Hospital Bound Once More

Here we go again...

I am currently writing from my hospital bed on the Respiratory Bay of Ward 23, Royal Preston Hospital, what can I say about being a patient except that you have to be just that; patient! Beyond belief to be honest...

In the Resuscitation Bay of Royal Preston Hospital on Friday

Being An Advocate

I am starting to realize the importance of being an 'Active Advocate' for my own Rare Disease, Melkersson Rosenthal Syndrome (MRS), with 27-years of first hand experience who is better placed to know what works for me and what doesn't? Of course I don't know of a cure, if I did I wouldn't be here, I can't keep my illness under control with the medications I have at home already, because if I could then again... I wouldn't be here!

So why, then, do they always ask the same question with regards to giving me IV medications? Be that; "But you aren't on IV Morphine at home for pain are you?" 

     "No, at home I am on 75mg Fentanyl Patches, which are changed every 72 hours, I take regular Paracetamol and Oxynorm 20ml as and when required. If they were sufficient for the pain I am currently experiencing do you honestly think I would put myself through another hospital admission?"

Does anyone else face this problem?

I also came across the dreaded 'Google Doctor' again, basically a Doctor who previously to meeting me had never before heard of MRS, then of course off he trots to google the disease and comes back arguing with my 27-years experience and my devoted partner/carers almost 9-years experience, the combative stance? facialpalsyUK's definition of the illness, which is severely lacking, outdated and incomplete.

He quoted the website verbatim, it was cringe-worthy for both myself and Amy, even his own Junior Doctor, who had spent the time listening to us and absorbing what we were saying, and had also met us previously, was shuffling his feet awkwardly and averting his eyes at particularly 'difficult' moments when the atmosphere dropped beyond zero and we clashed yet again in a head-to-head battle of wits.

The Outcome

So in the end the junior doctor won out, we won out but not before expending needless energy and struggling with anxiety and stress that exasperated my symptoms and then today the same thing defending my treatment plan to the nurse in charge of the Bay for today. 

Sometimes it doesn't matter how nicely someone says something when what they are trying to say is that they disagree fundamentally with something that is helping you. That they want to change it for their own reasons with no relevant background knowledge of your illness to something you know will extend your hospital stay by twice or even three times as long!

She advised me that she would be speaking to the doctor about changing my IVs to Oral medications, as the on call doctor would be completely unfamiliar with me and my rare disease I knew this could spell disaster, that I could end up stuck in hospital, backtracking and in agony for weeks...

Get some guts...

My veins, My cannula, My body, My Rare Disease; Please just make me better rather than argue with me...

So I puffed out my chest, mentally speaking, picked my guts up off the floor, and remembered what Amy said to me; 'don't back down, make them listen, they don't know MRS, they don't know it like you and I do, they don't know you. Do not let them change anything on your drug chart or care plan without letting me (as in her) know first."

So I said that I would want to speak to a doctor prior to any changes being made to my drug chart and that I was an advocate for my own rare disease etc etc. well it had the desired effect, I did feel again the unnecessary confrontation, which let's face it is really not needed. 

I am on a Respiratory Ward, I know I am not the average patient that comes here, I am not what they expect on a Sunday morning... So leave it to the doctors and myself to discuss my care and come up with a suitable plan of action, why try and interfere with that very delicate balance that was clearly having some kind of positive effect?

I have spoken and dealt with this all day since and as she pointed out during one of our conversations, we are all normal people outside of this hospital, with normal lives, and we have no real concept of what is happening in those 'normal lives' that each other are living. 

...So in essence, although she did not say this herself, she could very well be bringing a little of her home stresses to work, and who is not guilty of that at some point in their lives.

On-Call Doctor

Eventually a young on call doctor did happen by, but luckily by this time Amy was here, and she spoke up on my behalf, explained the illness and how we were worried that the medications and treatment plan might be altered too soon and I would end up back to square one. 

The young lady doctor was very understanding about this and explained that she had no intention of changing anything as it would clearly not be the right course of action at this stage, as altering a method that had only just started to work was detrimental to my care. Finally someone gets it!

Perhaps I'm Overtired?

Last night I was so damn tired; but this being the place that it is things happen, not such wonderful things, sad things, things that take you on a mental journey to a place you do NOT want to re-visit! 

So there I was back in that place where my heart starts to break all over again while listening to the very audible sound of another family's collective hearts crumbling as they said goodbye to their loved one.

You can't switch off from that, no matter how much you might want to and even if you could it feels kind of insensitive to try. To pop in a pair of earplugs and know that you will be snoring away, singing a background tune to their grief that is completely unsuitable. 

So no, I sat awake on my Ipad in a lot of pain but not wanting to ask for painkillers because it seemed again like an intrusion on their grief to start asking for something so ordinary and mundane as pain relief when they were visiting a section of their own personal Hell.

Waiting for the appropriate moment to catch a nurse when she is not busy with a grieving relative or crying herself is not a task that I would recommend. I don't know how they do this job... 

Sometimes I might get upset and annoyed when I am not treated the way I would hope by some, but at times they do really shine, admittedly some more than others, but even so... I couldn't do their job.

My bed for the night; I am hoping that tonight I might actually manage a few hours sleep in it...

Made An Impression

A few years ago I was a patient on this same ward, different bay, and again there was a very sick lady in the bay with me. She was rude and aggressive leading up to the days when she began succumbing to her illness. Not just to the nurses and doctors but to the other patients as well, and no I wasn't spared...

The only time I saw that woman smile was when her family visited, then she changed completely, her face relaxed, she became polite and gentle... A much nicer version of the person we were met with the rest of the time. 

Not to be cruel but she smelt to high heaven, I have never before smelt anything like it, she kept insisting on enemas that apparently she didn't need but could somehow talk her way into receiving anyway... And usually just in time for dinner too... :-(

What happened next...

... Then one night she took a turn for the worse, it wasn't really expected as suddenly as it happened as far as I was concerned anyway, it was shocking, suddenly I was awoken in the night to a lot of commotion around her bedside... then it went quiet.

The lady in the bed opposite me, who I had become friends with, text me and said that she thought that the lady beside me must have passed away. But then no sooner had I opened the message then we heard the nurses talking to her.

"Now... sweetheart, we are just going to roll you, we'll get you all nice and cleaned up **name omitted** don't you worry..." and so it went, the lady opposite and myself were very confused. The other lady in the bay was peacefully asleep and completely unaware.

Then one of the two nurses popped her head around my curtain, ours had all been drawn during the commotion, she asked me if I was okay and explained that the lady had sadly passed away.

Now I have known these two nurses for a number or years since then, back then I didn't know them too well, as at that point my illness was just getting it's 'nasty little grip' on me. Anyway it transpired that despite all the mean jibes and nasty comments, the biting, pinching and punching... yes all of that happened and to those nurses too...

... When it came to it, they showed that lady the most beautiful respect, when her family arrived, teary and sad, crying uncontrollably, I could hear but could not see. I heard the nurses give them respect and patience too, offer a comforting shoulder to cry on, give them the peace and space they needed to say their goodbyes.

During all that followed that family were not made aware of the special, or more likely typically respectful, manner in which those two nurses treated their mother, I wish I could have told them, because I only hope that my loved ones who have passed were treated so kindly.

It wasn't done for a pat on the back, or a well done, it was completely altruistic, there was nothing in it for them other than knowing that they had done the right thing by a very poorly lady who had passed on in their care. That night has always stayed with me and probably always will.

I have no end of respect for those two nurses and all the nurses like them, they are what 'being a nurse' should be about. It was a beautifully sad event to witness and in some strange way I am glad that I was there to listen in, to know that there really is goodness in the world, beating in the hearts of all the nurses who nurse because they genuinely care.

"I see the bigger picture, sometimes my lens is just cloudy"

I do see things as they are, sometimes I do get bogged down with the negative, who doesn't when they are always poorly and faced with people who simply wont listen, when their voices are not heard or heard but valued?

I challenge anyone not to feel that way at times when suffering with a rare and debilitating disease, especially one that causes chronic facial and body pain. Amy sees it every single day, she understands, she wants me at home, of course she does and I want to be home with her... 

So why would anyone imagine that we would prolong this agonizing separation? Why would they believe that being dosed up to the eyeballs on medicines that mean I have to stay here while on them was a plausible choice made by the both of us? The lovely free hospital meals? The great TV you don't get? haha

Being in hospital is a means to an end, the end of my current spate of unlivable flare-ups, that have been happening back-to-back. I have been enduring as much pain and 'zoning out' as I possibly can, but enough is enough and we decided the only way forward was to step sideways for a little while and come here.

Sometimes if you take a little detour you get to miss the excruciating bumper-to-bumper traffic jams, avoid the head-on collision or the 10-car pile-up, you take a sideways trip for a few days and you arrive at your destination a little later than planned but alive and a little better for it. 

'Zoning Out' Vs Seizures?

It has never occurred to Amy or myself previously that what I could be experiencing when I describe my 'Zoning Out' episodes could be seizures. Perhaps because like most people when we imagined seizures we pictured someone laid helpless on the floor suffering a severe Epileptic Fit.

However just the other day someone on a Rare Disease Support Group I have joined stated that mine and Amy's combined descriptions of my episodes sounded ominously like I could be suffering some type of seizures.

Well to have a possible answer, finally, after years of telling every doctor, nurse, medical personnel that we have come across about them, sounded like music to our ears. Admittedly probably the kind of music that would be fitting of a slow-build horror scene on some old Hitchcock Horror but nonetheless, we were intrigued.

So research, research, research... Brought me to varying types of seizures that all had similarities to my episodes, such as Temporal Lobe Epilepsy (TLEs) which combined the three most obvious candidates, due to their effects: 

• Sensory Seizures - Because I hear and see things that aren't there. 
• Autonomic Seizures - Because I suffer an extremely unpleasant sensation in both my head and chest.
• Psychic Seizures - Because I have garbled speech, problems with memory and get overwhelmed by sudden emotions such as utter dread.

There are so many different types of seizures that it really is overwhelming to a layman like myself, I am not going to pretend that I know much about them because when it comes to seizures I truly am a novice.

However it does upset and somewhat infuriate me that no one has EVER looked into what these episodes mean and that they can cause long lasting damage and even death if left untreated!

Perhaps it's not that, maybe it simply is a part of my MRS but shouldn't that be investigated anyway, should the 'route-cause'not still be identified if at all possible? Well we are going to start keeping a 'Seizure Report' that stipulates everything worthwhile and useful with regards to these 'episodes of unknown origin and/or pathology' we will get to the bottom of it even when the only way is to present our findings to the doctors ourselves.

In order for the relevant scans to show any Seizure Activity they would need to be undertaken 'during' the episode, seen as we can't get anyone to actually run a scan at all this seems like an impossible task and not likely to come to fruition despite all our best intentions.

So go Team Christie & Amy once more.

I have penned a letter of intent, along with Amy, to send to my Chest Physician, Dr Vyas, all our GPs, and any relevant specialist working for the Royal Preston Hospital, highlighting all my symptoms and any specific elements that may be of a particular interest to them with regards to their specialties and research interests.

The letter stipulates that we are writing to advise that we are looking to advance the current knowledge of MRS and to create a Charitable Foundation for it, that we are looking for a person(s) within the medical field who will be willing to step forward, take an interest and become somewhat of a 'Clinical Advocate/Point of Contact for the disease. 

The letter goes into a fair amount of detail and I have tailored each one to suit the relevant party while using my first penned copy as a general template, who knows if this will get us anywhere but we are at a stand-still without it. We can only try...

I have also been looking to join any current Rare Disease Foundations possible as well, in the interim, these will offer insight and knowledge and could open doors for our own MRS Charitable Foundation in the near future.

We want to help more people than just myself, yes of course I want to be benefited, I want to be cured or treated to the best extent of medical capabilities and beyond. 

At the same time, however, every time an image of a child starting with this awful illness pops up on the MRS Support Group Facebook site etc. it is heartbreaking, we do not want anyone else to suffer the way that I have... we don't want to give this cruel disease that chance...

There is more strength in UNITY than in NUMBERS alone

Feeling Hopeless - need your help...

At the moment I really have no capacity or ability to write anything other than despair because short of that I am inflated with nothing else.

I am the balloon left behind at the party because it has a slow puncture and will soon be nothing but a useless piece of what it once was with a little string attached, reminding you that at some point it was anchored to something. That's what has been left of me since my doctor pretty much gave up on my situation and walked away, the only dredges of air inside me are consumed by despair at my current situation and fear about where I can turn to from here.

Me swollen and in pain trying to celebrate my birthday.

Amy has been making herself busy writing to all sorts of people who work within the Rare Disease community and when you search those people out you realise that it is very much a community but yet again we are on the outside looking in; through windows kept pristine because inside are all the 'bigwigs' and outside arrives the window cleaner frantically wiping away the dirty unwanted smudges we are leaving to show of our presence. Will they ever know that we were even there? That we even existed?

The loudest voices typically are the ones that are heard again and again and then there are the rest of us, meekly calling out, a minority of a handful of people across the world, unable to even commune in the same space at the same time to make our voices that bit louder, we are quiet as little mice and probably just as annoying should we be discovered.

We convince ourselves that someone, somewhere will take an interest and want to help us, will have the means to help us; but we are the comedians at our own show, eagerly laughing at our own jokes. We are merely a drop in the Rare Disease ocean, there are currently approximately 7,000 different rare diseases in existence and more being discovered every moment, 

It is estimated that 350,000 people are living with a rare disease world-wide, add to that that approximately 50% of all rare diseases/disorders do not have their own foundation working with them and for them to try and find help and to raise their voices.

It scares me just how small we really are compared to the BIG illnesses and the not as BIG but still much BIGGER rare diseases, we are a molecule if you really look at it, our whole support group community on Facebook consists of a tiny 148 people, significant to us, because we have all suffered alone for so long but as a collective, as a group of individuals in dire need of being heard; our voices are just too small.

I recently took the time, along with my partner Amy, to write a questionnaire about Melkersson Rosenthal Syndrome and send it out into the world with my details attached; hoping against hope that everyone else with this awful illness would feel as passionately as we do about getting help, raising awareness, finding a treatment or better yet... a cure.

In the weeks since I have sent it out I have received only 2 copies back and a minor flickering of interest. I am hoping that after years of being let down it is a deep routed cynicism that is sitting squarely upon my right shoulder whispering cruel jibes, that people don't care enough, that no one will see the importance of trying, that no one else will bother to complete one...

However in our situation we have no choice but to put ourselves in that position;
Otherwise what hope do we have?

I thought now would be as good of a time as any to explain why we did the questionnaire; well here goes, since joining the Facebook MRS Support Group, I have spoken to many other sufferers with the illness and for some of those I have not spoken to I have read posts that they have written about their own experiences with MRS.

From reading these posts and speaking to these lovely people we have gained an insight into each others lives and there have been many moments of 'Oh My Goodness I get that too' for example. Things that our doctors have ignored, swept under the carpet, or left for the next doctor (who would never appear) to look into it. These things I started to realise are not only my cross to bare! They affect all of us on some scale, some may get away with some of the symptoms, others may not, some may get them in later life but they are there ready to rear their ugly heads at any given moment and no one is connecting the dots.

The information available on google about MRS is so severely lacking that it verges on being dangerous, in fact in my case it could well be considered dangerous, as my airways swell up significantly, this has long since been linked to MRS by my doctors, however with no treatment to offer me other than adrenaline nebulisers and IV steroids when I have a bad attack I am left huffing and puffing my way around. 

I have spoken to others who also have swollen limbs, which gives you excruciating pain and makes even the most menial tasks like holding a pen to write a challenge that cannot always be met.

There are so many links and crossovers in our symptoms and non of them are being formally attributed to Melkersson Rosenthal Syndrome!

Where does this leave us? Down a very dark alley, calling and calling into the abyss...

I am currently in agony from head to foot and my head feels like it is full of cotton wool, people expect sense from you're when like that, they expect you to be able to care about the little things that will pass in their own way with or without your input. 

No amount of pain relief is working for me right now, I am on analgesics amongst other strong medication but it wont even ease to be suitably comfortable, I feel like I have weights under my skin, all around my body, pressing down, pushing and digging, giving me no peace at the best of time and at the worst sharp stabs of agony.

I can tell these symptoms to any doctor I like and non of them will investigate, they will all just 'make a note' or pass me off to the next doctor who will then also 'make a note' occasionally they might run some random scan or try a new pain medication or suchlike that will prove ill-effective and then I am again back to 'square one'.

My 'Zoning Out' bothers me so much more than I can say, to feel like you are not connected to your mind anymore, that you are not in any control whatsoever is truly terrifying. I wish I could get across to someone, anyone how that felt, I wish for just a moment someone else I know could experience it just so that they understood.

I have tried so many times to explain what happens when I 'Zone Out' and I am still not sure I am getting it right. When we recently went to see Dr Vyas's nurse, I asked Amy to explain from her perspective, as I can only say that I feel disjointed from myself, that I go 'fuzzy' I get a pain in my head and chest, my eyes become unfocused, everyone, everything sounds distant, and I know I am slipping away...

A screenshot of our appearance on Jeremy Kyle when we desperately asked for help.

Amy said; "It is like a mini stroke, her eyes roll into the back of her head, she isn't with it, sometimes she can't hear me at all and sometimes she tries to answer questions I have asked but slurs incoherently. Sometimes she is unaware she has even 'Zoned Out' or for how long as it can last from 30 seconds to 30 minutes, she sometimes comes round and thinks that she has said something or that I have and it just hasn't happened. She then gets very disorientated and confused. The longest 'Zone Out' to date is probably about 45 minutes and we are extremely worried. No one is investigating it, no one is helping at all!"

With all of these varying and sometimes terrifying symptoms it would be a comfort to know that there was someone out there willing to do the work it takes to put all of our symptoms together, to see which ones are part and parcel of MRS and which may not be and to then encourage the possibility of creating an MRS Charity Foundation, in other words a VOICE!

If you can contribute to that in any way, shape or form, then why would you choose not to? Please I am begging all sufferers of MRS however mild, moderate or severe, to please fill out a questionnaire and return it by the end of April or sooner if possible, even let me know that you are interested. I am a born worrier and the idea that we wont have enough questionnaires to glean the results we need scares me. None of us know what the future 
holds but maybe together we can work to make it a little brighter for those of us with MRS and all others with rare diseases too.

We are a giant tree, full of beautiful, branches flush with flowers and leaves, seedlings springing off in all directions. That tree is Rare Disease and each branch is an illness or disorder and every seedling a new illness or disorder. Every single leaf or flower is one of us and we may feel very alone blowing in the breeze but together we are a magnificent, strong and majestic tree, we are part of something bigger, if we link into that strength then perhaps we too can become stronger, maybe we will finally be heard.

Shout for help

Heading back to that dark place?

I am at a cross roads right now, I have been backtracking recently - going back towards that very dark place where I previously existed, and it terrifies me beyond belief, I don't know how I would cope with another visit to that place, I don't know if I could survive it again.

I managed a few days away recently, it was a struggle but I enjoyed it, yes I was in pain... a lot of pain in fact, my breathing wasn't great, my swelling evident and I was extremely lethargic but battling insomnia at the same time. However regardless of all the negatives that could have ruined our little trip to see family, Amy and I, both, really enjoyed the break from normality.

On the journey home I started to deteriorate, I knew that I would be 'in for it' after such a busy few days and prior to our trip I had already started to come face to face with the possibility that the Stem Cell Treatment had run its course and that I needed my second (and hopefully final) dose long before the expected and hoped for 'few years' that had been proffered when I went to America for my 'life-changing' treatment.

The pain has become so bad, of late, that I am back to being practically bed-bound day to day, I can hardly differentiate between night and day as all I see are the inside walls of our home. I move - I hurt, I stand in the shower and the water falls viciously and carelessly onto all my nerve endings, sending pain ricocheting around my entire body, I lay in bed and my body aches for me to move, so I get up and my body craves rest but at the same time the pain keeps me awake, aware of all the different types of agony I am experiencing, unable to hide from it, even for a moment.

This poem is for Neuralgia however it seems very fitting for any chronic pain illness

I have gone from not being able to sleep for the past few weeks to now barely being able to wake up, I honestly can't say which is worse; they both carry their own demons, they both haunt me. I have started to suffer nightmares again, ones that are filled with trauma and anxiety, I am always trying to get away from something, to escape but I never can... whatever it is, it always catches up with me in the end but before I can face the consequences; I wake up. 

I am a big believer in what you dream being a reflection of your daily life, I don't believe that it mirrors your life, however the emotions you experience; the stress, anxiety, fear or if you are lucky enough happiness are all derived from the thoughts and feelings you carry with you from wakefulness to sleep. Judging by my dreams I am in a very difficult place right now, and I know that I am, that that is the truth. 

I have been having better times lately, where by, for example, I can spend time with my nephews at my sister's house, nothing anyone else would necessarily get too excited about. To me, though, those times are invaluable, they are something I simply didn't have before. I had drifted so far from my family because of my illness, because I never saw them, that I felt the thread connecting us weakening and tearing and I was at a loss to stop it.

So when people ask if the Stem Cell Treatment was successful all that I can go on is the fact that it has given me the chance to get to know myself and my family again, that I can sit in front of this laptop and write occasionally and that not every moment is spent in bed staring at the same four walls.

So yes, it is better, it may not be amazing to anybody else but to someone whose life had become a shell of what it once was, who existed at the far end of a dark tunnel and could see no way out... What this Stem Cell Treatment gave me was nothing short of amazing to me, a new lease on life. But now that I feel it all slipping away again, heading back to that dark, scary place I am afraid, no I am terrified, knees quaking, hands shaking, terrified... I don't want to go back there... I can't go back there.... because if I do then I am not sure I have the strength to fight my way out again or to wait until someone can come and rescue me.

My blog - my diary

This blog has become, amongst other things, my diary and all of you who choose to read it my confidantes and I appreciate you all. It means a great deal to me to know that I am not alone in this; that I have people that care enough to follow what is happening along the way.

Amy is trying to get in touch with rare disease specialists as it has now become apparent that after years of having Dr Vyas (A Chest Physician at Royal Preston Hospital) coordinating my care and making efforts to try to help me, he has now stepped back. 

The day we went away I had an appointment with his nurse, as he is unable to see me for the foreseeable (I still don't have an appointment with him), Dr Vyas was in the building and his nurse acted as a go between. 

We did our best to get across all of my symptoms and their seriousness, how they affect me day to day, we told her how hopeless it all feels, and she went to see Dr Vyas and came back to us saying that 'Dr Vyas is a very busy man, he is dealing with patients who ARE his specialty and he unfortunately doesn't have time to help.' That was a truly devastating blow! 

Dr Vyas has always been my only advocate, the only medical professional who took the time to coordinate my care, without him I am lost amongst a sea of patient files, nothing being linked together, my symptoms being treated separately, if at all.

He prescribed me some sleeping tablets, as at that point I was having trouble sleeping and my GP wouldn't prescribe me anything, he also wrote me up for a steroid nasal spray, which he advised via his nurse that he didn't hold out much hope for. He told her to tell me that the questionnaire was a great idea and that I should pursue it, that being proactive with my care was fantastic and I should look for an advocate for MRS with urgency. In other words; he couldn't be that for me anymore.

I don't even know whether he would put me on his ward if I get admitted again, I am so lost and alone in this battle that it scares the hell out of me! I am lucky I have Amy with me, helping to fight my corner or I don't know what I would do... probably lay down and never get up! :-(

It feels so desperate when a doctor you have trusted and put your faith in to help you gives up on you, when you are too rare, too much trouble, handwork, not their specialty... Where do you turn at this point? If anyone has any ideas then I would gladly receive them...

My nasal swelling is problematic as it means that wearing my Non-Invasive-Ventilation machine (via a nasal mask) is not always very easy. I have already been struggling for breath recently so this just adds to the difficulties I endure breathing-wise. 

NIV nasal mask

When I saw Dr Vyas's nurse she did a blood/gas test and discovered that my Carbon-Dioxide levels were higher again, which means that the NIV is not doing its job. I will have to have my settings upped again and again until it keeps my levels within an acceptable/safe range. In the meantime I wake up with excruciating Carbon-Dioxide induced headaches that refuse to respond to any pain relief/treatment at all.

The body pain I have at the moment is such that to simply place my feet on the floor feels like I have no protection on the bottom of them, no skin, no flesh, just exposed nerve endings that are being agonizingly crushed against the flooring.

My hands and fingers ache painfully, the sensation running up my forearms, feeling as though it is liquid pain pumping through my veins. My left thigh feels as though I have a trapped nerve the pain pinching and shooting in equal measures. 

My stomach, with its inflamed subcutaneous nodules, is swollen and bloated, making me feel sick and as though I have a wooden block across my middle making it stiff and making any movement distressing at best, excruciating at worst.

My back has swollen subcutaneous nodules all over it causing an extreme burning and throbbing pain through it, making staying in one position for any length of time impossible.

My face is swollen and painful; as is the rest of my body

My face is swollen, particularly near my mouth, my lips taking the brunt of the swelling. I have sores/ulcerations all the way through my mouth, along the side of my tongue, on my gums and down my throat. 

Eating is not a pleasurable task, brushing my teeth hurts and leaves the sink filled with blood as if to prove the fact that I have just endured pain in order complete such a simple, mundane task as cleaning my teeth.

My face and head feel like I have been beaten up, attacked without mercy, as though punches were rained upon me until I could feel the imprint of their fists on my skin, burning into me. My face is smarting from an attack that never took place; I am after all a victim of MRS not another person, not anything I have even the faintest chance winning a fight against.

I don't feel like I can win this battle, I am all alone against an invisible assailant, wishing that I could reach out and touch someone else who is suffering the way that I am, who understands what this feels like. 

Luckily for me, though, I have a partner who is trying to imagine what I feel, who is attempting to put herself in my shoes. This cannot be fully achieved but I know that she knows me and understands me better than anyone else because she sees it every day, she sees my struggle and she struggles through it with me. 

I know that like me she too is terrified of going back to that dark place so all we can do now is, together try to find a way to fight back, to not end up so far down that dark, tunnel that we can't get back to the light or indeed to each other.

This is basically Amy's promise to me

I truly hope that anyone out there with MRS takes the time to complete and return the questionnaire we have put together, it is the only means we have of finding out more about this horrible, rare illness and our only effective way of fighting back. 

The idea is to start our own MRS charitable foundation, to find doctors/medical professionals willing to step up and be advocates for our illness and to encourage treatments, trials, maybe even help to find cure. 

Amongst all of these hopes and dreams for us and every other MRS sufferer, and their loved ones, there is also the hope that we will no longer face it all alone, that we will find a way to find each other and fight the battle against MRS together. We don't have to be MANY to be heard we just have to speak collectively and loudly enough.

Questionnaire to help broaden our knowledge of MRS

Dear Fellow Melkersson Rosenthal Syndrome Sufferers, 

Please send an email to chrissyjade18@icloud.com and I will send you a copy of the questionnaire to complete alternatively you can find a copy on the Facebook support group for Melkersson Rosenthal Syndrome; https://www.facebook.com/groups/196578270455272/ 

Please take the time to read this little post, with the odd inspirational quote thrown in for good measure, I believe that we CAN make a difference. That when we speak alone our voices are quiet, if we all say different things at the same time we confuse... but when we all say the same thing, together and at once we are UNDERSTOOD and we are HEARD!

I have attached a questionnaire that I have created in order to gain as much knowledge about MRS and how it affects ALL of us. You may remember me saying that I wanted to get the information available about MRS updated so that we can receive much more adequate care and treatment.

Well... this is the first step in achieving that objective, the results will be available as soon as possible and hopefully this will go a long way in answering a lot of questions, for example; What symptoms experienced by how many people with MRS etc that are NOT currently associated with it.

I am sure that, like me, there is a lot you don't know about MRS for certain, and that the medical professionals 'helping' you would be helped greatly by having more thorough and up-to-date information available to them.

Please help me achieve this by filling out the attached questionnaire, details of the deadline and where and how to return it are included in the document.

If you are unsure then please still take the time to have a look and then decide whether you want to send it. This could really make a difference for ALL of us and for any future sufferers as well.

Thankyou :-)

Latest Flare-Up

Latest flare-up strikes me down

I am extremely swollen at the moment and it is getting worse every day

My chin is swollen, red and rock hard, the same description can now be used to describe my right cheek. My lips are grotesquely enlarged, so much so that my top lip has two very painful and visually vivid splits, running from my teeth up towards my nose.

My chin is rock-hard and very painful, this is spreading all the time

Melkersson Rosenthal Syndrome is an unsightly illness, that often leaves sufferers too embarrassed to leave their homes during flare-ups and understandably so when they become the butt of people's jokes. I personally have often been pointed at and stared at; public humiliation is not something any of us strive for after all.

Yes, I have been there myself, wrapping a thick woolen scarf around my face, looking like a cheap Mummy imitation as I made my way nervously to the doctors to beg for mercy, for some kind of help, any help whatsoever, just so long as (s)he made this Godawful swelling disappear.

It is extremely painful and I wish I could make it go away 

I can understand wholeheartedly why people with MRS still make themselves masters of their own disguise in order to navigate the streets but I refuse to be one of them any longer.

In fact if I catch myself cowering from the glare of other people's scrutiny I give myself a mental nudge and if that doesn't work I walk headfirst into the nearest wall (not really) but I do tell myself again and again that this is who I am, like it or lump it. I can't change who or what I am; I just have to accept it or spend my entire life at war with my own reflection.

Seriously?

I sound so 'together' maybe even a tad self righteous but if I am completely honest I will admit that I haven't had a hair cut in years, not one that anyone other than my untrained partner or equally untrained mother haven't performed anyway. 

Why? Because I am terrified! Absolutely mortified at the mere idea of sitting in-front of a great, big, mirror and staring at what has become of me, with a hairdresser and everyone else in the salon doing the same thing, staring... All just staring.

Maybe I am wrong to think like that, maybe I wouldn't hold any interest for the other patrons of the salon and the hairdresser would be so caught up trying to locate anything other than a mass of split ends that she would be oblivious to anything else but I am too scared to take that chance.

Years of bullying has taken it's toll; I am a shadow of who I could have been

After years of bullying, of torment at the hands of anyone and everyone who took exception to the way I look I have been left a wreck of a human being, I am the rickety, broken, old boat, with holes littered throughout that washes up on the shore and becomes nothing more than a tale of woe. 

I am the image of a story of what 'could have been' had it not been for that big, ferocious, wave that came out of the blue and capsized me. That wave in this scenario is of course Melkersson Rosenthal Syndrome.

So my horrendous dark brown mop of hair is going to survive another day, week, month, maybe even another year... Before you know it I will somewhat resemble a Yeti and the emphasis will no longer be on my swollen face and body... probably because you wont be able to see any of it under the avalanche of hair.

Being Rare

Having a rare illness does not give me a feeling of being 'special'. I don't feel like I have a golden ticket to some amazing club somewhere, which is reserved for people like myself who suffer with these illnesses day after day after day... no break... no rest... no cure...

Being rare makes me feel lonely, I am not alone; I have my lovely family, my amazing partner, my wonderful friends but you don't have to be alone in order to feel lonely.

Having an illness and symptoms from that illness that no one else has or understands is a very lonely place to be. I can't talk about it knowing the person listening to my troubles faces the same issues as me, the same trials and tribulations. I know that I am the only person that feels what I feel and hurts the way I hurt and that no one else can understand it completely.

When I told Amy that I keep 'zoning out' she had no idea what I meant, in all likelihood neither would anyone else as I have invented the term to describe symptoms that I suffer with on a regular basis, I would love to know if any of my fellow sufferers of MRS 'zone out' too.

I try my best to put into words what I go through when that happens, in fact I have penned a whole post on the matter but alas; here I go again...

My vision becomes blurred, my eyes wont focus, and according to Amy they roll into the back of my head. Everything I hear becomes echoey and distant, like someone is holding a long piece of pipe from their mouth to my ear and are then whispering secrets I am desperate to hear but can't quite make out.

My head feels 'foggy' and I cannot process my thoughts. Words come out slurred and incoherent, I imagine whole conversations have and are currently taking place then respond with an answer to a question, which was never asked.

Another day, another flare-up

It is now two days later than when I typed what is written above, I was far too poorly at that point to continue writing and trust me you should thank me for that wise decision as the 'foggy-headedness' that consumed me would have led me to write a lot of nonsensical gibberish. 

I have been in so much pain over the past couple of days that I have been battling with myself over whether or not I should simply give in and go to hospital. I have been taking Oxynorm as if it is going out of fashion and have been barely sleeping.

Amy has been extremely devoted to the task of helping me get as much sleep as possible by stroking my back and playing with my hair in order to make me feel relaxed. 

The problem is that I do then manage to fall asleep but still wake up a very short time later when the pain decides to give me a rude awakening or the inside of my nose is so swollen that I can't breathe using my NIV nasal mask anymore! Who needs alarm clocks?

It is now 8.16am and I have been awake since 1.30am, the pain a constant barrier between myself and sleep. I feel drained, nauseous and exhausted but I know that sleep will evade me even if I go and lie down once more. Like a cruel joke at my expense it is there placed just out of reach to tease me and make me desire it even more.

Painful Swelling

My whole body is a swollen mass of pain right now, everything hurts and feels tender to the touch, like I am covered in bruises that are invisible to the naked eye. 

My stomach is so swollen that if you didn't know me you would probably assume that I am pregnant, which is yet another cruel joke at my expense seen as that is something I want more than anything else but cannot have.

My legs, ankles and feet are extremely swollen and it is painful simply to walk. When they get like this I tend to hobble like I am an 80-year-old who should be using a walking frame, in fact yes, I do actually have one of those! It is hidden away from view in Laddy's room as I refuse to use it, I would see that as me giving up yet another aspect of myself and I am not ready to do that, no where near ready in fact.

The pain is intense, the swelling pushes against my toes and makes me cringe

I seem to have lost my ankles in the above image! lol, see I can laugh about it, in fact isn't that the way that most people deal with an embarrassing situation? They laugh and point out the issue before anyone else can? 

Amy is forever telling me not to do it but it's an automatic reaction, self defense...Really, if I am honest, I want to do anything but laugh, crying would probably be a more honest response.

The swelling pushes on my nerves and creates a mixture of pains just for good measure, the pressure as it pushes onto my toes leaves me with a cringe-worthy and extremely uncomfortable sensation, walking hurts so much that, for example, I find myself avoiding going upstairs until I have absolutely no choice.

My stomach aches from the swelling and my chest hurts with the effort of pushing my chest out to breathe. Having a paralysed diaphragm means that I must force my chest outwards to give my lung room to expand as normally your diaphragm would move down to make room for your lung's inflation.

Normal chest X-Ray

 

A chest X-Ray of someone with a paralysed diaphragm

The above X-Rays show the difference between what it looks like when someone has a paralysed diaphragm as apposed to someone with a working diaphragm. 

It is very noticeable to look at on an X-Ray and it is extremely noticeable to live with. Add to the breathing mix chronic asthma and swollen airways (Epiglottis and Larynx) and you are going to have problems.

Causes and problems associated with having a paralysed diaphragm

The cause of my paralysed diaphragm is of course, as always, Melkersson Rosenthal Syndrome. Although they have no clear explanation as to why it has happened the general consensus is that it is the root cause.

During a flare up when my airways are more swollen than normal my breathing becomes very problematic along with my swallowing. It kind of feels like I have a pocket at the bottom of my throat collecting fluid when I drink, making me choke and struggle to swallow.

I have been seen by the 'Swallow Team' at Royal Preston Hospital and they have confirmed this as the problem, I trust myself to be able to self diagnose these issues prior to an official diagnosis simply by the way I feel. 

Time and time again I have told doctors what I believe the cause of a problem to be, had them look at me side-ways clearly thinking that I am crazy or imagining things, only to then be proven correct.

I am a big believer in the fact that no-one knows you better than you know yourself and no one knows a child better than their parents (if they are good parents of course)because who pays the most attention after all?

So this 'fluid pocket' created by the swelling in my throat is driving me crazy and most likely sending Amy on that roller-coaster known as 'Momentary Insanity' listening to me constantly trying to clear my throat and coughing and spluttering because I can't.

Tough Decisions

Amy and I have now come to the conclusion that the best option, or rather the only option, is for me to 'give in' and go to hospital today. I have been getting worse and worse with this flare up and not getting any better.

We know from previous experience that if I don't go in now for a couple of days I will end up in later for a couple of weeks; and that is something that would kill my spirit rather than simply cause it minor injury like a couple of days will.

My lips are very swollen and I have painful splits in the skin of my upper lip and in the corners too; I worry about that too in relation to a hospital stay, as I have been targeted a few times now by people who were clearly once playground bullies and who unfortunately never saw the error of their ways.

I can't wear a scarf around my swollen face in hospital, I can't hide, I simply have to hope that no one 'picks me out of the crowd' to take issue with.

I will have to hold my arms out again and become a 'pin cushion' once again, years and years of having to have canulars (needles inserted for vein access) along with my MRS in general has caused my veins to be thin and weak. It is extremely challenging for them to get access in the first place and my veins often simply collapse within a day or two.

I have had canulars in my feet before now, they have threatened to put them in my neck and I have had a piccline (semi-permanent access to a main vein near the heart running mid-way down my arm) twice. 

So off I go, wearing loose fitting clothes ready for the inevitable monitoring equipment that needs to be attached and the needles that will be injected. 

I am hoping against hope that I will feel much better after a few IV medications, the usual suspects being Hydrocortisone steroids and Piriton Anti-Histamines amongst others, and that I will then be able to come home. 

I'm desperately wishing that I will not have to spend the night, or worse a succession of nights but  at this point I will do what needs to be done. I will do my penance and stay in for as long as I need to so that I can return home to Amy feeling somewhat human again and not like something out of an episode of The Walking Dead (one of Amy's favourite TV shows).

I may not be able to write a post for a few days, we will see what happens, but if I don't give in and go into hospital today I run the risk of ending up in Intensive Care again and that is not somewhere I am in a hurry to head back to.

Any good vibes and thoughts of a swift and problem free recovery from this latest flare-up will be gladly received. Thank you so much to anyone who has been loyal enough to read my post this far lol, it probably isn't up to standard as I kind of feel like I have been run over by a Jet and subsequently mauled by its engine! 

All the lovely people that follow my blog and anyone new to it too are all appreciated; wish me luck! :-)