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FOP Friends Blog

Thank you to everyone who attended or was involved in making the 2014 UK FOP Family Gathering a great success.

Twelve years after the first ever UK event, FOP Action held a conference and family gathering for families from across the country and the globe. The event was organised by FOP Friends, the UK charity for FOP, headed by Chris Bedford-Gay. Assisted by a grant from Genetic Disorders UK, the aim of the event was to gather together families of FOP sufferers in a relaxed environment allowing them to meet others living with the same circumstances. The reality turned out to be much more.

After a year of planning, the event started in the early hours of Friday morning with Drs. Kaplan, Pignolo and Shore flying into Manchester, England. They ran clinical appointments all day long, meeting many patients, some who they had met previously, others new – all overwhelmed at meeting their ‘heroes’. The waiting area overlooked a sunny, yes sunny, Manchester Airport and the atmosphere was happy and hopeful. Behind the scenes, the FOP Friends team were busy getting ready for the evening’s meet and greet reception as well as preparing for the following day’s conference sessions. At 7pm, families gathered for the reception with attendees coming from, amongst other places, the UK, Sweden, Greece, Malta, the Netherlands and even South Africa and Australia. The hour-long reception ended when the bar closed three hours later!

Saturday dawned and the families gathered. A crèche was provided for the young children which proved to be a real hit! Staffed by qualified teachers, the children had a great time playing and meeting each other and particularly enjoyed the trips to the hotel penthouse to see the planes taking off! The crèche enabled the parents to enjoy the conference, taking the time to listen to the presentations and meet other parents to have great conversations. The presentations were given by doctors and researchers from the Universities of Oxford, England, and Pennsylvania, USA as well as IFOPA and Clementia Pharmaceuticals. Delegates had the opportunity to ask questions of all the speakers in the afternoon which was extremely well received. The families had different levels of understanding of FOP, depending on when they received a diagnosis, and the doctors and researchers were able to answer all questions. News of Clementia’s clinical trial provided real hope for the sufferers and their families, many hearing about this for the first time. The day was topped off by a dinner and quiz, which ended with an unexpected karaoke round, creating some fun competition – Dr Christiaan Scott from South Africa was the unexpected star of the night! There was no rest for Drs Kaplan, Pignolo and Shore who ran clinical appointments all day Sunday.

The feedback from the three day event was overwhelming with the most frequent comment being ‘When is the next one?’!  Lasting friendships were made and many delegates received support they didn’t know existed. Tears were shed and hugs were exchanged: the FOP community came together in spectacular style!

 

Chris Bedford-Gay
Sunday, 8 June, 2014 - 23:48

In the middle of 2013 Chris Bedford-Gay (founder) and John Lever (trustee) of FOP Friends of Oliver successfully applied to a medical trust for £65,000 to fund the employment of a Senior Medical Chemist (and equipment) at the University of Oxford. 

The chemist will have the challenge of taking the Oxford FOP Research Team's drug designs and turning them into optimised drug candidates.  The trust awarded £35,000 towards the post, conditional on the commitment of an additional £30,000 from another party.  We are thrilled to announce the second £30,000 was secured early in 2014 and on the 5th March 2014 a total of £65,000 was transferred from Friends of Oliver to the FOP Research fund at Oxford.  The search for the chemist can now begin.

This photo shows the lab where our chemist will spend their day, building drugs in the hope of finding a treatment and ultimately a cure for FOP.  Excitingly, this chemist will join the exisiting excellent FOP research team at Oxford who still need our £125,000 to continue with their groundbreaking work. We look forward to providing you with full details in the near future, and to let you know when a chemist has been appointed.

Chris Bedford-Gay
Thursday, 6 March, 2014 - 23:34

All the money raised by Friends of Oliver goes directly to supporting the research into finding a treatment and a cure for Fibrodysplasia Ossificans Progressiva.

But where exactly does your money go and how is it helping the fight against one of the rarest and cruellest genetic diseases known to medicine?  We asked Dr. Alex Bullock from the University of Oxford some questions.

So Dr. Bullock, who makes up the research team at Oxford University?

The University of Oxford FOP Research Team is led by Emeritus Professor Jim Triffitt and myself and includes two postdoctoral scientists, Dr Eleanor Williams and Dr Georgina Kerr. The team operates from the SGC laboratory in the Old Road Campus Research Building, Headington, Oxford.

How much does it cost to keep the Team running per annum?

We need £120, 000 every year to fund the work of the two post-doctoral scientists.  The money raised comes from a variety of sources such as donations from Friends of Oliver and the families of FOP Action and FOP France.  We also receive generous donations from small businesses such as Roemex.  Donations from Friends of Oliver and FOP Action account for around 70% of the total needed.

What role did the Oxford Research Team play in the discovery gene that causes FOP?

Oxford scientists were instrumental in leading the race to find the rogue gene responsible for FOP.  Genetic linkage of rare families with inherited cases of FOP allowed the Oxford team to map the precise location of the FOP gene on the human chromosomes.  Oxford mapped the FOP gene to chromosome 2 and specifically to a small region between bands 23-24.  To explain: it is like looking for a single house in the UK.  

If you compare the chromosomes to a street map of the UK, this is equivalent to identifying the city and then the street.  

The next challenge was to ‘knock on each door’ to find the right house.  This was done in both the US and Oxford, if you like starting from opposite ends of the street!
  The ‘right house’ was with the half analysed by Dr Kaplan’s team in at the University of Pennsylvania.  Overall, it was a massive international effort, as the DNA had to be collected worldwide.  The linkage mapping was particularly challenging because there were so few families with which to build good statistics.  The location was twice miscalculated in the 2000 by international FOP labs.  It was only after the mapping was undertaken by Oxford, that the gene ACVR1 was finally discovered in 2006.  The eventual correct assignment was made possible by the expertise of Professor Matt Brown, a geneticist in Oxford who was collaborating with Jim Triffitt. 

What research is the Team conducting at the moment?

Mutations in the FOP gene ACVR1 give rise to a faulty protein which triggers extraskeletal bone formation.  We are studying the three dimensional structure of this faulty protein to learn precisely how it works, how it becomes activated and how we can design drug molecules to stop it.  We have identified how the first prototype drug molecules bind and are now working with other international groups to improve these prototypes to make them more effective.

Does research into a cure for FOP have any benefits for the treatment and cure of other medical conditions?

The research being carried out into FOP has many interesting implications for other, more common, medical conditions that affect much larger groups of people.  Researchers are looking how treatments and cures for FOP sufferers may also have positive benefits for people with childhood brain cancer, osteoporosis, chronic anaemia, stem cell therapy, atherosclerosis and vascular calcification (clogging of arteries causing heart disease) and heterotopic ossification (following military wounds or hip surgery).

What are the hopes for the research team both in the short and the longer term?

In the near future, we hope to define how the ideal drug for FOP might look e.g. what chemical groups are required.  In the longer term, we hope our learnings can be used by the drug industry to manufacture a drug candidate for clinical trials.  We must also further our understanding of the processes driving FOP to better design these drug trials and future treatment regimes.  

For example, are there alternative drug targets in FOP?  Should a combination of drugs be used to treat FOP?  How often and when should these drugs be taken?  At present it would appear that any drug would need to be taken for life.

Finally, what would happen to the FOP research team if they didn't receive donations from groups such as Friends of Oliver?

Without the generous donations we receive from Friends of Oliver and other groups, the dedicated FOP research of the Oxford group would be lost.  This would then have further repercussions as other funding sources would be less secure and perhaps lead to a more limited budget.

In turn, this would severely hinder the excellent research being carried out by the Team at Oxford and thus delay the race to find a treatment and a cure for FOP, before it is too late for children like Oliver and all his FOP friends.

Chris Bedford-Gay
Monday, 20 January, 2014 - 21:12

Doing Your Christmas Shopping?  Donate when shopping online and it costs you nothing.  Shoppers have raised over £300 for FOP Friends of Oliver so far!

Friends of Oliver is registered with easyfundraising.org.uk who help us to raise money when our supporters (you) shop online.  You can shop with over 2,000 well known stores and each will donate up to 15% of what you spend.  It does not cost you anything, you pay the same price as everyone else.  The only difference is your purchase also raises money for FOP Friends of Oliver.

easyfundraising.org.uk

Chris Bedford-Gay
Thursday, 10 October, 2013 - 22:58

 

There are lots of fantastic prizes to be won including a signed Manchester United shirt, a day's cricket at LCCC, pamper treatment at the Hale Country Club... and much, much more!!!  There's also 'wine for a line' to get the evening going!!  All proceeds go towards finding a treatment and cure for FOP. 

NOW SOLD OUT!

 

 

 

Chris Bedford-Gay
Wednesday, 17 July, 2013 - 18:06

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