Oliver and Samuel - the past year...

 Wow!! What a difference!!

From the licence being granted in Jan 2007, and knowing that in April the ERT would be funded, 2007 was to be THE YEAR!!

  Oliver and Samuel had port a caths inserted to receive their weekly infusions and they began their treatment at Great Ormond Street. We had eight infusions there and met with two other little boys who were on the Thursday list. All boys quickly bonded and Kingfisher Ward became a noisy playground once the infusions had fnished each week-football being a favourite!! Our play specialist, Rosa, was an absolute star and the boys love seeing her.

After 8 weeks we transferred to our local hospital for another 8 weeks and then to home with a nurse visiting each week from the start of the Summer hols. At home the boys were given portable drips so they could move freely around the house-or should I say from the X-Box to the telly??!!

By now their tummies were getting smaller and their appetites increasing. Behaviour??Hmm, well, that goes with the excess energy they had found-many parents have reported behaviour maybe not so good, but hey, I try to remind myself we are lucky the boys are well enough to be naughty!!

At assessments done in September, Samuel showed no signs of enlarged liver or spleen-amazing-and a few tears of relief were shed. His heart valves had got more leaky so he needs to start medication, but we were aware that the valves wouldn't be helped too much by the enzyme. He had grown alot too, Oliver had grown just slightly, but we have to remember that Oliver is two years older than Samuel, so has had build up of MPS for two extra years, and at least he has still grown.

In November, Oliver had an op to stretch his achilles tendon and after much pain and a very cute mini zimmer frame, he can now walk with his heel down!! (This problem is down to his neo natal brain haemorrhage-a mild cerebral palsy). He still wears his splint but it is so much nicer for him to actually be able to stand flat on the floor.

Samuel had his bits and pieces operated on in December-umbilical hernia, hydrocele, and carpal tunnel release on both hands. He woke up the following morning and holding up his bandaged right hand whispered -"this is the worst one". I felt really guilty as I thought he would be a bit of a drama queen over the whole thing-how wrong I was !!

We have also learned in the last week that Samuel's hearing is now better than it was 18 months ago!! That was fantastic news!!

I have now learned how to do the infusions myself so we are completely independent at home now, which is great-I feel like a real nurse!! I go into the boys' schools on a Tuesday at 2pm and put local anaesthetic cream over their port a caths. Then I pick them up at 3pm and insert the needle into their ports. Then the medicine gets made up and attached to the pump which then gets attached to their lines and off we go, gradually increasing the dose for the first hour, then, when the infusion is finished, a flush and heparin administered to stop blood clotting in their internal line during the next week. The whole process from cream to finish takes over 5 hours but at least we are at home-getting to Great Ormond Street used to mean leaving home at about 9.15am and getting home at about 7.30pm!! And I wasn't given any dinner up there!!

It is SO nice to get some good results-which really give us hope for the future. People have commented on how Oliver and Samuel's faces have changed, as their features are gradually softening-something which is hard for a parent to notice on a day to day basis.

Their hair is definitely softer, and so is their skin. Their cheeks feel so soft. Oliver's nose is not so taut as the build up reduces, and the same goes for the tips of his fingers-they used to feel like bits of wood they were so 'full' with the mucopolysaccharides.

The physio at their assessment on 12th March 2008 commented on how they are becoming more supple and the special computerised walking mat she has, showed their walking had improved-their gait, velocity etc.

Lung function tests showed their breathing has improved-which we knew as their snoring has decreased!! On occasion I have had to go right up to both boys while they were sleeping and get within about 6 inches of their face to check they were still breathing!! It was a bit of a shock to start off with, as I used to be able to hear them as I walked past their rooms-sometimes even as I lay in bed!! Elaprase, the drug they are infused with each week, has changed our lives. No, it doesn't cure the disease, and there will be problems, but hey-this gives them a chance to grow up. A fact I will be eternally grateful for.

We wait to see what happens next-I pray Oliver will start to grow quicker and his conductive deafness will improve. He will always need his hearing aids as he has sensori-neural deafness, and the enzyme will unfortunately not mend nerve damage.

What I want is for my two little men to grow up happy and confident and to not feel different. Oliver remains in his special needs unit at his junior school, as he has speech and language difficulties (but he couldn't hear properly until he was nearly 4, so it isn't surprising) and also he needs help with his leg, but there are some really disabled children there too, so I think he realises he is quite lucky in some aspects. He is an absolute whizz on the X Box and gets through levels without actually being able to read the instructions...

Samuel plays football after school and was overjoyed to have scored a goal yesterday. He runs as fast as he can and is very much into getting exercise as well as looking like a cool dude (his words, not mine!!)

We are blessed with two very happy little boys who can drive us nuts like any child but are very loving and caring, as well as being fiercely protective about each other.

Bring on the rest of the year-onwards and upwards...!!