Fragility…

All children are fragile, whether it’s physically or emotionally.  All parents want to make sure that their little ones are safe and happy at all times.  Everything we do, we do because it’s the best that we can for them.

All three of our children are fragile in their own way, Joseph is a sensitive soul and Alice and Ollie have thousands of pounds of sensitive electrical equipment embedded in their skulls.  We as parents try and balance normality with protection.  Today, Alice and Joseph went happily into school for another day of learning and wonder.  I smiled as I waved them both goodbye.  Half an hour later, the difference between a normal child and a CI child became clear, Alice’s processor has a fault.  She instantly goes from being a “normal” child, to one with a significant hearing impairment.  Her confidence slips and I turn on the rescue protocols.

The spare that lies under a layer of thick dust 99% of the time has to go and get reprogrammed, the school nurse and Alice’s amazing LSA do their best to reassure and repair, but ultimately it’s a replacement job.  We are extremely fortunate that we have a spare.  Due to a high level of losses, our CI programme is out of spares, so without the dusty old processor, Alice would have lost her favourite ear for days…. School would have been a nightmare.  It never ceases to amaze me how much on a knife edge we live.

If any CI company is reading this, our real list of wants as parents is as follows ….

  1. The most robust processor you can make (water, sand and falling over are all part of a normal life as a child)
  2. GPS and other localisation technology.  Ollie likes to crawl into small spaces and sometimes we find processors under drum kits, beds and other corners.  The park, theme parks, the beach and public transport are our idea of hell, 3 children, 4 processors, tickets, keys, phones it’s a lot to think about
  3. Spares – Cochlear Europe is 10 mins drive from Alice’s school, the MAPs can be carried on a USB and Alice could have been up and running in 10 mins.  Remote mapping helps those of us with spares.  But not everyone can afford to have £6k lying on a shelf for an emergency.

And as parents we need to make better promises, we need to take care of our children’s ears and processors and not leave our CI teams short of processors for the unusual occasion when something does go wrong.

 

One Thousand, Eight Hundred and Sixty One Days….

Since the 18th August 2008. A day I will never forget. The day we found out the our beautiful Alice was deaf. That is how long it has taken to get our answer…..

Today it came.

I have to say I am shell-shocked; not because it changes anything or anyone; not because we now know something we hadn’t already suspected, but because we finally have our answer; we finally know which little pieces of an infinitesimal code were out by a fraction and why our babies were born with wonky ears.

I am relieved, sad, happy, depressed, angry, elated, confused and exhausted all in one emotion.

Alice…Ollie….everyone, meet Otoferlin (OTOF) Exon 21 Mutation C.2348DEL>p.Gly783ALASFSX17

I told you so.

On the plus side, we also have a suspected additional mutation at c.1469c>Gp.Pro491Arg which is newly discovered and thus we are now part of some scientific study. How terribly exciting. Also, as they are recessive genes, it is both of our fault….

I’m sorry babies.

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I am now off to cry.

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