Easter Baking Extravaganza: Hot (Cross?) Buns

Being mostly bedbound and unable to eat DOES NOT have to mean you can't do something you love. For me, that's cooking and baking.

Mama and I had a busy fun filled afternoon Baking the first batch of Easter goodies: Traditional Hot Cross Buns, pepped up with cinnamon and citrus peel, as well as the ubiquitous sultanas. The day got off to a bit of a dodgy start. My scales were playing hide and seek, Mama forgot the second type of flour to make the essential crosses with, and the small measuring jug that used to be kept in the kitchen was turned into a bile bag measurer months ago.

Dry ingredients sifted & mixed

So, Mama made a mercy dash back to her home for the missing stuff as I pressed on, (weighing scales found), with measuring out the dry ingredients. I was so eager and focused on measuring out my two weighing tubs of flour, (my scales only go up to 450g and I needed 500g), that I had dumped the first lot of flour unsifted into our mixing bowl. It was only when I was halfway through measuring out the rest of the flour that I realised my mistake.

Big cook Little cook

Horrified at what Mama would say, the girl guide in me took over. I stopped my measuring,  took the tub off the scales so I didn't knock 100g of flour all over the bed, and got a nurse to help me spoon the mixing bowl of flour into an empty clean ice-cream tub. Cue some expert sifting, my mistake was rectified.

By the time Mama arrived back, all the dry ingredients, (flour, salt and sugar), we're ready in the mixing bowl and the yeast packet was in the process of being opened. Together we gradually mixed in the watered down milk until our dough was well formed but sticky. We turfed it out of it's pot and onto a chopping board on the bed. It was ready to knead.

Mixing decks

It's amazing what an impression something can make on you at a young age without you realising, even after only doing it once. When I was 12 I went on a school trip to France. On one of the outings, we went and made Pain Au Chocolat at a local Boulongorie. It was the only time I've kneaded bresd dough until today, but as soon as that dough was on the board and under my fists it all came back to me.

Rosie Kneads Dough

The hardest bit of the whole enterprise was getting the bloody sultanas, orange zest and mixed citrus peel into our dough. Oh my god! As soon as you pressed them in, the ones you'd put in earlier would pop out! It didn't help that I was trying to do this back in the mixing bowl as Mama cut the orange zest on the board. Occasionally I'd turf her off, Chuck the dough on the board and knead it a fresh. After a couple of repetitions, plenty of swearing and laughing later our dried fruit adversaries resolve started to wane. Exhausted, but with currents running evenly through we left the dough in its bowl to prove.

And boy did it prove. Double it's size, happy and majestic. I buttered the baking trays, and together we took handfuls of our springy dough mix rounding it into even sized balls until we had a good bakers dozen plus one good sized patties on our tray. Whilst waiting for them to prove again, we made our piping mix for the most scary and exciting bit yet: piping the all important crosses.

Crossing the Buns...

Ordinary flour, sugar and the rest of our milky water spooned into a crudely made piping bag, (plastic bag with the end chopped off). Both of us having never piped anything before, Mama had a go first to get the idea before handing over to me. Hmm... I'll say piping is not my forte yet but I managed 7 reasonable attempts all mostly cross shaped.

The next job was getting them home to Mama's house to bake. By this time my TPN nurse had arrived as well, (the clock had struck 8), just to add to the general melee. A washing basket commandeered for one baking tray, the other was placed in the box Mama had used to bring the equipment over with her. Both trays will be baked at home. Drive safely Mama, you are carrying precious cargo on board...

Tray 1 of Hot Cross Buns 

Tray 2 of Hot Cross Buns

The beast from the east

The beast from the east is truly upon us. Blizzards, gridlocked motorways, children off school, and the first 'Red' level weather warnings the UK has ever seen. It's safe to say that the weather is firmly at the front of everybody's minds. But for me, and other people with chronic life altering conditions, the beast from the east, (as this Siberian tundra has become affectionately known), is the icing on an already difficult existence.

"The Best from the East"
An original cartoon by Rosie P

Snow always highlights an internal struggle for me. The small child inside lights up with delight at the virgin snow outside my window, eager to jump up and get out there, whereas the adult old before her time worries and frets that her joints will be particularly stiff and that day. I want the snowball fights, the snowmen building. Walking in the crisp crunching white stuff to the park dragging my little orange sled behind me before hurling myself down a slope headfirst for an afternoon of endless fun. But that's in the past. Memories that melt away with the snow until the next time we are deluged.

Nowadays they're replaced with worries. "Will my nurse make it to do her shift?". "If she doesn't will the current one have to do a

double shift?" "Am I going to be able to get to my appointment tomorrow?" "What if I do go out to build a snowman and my wheelchair gets stuck, our I catch a cold that then won't shift". My most pressing worry is "What if the TPN nurse can't make it? What do I do about getting fluids and my intravenous meds?". Snow highlights to me just how dependant on others I truly am, and for someone who's worst nightmare is total dependency, that's a very scary feeling indeed.

Snow scape. The beauty of it never gets old

The worst part is the child like wonder and magic is still there, pulling my heart where it cannot go. I'm jealous of the kids out there shrieking with glee, there tiny footsteps peppering the soft white snow. They don't know how lucky they are. I feel barred from the fun, resentful that my vulnerability is being highlighted for all to see. Friends used to invite me out on snow days as a child. I didn't used to go, preferring throwing myself down the park hill with my Dad instead. I'd give anything to be able to do that now.

So as you cheer that school is cancelled, and another day of exploring your winter wonderland beckons, spare a thought for those of us hiding away from the snow restraining our inner child as they scream and kick with every fibre of their being to join in the fun. Think of the ones we rely on too. The nurses, police officers and fire crews who battle come what may to make sure we are safe. For they are hero's, literal life savers. And let's hope the beast from the east passes by soon. 

R wraps up warm indoors as the kids play in the snow

What it means to be "Rare"

Official banner for Rare Disease Awareness Day

28th February is Rare disease day. Social media is filled with the bright colours of the world wide "Rare" community celebrating and raising awareness for the myriad of different conditions. And why shouldn't we? No one else is going to raise awareness for us, and after all our battles, we deserve to be heard. To be understood. To be loved. There are more of us than you could ever imagine. More then 300 million worldwide to be precise. For a condition to be classed as rare it needs to have less than 200,000 sufferers in America and 1 in 2000 in Europe. Most are caused by faulty genes. We were born with them. They are us, and we are them.

I suffer from several, but the main two are a collogen disorder known as Ehlers Danlos Syndrome (EDS for short) and a Pancreatic disorder known as Hereditary Pancreatitis. Both have left me in agonising pain, tube fed and reliant on nurses and carers to help me with my daily needs. They impact each other too. Flares of one, often result in flares of the other. The almost constant tag teaming is exhausting and impacts every aspect of my life, all be it in differing ways. 

"Pancreas under attack"
 an original cartoon by Rosie P

Hereditary Chronic Relapsing Pancreatitis. Quite a mouthful, but very descriptive. I chose the wrong genes in conception, and as a direct result was labelled as being "failure to thrive" as a baby. Essentially, HCRP means my pancreas doesn't produce enough enzymes to break down my food, and the ones it does produce haven't been made correctly. As a result, when my food enters my digestive system, the enzymes my pancreas release start digesting the Pancreas instead. Its effects are chronic relapsing episodes of inflammation of the Pancreas, leading to pancreatic atrophy, pancreatic stones and many other nasties. During an attack I will vomit continuously for hours, blinding stabbing pain high in my abdomen going from the front to the back, radiating all over my belly. 

At aged 4 when I was first diagnosed I used to describe it as being "Here, here, here, and here" (all over the abdomen). I wasn't believed by the doctors and would cry in agony for hours despite the diamorphine syringe driver I was attached to in my hospital bed. A simple blood test from a curious young doctor saw my diagnosis rendered after a traumatic week long admission with these apparently non descript symptoms. They sat my mother down to tell her. It broke her heart. I was the youngest they had ever seen presenting with it. The disease only usually shows itself during teenage years. 

I still get attacks now, 21 years later. It's the worst pain imaginable. From a pain and relapse point of view the best I can hope for is the Pancreas "burns itself out" and becomes more scar tissue than working organ. Mines already nearly there. Only 10% working tissue. Brittle Diabetes and Pancreatic Cancer are complications I will likely need to endure in the future, but for now I wait. There is no cure, just symptom management. If I have children, I am 50% likely to give the faulty gene. Any child of mine with the condition has a 50% chance of having symptoms.

Some of the symptoms of EDS 

As for the Ehlers Danlos Syndrome, the effects of that on my body are wide spread. I have the Hypermobility Type (known as hEDS or type 3). It effects all my muscles, including the muscular organs of the bladder, bowel, heart and blood vessels, even the muscles that hold and focus my lenses in my eyes are effected. I have total bladder failure and am reliant on a catheter straight into my bladder to empty it, and wil be for the rest of my life. I am tube fed via my bloodstream as my bowel lacks the ability to move. It too is slowly falling. I suffer painful dislocations of my joints too as the lax muscles surrounding them struggle to keep my joints in place. This makes simple tasks such as opening jars, walking, writing increasingly harder. 

Like the Hereditary Pancreatitis it is a degenerative condition and there is no cure. I was diagnosed aged 21 after years of mistreatment by various doctors and medical teams. Again it took someone looking outside the box to make the formal diagnosis. Unfortunately I am not alone. On average it takes sufferers of rare diseases years of being turned away and mistreated to get their diagnosis, and many more besides to get the treatment they need. So next time you see someone hobbling about on crutches, or walking with apparent ease out of a disabled space spare them a thought. They might be battling a rare condition. There are more of us than you think.

Rare Disease Fact card by Novartis

For further information on Rare Disease Awareness Day please follow the link below.

https://www.rarediseaseday.org

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)