Sorry a couple weeks late I know., but here goes.

17th and 18th May 2012.
After arrival at the Kingfisher Ward on the Thursday morning a bed was assigned to Anabelle and the process of inserting the cannula began. Vikram, (the man with the light) was in intensive care and was therefore unable to attend. So it was left to Sinead who found a vein in Anabelle's arm and managed to insert it first time. The process was very quick with little stress to Anabelle. Her seventh infusion went extremely well and finished at around half four. Anabelle is getting more used to the infusions as the weeks go by.
Whilst the transfusion was happening we were were given the date for the bone marrow transplant. The date they are working to is 10th August. Relief and worry all at the same time!! Someone from the bone marrow team is going to come up to us in the following week to answer any questions we have. A meeting has also been arranged for the 20th June to talk specifics and to go through the technical stuff.
We have also been advised about her GAG levels. Her reading at her first appointment was 156, an unaffected persons generally are around 20. By the 2nd May Anabelle's GAGs have reduced to around 70. This is good news. Hopefully they should have reduced even more.
As it was my birthday we went to the Italian across the road for a meal before retiring to the patients hotel for the night.
The next day began with Physiotherapy at 9.00 am and Anabelle was grumpy through this. Cardiology then followed. This consisted of a ECG which she slept through then a Echo she was awake but I rocked her to sleep. We then saw the consultant. To sunmerise she has noraml heart sounds with no murmurs and no added sounds. She has very slight leakage of the Mitrial valve and even slighter leakage on the aortic Valve. The leakage is so slight that it couldn't be picked up by a stethoscope. MPS babies generally suffer from heart problems due to the Mucopolysaccharidosis. Basicallly Anabelle is asymptomatic from a cardiac point of view and only has mild cardiac involvement from her MPS. Good news indeed.

23rd May 2012
Infusion number eight was very smooth and the process very efficient. Anabelle is getting more and more active as the weeks go by. As suggested by the Physiotheripist we bought Anabelle some new shoes. Now all she wants to do is be on her feet. She has also learnt to point and to pull herself onto her knees. She is such a fun smiley child who always seems to be in a good mood, aprt from in hospital that is. It would be nice for the nurses to see her cheerful side!!. In the afternoon Victoria brough Nicky from the Bone Marrow Team down to answer any inital questions we may have. I think we kept them for about two hours. The 10th August is the date we will be working to although this is not set in stone as other factors may affect this. The cord cells are sitting in a jar in a fridge somewhere waiting to be united with Anabelle. Some of the things that we had been told at this time are that her treatment will start with Chemotherapy. This will be classed as day Minus 8 which will then count down to Day Zero which is when the transplant will take place. Only one parent will be able to stay on the ward over night with Anabelle and freash clean clothes are required every morning and night. No wooden toys or soft toys are allowed, generally only things that can be wiped down with no intricate parts where mess and germs can harvest. We will need to work out what to do with regard to the accomodation issue as this will need both of us with Anabelle.

Anabelle and her mummy

The next date at Great Ormond Street is 30th May 2012. the only thing that is due that day is her 9th infusion.

Getting used to this now!!!

Hi. Not a huge amount to report this week. Anabelle had her 6th infusion yesterday. It took 4 attempts to get the cannula in. In the end they had to get the man with the light back who managed to get it into her left foot. Bless her with her bruised little hands and feet. Although she kicked off throughout the insertion she soon settled down afterwards. I think I have mentioned it before that due to the MPS they have tough skin which makes it difficult to insert a cannula.
There has been no real feed back concerning a meeting with the BMT Team, but we have been told that the transplant will happen in August and is currently being scheduled in. The Enzyme Replacement infusions will continue up until this date. The infusion yesterday went fairly swiftly and Anabelle even slept through part of it. She is becoming more active week by week and on the way home she amuses all the commuters with her giggles and waves.
As we have an appointment with the Cardiology Clinic on Friday 18th May the infusion that week has been switched to the Thursday. This means we only need to spend Thursday night up there (my birthday by the way!!!).
We have also set up a JustGiving page and Team for the Great South Run. The link is   http://www.justgiving.com/LRShepherd and is also pasted on the right. If anyone would like to donate to the MPS Society or join the team it can be done through there.

Look At Me!!

Just a quick one. Anabelle took her first steps with the baby walker today. Well Proud!!!! The Enzyme Replacement Therapy is definately working.

View from Waterloo Bridge on way to Great Ormond Street. (Testing out my new poster app!!)

Another busy time at Great Ormond Street, but plenty of positives to be taken this week.
We stayed over on Tuesday night for an appointment with the Ophthalmologist (eyes) first thing Wednesday morning. The first appointment confirmed that she pretty much had 20/20 vision although they found it difficult to keep her attention on the task in hand. Well she is fourteen months old after all!!! They then put some eye drops in to dilate the pupils to prepare her for the second appointment. Fortunately she fell asleep which was a major bonus as the various test were carried out by raising one eyelid open and then the other. Apart from stirring a couple of times she didn't even notice. What could have taken ages to complete with lots of hysterics was completed easily within five minutes.

The potential effects of MPS on the eyes are clouding vision, intolerance of bright light, glaucoma and damage to the optic disc where the mucpolysaccharides build up around the nerves. The results from these tests showed that Anabelle does have some clouding but this is improving through the ERT infusions. She also hates bright lights but again over the last few weeks she is more tolerant of this. The even better news though is that Anabelle has no signs of glaucoma or damage to the optic discs.

Future Musician!!!

After our stint in the Ophthalmology Clinic we went back to the Kingfisher Ward for Anabelle's fifth infusion. Our specialist nurse, Victoria told us that the results of the CT Scan had come in and she confirmed some more good news...... There is no evidence of Craniosynostosis (I've tried to limit the big words but there are just too many!!). This basically means that there is no issue with how our little girls head has fused. there was also no fluid on the brain, these two items are symptoms of MPS and we had been nervously awaiting these results so it was amazing to hear of things that Anabelle doesn't have as opposed to things that she does.

We were also given the even better news that the BMT Team had been in contact and that they have a 10/10 cord match for Anabelle!!!!!!!!!!!!! July as been touted as a possibility for the transplant but I have a feeling it will be in August. This is great news although daunting. Our only niggle is that we will be in London whilst the Olympics are on. Me personally I'm really not fussed about the Olympics. If it was the world Cup however that would be a different story.

We will be setting up a JustGiving site for the sponsorship for the Great South Run for the MPS Society. Kate and Paul have also entered the Great South Run.(Well Done & Thanks)
We also need to mention the good people of N.A.T.S. where Anabelle's Aunt Sally works. (no not the one from Wurzel Gummidge for those old enough to remember!!) They have been doing some fund raising for the MPS Society and have raised lots of money already and other events planned. Don't forget it is MPS Awareness Day on 15th May 2012, where everyone can do their bit. 


N.A.T.S. They do more than keeping our skies safe!!

We are very fortunate to have such good family and friends who have been there for us and would just like to say a massive THANK YOU.  We are also overwhelmed with the support from people who don't know us personally but have taken the time to read our little blog and even comment.