Goodbye 20.

As I turn 21 tomorrow, I’m hoping for a ‘fresh’ start and a probably unrealistic turn in my luck, so I felt it appropriate to bring you all up to date on my health – as we don’t want to still be here at Christmas, I’ll keep it brief!

One of the most significant additions has been a currently unexplained Potassium deficiency; for those of you who don’t know, a normal Potassium range is from 3.5-5.0 mEq/l. I had a phone call from my GP after a routine blood test to say that mine had dropped to 2.8 mEq/l and so supplementation had to be put into place. A week later I had my bloods repeated, and thought nothing of them, until at 03.45am the following morning, our house phone rang and it was the path lab at Queen Elizabeth Hospital; they’d been processing my blood sample, and it had come back showing that my Potassium had now dropped to <1.2 mEq/l which is a critical level, I was at risk of a cardiac arrest and I was to be rushed into A&E.

My levels were ‘stabilised’ to a level which allowed me to return home, which had to be sooner than the doctors wanted as they couldn’t get blood from anywhere (foot, wrist, arm, groin etc) and there was no one in the entirety of A&E able to access my port-a-cath, and so we had to rely on the district nurses coming home every two days for bloods, and adjusting my dose of oral Potassium from there. This is still what we are doing at the moment, but there is currently absolutely no explanation as to why this problem has arisen. It could be due to my inability to absorb, although it seemed to come on too quickly for that.

In addition to this, over the past few weeks there has been a severe deterioration in my POTS. I can’t even always transfer without passing out, and with the maximum dose of a lot of the treatments tried (Midodrine, Ivabradine, Bisoprolol, Pyridostigmine, Fludrocortisone etc) we are beginning to exhaust our options. There is the possibility of starting Octreotide which is an injection 2-3 times a day, but it has gastro side effects and with my bowel already failing to work, my teams are still trying to weigh up whether or not this is a viable option.

Considering the complexity of my health conditions and the multi systemic nature of them, UCLH have decided that the only real option is for me to have a planned admission to get the various health professionals together and try to stabilise things as much as possible. To begin with, I’ll be admitted for a minimum of a fortnight and we will go from there as to whether it’ll be longer and what procedures/operations (such as the siting of a PEG) need to be carried out. The whole situation is far from ideal, but realistically there isn’t a choice as we can’t carry on with how things are at the moment. I hate admissions as it is, and the hospital environment is one I find extremely hard to cope with, but I am holding on to the fact that it should be worth it in the long run.

Despite it being my 21st Birthday tomorrow, I still have district nurses etc coming as, with chronic illness, there is never a day off even as much as I’d like there to be. However, my family and friends who have been there for me through the good and the bad times have done everything in their power to make it special, and for them I am eternally grateful.

Advertisements

EDS UK/ HMSA Conference

This weekend, my Mum and I went to Warwick to attend the joint conference by EDS UK/ HMSA titled ‘Management and Wellbeing’. We decided to purchase tickets to be able to listen to the various speakers, gain a deeper understanding of the illness and management options for it, in addition to meeting and learning from likeminded people. It also meant a weekend away to look forward to, getting out of the four walls that have become so familiar and it has been a weekend I could spend with my best friend since we no longer see each other very often after both being reliant on wheelchairs due to the condition.

The conference was spread over two days, and so I decided to split my blog post up into the two sections.

Day one;

The first day started early, with registration at 0830 and talks commencing from 1000. To avoid an excessively early morning and a stressful drive with needing to get here on time, my Mum and I stayed the night before locally. We registered, and had a look around the different information stalls; there were many organisations represented, including EDS UK, HMSA, Mast Cell Activation Syndrome and Stickman Communications. My Mum and I met up with Katie and her family, before going into the main room in which the majority of the talks were to take place.

The mornings talks were commenced by Dr Kazkaz who gave an overview of the new classification of EDS. The rest of the mornings talks focused on PoTS, Mast Cell Activation Disorder and the importance of nutrition within the conditions. After a break for lunch, the afternoon provided talks on swallowing and voice, there was a talk by an Occupational Therapist and finally a Mindfulness talk concluded the first day. In addition to the main talks, there were also ‘breakout’ sessions provided; My Mum and I didn’t attend any of these as we had an interest in all the main medical talks and obviously could only go to either one or the other.

There was a huge amount of information to take on board, from the combination of the talks, information stands, leaflets, health professionals and fellow patients. This, in a combination with the travelling yesterday, the early start and generally having chronic illnesses left me absolutely exhausted! We had a conference dinner, soon after which my Mum and I went up to bed, as we were in desperate need of sleep before the second day.

Day two;

The second day began with my Mum and I having breakfast, before heading back into the conference room where we met Katie ready for the mornings talks. These included one from a urology consultant I am under the care of, a talk from a GP who is also an EDS sufferer and a talk by psychologists based on managing pain and fatigue: The combination of talks which were from numerous different angles was so effective as there was a bridge between us as patients and the healthcare professionals whom we so heavily rely on.

As the conference broke for lunch, after a few final visits to the information stands, my Mum and I left. There was one more talk this afternoon, but in order to pace my day (which is a technique so frequently mentioned during the conference), we headed home as we still had a two and a half hour journey ahead of us.

The conference was such an informative weekend, giving us tools to cope with the debilitating impact of Ehlers-Danlos Syndrome, whilst also providing hope for future management options. I’m so very grateful for my Mum having no boundaries to what we do to try and improve my quality of life, and for Katie who sticks by my side no matter what, and makes my life with this horrific illness so much more worth living.

University.

As I see my friends returning for their third year of university and pupils I mentored at school become freshers, I felt the need to write a blog post with a heavy heart about my need to extend my intermission.

As most of you know, I completed my first year of university at Cambridge during the academic year of 2015-16; I then had to take the following year out due to a deterioration in my health, and since I’m still trying to stabilise my health and awaiting operations, I also have to take this coming year out with a view to return in October 2018. The decision to intermit both times was not really a decision and was taken out of my hands – the first time because I was in the middle of a three month hospital admission relying on IV drugs etc, and this coming year due to needing operations before I’m stable enough to continue my degree.

I can’t even begin to put into words how difficult this has been to come to terms with, and still now I don’t think I have. I see people returning to uni on various social media sites, and whilst I don’t begrudge people for what they have, I can’t help but think that it should also be me. I can’t help but scroll through with a lump in my throat and tears in my eyes. I have never worked as hard as I did for my A-Level results to get into Cambridge, it was the place I’d always aspired to get to and I just want to live my dream. I shouldn’t have a life ruled by my broken body, but instead should be back getting my degree; I would do anything to have this bit of normality back in my life again.

In addition to missing out on getting my degree, I’ve also lost my year group who have moved on, I’m losing out on having the experience of living independently and ultimately missing out generally on the entirety of my university life.

I don’t want this post to come across as selfish, and if it does it has been misinterpreted as that was completely not my intention. Of course there are people worse off than me, in more challenging situations, but it doesn’t take away from the problems I’m facing myself and my absolutely burning desire to be back at Cambridge; all the barriers preventing me from returning are so frustratingly out of my control.

It goes without saying that I’ll go through this year, have my operations, do my utmost to adapt to my new ‘normality’ and prepare as thoroughly as I can for my return for Michaelmas term 2018. I have to believe I will get back there as it’s the only thing keeping me going, the only thing worth fighting for. I have to have faith that I will make friends in my new year group, I will get my degree and ultimately I’ll be able to live my dream.

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.

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…