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My medical journey


  • Ehlers-Danlos Syndrome - Hypermobility/Type 3
  • Autonomic Neuropathy
  • Postural Orthostatic Tachycardia Syndrome
  • Orthostatic Hypotension
  • Chronic Intestinal Pseudo Obstruction
  • Intestinal Failure
  • Neurogenic Bladder/bladder failure
  • Spinal degeneration, progressive Kyphosis and Scoliosis
  • Osteoporosis
  • Hyperthyroidism (Overactive Thyroid) 
  • Wheelchair/bed bound
  • Total Parenteral Nutrition (TPN) dependent
  • Double lumen Hickman line (right side of chest)
  • Drainage PEG in situ 
  • Ileostomy (right side)
  • Urostomy (Ileal Conduit) (left side)

My Medical Journey

I was born, seemingly healthy, on the 2nd October 1993 and aside from minor health issues - all dealt with separately, including joint problems, severe reflux that stopped me breathing, anal fissures, flat feet and astigmatism - I had a normal childhood. I was fit and active, being a keen horsewoman I spent every spare moment at the stables and would think nothing of being out for hours walking my dogs. I was always outside and always on the go. However, pain was a prominent part of my life; I have always suffered with joint pains, and did not realise this wasn't normal nobody else complained, so neither did I. When I was 12, the difficulty I had with my joints reached the point where I was forced to tell mum because I was struggling with the pain, fatigue, weakness and stiffness. I started strapping up my joints and having physiotherapy, and carried on as best I could.

I continued to get worse however, and in January 2008, aged 14, I became housebound and started needing to use a wheelchair. Chronic pain and chronic fatigue had set in, and it was a difficult year for us all. My life had changed completely, from someone who was always outside to someone who was stuck inside, in a lot of pain, and with doctors telling us it was a school phobia, laziness, or "all in your head". Eventually, in October 2008, a few days after my 15th birthday, I saw a rheumatologist called Professor Rodney Grahame who diagnosed me with Ehlers Danlos Syndrome (EDS), hypermobility type. He said he could probably have diagnosed me at the age of 7 based on all the problems I had, but we're glad we didn't know.

This is when my journey with Chronic Intestinal Pseudo Obstruction began. It started with what we thought was oral thrush, but we couldn't get rid of it. I was also suffering with a lot of mouth ulcers and food intolerances, especially to milk and bread, but I struggled with sugary foods and acidic fruits too. Having put on a lot of weight when my mobility declined, reaching 18.5 stones, I was starting to eat healthily and losing a few pounds a week. With the trouble I was having with food, my diet was very restricted, and I was losing increasingly more weight each week. In early 2009, I started suffering with constipation, abdominal pain, nausea and bloating. My diet was getting more and more limited, and eating was no longer a pleasant experience. I also began having problems with my bladder. Then, by March 2009, I was having difficulty swallowing. I was having to wash my food down as it kept getting stuck, even though I was only eating green beans and carrots cooked until they were falling apart. Despite eating very little I felt full all the time and would suffer terribly after I ate.

By August 2009, things were out of control. I had lost 6 stones in 6 months, was not eating anything, drinking very little and barely awake - our conversations were only a few sentences long before I'd drift back to sleep. I had become bed bound, and mum was checking on me regularly, terrified she would walk in and find I had stopped breathing. I saw doctors and had a few trips to hospital in an ambulance, but would be discharged without any help. My GP gave me Ensure build up drinks, but I could not manage to drink enough to sustain myself.

Eventually a family friend called an ambulance and I was taken to my local hospital where, after a night in A&E, an argument with my paediatrician (who felt I had an eating disorder) and an emergency psychiatric assessment, an NG tube was put in and I began tube feeding. I was so malnourished and dehydrated that my kidneys were beginning to shut down, and nurses had to flush me out with plenty of fluids to get them working again. The tube feeding went ok, and I was started on Movicol to treat the constipation. I looked very healthy though, as the weight loss had brought me down to the ideal weight for my height, so I wasn't thin and didn't look malnourished.

In Southend Hospital, a few days after my NG tube was inserted
After just over a week in hospital, on a bank holiday weekend, NG still in place, mum having only ever set up my feed once and with no drip stand or support when we got home, I was discharged. Social services agreed to fund a carer to stay with me during the day on weekdays as my mum worked full time in a school, and we muddled our way through each day. I struggled to tolerate the tube feedings. My weight loss slowed down for a little while, but I didn't stop losing weight. We also struggled to get my bowel moving and I was needing increasing amounts of Movicol as well as trying things like Senokot and Lactulose. The pain was increasing, I was still bed bound and generally my condition was worsening.

It was agreed by consultants at my local hospital that tube feeding was going to be long term and so I had a PEG placed and my NG removed on 21st October 2009. We didn't know why I was having gut problems or what was going on, and so we went back to see the consultant who diagnosed me with EDS. He confirmed that it was secondary to the EDS and referred me to Professor Qasim Aziz at St. Bart's/The Royal London. I first saw Prof Aziz in April 2010 who felt I had gut dysmotility and ordered some tests. He also picked up on my low blood pressure and high heart rate and referred me to Professor Christopher Mathias to be investigated for Postural Orthostatic Tachycardia Syndrome, which I was subsequently diagnosed with after a positive tilt table test.

Prof Aziz's tests took place over the course of 2010 and included an Oesophageal Manometry, Small Bowel Manometry, Endoscopy, Colonoscopy, MRI scan, Barium Swallow, Sitzmark Study and blood tests and included a short admission in the October to investigate low haemoglobin and raised inflammatory markers, although the cause was never determined. The results of the tests confirmed I had severe gut dysmotility, and so I was started on a new medication regime to try and speed up my gut. I was still needing increasing doses of Movicol to keep my bowel cleaned out, and the new drugs didn't make any difference. I was anaemic requiring blood transfusions and iron infusions, potassium because my potassium was very low and I was ending up in hospital every few weeks, dehydrated and malnourished. We tried putting an extension through my PEG into my Jejunum (making it a PEG/J) to try feeding me straight into my small bowel, but this also failed. In this time I also had a suspected heart attack, which they put down to a virus that had attacked the heart muscle at the time (blood tests were all normal), but looking back they think it was my body beginning to digest my heart muscle. I had no other muscle or fat on my body, I was skeletal and covered in fine downy hair where my body was trying to keep me warm.

In May 2011, I was rushed to The Royal London under Dr Glynn to begin TPN. My weight had plummeted to under 7 stones, which for someone of 6 foot 1 inch is extremely low. It was touch and go for the first few days, as Dr Glynn did not know if I had been left too long. Thankfully my body accepted the new nutrition, and I had a Hickman line placed on 19th May 2011. I spent 5 weeks in hospital and we were trained how to set the TPN up and disconnect it, how to care for the line, and the aseptic principals. Since being on TPN my weight has normalised - in fact I've put on a bit too much weight! They're now having to cut the calories in my feed down. It was during this admission that we first heard the term "Pseudo Obstruction", and the first time we really understood the seriousness of my condition.

Despite stopping PEG feeding and needing increasing amounts of Movicol - I was having at least 40 sachets per day and still not going - it kept landing me in hospital with "bowel obstructions" and faecal impaction quite regularly. At this point, Prof Aziz referred me to Professor Charles Knowles for an Ileostomy, which was formed on 7th February 2012. It was successful, although my bowel still pseudo obstructs regularly. I have complete intestinal failure and Prof Aziz has referred me to Addenbrookes Hospital, Cambridge to look into the possibility of a small bowel transplant. We don't know if this will necessarily be the right course of action yet, since my TPN is going so well and I've had no major complications so far, but Prof Aziz wants them to monitor me regardless. we haven't heard back unfortunately.

In April 2013, after a few admissions for bowel obstructions, I had an operation to reconstruct my Ileostomy (stoma) and remove a stenosed (narrowed) and damaged section of bowel. This helped lessen the frequency of total bowel obstructions I had, until my bowel deteriorated again in 2014. 

My bladder is also affected by EDS and CIPs, and I see Mr Khullar at St. Mary's Hospital, Paddington. I have a chronic UTI which we cannot get rid of with oral antibiotics, and often not the strongest IV antibiotics, and I intermittent self catheterised from March 2010 to September 2013 but In March 2013 we found out my bladder was getting worse and so I needed a permanent catheter (suprapubic catheter) inserted. It was placed on the 24th September '13. 

Then in August 2013 we found out that my spine is degenerating (crumbling), which hadn't been present on scans I'd had previously, which means it has occurred quite rapidly. We don't know if it is a part of the Ehlers-Danlos Syndrome or due to osteoporosis caused by EDS and the TPN, or is a separate condition entirely. I am due to have a spinal injection into my coccyx to help with the pain, and if this fails, they will remove my coccyx to help with the excruciating pain I get in it. I was diagnosed with Osteoporosis in December 2013. 

Also in December 2013, I saw a urology surgeon to look at my options, and it was decided I need a Urostomy - a stoma bag for urine. The operation will take place on the 3rd October 2014. . My bladder infection is making me very poorly, I keep hallucinating and running temperatures. The operation can't come soon enough. 

We know my life will be limited, and our aim for now is to try and make me comfortable and improve the quality of my life, which at the moment is extremely poor. I am bed bound due to the EDS and POTS, with CIPS complicating the matter, and I struggle daily with the gut and back pain, for which I am on an intravenous infusion of Clomipramine, a Fentanyl patch and sublingual Fentanyl to try and control it. I also need regular antiemetics (anti-sickness) which help to control the nausea and stop me vomiting; I am on IV Cyclizine, IV Levomepromazine, Buccastem and Buccal Olanzapine. I am a particularly tricky patient because I don't seem to be able to absorb any medication orally/enterally, which complicates everything. I have 4 litres of fluid TPN 5 days per week and 3.5 litres of lipid TPN 2 days per week. Most of my medication is IV, buccal/sublingual or a patch, except for one oral drug which we doubt I am absorbing. 

My PEG and catheter sites are badly infected (2 infections each) and IV antibiotics don't cure them, they always come back. My PEG is used for drainage, it's on drain 24/7 because my bowel obstructs frequently and things go backwards and forward, so what should come out of my ileostomy frequently comes out of my PEG. I have it on drain to stop me vomiting and to allow me to drink as it drains straight off. 

I was due to go into hospital for a line change and PEG removal on Monday 7th July but things didn't exactly go to plan. On Saturday 5th my bowel obstructed and on Sunday night instead of driving to the hotel ready to go to The Royal London early Monday morning we drove to The Royal London A&E. I was admitted, given Morphine and extra IV Cyclizine (antisickness) but fortunately my bowel started to work in the early hours of Monday morning so my prearranged line change went ahead on Monday. I had my single lumen (one tube) Hickman line changed to a double lumen (2 tubes in one) Hickman so that we can run IV medication and IV antibiotics alongside my TPN. It's been a godsend. That went really smoothly, they managed to use my old site and tunnel which is good. They started me on IV antibiotics to treat the PEG and catheter site infections. Then on the Wednesday, in preparation for my PEG removal on Thursday, I was taught to put in my own NG tube and I did my first one all by myself. Unfortunately, within 2 hours I started vomiting and vomited for over 3 hours straight. I then started vomiting blood, and vomited up the tube. The vomiting stopped not long after that, but my stomach/oesophagus was still bleeding either from the tube or the constant vomiting and I started passing this blood through my Ileostomy. I continued to pass blood through my stoma for 12 hours, but finally it stopped. My haemoglobin dropped but stabilised once the bleeding stopped. 

On Thursday 10th July an endocrinologist (hormone doctor) came round to tell us that I have an overactive thyroid (Hyperthyroidism). This means my thyroid is producing too much thyroxine, making my body work overtime. It is causing my tremors, and is causing or contributing to my high heart rate, excessive thirst, excessive sweating, heat intolerance, restlessness and irritability, shortness of breath, chronic itching, more severe fatigue and more severe muscle weakness. I was started on Carbimazole, however it is an oral drug which we put down my PEG but whether or not I absorb it we don't know. 

Then came the disaster. In the early hours of Friday morning (at about 1am) I fell out of bed or tried to get out of bed and fell as I can't stand without help and I pass out if I stand for more than a few seconds. I don't know what happened, I just woke up on the floor in the most excruciating pain I've ever felt. I knew that my right hip/leg was broken. At first I tried to get up; I couldn't have got up off the floor in the six and a half years I've been ill even at my strongest, so how I was going to do it when I'm so weak and poorly only using one leg I don't know. Then I unplugged the chargers out of the back of my pumps and tried to shuffle to the door, but I couldn't move. I ended up calling nurse and they heard me after 20 minutes. They manhandled me back into bed and mum came straight away. Mum thinks that, as my catheter kept blocking on Thursday, that it must have blocked and I needed a wee and that I got out of bed to go to the toilet, subconsciously thinking I was still the healthy fourteen year old girl I once was. Except I'm not, and so my legs gave way or I passed out. After waiting 5 hours for a doctor I had an X-ray which confirmed that my hip was broken - in medical terms, I'd got a pertrochanteric fracture of the neck of my femur. It's an injury most common in geriatrics - I do have the bones of a geriatric due to my osteoporosis - and people only tend to get 60-70% function back. After 57 hours in excruciating agony, on Sunday 13th July I was operated on. I had a Dynamic Hip Screw fixation, so I have a long screw through the bones and a plate, with smaller screws holding the plate on. My pain was immediately halved, and I could start moving it almost straight away. I sat up in my wheelchair for the first time on the Wednesday and was allowed home on the Thursday. I'm working really hard on increasing the movement and strength in my right leg, and it's paying off. The only thing that isn't improving as quickly is bending that knee. I can bend it enough to sit in my wheelchair but it's very stiff and painful. I'm going to continue working on it, I can put some weight through it when I transfer but it was my bad leg before I broke my hip so can't put much weight through it without it giving way.anyway. I will get the most movement out of it as I can.

We were only home for 6 days when we had concerns with my new hickman line. After a phone call to my nutrition team, we found out that the cuff - a bit on the line that sits under the skin and irritates the surrounding tissue to make the tissue heal to it - was on the outside of my body; my line was falling out. It had slowly worked its way out after the fall. We had to stop using the line and had to go up to The Royal London A&E, the hospital at which my nutrition and gastro teams are, for IV fluids while we waited for a slot to come up to have a new line placed. My hickman line is literally my "life line", without it I can't have my TPN (my only source of nutrition) and most of my medication goes through it too. That was Wednesday evening. They got me a bed on the admissions ward, whilst we waited for a gastro bed to become available. Fortunately they managed to get me a slot on Thursday morning. When they started putting in my new line, using a new site and tunnel under the skin, they couldn't find the bit of my old line that sits in the vein leading down into the heart. They kept scanning and following down the vein when they found out that it had snapped; they couldn't find it because it wasn't there. After a lot of searching, they found out it had gone through my heart and was in my pulmonary vein. They got in a bit of a panic and called in the senior radiologists - I ended up with about 5 radiologists there in total. They made a cut in my groin, got access to my femoral vein and put a guide wire up through the femoral artery/vein and into my heart, then into the pulmonary vein. Unfortunately the wire kept coiling in my heart, irritating it and causing it to beat irregularly and feel like it was going to burst out of my chest. After a lot of trying, they finally got through into my pulmonary vein, threaded the clasp tool up the wire, got hold of the 3 inch piece of line and pulled it down through my heart, through the femoral vein and out through the cut in my groin. Then they could get on with putting in the new line, using a new site and making a new tunnel. Then they stitched me up and dressed the wounds and it was over. Poor mum had been waiting for 4 hours for me to get back, now anxious because lines don't take that long, but as she went to ask the nurse I was being wheeled into the ward. They kept me in that night due to my high heart rate (115-130bpm), and they investigated it with a chest X-ray, ECG (which showed sinus tachycardia as usual) and a nuclear (V/Q) scan to check for a pulmonary embolism (blood clot on the lung), which fortunately came back with very low possibility of a PE, and they came to the same conclusion as me - it is due to a combination of my preexisting conditions (Autonomic Neuropathy, PoTS and Hyperthyroidism) plus the stress on my heart from the guide wire irritating it the day before. I run at 100bpm laying down on a normal day anyway. They let me go home later that day (Friday). 

I then ended up in hospital again with an infection in the line site so was in hospital from Tuesday 29th to Thursday 7th. I had a PICC line put in to give me my TPN and medication whilst we had my line out of action (despite fact that the cultures from inside my line were negative, the site grew Staph Aureus (the same infection as my PEG and catheter sites, where it had spread from) and so they stopped using my line for everything except the antibiotics, just being on the safe side). Whilst I was in we found out my Urostomy on the 12th August had been cancelled and would not be done for 7 months due to my hip (despite my hip surgeon saying the op could go ahead) and I was, understandably, devastated. I finished the antibiotics on the13th August but the PEG site is still mucky. Blood cultures were taken on Thursday 21st and are clear, so the PICC was taken out on the 2nd September and we are back to using my Hickman line.

On Thursday 2nd October, my 21st birthday, I went into hospital (University College London Hospital) to be admitted and have a scan ready to have a Urostomy (Ileal Conduit) formed on the 3rd (my consultant agreed that the risk of me dying of the infections was greater than the risk of them causing problems with the screw and plate in my hip, so they agreed to do the op sooner). Though we had a disaster with IV access and pain relief, and the op itself was longer and more complicated than they expected, the recovery went well and I came home exactly a week after my op. Unfortunately I developed an infection in the incision wound, the Urostomy itself, my Hickman line and Hickman site (in addition to the infection already in my PEG) so I had to go back into hospital on Wednesday 15th October. My inflammatory/infection markers were through the roof, I was showing signs of a systemic infection (sepsis) and my haemoglobin (the oxygen carrying part of red blood cells) was very low. The Hickman line had to be pulled out in what turned out to be a difficult procedure, they had to cut a large wound and retract the skin to dig the old line out so I now have a nice scar instead of a small hole. They put in a PICC line (a central line that exits on the arm) temporarily for access as I am impossible to cannulate nowadays (despite the risk that this, too could get infected) to give me fluid, medication and antibiotics while I was without a Hickman line, I was on IV Teicoplanin to treat the line infection and the other infections although I had to go two days without fluid, medication and antibiotics as the PICC line was sitting too low in my heart and they had to keep x raying and then pulling out a bit more until it was in the right place, which took two days. I was due to have my new Hickman line put in on Wednesday 22nd October but a communication breakdown meant I missed my slot. Fortunately it went ahead the next day, though they struggled to put the new line in. Towards the end of the procedure my body went into a form of autonomic shock (a Vasovagal attack) whereby my heart rate rocketed, my blood pressure dropped, I was sweating profusely (like I had been in the shower), I felt dizzy and sick, my chest was burning and I couldn't breathe properly. I had to be given oxygen and IV Ondansetron and an ECG and chest x ray performed. Fortunately both were normal (except for the ECG showing sinus tachycardia, whereby the heart beats extremely fast but stays within a normal rhythm - my heart rate was 140bpm), but I had to spend the next 2-3 hours in recovery being monitored. They were able to stop the oxygen after an hour and they called my mum up to sit with me while I recovered. They had said I could go home after the Hickman line was inserted, but I wasn't stable and it wouldn't have been safe. Fortunately, after the PICC was removed the next day, doctors said I was stable enough to come home. 

However, my problems have not stopped there. My Hickman line and site, the neck wound from inserting the line and the wound from digging out the old Hickman are clear, but I still have Staphylococcus Aureus infection in my Laparotomy incision, my Urostomy and my PEG site, and something else (probably MRSA, which it was positive for when I went into hospital) also in the Urostomy. We're just waiting on cultures for confirmation. My haemoglobin is also still low and I'm very symptomatic with it. Generally my body is struggling. Mast cell issues that plagued me in the past have returned, I'm breathless and very fatigued, I'm running a temperature constantly and am still losing a lot of blood in my urine. 

The stents into my kidneys came out 5 weeks after my operation and since then the bleeding in my urine stopped. I still had Staphylococcus Aureus in my Urostomy, Laparotomy incision and PEG site and a nasty infection called Acinetobacter in my Urostomy. This infection could have been life threatening. I had IV Flucloxacillin for the Staph Aureus, then went straight into a course of IV Co-Amoxiclav which cleared the Acinetobacter infection and the Staph Aureus in the incision, allowing the laparotomy wound to heal. My Hickman line only had one working lumen (my line is a double lumen, which means the one line contains two separate tubes so two things can be pumped into my heart at the same time without mixing) so we were manically trying to fit all my IV meds and at least some of my TPN into that one lumen. I needed a new line and, now clear of Acinetobacter, had a new line put in on Monday 1st December. I just can't believe I've lost another line this year. That's 4 lines lost in 4 months. 

The curve in my spine has progressed rapidly over the last few years and I'm waiting to go back to see my consultant about it. In the meantime we've had to increase my Fentanyl (a synthetic Morphine-like painkiller) to cope with the pain from the degeneration. I've also had the backrest changed on my wheelchair to support my spine as it can't hold me up properly anymore. 

In December 2014 I developed a kidney infection and started IV Co-Amoxiclav 1.2g three times daily on the 24th until the 31st December. Unfortunately I did not improve and was admitted to Southend Hospital on Tuesday 6th January 2015 with a temperature of 39.7. I was started on a 7 day course of IV Gentamycin 180mg once daily. I had a kidney and bladder scan which showed my bladder was full of fluid (pyocystis) which was subsequently drained and revealed to be pus; approximately 1 litre was drained off and my bladder washed out. I finished the Gentamycin course and was discharged on Tuesday 13th Jan to wait for my surgeon to make a decision with regards to removing my bladder. I am awaiting a Mag 3 scan and CT Urogram under Mr Ockrim at UCLH and will see him urgently following these tests for a decision to be made about removing my bladder. 

I had the tests done for Mr Ockrim to look at my kidneys and bladder, which is full of pus again. Unfortunately I became septic again and ended up in The Royal London where I was admitted by my consultant after my clinic appointment via A&E. It meant I missed my appointment with Mr Ockrim, mu went without me, but we need to see him again in April to decide whether to remove my bladder or not, as he does not think I'm strong enough to survive the surgery. 
I had a battery of tests when admitted to find the source of the infection, I was put on 3 antibiotics (Amikacin, Ciprofloxacin and Metronidazole) and swabs taken from everywhere to find the infection. My TPN was stopped in case it was a line infection and my line kept having to be locked with Urokinase to allow it to bleed as when in A&E it took 8 peripheral, 2 arterial and 2 femoral stabs to get blood from me. They were very concerned about my heart which had a long QT interval and ventricular ectopic beats and were going to do a sleep study to see why I keep having apnoeic (stopping breathing) attacks in my sleep. Unfortunately, they did not do the tests they said I so desperately needed. My urine grew an infection and my PEG grew Staph Aureus and Candida (which are always present). The ultrasound showed that my whole bowel is so distended that it's enveloped one of my kidneys and also showed that the faeces they were unable to wash out of my colon when they formed my Ileostomy (3 years ago) is still there and is now a huge mass. We're worried that it could be that which is causing the infections instead of/as well as my bladder. We're going to speak to my colorectal surgeon about it. The doctors wanted to start me on Osteoporosis treatment (Zaledronate/Zaledronic Acid) and non-oral Hyperthyroidism medication but these didn't happen either. I had a subacute bowel obstruction while in too so vomited for 3 days straight until the obstruction resolved, but because they couldn't get a cannula in and they left doing a CT scan too late, we won't know what/where the obstruction was. The admission has got us no further forward, and already I am on the slope towards an infection, sepsis and a hospital admission. I just hope I can stay out for as long as possible as I have so many projects to get on with over the next 3/4 weeks. 

My sats keep dropping and are generally lower than they should be, running about 94-96% but have dropped to as low as 74% on more than one occasion. I had a spinal injection on the 14th April which sadly had no benefit. I also spent a few days in hospital due to infection. There's nothing we can do to stop the repeated sepsis I am getting, and removing my bladder won't help that as the sepsis is arising in the kidneys. It's a bit of a blow. At leas I have cardiac tests coming up and we can get to the bottom of that, and focus on other problems that haven't been a priority as other things have been more pressing. 

From April-December 2015 I had numerous kidney infections, including one lasting 6 weeks after numerous courses of IV antibiotics failed to clear it, my PEG site is a horrendous mess as always, my body is generally struggling and weakening and I am deteriorating. I had a few pseudo-obstructions too. I was diagnosed with sleep apnoea in August following a sleep study and had lung function tests which revealed I have (and I quote) "restrictive lung physiology with abnormalities in transfer factor". I'm waiting to see a respiratory specialist. They are also concerned about the fact that I cannot breathe when laid flat, and never have been able to. My spinal injection in April failed, and so I have been referred to look into a spinal stimulator to try and help with my pain. I am being referred to a "clued up" EDS/POTS endocrinologist to try and finally get my Osteoporosis treated after 2 years with no treatment for severe osteoporosis and despite it being 18 months post breaking my hip aged 20, and look at the Hyperthyroidism as that is also untreated. I am under investigation for other things and am just trying to manage everything as best as possible, to manage pain, nausea, breathlessness, headaches etc. I have episodes where my legs forget to work which is pretty scary, and have had some shoulder dislocations that have resulted in a completely numb and paralysed right arm (2 or 3 times now) that has taken hours post reduction to return (reduction = putting back in place, which I have so far managed myself). It's been a horrendous 6 months for us as a family for reasons I won't go into, and I'm now cared for by a team of ITU nurses (7am-11pm) and carers (10pm-7am), I'm on 21 hour TPN (4000mls) plus 1 litre of Hartmann's every day as my blood sugars were dropping and I was getting dehydrated on 17 hour TPN, meaning once my TPN was switched off I would crash and all I could do was sleep and I had the shakes, horrendous headaches and severe nausea, and without the additional Hartmann's I seem to be very dry and my urine gets dark - who knows why my fluid requirements have gone up again, I was managing so well on 4 litres (which is the TPN bag volume limit). I've managed to cut my medications down though, and Dr Gall my cardiologist was going to ask at a conference about non-oral drugs for POTS to try and improve my symptoms, aside from Octreotide which is an injection (I can' t take it as it caused my gut to shut down (resulting in constant faecal vomiting, dehydration, excruciating pain etc.), caused horrendous headaches and made my blood sugar drop very low). So it's been a busy 6 months on many fronts. 

My team are trying their best to help me get the most out of life that I can, and I am extremely grateful to them all.  


  1. Wow, I can't believe how much you are going through. I too have all the same problems, but my GI tract is working at 30%, so a Jtube has been useful thus far.
    I wanted to let you know, as far as the urinary problems. I have Fowler's Syndrome, aka Neurogenic Bladder, so I am not able to urinate on my own. I self catheterized for a long time but eventually had an Interstim Neurostimulator placed. I just though maybe this could be a better alternative.
    I hope things stay on the "good" side and that you have more better days. You are in my thoughts and prayers, my Zebra friend. I am sending Gentle Hugs your way!

  2. Wow, this was pretty sad to read, I hope you are keeping well. Email me if you need any support, I am in a wheelchair too, I am a T11 Incomplete spinal injury. rb1003@hotmail.co.uk :)

  3. Stumbled upon your blog and am sending positive vibes your way. Keep your chin up sister, you are a beautiful person inside and out. =)

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  5. Hi, I came across your page and just wanted to say that I hope you do get better. I know the medical aspect of the illness but there's always hope. Keep your head up and always think positive. =)

  6. Have you been worked up for mitochondrial disease?

    1. I'm currently under investigation for it x

  7. Hi just wanted to say thank you for writing this website. I can really relate to what you are going through, i have been unwell for the past three years with several things (including fowlers syndrome) and my life has completely turned upside down. Im in and out of hospital several times a month, i keep a constant hospital bag packed, i used to have a normal life (im 32) but now im dependent upon my mum, i cant really have a social life, work, go places and am in constant pain, it is also a struggle to eat. I find that although people try to understand what im going through, they cant really, and it is so lonely and isolating. Its also very scary and frustrating at times. Im sorry to hear what you have to go through i think your such a strong and brave person. Your story has really inspired me and i feel less alone knowing that there are people out there who go through all this too, especially as a younger person. Theres so much i want to say but it would take up too much room but thank you for this website and talking about what you have to go through, keep strong x,

  8. I am loving your blog just like my life with Irlen Lucy check out my blog Day of life of a person with irlen.

  9. God bless you. Just came across your blog. You are so resilient and beautiful. I hope your journey is filled with support and love.

  10. Lucy, one of the saddest medical stories I have ever read. How can one beautiful person endure so much, but there are no answers, only love to keep you going. I pray your life will hold deep meaning for you, and I am sure your Spirit is perfect for all time. I was seeking help for my daughter but now I realise how fortunate we still are compared to others such as you. God Bless you, Lucy, and your family who love you, and may all those who work to help you be workers in Light. Amen.

  11. I can't stop crying reading your story Lucy. You are really a bright star in the horizon of your unique sky which l can see filled with massive positivity and I feel with my heart that your health will improve one day.

    Loved ones go through so many tests and tremendous trials because they are loved dearly.

    Keep positive as you always have been.

    Please keep writing your updates as and when you are able to. I learnt so much reading your story, l'm going through EDS HT3 and all the complications with gut too but not as severe as yours.

    Thank you for the effort you are putting in sharing your story despite what you are going through.

    You will be in my prayers forever.d. X

  12. I was reading your story, and feeling that my problems were absolutely nothing. What you have gone through is utterly unbelievable, and I really don't know how you have managed to keep going. You are a true inspiration, and thank you for sharing such a gruelling journey. You are a true star. x


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