Purpose

On 6th June 2014 Uncle Danny, Gary (a pal from work) and I will be completing a motorcycling journey totaling 1800 miles!! 1500 of these over two days and this will be to raise money for the GEM appeal.

The GEM appeal fund clinical research into rare genetic diseases such as Hunter syndrome and my Godson Matty has been part of an early phase clinical trial - enzyme replacement therapy, for which he has had to endure a number operations e.g. to fit a spinal port and countless weekly trips from his home in the Northwest of England to Birmingham Children's hospital for administration of his medicine.

Around the time of Matty's Third Birthday he was diagnosed with Hunter Syndrome. This is a serious genetic disorder which interferes with the body's ability to break down and recycle specific mucopolysaccharides. These build up in cells throughout the body due to a deficiency or absence of a specific enzyme (I2S). The buildup interferes with the way certain cells and organs within Matty's body function.

Within the last week (first week of May), Gary has heard about our staggering mission and himself gone to the extreme of purchasing a motorcycle especially for this trip!!  Gary is no ordinary Dad he has two boys 9 and 11, works full time in our busy factory and sadly lost his wife recently. To compound things Gary's youngest is currently undergoing tests for a Genetic condition which may take up to 6 months to diagnose. 

Gary is currently in "training" to rack up 500miles prior to the 1st service otherwise is warranty is invalidated by our mammouth challenge.

We all have our reasons to make sure our efforts go to a good cause and I would like my efforts to go some way to supporting the GEM appeal for the support they have offered my Godson Matty and our family over the last 11 years. 

Although sounding straightforward our trip will be far from this. We will be on the road for approximately 15 hours each day focussing on the road, the conditions, scanning the road surface for obstacles and we'll be making progress at a rate of ~120 miles before each refuelling stop- so  add 3 hrs refuelling to this time on  the road (this will obviously become strategic in the highlands!). We will battle fatigue, our state of mind, our common sense,  and those age related problems our body chooses to throw up - all to get through 750 miles on day 1 (Swindon-Lands End- Carlisle) and if that wasn't enough the same distance on Day 2 (Carlisle-John O Groats-Edinburgh).  We pretty much reckon that is pushing it somewhat so Day 3 will be a gentle "potter" back to Home and preserve whatever we have left!!

So the mission that we have chosen to complete in the hope of raising some extra money for GEM is an 1800 mile motorcycle trip on my Triumph Thruxton (the classic looking thing in the pictures!). I will be supported on this trip by my Uncle Dan who will also be riding a Triumph although with somewhat more technology and considerably more power than I have to hand, he'll do this on his modern day racing Daytona (also pictured). Over the first 48hrs we will complete a staggering 1500 miles. Gary is the sensible one and has had time to choose his ride - a fuel efficient modern reliable Honda! Although he's complaining about the handling already - THIS IS NO MEAN FEAT, Please give what you can - THANKS! 

About "The GEM appeal" supporting my Godson Matty Burian.

The GEM Appeal was founded in 1994 by Karen Johnson after she was approached by Consultant Paediatrician Dr Ed Wraith with a request for money to be raised to hopefully extend the Willink Unit at the Royal Manchester Childrens Hospital and to fund research into the disorder that her two sons Simon and Mike were dying from. At that time there was no treatment and no hope for children diagnosed with these rare genetic disorders and inborn errors of metabolism and very little research was being done into these diseases. 

Along with Ed and a few other likeminded parents and health professionals, the fundraising began and The GEM Appeal was born.

The fundraising began in earnest and by 1999 the first £250,000.000 had been raised from numerous varied events mainly in the North West of England and the building began. Before long, the research was under way in a laboratory named after Karens sons and progress was rapid and successful.

Sadly, Karens two sons both died around this time aged 12 years but trials of treatment of enzyme replacement therapy became available to others with promising results.

As the years went by, other fundraising friends came on board with Karen and the GEM Appeal trustees and committee in Rochdale became the strength and drive for the amazing success that the GEM Appeal has become. Patrons Denise Welch and Tim Healy became staunch supporters and have contributed enormously to the success by endorsing the work that is done and by gathering support from other stars and celebrities from the world of TV . As the charity has become more well known, fundraising has spread across the country and events now are regularly organised in various locations far and wide such as County Durham, North Wales, Hampshire to name but a few.

By 2012, the Charity was proud and delighted to be able to announce that over £2 Million had been raised. Due to the research done and treatment becoming available, children and young people, who these days are diagnosed with these terrible life limiting disorders, now have hope and treatment available. In some cases this treatment is slowing down the progress of the illness but is currently by no means a cure. 

Amazingly, £2 million pounds in the world of genetic research is only the start and so much more research must still be done to hopefully one day eradicate these terrible diseases that devastate so many lives. Children and young people continue to die on a daily basis just like Karens sons did and to prevent this, the fundraising must continue.

The fundraising by The GEM Appeal and its supporters is totally voluntary with no Government or lottery funding and every penny raised goes directly to the research done at the Willink unit.