The (Rubbish) Week That Was

Last week was rough. I think I had a bit of a post-vaccination slump, but also, Littlest is so, so bored that things have become untenable. She doesn’t understand why she can’t go to school and she’s fed up with being at home with us all the time. Twice a week a carer takes her out for a couple of hours for a walk round in the woods—rain or shine, she’s a machine. But it’s not enough.

Every single moment of every single day, Littlest needs someone there with her. Unless she’s plugged into her iPad—but at the moment, she doesn’t want her ipad, she wants either my phone or Mr AL’s. If I need to use the phone she tugs at my arm and screams incessantly whilst I’m making the call, unless Mr AL is there to either occupy her or give her his.

Currently she is sat beside me in her wheelchair feeding Morris Skips. He’s delighted. She’s delighted. But when she runs out I’m either going to have to pacify her with my phone, or stop work and go and do an activity with her. But nothing holds her interest…a story, colouring, stickers, dolls house, brushing the guinea pigs, video calling with grandma, cutting things up, glitter, feeding the hens, duplo, music. You get about twenty minutes concentration for each activity, max. As much as we can we are trying to involve her in the household processes. ‘Helping’ pass laundry to fold, unloading the dishwasher—she can manage to hand someone the cutlery and some of the smaller plates, hoovering is hilarious, so is dusting. She sometimes plays Dora the Explorer or World Zoo on the Wii, but she does find that tiring.

Once she gets bored, she starts throwing things on the floor. Her doll’s furniture, DVDs, TV and Wii remotes, books, toys from the toy shelves. Cutlery. Crockery. We have tried sending her to her room for some quiet time, which at least gives us a break, but it doesn’t change her behaviour. We can’t leave her alone with a book any longer because she has begun to rip pages out.

There is nothing to bribe her with like I can with Talking Child—there’s nothing she overtly wants. And threatening to remove internet privileges etc. is pointless. She has no concept of punishment and reward.

This is on top of all the usual tube feeding, personal care, making-sure-she’s-not-choking-on-her-own-secretions, physiotherapy, phone-calls with professionals, ‘ordinary’ stuff.

My seizures are through the roof. Mr AL is randomly falling asleep mid afternoon like I remember doing when I was pregnant (he is not pregnant).

I have spent the last week finding out about vaccinations for Extremely Clinically Vulnerable children. There is apparently an expectation that there are between 1,500 and 2,000 children between the ages of twelve and fifteen who are fragile enough to need the Pfizer vaccine although the clinical trial for that age group is not yet completed. I have found a document with criteria from the NHS that strongly suggests Littlest falls into that category. A handful of Mr AL’s friends on the SWAN (Syndromes Without A Name) facebook group have kids under fifteen who have had it, their paediatricians working in tandem with Great Ormond Street.

I have phonecalls in to her two leading Paediatricians.

Regardless though…we have arranged with school that she is going back in on a Tuesday and a Thursday from the end of this week. Mr AL is going to take her rather than the school bus. Her class has six children and they don’t interact with the rest of the school. The teachers have all had their first vaccine dose, as have we and as have her carers.

We have kicked it around with our palliative care nurse, with school, with the children’s hospice, with other parents and we have made the decision because she’s clearly jumping out of her skin…her mental health is rubbish and Mr AL and I are going to snap before long—it’s happened before, before we got help with her care, a few years ago, and I recognise the signs.

I hate this shitty, shitty situation. I know everyone else does too, we are all in the same situation on one level or another. But I guess I needed to vent…and if you feel like this too, you’re not alone.

We’re here with you.

Personal post: it’s a lot, folks

I’m genuinely struggling to know what to post about this week. I’ve got all sorts of things going on–Littlest had another COVID scare in the week and had to go in to the Children’s Ward to be tested and my Mama had her radiotherapy orientation yesterday and has her fortnight’s treatments scheduled to start in a week’s time and both kids have needed bloods taken and we’ve all had flu-jabs.

open book on table
Photo by Polina Zimmerman on Pexels.com

Then…Morris got out whilst Mr AL was taking Littlest in to the ward on Wednesday and when I went to collect him up, he wouldn’t be collected (because badger setts are more interesting than people, doh) and I ended up having a mahoosive seizure in the middle of a ploughed field a quarter mile away from home, in the dark, in the rain. Without my locator-watch gadget, which meant that poor Talking Child, the passing Lovely Jogger, my sister and my friend–both of whom I managed to call in a garbled fashion before I went full-on kipper–spent quite a while searching for me. I’d just got out of the bath and was in my nightie and house-trousers and unsuitable shoes, and I’d semi-fallen in to the ditch whilst trying to jump from one field to another.

Morris Dachshund of Lester Towers, Badger-Hunter Extraordinaire. Butter wouldn’t melt, obvs.

I felt pretty grim on Thursday–I get a sort of hang-over after the siezures–and that was made even worse by feeling so useless with everything else that is going on. Mr AL will be driving my Mama the ninety minutes up and down to the hospital, with my sister. It’s more comfortable and less tiring for Ma to travel in her powered wheelchair in our vehicle rather than transfer in and out of my sister’s and take a folding chair. I can’t participate in any of that, because I can’t drive any longer and no-one wants me to ride along only to have a fit in the middle of Mama’s appointment.

In addition, it’s not really safe for Littlest and I to be on our own without supervision. She’s a choking risk and I’m a fitting risk and if those two things happen simultaneously, well, that’s not ideal for her. This has been the case for quite a while and we work round it, making sure we always have oversight.

But after Wednesday, my nearest and dearest are having a conniption fit about me being alone at all.

Littlest, when she was in hospital for something-or-other earlier in the year. Honestly, we lose track.

I’m really, really pissed off about it. I relish my time alone and need it to recharge. Being stuck with a carer in the house is horrible, however much I like them personally. It’s not that I want to hoover naked or turn cartwheels in my underwear; but that possibility doesn’t exist at all if you have someone else in the house. And if they are pottering around unloading the dishwasher and changing the beds…both of which I am massively grateful that they help with…I feel guilt-wracked sitting on the sofa watching them and not helping.

In addition to all that, we have lost our usual support from the Children’s Hospice. Because of bloody COVID, they have had to change the way they look after their families. Usually we all go for three or four nights every three or four months, Littlest gets twenty-four hour care and we get looked after too. There are people to talk to and discuss different care approaches, there are comfy sofas and a nice garden and lovely food and we have a real rest. Now though, they are only open for end of life care for children (both COVID and other ‘normal’ conditions) and emergency stays for children when families are on their knees. Littlest went in the summer when we were doing really badly and she didn’t enjoy it as much as she usually does–it was all a bit different, the main parts of the house were shut off, she couldn’t interact with everyone like she usually does and of course all the carers were in scary PPE. They have us on the emergency list again, but it’s not the regular respite that we have been relying on.

Plus I’m bricking it because although we’re in a relatively low-incidence area COVID-wise, it’s on the rise everywhere and if we get locked down again we will have to cut back on some of the carers we have coming in to the house, just to keep everyone safe. That will mean we get progressively more exhausted–Littlest needs help in the nights. And there’s the risk to Littlest on top of her general respiratory fragility. And the risk to my Mama, with the lung cancer.

My mental health is for shite, basically, and not really because I’m becoming extra-depressed, but because there’s so much going on.

I’m trying to crack on with the rewrite of The Hunted and the Hind, but I’ve stalled a bit. All I want is to be in an alternate fantasy world, to be honest. Somewhere I have control over, unlike here. But I’m not sure that makes for good writing.

That’s it. That’s the blog post. Tomorrow is another day, we’ll laugh about this later etc..

It’s just a bit tiring, is all.

disambiguation: mince pies

Proper British Mince Pies ™

You need:

  • Mincemeat
  • Plain Flour
  • Butter
  • Lard or vegetable shortening
  • Water
  • Sugar
  • Wine
  • 12 muffin muffin-tin

Step 1

Procure mincemeat. THIS DOES NOT HAVE ACTUAL MEAT IN IT.

It usually comes in jars and if you can’t find it locally, you can make your own. Mary Berry, who is a veritable saint among food creators, has a good recipe.

NOTE: In Ye Olden Tymes, mincemeat DID actually have meat in it. In Tudor England, this was often mutton or perhaps beef. There’s a traditional recipe from 1591 here.


Step 2

Make (or buy) pastry

You can use shortcrust or puff pastry. We use shortcrust because it’s easy to make, especially since I prefer things to be gluten-free:

  • 8oz plain flour
  • 2 oz butter
  • 2 oz lard (or vegetable shortening)
  • pinch of salt
  • splash of cold water

Put the flour in to a bowl and chuck in the lard and butter, cut in to lumps. Take the knife and keep cutting the fat, so that it gets covered in the flour. Then rub it in gently with your fingertips, lifting it up out of the bowl and letting it fall back in, to get air in it. When you have a bowl full of crumbs, add a table spoon full of cold water and use the knife to mix again, to start it sticking together. Eventually, you’ll need to use your fingers again. Don’t make it too wet, but if you do, you can add a bit more flour.

Stick it in the fridge for half an hour and have a glass of wine whilst you grease the muffin tin and wait for the oven to heat up to 220c/gas mark 7.


Step 3

Compile your pies

  1. Roll the pastry out on a floured surface. Use a pint glass to cut circles and put them in your muffin tin, pressing them down.
  2. Put a tea-spoon or two of mincemeat in each of the 12 little pies.
  3. Use a smaller glass to cut a smaller lid for your pies. Or a cookie-cutter in a star or similar. Or you can make lattice strips for the tops. Or stack a little a pile of almonds on the top of each one. Or a couple of slices of apple.
  4. Arrange your lids on top of your mincemeat. Glaze with milk and sprinkle with sugar.
  5. Cook for about 15/20 minutes.

DO NOT EAT IMMEDIATELY AS THE MINCEMEAT WILL BE THERMONUCLEAR IN TEMPERATURE AND BURN YOUR TONGUE.

We sometimes eat these with no topping, sometimes with cream and sometimes with custard. Custard is a whole different can of British Worms that I will address on another day.


Fashion Museum, Bath

Firstly apologies for the lateness of this post. However, I’ve been collecting blog material! We’ve been on holiday near Bath and we went to the Fashion Museum earlier in the week. I was primarily focused on looking at clothing from the 1770s and 1780s for Edie and for Jones.

The trouble with collections of historical clothing is that you only get the really expensive things or the things their owners didn’t like much that survive. And you don’t get a great deal of working people’s clothing, because they literally wore it until it had holes and then it got cut down and repurposed. Clothing was so much more expensive and energy-intensive than it is today. Everything was woven and sewn by hand.

These gowns and petticoats from the 1770s and 1780s are much more Edie’s sort of thing than Jones’, although I do imagine Jones stuffed in to the one with the blue quilted petticoats when she was visiting her aunt in England. And perhaps the one with the yellow gown and stomacher for more formal occasions. I can definitely see Edie in the pale pink effort with all the embroidery on the front when she first meets Jones at the ball. (High waists a la Jane Austen only came in around about 1794 as far as I can make out).

Once the pair of them are travelling, they revert to much simpler clothes. I imagine Jones wearing something like this… it’s based on a working man’s coat from about 1780, made of wool.

I am still in debate with myself over whether Jones would wear local clothing once she gets home to the mountains. I think she might need to stay in western garb because I am not confident enough to write about regional clothing without getting it wrong and that seems disrespectful.

Edie doesn’t feel right going for breeches, however comfortable they might be. So she compromises by wearing ‘stays’ or ‘bodies’ (which is what she calls them) that lower class women, who had no help getting dressed, wore. They lace up the front rather than the back, so you can do them yourself. This is really interesting little video of a working woman getting dressed.

The little things… how you deal with menstruation, what pins you use in your hair, how often you change your stockings… those are all things that tend not to get referenced in contemporary texts because it was all such normal stuff that you didn’t need to. Everyone knew about it. There’s a good blog post about Georgian personal hygiene by the Word Wenches and I think I may have mentioned Madame Isis’ blog before.

Next week I am back to regular scheduling and I am interviewing Naomi Aoki!


PS: As we came out of the museum and went to find the old fashioned sweetshop, we fell over a coach and four. Netflix are filming the Bridgerton series of books by Julia Quinn.

coming to terms with CTBP1

If you follow me on twitter and to a lesser extent here, you’ll know that Littlest has very complex needs and is classed as ‘life limited’. Well. Something has happened and I’m not sure how I feel about it, so where better to process my feelings than here, publicly?

Littlest is ten. She was born with pneumonia and spent some time in an incubator. She was very ill and on CPAP when she was eight weeks old and had swine flu the summer after she was born. She never met her milestones- rolling over, walking, talking. And as she got a bit bigger, everyone started to believe us when we said there was something not quite right about the way she was growing. Eventually we got referred to the bigger area hospital and then they referred us to Great Ormond Street. Lots of tests got done for rarer and rarer conditions and all drew a blank. GOS thought she was interesting and did a muscle biopsy and it turns out that the nucleus of her muscle cells are in the middle rather than on the edge as they should be, so her muscles don’t sproing properly and she’s underpowered. This presents similarly to Muscular Dystrophy, but it’s because she’s outgrowing her strength as she gets bigger.

So, she no longer walks at all, except in a walker. She is classed as having an unsafe swallow and is fed by tube. She can’t turn herself properly in bed. She can’t vocalize words, although she can make sounds. She communicates with sign, but that is getting harder for her as she is losing her fine motor control. She has a learning delay and is probably around about four intellectually. She can’t read but she recognizes some letters. She can count a bit. She understands pretty much everything you say to her and really wants to learn. She has a wicked sense of humor. She likes Peppa Pig and books and her iPad and any animals she comes in to contact with love her reciprocally.

We had no diagnosis. And therefore we had no prognosis. We were referred to the Children’s Hospice seven years ago. The criteria for a referral is that they think that the child has a fifty percent chance of surviving til they are nineteen.

This took some coming to terms with.

CTBP1 Gene

The thing I have found absolutely hardest to process, though, is the lack of certainty about it all. Four years ago I started having these stupid stress-related seizures, which were eventually diagnosed as ‘Functional Neurological Disorder’ or a learned reaction to stress. Mindfulness, relaxation and avoiding stress is the only way to manage it. It’s taken me years, but in the last twelve months or so, I’ve finally begun to live in the moment, appreciate what we have and just seize the day. Enjoy what we can. Live our life in the cracks of appointments with Littlest’s fifty-ish different professionals. Spend time with Talking Child. Fit friendships and self-care and tending to my relationship with Mr AL around the chaos.

And then. Yesterday. We had a letter from the 100,000 Genomes Project. We had had swabs and bloods taken years ago. I’d put it out of my mind as something that was yet another test that would be futile. A hoop for us to jump through that wouldn’t give us any answers but which might help future children and parents.

Littlest has a small alteration to the gene CTBP1.

We have an appointment on Tuesday at the area hospital to go and discuss it. Obviously in the meantime I have played Internet Misery Bingo (TM) and found a load of articles I don’t have the education to understand and also, a support group set up by a genetics study in the US, which has ONE (1) other parent on it, from the Netherlands, with a slightly older child who has hauntingly similar symptoms.

Mr AL and I are both a bit weepy and shocky. After so long and so much adrenaline, I have no other emotions left. How am I supposed to feel about this? Materially, it changes nothing. The sample size of children with the alteration is vanishingly small, so we are no closer to a prognosis than we were before.

All information is good, but I simply don’t know what to do with. A very long time ago I blogged every day about life, feelings, the usual introspective noughties stuff and it seems like that’s where I turn again when I need to process.

Thanks for listening, if you’ve got this far.