Two little stories form Greece

We're visiting Super Papou in Greece - with Yaya and Dimetres, but without Mummy. You and your sister are having so much fun. Today we ate lunch with Hella in Loco Bus and went to the play area, which had a bouncy castle, a ball pit and a toddler climbing frame. Maybe I'll post some pictures later.

Seeing Super Papou and Minnie sitting next to each other, I imagine Super Papou being Minnie's age and sitting next to someone of Super Papou's age - who would have been born in 1833!

I wanted to tell you two funny stories from today.

You made up your first joke. You made a string of spaghetti out of Play-doh with Dimetres this morning. You put the spaghetti on your nose and said, "Look! Bolog-nose!"

Dimetres laughed so much that you kept on going - you put the spaghetti on your eye and said, "Bolo-eyes!" then "Bolo-ears!" and so on.

Later, in the play area, you were having fun in the toddler climbing maze, which was full of little padded rooms with net walls, and slides, and ball pits. At one point you shouted, "Daddy, Daddy, Daddy!"

"Yes, Penny?" I said. I couldn't really see you in there, but I could hear you well enough.

"I'm a stuck princess!"

"Oh, really?" I said. "Will you be stuck a hundred years and grow old in there?"

"No!"

I laughed to myself, and went back to chatting with Yaya.

Then five minutes later you started crying. "What's wrong Penny?" I asked.

"I'm a stuck princess!"

"You're really stuck?"

"Yes!"

So I ventured into the child-sized climbing maze and found you. Sure enough, you were totally stuck! You had unzipped a netted wall, snuck behind the maze and zipped it up again behind you - but the zip was only on one side! You were huddled into a tiny space, smiling at being rescued. I know you like pretending to be a princess, but it was very funny to hear you refer to yourself as one in that situation!




Also, soupcases.

Medical options

Minnie's medical status is constantly evolving. No doubt in a few months' time things will have changed and we'll have forgotten what decisions we were wrestling with, so I thought I'd capture where we are right now, for posterity.

Min is healthy, having fully recovered from her various medical problems last year, except for two threads: her deafness and her pyruvate kinase deficiency.

As far as her deafness goes, we had 11 appointments put into our diaries in the two months leading up to Christmas to go through her assessment for cochlear implants. We're halfway through them as I write this, and Em and I reckon it's reasonably likely they'll recommend that Minnie should have an implant in her right (profoundly impaired) ear, while her left (severely impaired) ear should remain aided.

As much as we'd prefer not to install a piece of kit into Minnie's skull, the benefit to her hearing and speech is potentially huge. Her ability to develop speech is greatly helped by the small amount of hearing in her left ear (she can hear low frequencies with only mild impairment), but right now, Min has never heard the high-pitched consonant sounds like 's' and 'sh'. With an implant while she's still small she could learn to interpret and use those sounds quickly - and she has a chance to be talking normally by the time she goes to school.

The other thread is her pyruvate kinase deficiency, which means that her haemoglobin count gradually reduces over time - she becomes anaemic and a bit yellow. She's been having a blood transfusion once every six weeks to top her up and keep her healthy.

Next year we are going to try and wean her off blood transfusions - extend the gap between transfusion by an extra week each time. The idea is to test whether her body can maintain her haemoglobin count at a reasonable level. She will certainly be anaemic by normal standards, but if she can still grow and be healthy and have energy then she can give up the transfusions.

A normal Hb count is about 11-13. Min has gone down to around 8 before each transfusion - and that's pretty much the lowest that the haemotologist would be comfortable with leaving her at. If she could maintain at 9 or more and still be healthy and have energy, that would be ideal. But if she maintains at less than 8, she'll definitely have to go back onto the transfusions - probably every four weeks, indefinitely - and she'll need chelation as well.

Regular blood transfusions mean that iron builds up in her blood and can eventually lead to organ damage. Chelation (which is basically a pill once a day) counters that, but can have side effects including the loss of her precious remaining hearing.

If weaning Minnie off transfusions doesn't work, that'll be disappointing, but transfusions are still more appealing than the other option, which is to try and cure her. The cure for pyruvate kinase deficiency is a bone marrow transplant, which requires chemotherapy, which would almost certainly leave Minnie infertile. We are not willing to make that decision for a one-year-old. Better to continue with transfusions until she's old enough to be part of the decision - and maybe by then medical technology will have progressed enough to provide us with more alternatives.

Thank goodness for the NHS.

Signing the Alphabet

We still don't know many signs.  But Daddy did a sign language course many years ago, and the alphabet stuck.  So he signs the alphabet to you and your little sister.

You get lots of the signs right, and it is funny watching Minnie try to.  She loves it!