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. 

Relentless.

My ill-health recently has been relentless; when do I get a break? I promised myself I’d treat the new year as a fresh start but already 2017 has been a struggle. 

Jeffrey is my one focus away from my very medicalised life, and since we’ve had him he has made me smile when I didn’t think it possible. So many family friends have come round to meet him and he has been so lucky with all the treats and toys he has been given. Jeffrey’s arrival has brought us closer again to friends we haven’t seen in a while which has been  really lovely and I’m so fortunate for all of the support they have provided and continue to do so. 

Apart from having Jeffrey in my life, this year so far has been full of hospital appointments, procedures and decisions which I’ve had to make about my care. I continue to have physiotherapy at home and I’m having hydrotherapy which is such a relief to my joints. I’ve had a gastro review and my immunosuppressants have been increased to try to prevent my body rejecting the Humira which I inject. 

I was seen in the liver department of King’s College after the scare that I may have Wilson’s disease which was picked up during my most recent admission. Although I’ve had a few abnormal blood results, thankfully it is looking less likely that I have the condition and we are waiting for the results of a blood test that checks for the gene causing the disease which will take around 6 weeks to come back. My port care is still at the Hammersmith Hospital which is such a treck; the visit to the chemotherapy department there to have the port flush really makes me grateful for what I have and the reality hits that there are so many people worse off than I am. 

I had my suprapubic catheter formation scheduled as I had made up my mind that I wanted the procedure done. However, after seeking further advice and opinions (which are usually conflicting!) I had a wobbly so have put it on hold for now. It is still an infection risk, and I’m not sure I’m mentally prepared to have another tube coming out from my body, making me feel even less ‘normal’. There have been talks that I need a further cystoscopy done to look into my bladder, but my body just isn’t strong enough for the anaesthetic at the moment. The prophylactic antibiotics which I’m taking are having significant side effects so I’m going to go to King’s for regular bladder installations to try and prevent infection whilst I try to get myself together to make a decision about longer term plans.

Last week I had an appointment with the respiratory consultant to follow up my periods of desaturation when my oxygen level drops and there has been no real explanation as of yet to why it has been happening. I had my lung function test repeated as my previous one had showed my lungs were not working as effectively as they should be. I’m to continue having respiratory physio to try and retrain my breathing patterns which are abnormal due to my PoTS syndrome and will be continuously monitored in the outpatient clinic. 

On Thursday I had to endure some hospital tests requested by a neurogastroenterologist as my EDS has been significantly impacting my bowel. Five days prior to the test I took capsules containing 50 shapes which show up when an X-ray is taken; in a healthy person when the test is performed there should be less than 10 capsules remaining, but on my scan all 50 shapes were still present, providing categoric evidence that my bowel isn’t working. I had some further physiology tests on the same evening to test how the muscles etc of my bowel work, and I’m waiting for the results of those in my follow-up consultation next week to see what the next treatment to try will be as so many previously have stopped working. 

The severity and multi-systemic nature of my EDS has worsened significantly recently and I’m finding it increasingly difficult to cope with. I’ve been in touch with university to arrange the exams I have to sit prior to my return, and I’m so desperate to get back to some sort of normality and away from how medicalised my life currently is. It’s so very easy to take things for granted and I would be grateful beyond words to have just a fraction of my independence back. I most definitely don’t have the life I imagined ’20 year old me’ would, but I have a different way of life and one that I must adapt to. I’m lucky as I don’t have to adapt to it alone, but instead I have the most supportive, loving family I could ever wish for who make sure I never face my struggles alone. 

2016.

To say the least, 2016 has been an absolutely awful year and I can’t wait for the closure from it that the arrival of 2017 will bring.

I’ve had four separate hospital admissions, spending two of them in Addenbrooke’s, a fortnight spent in Darent Valley Hospital and then the most recent three month admission being split between St. Mary’s hospital and King’s College Hospital. It’s been a year that has brought my parents, sister, Nan and I closer as a family, and made me appreciate the constant love and support from them more than ever. 

I’ve faced losses due to illness; friendships, holidays, a year at university, body image, confidence and greatest of all a sense of normality. There are things that would seem insignificant to anyone on the outside, but it’s the smallest things that mean the most to me; getting up every morning and seeing my scar and lump of the port in my chest, my puffy face and weight gain after nearly three years of high dose steroids and having to rely on my wheelchair feeling so self conscious and like I’m a constant inconvenience. Whilst I know a new year won’t cure any of my health problems, a fresh start of coping with them all helps me to move on from some of the experiences of the past twelve months and gives me the ability to consider what I’m facing in perspective. At the same time I know I’ll still wake up on new years day and still be unwell.

I’m hoping to return to university in October 2017, and so I’ve still got quite a large period of time to stabilise my health before then. My EDS and PoTS is at the severe end of the spectrum and as it’s so multi-systemic there are still multiple consultants needed to manage it. There have been talks about operating to insert a suprapubic catheter which will be a tube out of my tummy and can have either a catheter bag or a tap at the end of to help reduce the constant infections caused by self-catheterisation. I really was hopeful about being suitable for Neuromodulation which I’ve mentioned previously in blog posts, but it involves quite significant procedures which my body wouldn’t be able to cope with at the moment and due to the faulty collagen as a part of the EDS, the consultant didn’t think that the device would hold in my back, leading to requiring further surgeries. Whilst I appreciate a professional opinion, I was disappointed that we aren’t going to pursue the Neuromodulation now as I thought that would get me as close to normality as I could get. The suprapubic catheter has been strongly advised given the severity of my health issues currently, but I’m finding it hard to come to terms with the fact it’ll be another tube and another step away from my life as I’d like it to be. I’m just having to remain hopeful that research will be done and the Neuromodulation type treatment improved so there can be more of a ‘cure’ in the future. 

I have so many outpatient appointments scheduled in 2017 but with chronic health problems that is inevitable; I just need to face them with a more positive outlook. My life has become so medicalised and the mental struggle coping with it all has been overwhelming. In January alone I’ve got consultations for my liver problems, with the pain team, with my gastro consultant and with an immunologist just to name a few, in addition to the possibility of the procedure to site a permanent catheter. Sitting at hospitals waiting for appointments is so draining, but I just have to take each day as it comes, make the most out of the good days and appreciate the things I can do. Jeffrey’s arrival on Tuesday has made this week a bit of a whirl-wind; he is absolutely beautiful but also a bit of a terror at the moment! I’m so grateful to have him as a distraction from my struggles and as something to focus my time on – I can’t believe it’s taken 20 years and a stay in intensive care to finally get a pet! 
As this year comes to a close, I can only hope 2017 is better; fewer hospital admissions, accepting the long-term nature of the health issues I do have, getting back to uni to have some sense of ‘normality’, and to have quality time with my family who have been everything to me. I never imagined I’d be 20 with multiple chronic diagnoses, having to rely on a wheelchair, needing to take in the region of 60 tablets a day, having to inject myself as part of my treatment, being constantly unwell and having my life torn up into little pieces and thrown up in the air like it feels it has been. I am fortunate as there are people worse off than me and so, as a resolution for 2017, I will appreciate what I do have much more, spend more time with the people who matter and enjoy life as “you don’t know how strong you are until being strong is the only choice you have”.