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