Thursday, 24 October 2013

plan a? or plan b?

Would you all care to join me in thinking the unthinkable for a moment?

I read a thing, a while ago, by someone with a severely ill child. She wrote that most people in that situation had a Plan A and a Plan B.

Plan A is how you are going to provide for your disabled child if you die before them.

Plan B is what you are going to do if your child dies before you do.

No-one should have to make these decisions, should they? But we are.

We've got Plan A more or less sorted. Life insurance, guardians*, letters of wishes, all of that. It's nice to feel that it's all tied up and it will all become Somebody Else's Problem if we both cark it suddenly in a Statistically Unlikely Electric Blender Incident or similar.

Plan B and I'm fucked.

Plan B varies from day to day, swinging so wildly from one end of the spectrum to the other that really, there IS no plan.

A part of me thinks that we will just trundle along as we are. Same house, same routine, same work, same school for Leo. Routine, Routine, Routine. Good for us, good for Leo. He'll need stability, he'll need his friends around him, WE'LL need the same. My mother, my sister. People to lean on when we sit at the top of our metaphorical stairs, sobbing too hard to be able to descend them and start our day.

The other part of me is planning a Great Escape. Drop everything that can't be fitted in to the camper van and leg it. All our roots, all our books, all our bedding and the tea-spoons; all the nik-naks of our past; shared or pre-marriage. Freecycle the fucking lot and drive off in to the sunset. Take Leo out of school for a year or two. Circumnavigate Europe. Attend mass in St Peter's Square and dance on the bridge at Avignon. Travel the Silk Road to Saamarkand and see Tamburlain's grave. Spend six months walking the Camino from Vezalay to Santiago de Compostela.

I am flailing. I can't see where we're going and I can't see a way out. I feel like every aspect of my self and my life is being squeezed in an enormous vice - there's no way out, no way back, no alternatives. All I can do is move forward, squeeze through each hour, each day, each week.

How can we work out a Plan B without knowing how long we've got? All we can do is go from day to day and try to keep all the plates in the air. And when we get there, we just have to hope that we'll know what to do.

What would you do?



* waves

Wednesday, 23 October 2013

internet disease misery bingo. a game the whole family can play.

I need somewhere to pin these after spending a morning playing Internet Disease Misery Bingo from the key phrases the consultant wrote down for us yesterday.

Centronuclear Myopathy (confirmed by biopsy - the nucleuii of her muscles are in the centre rather than on the edge, so they don't sproing as much as they should, as if she is running an underpowered engine) - http://www.muscular-dystrophy.org/how_we_help_you/publications/1978_myotubular_centronuclear_myopathy . There is also another muscle problem with the rods in muscle fibres being a different length to what is expected. You normally get one of these things going on, not both.

Mitochondrial Complex IV Deficiency (slight, but confirmed by biopsy) - http://rarediseases.info.nih.gov/gard/48/mitochondrial-complex-iv-deficiency/more-about-this-disease

Vici Syndrome (possible from her displayed symptoms) - http://quinnandkristi.blogspot.co.uk/2013/02/were-published.html

She also has a small Cerebellular Folia, examined by MRI last year, which is what is causing the Ataxia. I can't find anything helpful about that that I understand.

These four things have pointed them to various gene groups. The genes are checked one by one and as each one comes up clear they move on to the next. That is why it can take up to twelve months. I don't know whether there is a standard order in which they are tested, or whether they start at the best guess and work out from that.

In other news:

1. Nenna is sitting on the sofa with her head under a blanket, watching Charlie and Lola on her iPad and occasionally sticking her head out and shouting "BOO!"

and

2. Arvo has inadvertently taught her to say 'shitbags!'. Luckily her speech is so poor that it just comes out as 'BAGS!'.

For today, that is all.