Almost Christmas

It always seems the time flies by now, with two little ones.  Just as a quick update, Miya is FABULOUS!!! She can write her own name, is potty trained (except for the odd accident) and LOVES going to nursery.
Scout is full of mischief and is into EVERYTHING!!! She started crawling a month ago (at 6 1/2 months) and now thinks she should be able to walk.

Miya had her very first trip to see Santa this year and loved it!!

Both girls have had the chicken pox, although Miya got off quite mildly with only a few on her face and some on her backside.

I should also point out that Miya is growing like a weed and I have to buy size 5 clothes for her!!

From our family to yours, We Wish You A Merry Christmas!!!!

Now Where Was I....

Oh yes, I remember...When I last updated, we.....

Minutes after her birth, 16/04/2013
6lbs 13oz 

Scout Eiko Hulse, 1 day old

Actually, I don't remember.  I remember nothing.  I have good reason to remember nothing.  I had a baby...a baby sister for Miya!!

39 + 6, Three days before baby's birth

Scout Eiko Hulse was born at home on 16/04/2013 at 03.09hrs, weighing 6lbs, 13oz.  I've received lots of comments regarding my "bravery" at having a home birth (my view is that birth is a natural thing, not major heart surgery. There were no major complications and all indications pointed toward a normal delivery. I felt that there was no reason to leave the comfort of my own home. But that's a debate for another forum.) and Chris and I have both received some nasty, nasty comments regarding Scout's name.

Scout was named after the main character in the book To Kill A Mockingbird by Harper Lee, and  in the words of a friend of mine, is named after a literary social pioneer. Some members of the family nearly disowned us over the name.  I have my own opinion about this and suffice it to say that I don't give a s*!t and neither does Chris. If we had wanted to name her Sunray Moonbeam or Octavia Munroe, we would have.  End of.  Scout means "explorer" and Eiko (pronounced 'I-ko') is Japanese for "prosperous child". It's a unique name and it suits her.  

Miya has been brilliant and has never shown one bit of jealousy.  She accepted from the beginning that Scout was going to be born and would live with us and she was going to be a big sister.  

Miya turned three in July and is doing marvellously. She now enjoys weekly swimming lessons and has no fear, jumping into the water without floats on.  Her spine is good and her vision is good (both recently evaluated) but her hips are showing some sign of wear.  The femoral heads (round in unaffected people, more square-shaped in people with Hurler's) aren't sitting in the joint very well and have become uncovered. She has been referred to orthopaedics to see if she needs surgery to cover them, or if they just want to monitor her situation for a while.   

For a while, I have noticed that she seems to be tired after using her legs.  She can't pedal a bicycle and she often asks to be carried after walking for only short distances.  We mentioned it to her consultant at her clinic appointment yesterday and after watching her perform some basic tasks, he seems to think that it might be muscular.  Miya is having a physio evaluation on Monday and she has been referred to Cardiology and may possibly have an MRI. He doesn't seem to think that it's a problem related to Hurler's (as Hurler's affects bones and joints, not muscles).  

Wearing my Doc Martens

Super Tall!!

Our Family At Whitby Abbey

Scout Loving Bath Time 

Miya & Mummy In Whitby 

Scout, 4 Months Old

Miya starts Nursery full time next week.  She loves it (she went a day a week before school broke up for summer holidays) and she's all ready to go with her new ladybird backpack and lunch bag.  She's pretty much toilet trained now, except at night.  She's really creative and likes to draw, paint, sing and dance. 

So that's about it, really.  That's what's happening. I'm off to have my fifth cup of coffee...this momma runs on caffeine!!

An Update..Three Months' Worth Of Stories

 It has been a little over three months since my last post (sorry).  The last three months have been busy, tiring, funny, not-so-funny, sleepy, sometimes lonely, and always entertaining. 

In November and part of December, Chris was away with work and Miya and I found ourselves on our own. During this time, Miya had an audiology appointment in London so we bravely took the train to London all by ourselves (with horrible colds) only to discover it was a wasted trip - Miya's ears were too full of congestion to properly conduct the hearing test.  It has been rescheduled for March. 

In October, Miya was discharged from BMT and back to our regular Metabolic team.  Yesterday, we had our first Metabolic clinic since being discharged!!!  I have to gloat a little, so be prepared for some proper boasting. (Let's be honest, shall we? If I don't, who will?)

Miya's height and weight are good, at 15kg (33lbs) and 95cm (37.4in ).  Her hair is growing back nicely and she only has a small amount of her "monkey fuzz" left on her back, a reminder of the Ciclosporin days.  We talked about Miya's cognitive skills and have determined that she is achieving normal to above-normal cognitive abilities.  Her skeleton was found to be "better than expected" for this stage in her life and although Hurler's is the most severe of the MPS 1 family, at this time, she is prooving to be at the milder end of the spectrum within Hurler's.  Miya's enzyme level is normal for a post-transplant Hurler's patient, but lower than normal for an unaffected individual.  The GAG levels in her urine are still slightly elevated, but we were assured this is normal (they can take some time to come down) and no one is overly concerned about this.  We will provide the Metabolic team with a urine sample in March and her GAGs will be tested then, as well as her enzyme level via a blood test.

She is not without issue, unfortunately.  She has developed mild knock knees and toe walking (both common in Hurler's kids).  She will have some x-rays taken, and be evaluated by Physio in March.  A treatment plan for the knock knees and toe walking will follow evaluation.  Miya also requires a dental review which can be done locally at the RUH in Bath. Hurler's kids tend to have weak enamel and the root can degenerate. Cardiology will also be followed locally, with any surgery that might be required to be done at GOSH.  Audiology and Opthamology will continue to be followed at GOSH.

Miya's vocabulary is huge and she knows how to count to 20, can count backwards from 10, and knows her alphabet.  She can tell you what different words begin with by sounding the first letter.  She is developing deduction skills and can quickly use what she learned in one situation to be applied to another entirely different situation.  We are beginning potty training, and she has peed in the toilet twice.  She shows enthusiastic interest, which wanes from time to time so we aren't pushing her.  When she shows interest, we encourage her and when she doesn't, we remind her about using the potty but don't force her.  She doesn't seem to have developed the proper bladder nerves yet, so I'm not expecting a toilet training miracle at this point.  She'll go when she's ready.

For those of you that follow this blog and didn't know, I am nearly 27 weeks pregnant with an unaffected baby sister for Miya.  We had CVS at 12 weeks, which determined I am carrying a girl and she does not have chromosomal abnormalities.  We will be able to find out if she is a carrier (like Chris and me) with a simple blood test after she's  born.  Since this is a normal, uncomplicated pregnancy, we have opted for a home birth.  I had a traumatic experience in the hospital when I had Miya and the thought of birthing another baby in the hospital sends me into a million panic attacks.  Some people have voiced their disapproval over this choice, and I really don't care what other people think.  Our decision to have a perfectly safe home birth in our own environment is not up for debate. Although not common in North America, it is common here and perfectly safe.  If complications arrise, I will be transported to hospital...easy as that.  End of story.

Miya has various appointments at GOSH between now and March and I will update you on those.  And of course, I will update you on the arrive of Miya's sister.