My Illness

Well, where to start?

My whole life has been changed over the five years. As I’m writing this I’m looking at a photograph on my pin board of me walking, smiling and having fun at a dinner party with my friends. But the story starts before all this.

Growing Up

As a child, I was often unwell. Simple things like walking and talking I developed slightly later than other children my age. My speech needed intervention from a speech therapist. You wouldn’t have noticed it at the time because we weren’t looking for it but some of my joints were more hypermobile than my peers and I was always the one who was off school poorly and caught everything under the sun.

I was in pain, but in my early teens I started to get strong and chronic pain. I also had a lot of bowel and bladder problems as a child and into a teenager and I was always falling over/fainting and this led to investigations in hospital. They ruled out major illnesses and concluded I must have¬†IBS and bouts of intermittent bladder retention, growing pains and just clumsiness as well as anxiousness because of my fast heart rate/dizziness. From the age of 6/7 I got constant UTIs and other infections. My immune system seemed non existent. I would fall over and collapse a lot as a teenager, be dizzy and feel palpitations, be in constant pain, my joints feeling so stiff and painful and sublaxing and even having seizures, but because they were intermittent they weren’t deemed serious enough to be investigated fully.

Things Getting Worse

Two and a half years ago I started fainting more regularly and I got a number of injuries from collapsing. My friends saw some of these and being honest, only a few completely believed me and stood by me in finding out what was wrong. After one collapse and a bad injury to the head, I ended up in hospital for a few days and the doctors were baffled but ordered a head MRI.

They were looking for something significant like a tumour or a bleed, but couldn’t see anything obvious that would link to why I was collapsing so they dismissed it as being a young girl in hospital with dizziness and fainting and a number of other problems that you wouldn’t normally group together. They took my blood pressure lying and standing and it dropped, but they said it was normal and never checked my heart rate or oxygen levels.

I said I didn’t feel safe going home but I got discharged. I collapsed in the car park and my friend lifted me into the car, carried me up 3 flights of stairs to get into my flat and I spent a week crawling around my flat because I lost consciousness every time I stood up. My friends bought me shopping and I was basically under house arrest.

I had to get out the flat and I couldn’t get to the doctors appointment I had because I couldn’t stand up. We borrowed a wheelchair from our local British Red Cross centre. At this point I was waiting for an outpatient neurology appointment at the hospital but over the course of this month I got no support from my GP and at one point he said to me “I don’t know what to do, there’s no medical reason for what’s happening to you”. I had friends telling me that it didn’t make sense and must all be in my head and even members of my family who love me doubted me.

Two friends in particular saw me continually collapse and sometimes go on to have seizures from simple things like cooking food. I had to lay in the bath and get a friend to use the shower to shave my legs and wash my hair for me because I couldn’t sit up without passing out. I had a seizure in the bath even and couldn’t be safely left alone.

Diagnosis

The neurology appointment came and by this point I had found information online about POTS and mentioned to the doctor that this matched a lot of my symptoms. He quickly dismissed this, but when he examined me and I stood up, my heart rate went up to 180bpm and I lost consciousness. He told me I needed a cardiology appointment urgently and that he’d never seen a case like this.

The cardiology appointment was 6 weeks away and with my symptoms being so debilitating my family were left with no choice but to book a one off private appointment to speed the process up. He thought that my blood pressure drops as I stand up and my heart rate rises to compensate for this. He started me on medication to increase my blood pressure but it had no effect on the heart rate.

I was admitted to High Wycombe General Hospital on 1st May 2014 and was on a cardiac monitoring ward for 3 weeks. One of the top consultants said “in 20 years I’ve never seen a case like this”. Just sitting on the edge of the bed, my heart rate went from 90 lying to 170bpm. As soon as I tried to stand it rose to over 200 and brought by oxygen levels down at the same time. I had palpitations as the room was spinning and I was sweating lots. Everything blurred and I got tunnel vision, and I woke up on the floor in pain from my fall and lots of staff standing around me. I was hoisted back to bed and despite numerous physiotherapy attempts, that was the last time I stood up independently.

I was transferred by ambulance to NHNN, Queen’s Square, London where I had my tilt table test and was officially diagnosed with PoTS and Autonomic Dysfunction. They asked about my childhood, my joints and my digestive system and said that they were confident my PoTS was caused by EDS. They took every system one by one and came to a whole body conclusion as opposed to individual symptoms. They could not officially diagnose me as they were not a specialist in EDS but from then on my symptoms were treated as EDS symptoms and I was put on a waiting list for an EDS specialist (Dr Kaz Kaz) who could give an official diagnosis (whom which I saw in December 2015 and got the diagnosis).

Complications

Whilst I was in London I had more seizures, breathing problems and at one point I crashed and was admitted to intensive care for 48 hours of close monitoring. I developed two kidney infections there and that was the first time I needed to have a catheter put in. I had major retention (up to 1.5 litres) but no feeling or urge to go. Over time, this led to complete bladder failure and I have impaired sensation between S2 and S5 in my spine. I don’t get the need to go to the loo, and the EDS causes the bladder collagen to be stretchy and therefore it can’t function properly. Now, writing this in January 2016, I have had a suprapubic catheter for the past 6 months. This comes with it’s own risks and complications but gives me a better quality of life than it’s alternatives and I didn’t have many options once my bladder decided to fail completely.

The second half of 2014 was when my mobility deteriorated. I was forced to come to terms with the fact that the likelihood of me walking again was slim, and despite constant physiotherapy and leading an active lifestyle I had little use of my legs and would need to use a wheelchair full time. I was on a new cocktail of medication meaning I could sit up and keep my POTS under control to an extent, but with the various conditions I have added together, standing up and maintaining balance is nigh on impossible especially after my head injury in February 2014 which really affected my balance and coordination. I still have basic movement of my legs but with very limited sensation and bad proprioception Рwhich is the ability to sense where your limbs are within space and my aim through physiotherapy is to be able to transfer to my wheelchair safely and independently by having enough strength in my legs that they will not give way on top of my joints which are fragile.

In 2015 I was admitted to Wexham Park Hospital in January and ended up staying in hospital for 7 continuous months. I spent the first in cardiac high dependency as despite medication, my POTS and autonomic nervous system weren’t under control properly. During this time I was on full time oxygen and monitoring. I had a number of procedures and we spent a long time trying to get the right cocktail of medications together.

Through the next few months I carried on getting constant infections through my low immune system which led to me being neutropenic and I was put in isolation. With all my complications I need multiple courses of IV antibiotics so was kept in hospital.

With one very bad infection I arrested on 13th March 2015 which led to me being intubated and ventilated; I no longer had control of my own breathing. I had hypoxic seizures and my oxygen levels dropped down to 60-70%. This lack of oxygen in my brain is what caused the seizures. My body has a tendency to retain carbon dioxide and not breathe it out fully, which over time leads to respiratory acidosis and is a medical emergency. I chronically retain carbon dioxide, and when it got to it’s worst I arrested. The ventilation cleared out my lungs and after 4-5 days I was extubated and managed to maintain my own airway with a lot of oxygen support, but don’t fully remember the next 2 days as I began to come round.

Between March and November I crashed several times and had the ITU “Outreach Team” come out to me on the ward several times to maintain my airway. In May I was admitted to ITU again after going into status epilepticus, which is continuous seizures. My breathing again, wasn’t enough on it’s own I went hypoxic (and that’s happened a lot since) so I was given oxygen support/ventilation when getting really bad/dangerous.

With my immune system struggling I was getting constant infections and on numerous occasions, it developed into septicaemia (blood poisoning) and again I needed multiple teams involved including the ITU “Outreach Team” and ICU. Instead of a traditional canula into my hand as most people would have in hospital, I have had to have multiple central lines (both in hospital and at home) from my neck (CVC’s) to my chest and my arm (PICC’s) to feed me steroids, antibiotics, fluids, medications and at my worst with my digestive system, despite trying multiple alternatives to feeding I had to have TPN (Total Parental Nutrition) which fed nutrition directly into my heart.

Coming Out of Hospital

On December 2nd 2015 I was discharged from Wexham Park Hospital on writing this on 20th January 2016 I haven’t been admitted back into hospital for more than a few days at a time. Here’s to living this year to the full, with minimal time in hospital, and maximum time with the people I love.

(It’s now the summer of 2018 and I’ve not updated in two years? I can’t believe it’s been that long! Been very busy though, lots of things happened in that time both good and not so good but all about living life to the full and making the most of what I can! So for that reason I’ll do a little overview/update of the main events/admissions over the last few years on my health to keep you up to date but not a full blown account like the above. If I did a detail by detail account of every health update/admission and news I’d be writing for another 200 pages.. so I’ll do a shortened version. But if you’re reading this and have any questions at all please just ask. I’d be more than happy to answer/help if I can).

Update

Throughout 2016 I had multiple hospital admissions including more stays in intensive care my first being in February when I was abroad (trust for it to happen the first time I go abroad in years!) I was in a coma again for five days and eventually brought round safely once I could breathe on my own and my body could cope. I collapsed because of my seizures which unfortunately often make my lungs struggle and then can often stop me breathing and this time it went on too long and I needed ventilation support. The teams were amazing though and got me the rapid treatment I needed as well as realising I had a raging infection too. My family and Oli drove out to be with me in the ICU (intensive care unit) in the main hospital. I was there for 10 days before being taken back home to the ICU team in England escorted by brilliant team including doctors from critical care on a special medivac plane (wish I could have seen it as mum said the view was incredible but I was asleep – sedated). I then spent a little while recovering in my local hospital back in Wexham as well fighting another infection. End of March/April I went home. Over the next couple of months I spent as much time at home as I could in between short admissions for a few weeks at a time for infections needing IV antibiotics, surgeries and A&E visits for acute problems such as falls/faints due to my POTs, my pain and joints, reactions with my MCAD (mast cell activation disorder) and ongoing bowel, bladder and urogyane problems.

Then came around 2017 and another year had gone by, but another 365 days survived, got through and achieved which is always something to celebrate. The ongoing battles continued with early infections after new year being the first of many battles that year, some managed at home with oral antibiotics and others needing IV’s. We try to stay at home as much as possible though. That February I had another intensive care admission due to a severe bout of pyelonephritis and hypoxia. I then went on to slowly improve but unfortunately soon after developed another infection and this time it went straight to my bloodstream within 24 hours and I was septic, turning into sepsis again (life threatening blood poisoning). I was rushed to John Radcliff Hospital in Oxford and was admitted into the ICU there where I remained for the next 2 and a half weeks. We had a lot of complications within that time and it was a really difficult admission but the team at Oxford were amazing and saved my life (once again) and I’m always so grateful. Mid March I finally went home and I was over the moon. It took quite a while to gain back my strength as that was a really tough and critical care admission but I did it before so knew I could it again especially with the support of my family and Oli. So slowly but surely I built back up my strength back and focused on my list, having fun and making the most of all oppitunuites.

In the end of April I was rushed back into hospital as Callie (my SPC catheter) fell out, with the balloon still intact and inflated which keeps it in! (well didn’t this time lol). Unfortunately you only have a very short window of less than an hour until the tract closes so it was a rush to get to hospital in time and this time we didn’t so once I got there the tract had closed and that meant a urethral catheter went in, I was admitted and sent to a ward, theatre team planned and surgery booked for a new site and SPC to be reinserted. This was then another two week admission in hospital, because of my complexity any surgery is a big deal for me/my body especially my breathing when put to sleep so I have to have ICU involved each time and then on top of that my bleeding and immune system/infection risks makes it even harder. So where as a ‘normal person’ should have blood in the urine for 48 hours 72 hours at most well me, I had continuous bleeding for 12 days/near to two weeks and infections on top of that. Thankfully despite a few complications it went to plan and Callie 2 was in and I was discharged end of April/May. I also left with a permanent PICC line in, Percy (my 8th one) as since I’ve got unwell my veins have slowly but surely been scarred, damaged and many now unusable and thrombilised. Also because of the EDS they are incredibly fragile, small and when you go to put a line in they just flop/move like jelly this is both in peripheral and central. So to protect the rest of my central line veins we kept it in as we knew I had multiple surgeries coming up and any admission to hospital now needing IV’s more than 24 hours required a central line anyway. It’s a very difficult balance, not black in white and every case is individual but in this instance it was the right thing.

I then had some more time at home before my immune system decided to put me through multiple infections from anywhere and everywhere and break down my poor liver too. I then spent weeks in and out of hospital whilst having multiple IV antibiotics both in hospital and at home (thank you Percy!) As well as managing my liver and very high blood enzyme and ALT levels. Over the summer months and into autumn I had more time both at home and back in hospital for a variety of reasons consisting of infections, hypoxic/RAS seizures, my difficult and troublesome bladder and bowels and a three week admission for one of my gynae surgeries to remove and untangle severe adhesions inside and to also take more biopsies and put the coil in to try and manage my heavy bleeding, periods and (horrific) gynae pain which thankfully the coil has helped and slowed my bleeding too, not eradicated but definitely helped a bit and I’ll take anything! You have to find the silver linings throughout all of this however small, hold on to hope and don’t let go. I managed more time at home and in between appointments was even managing to tick some things from my list and write a few blogs (which is not easy for me as it take a lot of energy and strength to write each one). Then on 2nd December I woke up feeling a little more unwell than usual and to cut it short hours later I was being blue lighted to hospital as I had developed sepsis and was back fighting for my life once again. I was so poorly because I was not only fighting one bug but two from my bladder into my bloodstream and my PICC into bloodstream. I then spent the whole of December in hospital on a cocktail of strong antibiotics and treatment for the sepsis and it was close but thankfully I was discharged just two days before Christmas (YAY!) That was one very special Christmas and we are always thankful but after having sepsis five six times I know just how lucky I am to survive as not everyone does.

New year came around soon after (and I wasn’t in hospital this time thankfully) and next thing you know we’re into 2018, another year done and dusted! Another year survived and I don’t just mean that physically I mean mentally too, it’s not easy battling chronic illnesses and there’s no getting round that it isn’t easy, it’s okay to say that, to not be okay and to also give ourselves a little praise and credit when we hit milestones and achievements however big or small.