Time Flies!

Happy 18th Birthday Sarah!
Thank you for loving our Rosie...everyone can see the love between you here! Pictures from last Mondays shoot!

I cant believe it is almost a week since I last posted on here. The time goes buy so fast! Tv Lady arrived and did not a bad job of showing life in our house along with making a point about Rosie and CdLS. We all watched with bated breath as the article came on...three hours filing for a 3 minute slot but it was quite good I think and everyone who saw it has said so too, Now I have a link to a video of the article. IT is not great quality as a friend videoed it on her mobile phone from the TV and then uploaded it.....but it does let you see what they showed...and I am extremely grateful to Lori from http://www.nosteptoosmall.blogspot.com/ for putting it up for us.

http://tinypic.com/player.php?v=znnfoy&s=5

So fast forward to Monday and our trip to Sheffield. We decided to make the most of a late appointment and investigate Sheffield City Centre...next time we will go back to Meadow Hall!!!

I did manage to buy Christina a pack of knickers and Eve a pack of socks but other than that we bought lunch at Pizza Hut which was a lovely treat and then heded back to the hospital in the hopes of a parking spce where we would not have to worry about moving it later! We arrived at the hospital to cars parked on both sides of the road on the double yellows and a queue of at least 5 cars for the hope of a space coming free in the car park....there are apporximately 30 spaces for a fairly large children's hospital...diabolical! Thankfully just as we got to a point in the queue where we could escape to try on the road someone pulled off from the double yellows and we squeezed out the queue and parked! We had 3 hours on the Blue Badge so we hoped to get parked properly for the night then.

We were very early for our appointment so decided to relax on the cafe with a coffee. Eve had fallen asleep in her chair....heart rate racing above 175 so I eventually upped the alarms to try and keep ehr a bit queiter.

We had a lovely consultation with the respiratologist and were fairly hopeful that the upcoming sleep study would show us which option was going to give us the best chance to Maximise Eve's quality of life. We were hoping that tracheostomy would not be too high on th elist of options....knowing we would deal with it if we had to but preferring to to have to! The Respiratologist spoke of the problem of long term Hypoventilation causing a rise in CO2 levels and this being a concern for administering Oxygen. However she also explained that if that was the case then BI-PAP would be an option that may help more.

We left this consultation with high hopes and expectations of a useful sleep study with hope for a better quality of life for Eve.

We presented at the Sleep Unit at 6pm and the staff, all wonderful kind and compassionate ladies, started with the wiring up of Eve! there were 6 electrodes on her head, one on her forehead, one either side of her eyes, one behind each ear, a microphone pad under her chin, a vibration recorder also under her chin. 6 ECG electrodes on her chest, two movement senosrs on an arm and two on a leg, nasal canula for oxygen and to measure the emision of CO2 and the flow rate of expired breath. a sensor on her toe and one on her ear for CO2 and oxygen saturations, a probe below her nose and above her mouth to measure any temperature change. I think that was it....I did consider taking a photograph but only having my phone made that a bit pointless as I cant upload from it!

The study started and her oxygen levels rapidly crept to 99% and hovered between 97-99 all night!

Her heart rate remained tachycardic and I think her breathing rate was rather fast too but not been able to clarify that...20 breaths a minute seems a bit fast for a 5 year old asleep to me.

the night passed slowly...by 3 am Eve was having significant Myoclonic jerks and so far nothing dramatic had happened to my knowledge. There was a big confusion over the time she spent being recorded in oxygen, it seemed rather bizare to only give her an hours worth of 1litre before turning it down then down again until she was on .4 of a litre...her heart rate did not respond to the oxygen and I had assumed that was their aim...to see how much oxygen she needs to maintain her heart rate at a better level??? It turned out they were watchign the changes in CO2 when in and out of oxygen and seeing if the level of Oxygen affected the level of CO2...it didnt!

So the morning arrived and we were transferred to day care to allow Eve to wake up more naturally nefore removing the probes. Then we met again with Dr E who assured us the Sleep Study had demonstrated no obstruction, no evidence of Hypoventilation and only three minor central Apnoeas that had not really been of a concern. Therefore she feels there is nothing to offer Eve to improve matters and O2 should continue at 1 litre as it does appear to be having an affect!

So we left the sleep study with no answers we didnt want....ie trachy.....but no answers we did want either....we so desperately wanted to do something to help eve, to enable her to enjoy life to enjoy life more again....this is not to be!

We left the Children's hospital to walk up to the Hallamshire for the nerve testing...it was a pleasant walk apart from the last 5 minutes uphill.....David took the chair for that and I hobbled up the side!. Once in the Emg Lab the tests were explained and the Doctor assured us that if Eve found it too destressing they would stop! Eve found it quite funny at times even though it did trigger some Myoclonic Jerks. During this consultation I was able to se the referring letter from the neurologist...and on their under the heading of diagnosisI saw written

Undiagnosed Mitochondrial disease/Leigh Like Disease!

This is the first time I have seen this written down and no one has discussed this with us. However I suspect it came about from our last appointment where he could no longer illicit reflexes from Eve and copuld see significant progression of the disease in her.

Walking back from the Hallamshire to Children's I rang through to the metabolic Nurse who offered to meet us for a coffee at the Starbucks Cafe.....that ws so kind of her and we had an opportunity to offload the events of the previous few hours before our drive home.

It was good to get back home.....but where from here? It seems we have reached the end of the road for trying to find some way to improve things for Eve.....

The words of a chorus we sang at Camp come back to me

I am trusting thee Lord Jesus

Trusting Only Thee

Trusting thee for full salvation

Great and free!

We also sang a Hymn I barely knew at Church on Sunday.

I love the way you Father me!

It was very poingnat for me....and is a Hymn I hope we repeat in the not too distant future....it is by Graham Kendrick

O father of the fatherless

In whom all families are blessed

I love the way you father me

You gave me life, forgave the past

Now in your arms I'm safe at last

I love the way you father me

Father me,

forever you'll father me

And in your embrace

I'll be forever secure

I love the way you father me

I love the way you father me

When bruised and broken I draw near

You hold me close and dry my tears

I love the way you father me

At last my fearful heart is still

Surrendered to your perfect will

I love the way you father me

If in my foolishness I stray

Returning empty and ashamed

I love the way you father me

Exchanging for my wretchedness

Your radiant robes of righteousness

I love the way you father me

And when I look into your eyes

From deep within my spirit cries

I love the way you father me

Before such love I stand amazed

And ever will through endless days

I love the way You father me

Graham Kendrick Copyright © 1992 Make Way Music, www.grahamkendrick.co.uk All rights reserved. International copyright secured. Used by permission.

http://www.grahamkendrick.co.uk/songs/lyrics/father_me.htm

I wil leave you with a knowledge that prayer will be forthcoming for us...I thank each and everyone of you who prays for us....I probably dont know some of you and others I never realise who reads my blog unless you leve a comment....I urge you to just leave a comment to say you have read and maybe to say you are praying....you cannot begin to imagine the encouragement that is to us.

Underneath are the everlasting Arms!

Hugs