Time is a peculiar mistress

I wrote this earlier last week, and had been waiting until I had the energy to draw an accompanying cartoon to publish it. But here we are, a whole week later drowning in assignment tweaking and writing, with still so little energy that no cartoon has been completed. 

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Another night shift. It's been 3 weeks since I last got more than 5 hours sleep, and it'll be another 3 weeks until the end of this permanent dehydrated and sleep deprived fog. A new life line is to be inserted. But for now, I fight to maintain my grip on my existence. I've got into a rhythm now. Tablet on. BBC iPlayer or Google Play are my new best friends. Heat pads warmed, nurse sat on my sofa pressing the buttons when my fingers start to ache. The constant alarming drives through me like a knife. Piercing. Insistent. Soul destroying.

Time has become warped. Reduced down to infusion volumes and the witching hour. Two magic numbers. I look forward with every fibre of my being to seeing the volume fed turn over to 400ml. Or the clock ticking ever onwards, until the hour hand reaches 3am, which ever happens first. Then blissful beautiful blackness and sleep.

As the days draw on, the more I notice the changes. My hips protrude like too mountain summits, separated by a desert plain. Ribs and the buttons of my spine now resemble some kind of weird skeletal xylophone, my legs fragile as matchsticks in an open fire. I hurt. Bones are sharp and even sitting on layers and layers of padded cushions doesn't alleviate the pain for long.

I hate this. I feel like I'm disappearing, and not just from a weight point of view. I feel like I'm sleepwalking through things at the moment. I'm permanently exhausted. Lips cracked from dehydration. A permanent headache from loopy electrolytes, dodgy sugars. I feel like I'm lurching from one crisis to the next. Everything is falling apart.

He lied to me one time too many. As my doctor he should at least be talking to me directly about decisions about my care, NOT passing messages through his minions, ignoring me, and then lying to my face when I confront him about it. His tentacles have reached into my home life, it's disintegrating too. Problems are being brought to light that I had no idea existed, and to be honest STILL don't know if they exist or are a manufactured way of making me feel bad. I feel lost. Confused. Angry. Scared. The situation feels unreal. Like I'm loosing my grip on my reality.

The last few weeks have been full of so much stress and angst. Severe weight loss, sleep deprivation. Add into that unexpected withdrawal symptoms and spasm inducing pain, from the GP slashing my dose of pain relief. When will it all end? Every time I think I've hit rock bottom, the ground swallows me up and spits me out once more. 

As I said, time is a peculiar mistress. It's funny what a difference a few weeks can make.

Under Pressure (Day 10 of no TPN)

I promised myself what I first started writing this blog that I would do at least one of two things:

1. Always be honest about how a situation makes me feel, no matter how unreasonable my reaction might feel.
2. To record this for myself first and others later. 

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This post is about my mood, which at the moment can only be described as absolutely foul. Everyone and everything is unbearably frustrating and in my eyes nobody can do any right. The stimulus for this sudden and thunderous change puzzles me even more, and is completely out of line with the proportion and strength of my emotional reaction. My carer asked me what I wanted to wear tomorrow for my morning at uni and would I like a wash now as well to be woken up early for one tomorrow.. 

Immediately I felt a hot surge of anger. "And be up before even 3 hours of sleep?! I howl mentally "No I don't want a fucking wash!" The voice in my head screams, internal me leaping off the bed to shake my gorgeous carer warmly by the throat. "Fine" my mouth says coolly. Smiling, the carer bustled into the bathroom. "Rosie, do you want your shaving things". A muscle I my clenched jaw twitches. No I don't. I don't even want a wash. I feel forced. Entirely of my own making, but still real nevertheless. 

I can hear the TPN nurse busy preparing her trolley to connect me up to my torturous ever beeping pump. My heart sinks and my frustration and anger grow, like gasoline poured on flames. I  don't want my stupid pain in the arse TPN. I don't want all these people in my house constantly asking the same stupid questions over and over again. I want to scream. I just want everyone to bugger off and leave me alone. I want to get a full night sleep uninterrupted. Is that too much to ask?? Evidently so. 

As I said, completely unreasonable. I'm suddenly aware of the light bulb burning bright above my head. It's too bright. Making my eyes hurt. I resent it and want to rip it out of the ceiling and smash it into a million pieces. Woah! Talk about over kill. 

"Stop the world, I wanna get off!"
An original cartoon by Rosie P

My eyes sting and start to blur. My throat feels too tight, nose runny. My body prepares itself to cry, the normal measured response to being extremely overtired and emotional. But destructive angry me says don't you bloody dare, putting energy it doesn't have into suppression. It sees it as a sign of weakness. I know there is only blocking the dam for so long. I know letting it burst in front of someone will only attract sympathy. I can't stand all the questions, the attention. The perfect reaction for me is just for it all to be ignored, if I want to talk I will. 

The carer approaches, the commode rumbling down the hallway into the living room, water gently sloshing over the sides of the sides of the wash bowl. I hurriedly sniff and wipe my eyes. Now the rage is starting to ebb, I feel like the stuffing has been knocked out of me. I don't care what I look like at the moment. Not in the mood for anything except the sweet oblivion of sleep. Stop the world, I want to get off. 

Simple things such as washing and getting dressed when connected up to my failing life line 24 hours a day are mammoth proportioned in terms of effort. I am pinned to the bed thanks to the drip stand. I do have a rucksack, but by the time I've faffed about with that, my slim opportunity to grab clothes, wash and change my upper and lower halves would have been lost. 

I'm currently averaging 3-4 hours sleep a night, thanks to the almost constant mysterious alarming of the pump. In the morning I only wake up when the nurses are in the process of doing the morning medication and waft those heinous chlorohexadin wipes under my nose. Washing before being connected at night is not much better. By the evening, I often simply don't have the energy to wrestle the fluids through my top, change, shave, wash, moisturise etc. And also by that time I just don't care, all I want to do is to sleep. 

It's a race against the TPN nurse next door as she prepares the syringes of intravenous medication. Stressful and worrying. I can hear her opening and closing draws, as I half heartedly throw my flannel into the basin of warm water. I speed through my routine when my carer arrives back with clothes for tomorrow. The exhaustion induced rage rises again, as does the mantra "I don't care". Only, the more I say it, brown hair bouncing in angry defiance, the less I'm sure I mean it. 

The wheels of the TPN trolley rattle down the hallway, I hurriedly pull on my trousers. My carer still stands, arms full of clothes I don't want to know about. It's into this scene, arms half in to my top, hair sticking out like a bush through an arm hole, that the unfortunate TPN nurse emerges with the speed of a souped up Ferrari. "Ready to be connected?" she exclaims happily. I've been told my hard stares can melt mercury, even through a mess of hair and clothing... as if pulled by an overwhelmingly strong force, the TPN nurse backed out slowly without a word. 

Is it a bird? Is it a plane? No, it's Flamazine!

"Is it a bird? Is it a plane? No, it's Flamazine!" An original cartoon by Rosie P

Well, my course of the mysterious cream is at an end. Although the PEGJ* infection is definitely still there, it is by no means as swollen or painful as it was. And as a bonus for ten it didn't dissolve my surrounding skin, or finger tips when applied AND even helped the burnt skin surrounding it heal- Yay! 

As ridiculous as it sounds, the cream dissolving my skin was a real fear of mine. It's main component, Silver Sulphadiazine, is great at dissolving extraneous tissue and has antibiotic type properties. As a result all the literature says to use gloves and a spatula to apply it. Of course it didn't come with either of these, so I had to improvise. Thanks to my care company's constant over stocking of supplies, I have more than one spare set of gloves. As for the spatula replacement, that was easy- cotton buds! Which is also a lot kinder on the sore tissue than a sharp edged plastic spatula. 

Of course, in true hospital style, the cream just turned up in my bedside locker one day, with no explanation, let alone any instructions as to how to use it. Hell, it took me the best part of two weeks to work out what it was and where I was meant to be putting it, and that was all by complete accident. Once again, thank god for instruction books and not being a half wit. 

One of my dear friends told me it had been used extensively before the discovery of antibiotics to help treat burns, partial finger amputations, severe ulcers and skin grafts. It was the go to cream by all accounts. But nowhere did it explain what to do with PEGJ stomas. Queue plenty of beard stroking and discussion. I had detailed instructions of what to do if it was a burn or an ulcer, and due to stomach acid leakage there was one present, so... that will do. 

It may not be completely infection free yet, but this is the most comfortable my PEGJ stoma site has felt since it was placed 3 months ago by far. One more course of antibiotics, which my GP prescribed yesterday and I can hopefully wave goodbye to this Staph bug once and for all! 

Zebras vs Horses: What is a Zebra?

I have been asked a lot recently what Zebras have to do with Ehlers Danlos Syndrome. A Zebra in the medical world is someone with a rare and highly complex condition, such as EDS:

 “When you hear the sound of hooves, think horses, not zebras.” (Ehlers-Danlos Support UK, Why the Zebra)

The expression above is hammered into medical students throughout their doctoral training. The analogy serves to remind them that the simplest explanation and diagnosis for an illness,

"We are a Rainbow of Possibilities" by Rosie P

(the horse) is often the right one. As a result, when diagnosing a patient, doctors learn to expect relatively common ailments. Rare gets forgotten. This tends to lead to years of misdiagnosis, sheer ignorance and sometimes the sufferer being forgotten altogether and pushed from pillar to post with little in the way of answers.

Diagnosis usually comes years later, from some bright spark who considers rare and embraces it.

One of the things that makes EDS hard to diagnose is no two sufferers present in exactly the same way. Just as no two zebras bare exactly the same pattern of stripes on their coats. Some have different types of EDS. Others with the same type may have only joints affected, whereas others joints are fine but their internal organs are not. There are an infinite number of combinations, each unique to the person they belong to.

“Sometimes when you hear hoofbeats, it really is a zebra.” Ehlers Danlos Sufferers are known as Zebras. Unique, rare and proud. 

"Why the Zebra?" Explanation poster from dailykos.com

Sources: Ehlers-Danlos Support UK (2017), Why the Zebra- Ehlers-Danlos Support UK, [online] Available at https://www.ehlers-danlos.org/about-us/charity-aims-and-focus/why-the-zebra/ (Accessed 7 February 2018)

The Ehlers Danlos Society (2017), Why the Zebra- Ehlers Danlos Society, [online] Available at https://www.ehlers-danlos.com/why-the-zebra/ (Accessed 7 February 2018)

Dehydrated Musings of a Zebra

**BEEP BEEP BEEP BEEP** I sink under the covers. The shrill yelling of my TPN pump pulls me reluctantly out of my light doze. My eyes snap open. Immediately alert. I roll over, muttering darkly. **BEEP BEEP BEEP** Erghh! "I hear you" I soothe. The pumps green backlight on its display screen flashes angrily at me. The alarm still squawking.

Ambix Pump with tonight's bag of TPN 

I already know what's wrong with it, down stream occlusion. It has been the same for the last 5 nights on the trot. Not more than 600ml of my precious nutrition has managed to be infused per night. If I'm extremely lucky I will get 30 minutes between beeps. But more often than not it's been every 10 minutes.

The lack of food and hydration is starting to have a noticeable affect. I have an almost permanent headache, am increasingly grumpy and distressed, and am lacking in energy. As for my mouth feeling like something has thrown up in it, than curled up and died, things are not quite as grand as they could be.

My brain tortures me when I do manage to drift off. I dream about nice cold thick milkshakes. Strawberry with a large blob of vanilla ice cream in it. My favorite. The ice cream floating like a beautiful creamy iceberg. Then I wake up, drooling. Pancreas throbbing from starting to work to produce enzymes to break down this mysterious mirage.

** BEEP BEEP BEEP** instinctively I stop and re start the pump. But it's only as good as shoving a dummy into the mouth of a screaming toddler. It spends a few moments sucking, slightly dumbfounded that you had dared to stop it mid tirade, before spitting the dummy out and screaming hell for leather once more.

We have tried everything and still it alarms. Sitting up? No. Lying down? No. On my side? No. X-Rays, and a procedure to check the position of the line. All came back clear. We have tried two different pumps, changing the pressure gauge setting on my current pump, putting it to the maximum it will put up with before alarming. And although it had made some difference, it hasn't been enough to get a viscous bag of fat content TPN (known as lipid) to go through successfully. I can't help but wonder what the team will come up with in the morning during their special consultants meeting. Whatever it is, I hope it helps.

I'm at the end of my tether. My get up and go got up and went days ago. But still I battle on. This will not break me. I have been through worse, and came through the other side more determined and stronger. I might be down at the moment, but I'm definitely not out.

An Exam Officer Calls...

Doing any type of exam is scary and stressful. Add in a malfunctioning body with several complex and chronic illnesses, each with their own set of difficulties, and suddenly those stress levels find themselves in danger of shooting off into orbit.

The email from the University sits in your inbox gathering dust with the other emails that are too scary to act on, but too important to ignore. What if they refuse my request? What if they won't allow me the help I need? Hell, what help DO I need?! I mean you can't exactly phone up and say, "hi I wonder if you can help me. I have extra requirements for the exam... what help do I need?... good question..." you'll be a laughing stock! No much better to wait until you've worked it out, then you can go in and ask them straight. That's what they expect.

"Charlotte Rides in on her Red Charger"
An original cartoon by Rosie P

But life happens whilst you're making other plans and before you know it, it's a month before the deadline and you've thought no more about it than when the letter first came through the door. Its almost too late. And it's into this internal chaos that Charlotte from the University's Student Support Team rides one day completely out of the blue, her stead rearing dramatically. Her voice over the phone is quiet and gentle, yet firm. You know from that first exchange of hellos that you're in safe hands.

"Do you have any ideas as to how we can support you for your exam?"she asks innocently. Immediately that panic rises up from no where, and your brain goes into panic screaming mode.
Eyes wide with fear, you manage to squeak out a "No. None".
"That's fine" she replies brightly, "I didn't either when I was doing my finals". A tidal wave of relief halts screaming brain in her tracks, like a slap to the face.

Charlotte suggests you look at the statement of disability paperwork you filled out when you first joined up. It's years out of date, but that alone is a good start. It's now Ehlers Danlos Syndrome rather than plain hypermobility. Catheters and Hickman lines. She knows a bit about EDS does Charlotte. She's arranged many a special requirement package for students with the condition in the past. She knows just where to start

A Computer written exam, possibly with voice activated software. Maybe an amanuensis if I would prefer? From talking further about my needs, especially the difficulties around medications and the need to rest my hands, rest breaks, she decides, must also go on the list. As must extra time for the brain fog and also typing speed, if I do the computer exam by hand, that is. She will speak to her boss to find out details such as what a home exam would entail and whether I would be able to practice with the selected amanuensis, (if I did go down that route), before the exam itself.

Just leave it to her she says. In the meantime, I need to have a think about the options we have discussed, and start writing a letter to be signed by one of my consultants that explains to the exam board my conditions, how they effect my ability to carry out an exam and how these will be life long. She signs off with those all important words, "Don't worry. It's all under control"...

Perfect.