!) on my blog of what has been going recently – I’ve had another few weeks full of hospital appointments, physio, hydro etc. you know… the average wild life of a twenty year old.

As a result of being on such high doses of Prednisolone for long periods of time, I have adrenal insufficiency where basically my adrenal glands have become lazy as they’ve been used to Pred providing the necessary cortisol for so long. Last Monday I had a short synacthen test to see if my adrenal function has improved. If it hasn’t, I’ll be swapped from Prednisolone to Hydrocortisone and stay on that long term. If it has improved, I’ll be able to very slowly wean down (and hopefully eventually off) of steroids, but I have to wait for a telephone consultation at the end of next week to get the results and find out the plan going forward.

I also had a repeat video fluoroscopy which I just yesterday got the report of; the test is so vile and due to my bowel not working they couldn’t use barium, and so had to use Omnipaque which was so bitter! The report showed a moderate reduction in my swallow function, with some aspiration. I’m waiting for my SALT to come round in the next couple of weeks to see if anything else can be done to help me, if there are any other interventions such as my current use of thickener or if they will proceed with a PEG. Whilst there can be swallowing issues in EDS, there have been suspicions of me having MS in addition due to the nature of my problems, but I’m not able to cope with having tests for that at the moment on top of everything else so will just be something I’ll keep in the back of my mind.

I finally have positive port news, as my new port seems to be working – third time lucky! I have my bloods taken from it, can have IVs through it as I did for my short synacthen test, and I hope it’ll continue working and make my life slightly easier.

My bladder problems have been worsening; intermittent self catheterising was do-able, but now, no matter how often I catheterise, my bladder leaks in between. I have to use incontinence pads to deal with it but at 20 years old I find the situation devastating. I had waited for a urology appointment for months that was yesterday, but I got there and there was a mix up meaning the clinic should have been cancelled as the consultant wasn’t available. I had to sit and wait for around 4 hours, and was squeezed into the registrars clinic; it was a bit of a waste of time as I needed to see my consultant considering the severity and complexity of my history, so I’m hoping I’ll be squeezed into a clinic on 10th October, but I was told that they will consider Sacral Nerve Stimulation. If I am eligible, there will be a trial phase and my symptom improvement will be evaluated, although there is a concern of the wires migrating due to the nature of my connective tissue disorder. I’m not getting my hopes up and will wait to see what my consultant says, but if not a Supra-Pubic Catheter or a Urostomy will be considered.

I am still waiting to hear for a date for my ileostomy but I’m hoping if both operations go ahead and are successful, they will help stabilise things and give me a new ‘normality’ to adapt to.

I thought I’d end my post on a positive note (I know… it’s unlike me!) but we have to appreciate the good things in life. I was absolutely BURSTING with pride this weekend as my sister, Laura, completed the Great North Run. There was some complications with running it for EDS, so instead my sister did it for Crohn’s and Colitis UK, another charity that is very close to our hearts. Raising over £500 and being motivated herself to train for it, Laura completed the run and I could not be prouder.

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Port Problems are Persisting…

As you know, I went to UCLH yesterday to have my new port, which has only been sited for three weeks, looked at. The district nurses were struggling to flush it and couldn’t aspirate from it at all, and so knew something was wrong.

I went to the imaging department yesterday who did a chest X-Ray which showed the tip of the catheter attached to my port right in the apex of my heart. In the short while it had been sited, due to the defective tissue as a result of my EDS, the port had migrated and so when we tried to aspirate, it was simply suctioning to the bottom of my heart. As a result of the findings, the consultant decided he would have to entirely remove the port, and site yet another new one.

The procedure that I was just recovering from since having it done a few weeks ago had to be repeated, and this news was devastating. It’s not the biggest of operations, but it’s not without risk and, for me, healing is very slow and there are now another three incisions for me to dress and hopefully prevent from becoming infected.

With the nature of EDS in mind, the surgeon has sited this port in the middle of two pieces of scar tissue, hoping that it’ll hold better there and so function. Today I’m having a PJ day as I’m having to deal with the recovery process again and I’m exhausted from being up in the night with pain, but without the support of my family and bestest friend I could ever wish for I would have given up a long time ago.❤️

Port Problems

I thought with my new port-a-cath that problems would be a thing of the past…oh how wrong I was!

A fortnight after it was sited, the district nurses came round to access it to find that it was very resistant to being flushed and no blood could be obtained from it. After some further investigation, it is thought that my body has tried to reject the port so has formed clots etc around it and it has stuck to the wall of the vessel it’s in, creating a vacuum.

This means that tomorrow I’ve got to go into UCLH for an op to try and ‘strip the port’; basically go in via a vein in my groin to try to remove the clots etc and detach the port from where it shouldn’t be in the hope it’ll work. I’m only having sedation tomorrow as the local anaesthetic they originally offered doesn’t take with me having EDS – if it’s unsuccessful they’ll try again on another day under general anaesthetic and if that fails it’ll have to be removed and a new one sited. The least anaesthetic we can get away with the better as it often flares things up.

I’ve got to get through tomorrow, but I honestly feel like the whole world is against us. Surely our luck has to turn at some point!?

Port-a-cath surgery.

I’ve had this saved in my drafts for a few weeks so thought it was time to post…

On Wednesday 9th August, I went for my port-a-cath re-siting operation at UCLH. It was decided that the original port couldn’t be saved, and so it was planned to take my old port out and start a fresh with an entirely new port. I was so pleased that the procedure actually went ahead, as I’ve been told so frequently lately that I’m too ‘high risk’ for the operations that should help improve my quality of life. There was some uncertainty at the pre-operative assessment, but it was fortunately signed off by the anaesthetist after I sent in sufficient medical documentation.

Three hours in theatre later, and with three new scars, I finally have a new port. It can’t be accessed for 10-14 days until the swelling etc has gone down, so I have the District Nurses coming out in a couple of weeks for them to assess it. I hope that it is more successful and accessible than my previous one, and that going forward my weekly bloods can be taken from my port rather than the nurses spending way too much time trying to find a vein that they can get blood from, and when I’m admitted any IVs can be given via the port too.

To try to kill two birds with one stone, before having my operation I had an appointment with a colorectal surgeon. We had a chat as I really have exhausted all other options now and an ileostomy is the only way forward. The irrigation I’m doing isn’t always successful, I’ve tried all laxative regimes, and the only hope of getting a quality of life back will be to have the ileostomy. At 20 years old, this has been really hard to take; even more so as there really isn’t a decision in it, there is no choice. I’m on his waiting list, and I’ll see the stoma nurses in the meantime. There will be a stay in hospital and I’ll have to get used to living with a bag; we did speak about the problems I’d had with getting a surgeon to perform the operation, and despite the risks associated with my health issues, this surgeon was happy to take me on.

The thought of the operation is one I can’t face at the moment. I haven’t got over my port procedure yet I’m having to think about what else is coming just isn’t bearable, but one step at a time…

A piece of positivity.

As it is primarily about my health problems, my blog can come across as being quite depressing. The past few weeks have been the most challenging to me, being a constant battle with hospitals and the logistics of the NHS to get the operations/ procedures I need done and to organise appointments which should have been made. 

Saturday was the first day that I can remember looking forward to in a long time; I had arranged to go to Brighton to see Katie, my best friend. It happened that on that day, a couple of members of Katie’s family were taking part in ‘The Big Fun Run’ in Hove Park in support of EDS UK and so once I knew I was eager to support them.

When Saturday morning came, Katie and I sent each other a few panicky texts; we know how unpredictable both of our symptoms are, and so we were dreading our plans falling apart at the last minute! Katie and I have gone from seeing each other daily, to only seeing each other once or twice in the space of a year since we’ve both suffered deteriorations in our health and become reliant on wheelchairs and on other people to do simple, everyday tasks for us.

Despite being far from well, Katie and I were both in positions for our plans to go ahead; I wouldn’t have missed it for the world. Things that healthy people take for granted mean more to us than I can put into words. To just be able to spend a couple of hours together meant so much to us and made me realise that, if nothing else, our friendship is worth the fight we are both facing at the moment. 

“True friends are never apart; maybe in distance but never in heart.”

Silently Screaming.

If I didn’t know what frustration was prior to the past fortnight, I most certainly do now. When you’re relying on hospitals and the NHS for so much, as I do, it’s hard to appreciate the sheer enormity of the organisation, the amount of people they deal with and that to them, my case is no more important than the next persons. My ultimate goal, to get back to uni, is the only thing left which encourages me to make the constant phone calls and emails to move my care forward. 

As I mentioned in a previous post, the majority of my EDS care is being moved to UCLH. Whilst it won’t happen overnight, this will hopefully result in more co-ordinated care with everything ‘under one roof’ and a team who can work together. One of the things that UCLH were going to take care of was my ileostomy, which initially was planned to be done at The Royal London. After three months of phoning The Royal London to chase up the clinic letter and my appointment with the stoma nurses, it has come to light that my notes and the dictated letter were apparently lost in the NHS cyber attack, and the consultant claims he has no recollection of my consultation. To say this was frustrating would be an understatement; since my appointment in April I thought I was three months along on the waiting list by now, and it turns out I wasn’t even put on it! Waiting for another appointment at The Royal London seemed like a waste of the next few months, and so with confirmation from the consultant there that the situation of not even being on the list was indeed the case, I have decided the best thing to do will be to allow UCLH to coordinate my care, and see what the team of numerous specialities feel would be best for me.

A multidisciplinary team has been set up at UCLH, and they met a couple of weeks ago to discuss the procedures such as my port replacement, the ileostomy and formation of a PEG; I am yet to recieve the report of decisions made but I hope however things move forward there will be successful intervention to stabilise my condition and improve my quality of life from what it is now. More than enough tears have been shed over the past few weeks – to put the frustration into words is almost impossible. I’m so beholden to hospitals and medical professionals, and I have next to no power in getting things done. A complaint to PALS may at least make me feel like there is closure on the situation, but it won’t get the past three months of waiting for nothing back. 

My dislocations have been getting more frequent, particularly my right knee which has been so problematic. I had an MRI on it, which showed that my kneecap is at a complete slant where my tendons are not strong enough to pull my knee-cap back into the place where it should be. The angle at which my knee is results in it being weaker and so more prone to dislocating, so I’m in a vicious cycle with it. I’ve been referred to Guy’s hospital to a surgeon specialising in issues such as mine, who will consider operating on it. I’m open to any suggestions he may have, although I was told at a local hospital that surgery may only provide around a year of relief until the tendons have stretched again and the problem will return. The other worry I have is that, as with all my operations, I’m often classed as too ‘high risk’; I have a condition that needs surgery to help aspects of it, but the condition makes me too risky to have the operations… I can’t win! 


Even more challenging than the physical symptoms I find are the mental implications of having conditions such as mine. Living day in, day out with things such as catheterising, irrigation, requiring wheelchairs, dislocations and taking numerous medications is so hard. Whilst my anti-depressants have been increased the benefit is limited, and I would do anything to have a sense of ‘normality’ back in my life; to do simple things that people take for granted everyday such as walking, getting out myself and having independence is what I long for more than anything. In the middle of the frustration, upset and depression that I am currently experiencing it is hard to remember that I should be grateful for what I do have, in particular my family who I would be nobody without. Jeffrey’s cheekiness also brings light and laughter to even the darkest of days and I couldn’t imagine life without him.

What next? 

The past couple of weeks have flown by; they’ve been crammed full with appointments but I don’t have anything significant to report apart from my continuing frustration which seems to be a prominent theme throughout my blog.

Powerchairs;

One of the main things that I’d been waiting a while for was a meeting between my OT, physio, wheelchair service and I about a power chair. I feel an electric wheelchair/scooter of some kind would improve my quality of life as I’m unable to self-propel my manual chair due to finger dislocations, but the wheelchair service pushed their concern about me deteriorating with an electric chair through becoming lazy and not using my gutter frame when I am able. For all my faults, I’m not lazy and so would use my frame on good days, but the meeting was left with the council not providing a chair, and I have to contact a list of charities to apply for funding in order to be able to purchase one. Just another thing to add to my rapidly increasing to-do list!

Port-a-cath;

I have had a port-a-cath in my chest for almost a year now, and it has been troublesome from the start. It was poorly sited at St. Mary’s in Paddington, making it really hard to access and the problem has only got worse with time. There are only very few nurses who can successfully access my port and it’s just not feasible to get to St Mary’s to the one vascular nurse who can access it for my bloods on a weekly basis.


It was back in March when the district nurses reported to the vascular team at St.Mary’s that there was the fear my port was turning, but in any case it was getting increasingly difficult to access. Over the past four months, I have written countless emails, obtained letters of support and done anything in my power to push for my port to be replaced. This is because I need a reliable port for sedation/meds to be administered via for some major operations, and I don’t want an unreliable port delaying treatment and procedures I need doing.

There has been excuse after excuse to why my port is not being dealt with, but it all came to a head on Friday when I received the letter saying that, as I’m not under a Doctor in the hospital, they are having no more to do with my port and I have to go to my GP to be referred for this port to be removed and the new one inserted.

Percutaneous Endoscopic Gastrostomy;

I have been waiting months for my PEG, with the need for it rapidly increasing as my swallow function is deteriorating. I finally felt like I was getting somewhere when I had an appointment at King’s to discuss it on Friday, with a provisional view to having the procedure on 11th June. This was all too good to be true, as when I attended the appointment, the consultant told me that I’m showing some signs of MS, and so a brain scan should be done before the PEG to see if there is anything else going on which needs to be treated.

The way I feel at the moment, I can’t face any more tests and to be honest, if there is anything else sinister going on I’d rather not know. I came out of the appointment in tears as I’ve been so desperate for my PEG, to take the pressure off me when I am unable to swallow and more than anything to provide an alternative means of getting my tablets in when I’m unable to swallow them. The consultant has to now discus the situation with some of the other doctors involved in my care and so it is another waiting game until I know what will happen going forward.

What next?

Every aspect of my health care recently has been a fight; phoning to chase up appointments, writing emails, having arguments, chasing up prescriptions and filling in forms just consumes the entirety of my days at the moment. I had improved with coping slightly, but this past week I’ve really been struggling with my mental health. I’d rather have 100 more physical symptoms than have to suffer the mental implications of ill health, but there’s just no getting away from it. There are days where the inability to carry on just becomes overwhelming; this week has been particularly hard seeing the graduation ceremony’s at Cambridge, a place where I should be next year but my illness is stopping me from doing so. I can’t even express how much I want to be back at university. As I write this a tear is rolling down my cheek- I didn’t work the hardest I ever have to gain my place at Cambridge to lose it to illness. I can’t wait to be back there, but I’ve got to get through these rough times first, endure a few operations and hopefully be in a more stable position to resume my studies. 

“Your illness does not define you. Your strength and courage does.”