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.

  

The Kindness Of Strangers - A Family Holiday

A while back, Miya's story ran in a local newspaper where Chris is from.  A fundraising event was taking place, organised by Chris's family, to raise money to take Miya on a holiday and the newspaper ran a story on it; we were thinking enough might be raised to take Miya on a UK holiday. 

We were contacted out of the blue by a man who wanted to run in the Manchester Run and the Great North Run for Miya's benefit.  After the runs were completed, he contacted us to say he had a cheque ready to send to us.  The money raised was more than we could have dreamed or hoped for.  We took a family vacation to Menorca.

For a whole week, we didn't have to think about hospital appointments or illness.  We didn't have the stresses of everyday life, and the stresses that built up over the almost two years since Miya's diagnosis.  The holiday was great for our family and great for my marriage.  We never, ever would have gotten away anywhere any time soon had it not been for the kindness of strangers.  Somehow, "Thank You" doesn't seem like enough to say.

Here are some photos of our holiday.  I know my dad has been itching to see them!!

With mummy, hot and humid outside

With daddy on the balcony

Watching the "Big Red Bucket"

The children's pool, and view from our balcony

The "Big Red Bucket", Miya's absolute favourite

At Gatwick Airport, waiting for our flight

On the plane, ready for takeoff!

Swimming with Daddy

In the pool

A small beach near our hotel.  Suitable for playing in the sand but too much seaweed for swimming

We're on holiday!!!

Playing with Daddy

Building sandcastles with Daddy...first time playing in the sand!

Loving the beach!

Having a drink from a big girl cup

At the airport in Menorca..Super cool in sunglasses

A 2nd Birthday & A Big Girl Bed For A Big Girl

Miya's second birthday was celebrated on the 7th of July.  Just as last year, it was a party for three (Mummy, Daddy, Miya) and Miya enjoyed herself imensely.  Once I persueded her to stop playing with the balloons, she enjoyed opening her gifts and eating a slice of birthday cake.

Miya's Giant Cupcake Birthday Cake

Miya Examining The Gifts

Now THAT'S How You Eat Cake!!!

Miya's 9 month post-BMT appointment took place last Wednesday at Great Ormond Street Hospital and it was uneventful.  Despite it being two days before the Opening Ceremonies for the Olypics, the driving was good (for London) and we got in and out more quickly than usual.

The usual blood tests were taken and the usual glowing reports were given. Miya's blood counts are really good and we are waiting on the routine engraftment test results (the actual blood test will be done on the 10th of August, locally by our community team).  However, I am confident they will return the desired result.  Miya's last engraftment test was done in April and was 99%.

Last night, Miya spent her first night in her "Big Girl Bed" because she is now a "Big Girl".  She will not hesitate to tell you this. (Incidently, she will also tell you it is "Very Important To Brush Your Teeth!!!".  I went in to check on her about an hour after I put her to bed, and she was laying in bed, looking at a book my dad sent her.  She still wears corrective boots with a bar between them at night (for the correction of club foot), so getting out of bed will require some determination on her part.  I expect, if she ever tries it, I'll hear a "Thud!" followed by crying.

Looking Proud For Having Her Feet Buried In The Sand

Excited To Be In Her Pool

Miya's New Sandbox

Miya Trying Out Her New Bed (With Bed Rails For Safety!)

 The weather this summer, in our part of England, has been rainy for the most part.  We seem to get a week of sunshine followed by about 6-8 weeks of rain.  This past week has been lovely and warm and we've all been outside as much as possible.  Miya has been in her paddling pool and got a new sandbox as well as a Hello Kitty bucket and spade set. We've also been to several parks and soft play areas. Miya adapts to new situations quite easily and her vocabulary is growing every day.  She can put together simple sentences (five-six words in length) and chatters away for most of the day.  She asks me for chocolate milk on a daily basis and only seems to want to eat sausages (a typical two-year  old).

Below are some photos of our outings and our everyday life.

Smiling In The Soft Play Area

Out For A Walk Near Our House

Showing Off Her New T-Shirt

First Encounter With Pine Cones

Miya & Mummy

Little Girl Under A BIG Tree!!

 

 

Looking Humiliated In An Elephant Costume