Zoe

I have been poorly my whole life, fast heart, fainting, nausea, etc, but no one could tell me what was wrong until… I also have Ehlers Danlos Syndrome (EDS). EDS affects all of my joints so I dislocate and sublux (joints partially dislocate) daily, it also causes problems with my internal organs and my collagen. Recently I’ve been rushed to get an appointment with the geneticist as they now suspect I have a rarer and more life-threatening type… it’s a waiting game now. I’ve been enrolled in the 100,000 genome project on the NHS.

As well as EDS and PoTs I have a ventricular septal defect (VSD), which is a hole in the heart, and my mitral, tricuspid, and aortic valves regurgitate, this means that un-oxygenated blood (the wrong blood) goes around my body. I also suffer from different heart arrhythmias. I had a pacemaker put in on 30th March 2017 as my heart was pausing for too long and was also going far too slow.

I also have asthma, fibromyalgia, gastroparesis, mast cell activation syndrome (MCAS), genetic-related high cholesterol and seizures.

On Christmas Eve 2015 I was told that I had bad lung function and bilateral weakness in my diaphragm, especially on my right side in the supine position. This means I cannot lie down: by doing so my oxygen levels drop dangerously low, I turn blue and go into hypoxic seizures. Between Christmas and New Year 2016 I fell into a coma for 7.5 hours and after I woke suffered a number of seizures.

I have to sleep with a ventilator

I’m currently under the care of Royal Brompton Hospital in London and on 23rd June 2016, I had a sleep study there alongside other tests. The outcome of the study led them to suspect that I had an extremely rare condition and so they put me on ventilation. On the 4th of August, I went back to repeat the sleep study and it was confirmed that I have Congenital Central Hypoventilation Syndrome (CCHS): this means every time I go to sleep could be my last. I have to sleep with a ventilator (life support unit) now otherwise I would die. What happens is that when I fall into a deep sleep my autonomic nervous system stops working and so my brain doesn’t tell my lungs to breathe.

I’m currently under seven consultants, all at different hospitals. My health has got a lot worse over the past year, but you’ve just got to deal with it step by step.

My gastroenterologist consultant has told me I suffer from gastroparesis which means my stomach is partially paralysed. I have lost six stone in eight months and only have liquid food and soft food, I may need a central line. I also have a megacolon and it’s redundant. As this is so complex I have now been referred to a specialist in London. I have a continuance nurse and use an iry pump (irrigation pump). I also have to self-catheterise as my bladder is failing.

I have been wheelchair-bound now for 5 years now and need 24-hour care. My home is finally adapted for the wheelchair and I have a wet-room built to the side of my home, with a dropped kurb and drive with ramp access to the front door built-in, and my doors widened to fit my wheelchair through, and this has made my life a lot easier and a little more independent.

I don’t know what lies ahead

I am weaker now and I am poorly, all my organs are failing. My whole life has turned upside down. The consultants and I don’t know what lies ahead. All we know is there is no cure for anything I have and with time it will only get worse.

Despite everything I am still the same Zoe: cheeky, stubborn and I like to take the mick out of myself in jest along with others. I’m still positive and every day is both a struggle and a blessing. I have to fight but we all have our fights. I make the most of what I have and try to do things on the days where I feel half-human, even though every day I have severe pain which never goes away.

On top of pain medication which I try hard not to take unless I really have to, I rely on 30 + different medications a day to keep me alive. I shouldn’t still be alive but I am so making the most out of it!
I died on 19th October 2017 but they managed to bring me back so I feel very blessed. I’ve also died many times now after this date. If it wasn’t for my husband I would be dead by now.

Before I got so bad I used to work in travel as a cruise specialist and then at Manston Airport. I worked in Airfield Operations and Dispatch, this is where I met Carl, my husband. Carl worked for 24 years in the fire service as a watch manager, we didn’t take to one another at first (!) however over the 12 years we built a strong friendship and then we started dating. He wanted me to show him the world so that’s what I’m doing. He proposed on 9th Nov 2016 while at sea heading to Aruba and of course I said yes! And on the 10th March 2018, we tied the knot on Waimanalo Beach Oahu! Carl has always been by my side and when I got more poorly I gave him the option to leave as he didn’t sign up for this but there was no chance he was leaving as he said he loves me – so from then on, he continued to save my life more then I can count and he truly deserves an award for what he does as we don’t get any help from social care!

We lost my dad on the 12th August 2018, 5 months after our wedding which is so hard in many ways as I was a complete daddy’s girl and he was also my carer so my husband could go to work knowing I was safe.

We have CCTV installed

Carl runs his own business where he trains people in basic life support, fire training and much more, in between looking after me. We have CCTV installed so he can see me while at work to know I’m ok and we also have a lifeline.

We own our home and have 3 cats, my boys!

You will find us in one of three places, home, hospital or on a cruise. That’s what has triggered me into blogging for wheelchair users’ accessibility on cruise ships, excursions, and so on. I’m going to try my best to provide useful information/pictures to hopefully help other wheelchair users. You only live once after all….

My motto is “Everyday may not be good, but there is something good in every day”