It is a year to the day that Anabelle was diagnosed with MPS1 Hurlers Syndrome and our journey began. So much had happened to our beautiful girl within in that year and she faced it all with a smile. From ERT to chemo, through the stem cell transplant and isolation. She has been and always will be an inspiration.

On our last blog we believed Anabelle died of septic stock and we were waiting on the post mortem results.  Sadley we are no further forward on this and we still do not know what finally took her from us as the post mortem report has not been completed yet.  Once this has been completed a date for  the inquest will be set.

Our little ones funeral was on the 18th January and it snowed heavily, we were worried about the turnout and although alot of people couldn't make it into town there was still a very good turn out. After the service white balloons with purple stars that everyone could write a private message on  were released within the grounds of the church. Our friends in Perth also released balloons at the same time, although their weather was a bit different to ours as you can see in the photos!!!

The cemetary looked extremely peaceful covered in snow and had a Narnia feel to it, we like to think that the snow was Anabelle's tribute to us.

There isn't a moment that goes by where she isn't in our thoughts and our lives will never be the same again. The message she left us was that we shouldn't get stressed about the small things in life as these generally have no consequence. She reminded us about the important things in life. The important things are family and friends and these should come before anything else.

On February 18th Anabelle would have been 2 years old, she had a lot of visitors and her grave looked very colourful

We would like to take the opportunity to thank everyone that has supported us, either through cards, facebook, text, in person, money sent via the funeral directors or the 'rembering anabelle' justgiving page.

Even though words cant describe the emotions we go through on a daily basis we are coping and still feel very strongly that the journey we all went through was the right one even though the end result was not something we expected at all.  The last few months with Anabelle, seeing her grow and develop will always be rememebered with a smile.  All MPS syndromes are horrific and this has changed our lives and will always remain very much apart of it.  In the next additon of the MPS magazine, Anabelle will be on the front cover and my tribute that I did for my baby girls funeral will also be in it. 

It is extremely painful to be writing this but the sad news is Anabelle past away on Christmas day. 145 days post transplant.  The pain is overwhelming and not a moment has gone by without the thought of desperately wanting her back. We are still at a loss and totally shocked by the suddenness of losing her.

We were only at day clinic at Great Ormond Street on Wednesday 19th December and the feedback then was how well she was doing. Her levels were good, her ciclosporin was being reduced and by 9th January Anabelle would have only been on two meds - Penicillin (which she would have been on for life) and Aclovir for a year.   We discussed Anabelle' Hickman line and had planned for this to come out by the beginning of February.

We also discussed long term prognosis and how she would live a pretty much normal life.

Anabelle was starting to say a few words, bye bye, hiya.  Her makaton sign language was very good and she was walking holding only one hand.

On Sunday 23rd December she woke up out of sorts and wasn't really herself, but had no temperature. As per protocol we phoned GOSH who advised us to take her to our local hospital,who were aware of her condition.

Anabelle didn't get put on IV antibiotics until 36hrs after we took her to hospital, they gave them orally which she vomited as she hadn't eaten for 14hrs.

Early hours Christmas morning Anabelle had a seizure and was eventually rushed to Southampton General Hospital on Christmas morning.  After 12 hours the infection had spread and had started to attack her organs and she sadly past away at 2.40pm from septic shock.

We are currently waiting for the results of the postmortem as we would like to know the name of the infection that killed her as well as other questions we need answering.

The news is devastating but we don't regret our decision to go through the journey we have shared with you.  BMT gave Anabelle hope for a normal (ish) life and we have shared many many good time since she got diagnosed with Hurlers .

Anabelle was the most happiest, loving little girl with the biggest smile and we are very proud to have been her parents, she was our little angel, our shinning star and we will love & miss her everyday.

"Even the smallest of feet have the power to leave everlasting footprints upon this world".
 

 

Our little angel.

 

Hi, Still going well.
Anabelle is still having the weekly visit by the community team, having her dressings changed, bloods taken and blood pressure monitored.
We last went up to Great Ormond Street Hospital on the 28th November. The doctors have produced a schedule to wean Anabelle off the Ciclosporin. This is good news as she is now getting really hairy. Her T cells have gone from 70 to 150 which we are really pleased about. Her neutrophil levels are also really good. As I understand it (I could be wrong and often am!!!) the T cells need to be about 300 to come out of semi isolation. Now that the Ciclosporin is being reduced her T cell levels should keep on rising. She has also now come off her blood pressure tablets and her current medication protocol runs like this
7.00 am Ciclosporin, Aciclovir, Posaconazole,
12.00 Penicillin, Aciclovir, Posaconazole, Magnesium
5.00 pm Ciclosporin, Aciclovir
10.00 pm Peniclilin, Aciclovir, Posaconazole, Magnesium
In addition to this she also has Septrim on Mondays and Tuesday twice a day with Calcium Folinate on a Friday.
She still refuses to take any form of fluid and has 480mm milk fed through the pump overnight via her peg and two lots of 200mm sterile water through out the day.


A nice picture she will thank us for when she is older!!
 

One of the few places we can take Anabelle. It could be worse.

The Semi isolation can still be pretty monotonous although the walks along Southsea Sea front can provide a welcome break. We are next back at Great Ormond Street on the 19th December and hopefully her T cells will be where we would like to see them. That would make a great Christmas present!!!

It is Day BMT Plus 101, and everything is going well.

Loving a cheeky swing!!

Life is continuing now we are back home. We both are working 3 days a week each to help bring the pennies in and also to cope with the semi isolation that goes with looking after Anabelle. We are still not able to take her to any public places such as shops, restaurants etc.. Our only solace is that the beach is just down the road where we can take her for walks. On the way to the beach the other day we noticed the park was empty so had a little blast on the slide and swings, which she absolutly enjoyed.  Great Ormond Street are pleased with her progress though and we now are only required to go to Day Clinic at Safari Ward once every 4 weeks. We still get the community team coming round on Mondays to change dressings and take bloods.
She has now stopped taking the MMF and the only immune suppressant she is now on is the Ciclosporin. Although her blood counts are progressing well her Immunity level is still very low. This is will in part be down to the Ciclosporin. Her current counts are 70 and we need them to be at 300. A side effect of the Ciclosporin is increased growth of body hair and we are now starting to see this on Anabelle . Most notably on her eyebrows, face, shoulders and back. As she is progressing well we have been told that at the next visit on the 28th November to Safari Ward they will look at taking her off the Ciclosporin. Hopefully before she turns into a little monkey!!!

The Great South Run came and went in Portsmouth last week. We had a few runners running for the MPS Society. I want to say a massive thank you to all these runners, supporters and all those who sponsored us. If you haven't sponsored but would like to do so, good news, you still can!! Just click the link on the right hand side.

Runners, supporters & Anabelle braving the cold

Some of the runners re - de hydrating after the race in the pub!!

It is now just over a week away from the Great South Run. Anyone who wants to sponsor us can do so by clicking on the tag to the right. Its for a great cause as the MPS society provide tremendous support for affected families and also fund research into treatments for these varying diseases.
The link is as follows

http://www.justgiving.com/LRShepherd

We also need to thank a special young lady from Western Australia who took it upon herself to arrange a sponsored trek. Mimi Cardy-Newman who is only 13 and the daughter of Jackie and Billy (friends from down under) organised the trek and arranged the sponsorship herself. She also wanted Anabelle to benefit directly from this. We are really touched by this act, so a big cheers mate to Mimi. Cant believe how grown up she looks, what an intelligent and beautiful girl, (these things must skip a generation!!!!) No seriously Jackie and Billy you must be so proud.

Got to say the weather looks lovely there. It probably wont be the same at the Great South Run!!!

Mimi at the finish line

Just completing the trek.

We’re back home in Portsmouth!!!!!

After 60 days of being in isolation on Fox ward, Anabelle was discharged from Great Ormond Street on 21^st September.

7.00 am medicines!!!!

Prior to leaving GOSH we had training on how to do her medicine as we have to provide medicines at 7.00am, 12.00, 3.00pm, 5.00 pm and at 10.00 pm.  Not just one medicine mind but a whole bundle of them!!!  In addition we have to make sure that Anabelle takes in at least 1000ml of fluid each day which is a mission as since having mucasitis she will not even looked at a bottle so all fluids are currently been given into a pump machine via her PEG  - an overnight milk feed of 480ml, then during the day two lots of  water bowless providing the remaining of the water. Our first week at home took alot of getting used to.

Even though we are home we are in semi-isolation, which means we are unable to take Anabelle anywhere public i.e. shops, restaurants, pubs; anywhere where there is people which pretty much means everywhere. Our only outlet is to take her for strolls down to the beach.  We also have to be very very careful with people coming to visit, which being social people as we are is taking time to adjust to.
However all that said, Anabelle is coming on leaps & bounds, her tantrums/irritability has now calmed down and she is smiley  again, enjoying being on her feet & even giggles daily, which is a sound we didn’t hear for a long time – this all makes being at home so much easier. She is becoming more like her old self each day.

On Mondays, our community team comes to the house to change Anabelle’s Hickman line dressing & take bloods.  We also still go to Day Clinic at Great Ormond Street which gives us a chance to pop back onto Fox Ward to say hello.

Our time on Fox Ward had some high & lows, starting with the highs; we made some lifelong friends & really had a laugh and when you’re in the situation we were in you really need that.  All of the nurses on Fox, also made our journey so much easier, by not just only doing your jobs caring for Anabelle but again having a laugh with us. Coffee being brought first thing in  the morning is a real bonus.  A big thank you as well to Adele (play specialist), student nurses & volunteers who everyday came in and played with Anabelle so we could go & have lunch together most days, again when you’re in isolation to go and get lunch together was important for our relationship & sanity!! The jobs all these people do is really unbelievable when you consider what they have to face on a daily basis, which brings us onto the lows. We were very unfortunate that it was one of the worst times they have ever had on Fox Ward as four of the little ones lost their battle far too early,  our hearts go out to the families of Fiz (11), Rosanne (9), James (20 months) & last but no mean least beautiful little Richard (10 months).

On more of a positive note, we would like to congratulate Theresa & Shane on the great news of your impending pregnancy – Thank you for your support via this blog on your experiences & please keep us up to date when Corey s brother arrives.  Next year we will definitely have to catch up.

Anabelle  has been on such a massive journey  and although we know she is not out of the woods just yet, her progress has been amazing and we look forward to seeing her progress each & everyday.

To say this has been an emotional rollercoaster is a massive understatement!!  However we must apologise for not updating the blog as regularly as we would have liked, however in the bubble world that we live in, called ‘fox ward’, everything changes so quickly that we just haven’t had the time.  Let me try & break down what’s happened in the last 3 weeks for you...........

Ok so a week after the last blog, Anabelle had an allergic reaction to  Ambusol, literally in 10 mins her whole face had swollen & went bright red, poor little might could only just open her eyes – she really did look like she had been in a car crash. The doctors gave her piriton which took some of the swelling away.  A couple of days later she was having high temperatures (over 38), as well as needing oxygen constantly, so the decision was made for her to undergo a CT scan on her lungs as this would determine if she had an infection or if it was graft verse host.  Due her not being well we were both very apprehensive with her going under general anaesthetic, however we pushed on as we needed to know what was wrong so the team could treat her.  That evening however, her high temperatures escalated to fevers (over 40), her body was starting to shut down, whereby her hands & feet went freezing and she would be shaking as if she was cold. Along with all this going on we also got the results from the CT scan which showed that Anabelle had a chest infection & there appeared to be a section of collapsed lung. The doctors & nurses were constantly in our room that night, but they did say that Anabelle was on every antibiotic & anti fungal medication possible so it was just now down to Anabelle. If she got worse, we would be in PICU (Paediatric intensive care unit).  48 hours later and there was still no improvement. She was spiking through the paracetamol which had held the temperature before. The decision was made to put Anabelle on steroids to try to combat this.  It worked – within 6 hr she had gone from 40.9 to too cold to read on the temperature dot thermometer!! 3 days later & her oxygen stats where back where they should be – no more oxygen required & temperature returned to normal.

However the good news in this time is that Anabelle's neutrophils had risen so she wasn’t neuatropinic & could help fight the infection.  Another chest x-ray has been done & her lungs are all as they were on the last blog – panic over.

Day 33 - 5^th September we got the great news that we had been waiting for....... Anabelle is 100% ENGRAFTED!!!  And we maybe home in 2-3Weeks!!!

Day 34 – Anabelle tried her first food in 45 days as she has been nil by mouth due to having a cord donor.  I really thought this would be a struggle but seems to be loving food, even though we can only feed her bland, bland, bland food – no dairy & only introduce one new food a day.

Day 35 – Anabelle has developed a mild hernia & is very unsettled.  She has cried a lot, which is unlike her & will only stop when we are standing up holding her. She also has high blood pressure – god it feels like we take one step forward, one step back!!

Day 37 – Anabelle has not stopped crying now for days & we just don’t know why  - however the only time she has been like this was due to the cyclosporine so  her levels will be checked again as this can cause bad headaches, she has also stopped codeine & is back on morphine (orally) .  Fingers crossed we get out happy baby girl back & some much needed sleep

Day 38 – Its official we are in ‘Yellows’, this is a step closer to going home – no more having to wear plastic aprons in the rooms & Anabelle can now go out of this goldfish bowl & into the corridor!! By Wednesday we hope she can outside in the pram, this is all dependant on her high blood pressure.

Today is day 39 & so far we haven’t found anything that is upsetting her stomach – hooray.  Her milk is given via a pump over night & this is now at 30ml an hour, which increases 5ml every night until she gets to 40 ml, she will still be on this when we go home. Her neutrophils are 2.07

Anabelle has now had 5 blood transfusions & 8 Platelet transfusions!! She has also stopped ERT as her body is now creating the enzyme that her body didn’t produce before.

Throughout this process her general development has been pretty good. She has gained new skills such as unscrewing caps and being able to place small objects into bottles. She is showing aptitude for meticulous play and music. The play therapist and Occupational Therapist visit regularly to help stimulate her mind and boost her development.