I can’t stay away.

Agony. I started this week off with a knee dislocation.

I had only been discharged from Medway Hospital for a week and still suffering the ill effects of glandular fever when my knee decided to come out of it’s socket as a result of very little movement or pressure. The pain was overwhelming, and whereas usually I can manipulate my joints as needed, my knee just wouldn’t stabilise and I was relying on topping up my usual Fentanyl constantly to try and control the pain.

On Wednesday my Mum and I decided that we really did need to go to A&E. As time has gone by, my ability to know whether or not hospital intervention is needed has greatly improved.

Costa to get us through the waiting times…

 The hospital was packed; a constant stream of paramedics were bringing patients in, there were people covered in blood, people being sick, patients shouting out all making an environment that was very hard to cope with. Sitting hoping the five hour wait would hurry past, the overwhelming thought that I long for nothing more than to be ‘normal’ came to the forefront of my mind. Is it really too much to ask to not constantly be in hospitals? To have a day that doesn’t revolve around taking medications or partaking in treatment?

A sight that I see way too often

We were finally allowed home just after 3am, and as a result of seeing the doctor, my knee was back in place, I was fitted with a ‘cricket bat’ style splint which I have to wear to immobilise and support the joint and I have been made an appointment in the fracture clinic for the 19th June. As is the case with all my dislocations, I could have come home with my leg in plaster for the initial recovery period, but between the doctors and I it was decided that this wouldn’t be the best way forward as my other joints aren’t strong enough to support the cast.

I’ve got another 10 days before I’m seen in the fracture clinic for the initial swelling to settle, and I will then be fitted with a hinged brace. This will enable me to slowly increase the angle through which my knee is able to bend, enabling it to heal as much as possible and decrease the chance of a subsequent dislocation (although I’m not sure how successful that’ll be!).

Being unwell recently has been relentless, and I need a break. There isn’t one part of my body that isn’t impacted by illness; infections, dislocations, catheterisation, irrigation, taking meds, injecting myself and the list goes on. I would give anything for a day of feeling ‘normal’ but I am grateful for what I do have, and part of that is the most wonderful family I could ever wish for who are there and constantly support me no matter what. It may just be me with the official diagnoses, but my family have them too as they never make me feel like I’m facing my battles alone. ♥

Glandular Fever.

The past week has been an absolute whirlwind.

I knew something was coming on as I felt so achey and fluey but had nothing to show for it; on Sunday it all came to a head. I was really struggling to swallow (even more than usual) and so my Mum took me to urgent care. My tonsils were huge and after seeing three different medical professionals there they sent me to a local A&E due to the complexity of my other health conditions.

 

We arrived at Darent Valley and after the doctors had a look, I was told I really should have come earlier. My tonsils were huge and obstructing my airway so I was given strong IV antibiotics, IV steroids, fluids as I was struggling to drink and goodness knows what else. I was then blue lighted in an ambulance to Medway where they have the local ENT specialists, but this was done as a matter of urgency as the integrity of my airway couldn’t be maintained.


When I got to Medway I had various IV treatments and was told I’d have to be admitted – what I’d been dreading😫. My bloods came back showing positive for glandular fever and gave me a reason for why I was feeling so unwell. After three days in hospital I managed to convince them to let me home. My tonsils had shrunken slightly due to the steroids, and I was sent home with a course of another 10 days of antibiotics, and an appointment to be seen in clinic in 6 weeks to consider having my tonsils taken out. 

I feel unbelievably exhausted, but I’m so glad to be home; the main reason for this was because my best friend was 21 yesterday. I hadn’t seen Katie since briefly at Christmas, and we have both been battling horrific illness and dealing with hospital admissions etc ever since. I wasn’t going to miss her birthday for the world, so with the help of her step-mum I arranged to come and suprise Katie in Brighton with my supportive parents agreeing to get me there.

Yesterday was one of the days that to me shows why it’s worth it to carry on battling. I had tears of joy when I saw my bestie, and we were both so overwhelmed. Despite wheelchairs, medications and illness, I had a day where I felt like a ‘normal’ 20 year old.

I feel so lucky to have a best friend as supportive as Katie, and despite not seeing each other for six months it was like we had never been apart.❤

Jeffrey loved his first trip to the seaside and coming to meet his Auntie Katie on her birthday 🐶

 To say the past week has been difficult would be an understatement, but every cloud has a silver lining; the one that has come of illness being the strongest, most wonderful friendship I could ever imagine. 👭


Side by side or miles apart, true friends will always be connected by heart.

Ileostomy.

29th April. Today. It’s the day I’ve been waiting for throughout the past few months; an appointment with a specialist colorectal surgeon who has a lot of experience with EDS patients. 

The degree to which my EDS impacts my bowel has been getting increasingly worse. I’m fortunate that my Crohn’s has stabilised on Humira injections as the EDS has led to numerous laxatives being required, in addition to irrigation, enemas and frequent colonoscopy bowel prep. My most recent hospital admission was due to my bowel being on the verge of perforation, and it was evident that the regime of medications I was on were no longer working, and something needed to be done. I was fortunate that on that occasion the bowel prep finally had some effect and I avoided emergency surgery, although I didn’t think it’d be long until surgical interventions were needed. 

I went to the Royal London Hospital today with my parents who have been there for me through everything life has thrown at me. The surgeon was very understanding of my position, but told me what I was hoping not to hear. I had exhausted every medication regime, irrigation pattern, enema frequency, and he advised me that the best way forward would be to have surgery to form an ileostomy. 


Ileostomy. Stoma. Bag. I had been expecting this news, although I couldn’t accept it myself; as I write this post my vision is blurred by tears. Now I’m home, I still don’t think it has quite sunk in. I’m 20 years old, and I’m going to have a major operation to change how my body looks, to make me slightly different to healthy people and make some changes in my life. I’m holding on to the hope that in addition to the physical changes, it’ll also improve my quality of life as the truth is I just can’t carry on with how things are at the moment. In theory I have made the decision to have the operation, but quite honestly there wasn’t a choice. 

It’s going to take a while to sink in, and I just hope I can get my head around things; I have a bit of time as the waiting list is months long as I want the particular surgeon to operate due to the complications my EDS brings. I’ve been told that the stoma may hernia which is common in EDS patients, but that can be dealt with if they arise with revisional surgeries. I’ve also been told that, as my bowel isn’t going to be taken out initially, I may still need my Crohn’s medication although as my colon isn’t being used, the problems may just ‘fade away’ which is the topic of a really interesting area of research at the moment. As my colon will still be there, it’ll still produce gas and mucus and if this becomes troublesome the surgeon will operate again to remove my bowel and potentially form a different kind of stoma. 

Writing my blog posts help, but it all still doesn’t feel real. I really hope that things will get easier with time and next time I write a post I’m in a better position with things. I really am so fortunate for everyone who has text or been there today; I really wouldn’t cope without you all. 

Sinking.

Another month has passed and my ill health has continued to be as relentless as ever; trying to keep positive is getting increasingly more difficult and any sense of normality has been slowly slipping away. 

I had a blood result relating to the function of my liver come back at over double the normal range, indicating that my liver wasn’t working as it should. It was thought that this was caused by a gradual increase in one of my Crohn’s medications, and so I’ve decreased the tablet back to the original dosage and am having to have repeat blood tests weekly until it stabilises again. It’s a worry as the level of the medication in my blood was very low hence the dose increase, and I need it to help stop my body rejecting the Humira. My biggest fear is having to go back on high dose steroids as the Humira has been the ‘wonder drug’ in enabling me to cut down the steroids, but it’s something that very frustratingly is out of my control. I’ve continued to have hydrotherapy, and I really enjoy being in the pool but despite that and community physio my dislocations have continued to be relentless. 

Mentally I’m really not coping. There’s no denying it and there’s very little that can be done to help. I’ve been tried on various antidepressants at different doses and seen counsellors, psychologists and psychiatrists with no improvement. I’m 20 years old and I’m dealing with catheterising, irrigation, wheelchairs and countless medications just to name a few; I’ve been totally robbed of my independence and of the life at university I’d dreamed of having. I’m beyond people telling me to ‘stay strong’ and ‘keep going’ as there is no choice but to do that, but having to deal with illness and it’s effects day in, day out is something I can’t cope with for much longer; I’m sinking. 

I had an assessment at Stanmore and have been accepted onto the three week inpatient rehab programme there. In a couple of months I’ll be admitted for three weeks from Monday to Friday for a pain management scheme with physio, hydro and psychotherapy support. I’m hoping that’ll give me an alternative view on my life as it is and help me to accept what is happening and help cope with the situation. My bowel function is deteriorating rapidly and a stoma is now being seriously thought about; I have an appointment with a colorectal surgeon at the end of April to discuss the options. Daily irrigation, a complex laxative regime and enemas just aren’t working and are hugely decreasing my quality of life – I’ve exhausted all the options and I think the operation may be the best option. It’s such a hard decision to have to make, and if my bowel had previously perforated and I’d been rushed in for emergency surgery I wouldn’t have the option; I, of course, wouldn’t want that to be the case but to actually have to make the decision really is difficult.

I had test results back showing that my lungs only work at 46% of what is expected; the cause of this is unknown but the test has to be repeated on Friday at King’s College with the consultant observing so we can gain a greater understanding of what is happening. It is also thought that I have a type of sleep apnea with the top of my airway collapsing as a result of the EDS and so I’m waiting for further respiratory reviews.  I now have splints for my thumbs and fingers which I’ll use when they’re at the greatest risk of dislocation and I’m waiting to be seen by the appliances service at the hospital to see if any other splinting etc could be of benefit. It’s so hard when the weakness is so widespread; I can have a splint on a joint but then another one dislocate (which is what happened earlier with my ankle!), so managing the condition isn’t easy.

I truly do wish I could come on here and write a really positive post, but unfortunately it wouldn’t be a true reflection as I really am struggling at the moment. I still do recognise that we have such supportive friends; organising craft sessions to help me take my mind off things (I’m so proud of the doll I made!), just popping in to see how things are and sending little cards or texts remembering that I have certain appointments really are so kind and do help to lift my mood. I can only hope that going forward each little part of my illness can be managed better and co-operation between different specialities can help improve my condition. ‘Happiness can be found, even if the darkest of times, if one only remembers to turn on the light’. 


Another Admission…

Admission. The last thing I really wanted to think about since being in hospital for three months last year, but all too soon I’ve been admitted to hospital again. It was only for four nights this time but that still was four nights too long and full of stress, worry and a lot of time spent in tears. 

My bowel has been gradually stopping working despite different laxative regimes. I’ve also had to try irrigation but have to be careful not to flare up my Crohn’s (nothing is ever straight forward). The laxatives I take are rotated weekly to try to keep my body’s response to them as effective as possible but recently nothing has been working. I was then advised to take Picolax which is used for colonoscopy preparation, and despite taking nine sachets of that in a week it still didn’t work and there was nothing else the GP could suggest; after taking advice from the district nurse I came to A&E last Thursday.

I was sent for scans and blood tests, and they revealed that my large bowel was completely full and on the verge of perforation. We then had to wait to be seen by a surgical team to know what the next step would be. By this time it was almost midnight and my Mum, sister and I were drained. It was agreed that I would come back the next day into the ‘Ambulatory Care Unit’ and have Gastrografin which would hopefully work to clear my bowel out. I returned the next day and was seen by a lovely consultant who explained to my Mum and I that I really did need to be admitted so any medication administered could be monitored and the risk of my bowel perforating minimised. 

A surprise visit from Jeffrey!

I was started on Moviprep which I’ve had before in preparation for colonoscopies etc; it’s so very hard to get down and regardless of it’s previous potency (when I had to drink it in the loo!), my bowel function has deteriorated so much that it had no impact whatsoever. As usual it was a struggle to get a cannula in, and I was given IV fluids which, when I woke up in the morning, had all gone into my arm as the cannula had tissued, and my arm and hand severely swollen from the fluid. I was eventually visited by an oncology nurse who had experience in accessing port-a-caths and so further fluids and anti-emetics were given via that.

 

A sight that’s been too familiar…

The staff here were at a loss as to what to do next and were trying to contact my other consultants for advice. Daily I had two or three jugs of Moviprep and there were talks of surgery, stomas and needing a bag. Gradually the laxatives began to work, not as much as was expected, but it was still an improvement and enough to buy me time between now and my next specialist gastro appointment to wait to be able to discuss longer term plans and the need for surgery then. I’ve managed to persuade the staff to let me home and I can continue the recommended medication etc there; I can only hope that I manage to stay out of hospitals for a while but unfortunately the admissions are inevitable. 
I’m so grateful for my family and friends who have been my absolute rock throughout this nightmare admission; it’s the most difficult thing watching everyone carry on with their lives whilst I’m lying in a hospital bed with my life falling apart. I never imagined I’d be coping with these health issues but I just have to make the most of every day and be grateful that, thanks to my family and best friend, I’m not facing them alone.

The lack of care about rare.

February 28th marked ‘Rare Disease Day 2017′ which helps raise awareness of conditions such as Ehlers-Danlos Syndrome which are severely underdiagnosed. EDS being classed as rare has become very significant to me recently; it has been daunting speaking to various healthcare professionals who do not have any knowledge of the condition or its impact on patients’, such as my own, lives. 

I like to look positively on things when I write my blog posts, but I’ve come to realise recently that isn’t always possible. I get lovely, supportive comments from family, friends, strangers, fellow EDS sufferers and people who just come across my blog which I really appreciate, but there is a time, such as now, where I don’t want to be inspirational. I don’t want to pretend that ill health has only a positive impact on my life, I don’t want to come across as being able to cope with it all and it is true that I am absolutely exhausted and drained from being unwell. 

The reality of having a condition that is for life, that is impacting more and more systems in my body and that very few people understand is terrifying. My bladder doesn’t work, my bowel is stopping working, I’m struggling to swallow, I have multiple joint dislocations every day and I pass out when I change posture.

If the symptoms themselves aren’t hard enough to deal with, there are side effects of medications and further tests and procedures with further complications to be endured. A slightly odd aspect of EDS is the lack of effectiveness of local anaesthetic; this week when I needed a filling cleaning out and re-filled (after a chipped tooth due to my steroid use), I had the strongest anasathetic and I was still constantly needing it topped up, barely taking away any of the pain which I had to tolerate. It also happened to be Jack’s birthday and even seven hours after the procedure when we had dinner I couldn’t move half of my face – the numbing didn’t work for what it should have, and made everything else difficult!

 
I had a Flexible Sigmoidoscopy this week after a narrowing in my bowel was picked up on during a recent test. Before the camera could even be organised, there was a lot to put in place in order to have a vascular nurse present able to access my port to administer the sedation and pain relief as my veins are so poor a cannula cannot be used. When we got to the hospital, the nurse accessed the port but it soon became apparent that the hospital had no Heparin and so wouldn’t have the ability to ‘seal’ my port after using it. This ended up meaning that I had to come home with the needle still inserted into my port-a-cath and contact the district nurses to come home, flush and ‘Hep-lock’ my port, preventing blood from clotting in it. The lack of understanding of EDS and lack of familiarity with the devices which I have to rely upon causes so much additional stress to me which has been very hard to accept recently. 


As another example of added complexity the lack of knowledge of EDS brings, I also had a Video Fluroscopy this week to assess my swallow function which is deteriorating. In order to do the test I had to ingest Barium, both as a drink and pasted onto different foods. Even in a healthy person the Barium can cause the bowel to slow down, but as my bowel already barely works it hasn’t been able to process and get rid of the Barium, and instead I’ve been taking Picolax (which is the laxative used for colonoscopy preparation) with absolutely no result. I’m yet to know what the next step is if the Picolax continues not to work, but currently have increased the Picolax to every other day to try and clear everything from my system.
Even after my recent struggle to cope with everything and the realisation that the symptoms will never all ‘go away’, I have to hang on to the hope that I’ll have periods of relief from it all. I’ve been referred to the inpatient rehabilitation program at Stanmore; this will be a three week Monday to Friday inpatient stay where I’ll receive intensive physio, OT input and Psychological support whilst meeting people in a similar situation to myself. I’ve also had a meeting with local social services about a range of referrals which they are going to make to hopefully help me in various ways, and also equipment and adaptations at home to make life easier. 

There are illnesses both more and less uncommon than Ehlers-Danlos Syndrome, and my eyes are only open to a tiny window of rare syndromes and their implications through my own experiences. I hope one day EDS will be a diagnosis more well known, not due to more people suffering from it but from greater research being carried out into it. I can’t put into words how isolating the fight with my health has been at times; not fitting into a ‘correct’ ward in a hospital and not being suited to a certain regime of support has made my illness that bit more draining but I have the most supportive family I could ever imagine and for that I am truly grateful. 

When does this all end? 

Another blog post and unfortunately still battling with my health; it continues to be a really difficult time with no reprieve.  The lack of knowledge of EDS has been so significant to me recently, and whilst there are many illness still requiring greater recognition and research, an increase in EDS awareness would be amazing too. 

I was referred to have a ‘Short Synacthen Test’ as, after being on high dose steroids for the best part of three years, it is very unlikely I’ll be able to come completely off of them as my body has lost the ability to make Cortisol myself and so a maintenance dose will have to be found. I went to a day unit for the test, and despite numerous medical staff trying they were not able to get a cannula in to administer the substance for the test nor to take bloods needed. In the end the test had to be abandoned and re-arranged for a day when a member of the IV team could come and access my port which enabled the test to be done; I say it far too often but my port-a-cath is such a God send. Hopefully the results won’t take much longer and I can wean off to the lowest safe steroid dose.

One of the most challenging parts of living with chronic illnesses is dealing with pain; joint pain, stomach pain, headaches – the list is never ending. I’m under the pain team at King’s College Hospital whilst I’m waiting for a referral to a more specialist clinic and my medication regime has been changed to try and better manage my symptoms. My course of hydrotherapy has finished and it’s been the most effective treatment I’ve had – being in the water takes all the pressure off of my joints and the relief is overwhelming. I’m being referred again which I can’t wait for especially as I am unable to go swimming myself as I can’t get the wheelchair close enough to the pool. I continue to have psychological support but coping with my ill health mentally has been the greatest challenge for me; my illnesses have forced me to drastically change my way of life and live my days around taking medication – that’s a change I’m still struggling to come to terms with. 

My bowel not working continues to be problematic and I have had a different medication schedule implemented to try and help alleviate symptoms; I’ve also been referred to a nurse who specialises in ‘Biofeedback’ and bowel retraining and has suggested I use a gentle irrigation system as we also have to be mindful of flaring my Crohn’s Disease up (when is life straight forward?!). It has been suggested that I may be suitable for a sacral nerve stimulator which I was previously dissuaded from and this keeps me hopeful to regain some function and sensation in my bladder and my bowel although there is a fairly significant procedure which isn’t always successful to do so.  

The most significant medical thing recently has been an appointment at Stanmore. I was referred there after constant dislocations, joint problems and getting no relief from more local services. I’m over the moon as I’ve been referred to a three week inpatient rehab programme there which consists of intense physio, OT input and psychological support as well as the chance to meet others with similar conditions to myself whilst staying on the ward. There will be a wait for this but I’m hoping this will be the admission I need to stabilise my conditions as much as possible, getting my health into a more manageable state to be able to return to uni. 

Aside from my very medicalised life, I’ve been taking Jeffrey to puppy training each week. My Mum is able to take me in my wheelchair and when I am able to I can do the training with Jeffrey, and my Mum gets involved when there’s something I’m unable to do.

 I’ve also visited Cambridge with my parents where we have secured an accessible, disabled room for next year which is so reassuring; I can’t wait to get back and have some ‘normality’ back in my life. There is still a lot to be put in place to support my needs and there are exams to be sat before I return but slowly ticking things off alleviates huge worries of mine. I still have quite a lot of appointments coming up in the near future, but also am making a concerted effort to organise some nice things to help keep me positive throughout it all.