Perhaps not a big surprise, but the OIFE Executive Committee has in collaboration with the local organizers decided to cancel the OIFE Youth Event 2020 that was supposed to take place end of August, because of the Corona virus situation. Our plan is to do the OIFE Youth Event in Poland i 2021 and in Belgium in 2022. We will keep you updated!
Each year all delegates are invited to the Annual General Meeting (AGM) of OIFE, which is normally the only time a year the whole Board comes together. At the AGM the Executive Committee (EC) accounts for OIFE’s activities in the past year, future plans and policies are discussed and members exchange news and ideas.
Our original plan for OIFE AGM 2020 was to meet in sunny Valencia from May 8th to May 10th. But the Corona virus put a stop to that, as it has done to so many other meetings and conferences. But we would not let it stop us from having our annual meeting. The EC therefore organized a two part meeting on Zoom on May 9th instead. Part one included approval of annual reports, budget and elections. And part two included a webinar on new treatments in OI.
People from all continents took part
Anyone from our member organizations can attend the OIFE AGM. In addition, OIFE volunteers, volunteers from other organizations, professionals and other people with an interest in OIFE can be invited.
62 participants attended part one of AGM 2020. 66 people attended the webinar (part 2) on new treatments. What was unique about this year’s event, was the record number of member organizations that were able to attend. We had all the national organizations from Europe except one attending. In addition to European organization and three supporting members, we had representatives from organizations and invited guests from all different continents including Peru, USA, Chile, Ghana, Nigeria, India and Australia. This year we had also invited people from Mereo Biopharma, our Medical Advisory Board as well as contributors from other rare bone communities, including XLH and HPP. Between 70-75 people attended the event in total
Bruno elected as 2nd Vice President
AGM part one included approval of annual reports, budget and elections. All proposals from OIFE EC were approved. We welcome Bruno van Dijck to the EC team, as the new 2nd Vice President of OIFE.
In addition to reports and elections, OIFE president and OIFE’s Communication Manager gave an update on current happenings and news and a summary from the national reports. The last part of OIFE AGM part one was dedicated to a presentation of a new collaboration on a planned global survey between OIFE, the OI Foundation and the company Mereo Biopharma.
Survey on the impact of OI
OIFE president presented the project, which is an initiative from Mereo Biopharma which is formally supported by OIFE and the OI Foundation. The plan is to do a global survey on the impact on OI to:
understand the patient journey better in X & Y country
to understand better what OI means for children and adults beyond fractures
what the economic impact of OI is on people with OI and their families (direct and indirect costs)
how we can measure quality of life in OI in the best possible way
what kind of data we should collect and how
Three representatives from Mereo Biopharma were present to answer questions and comments from the participants. Mereo informed us that a steering committee will be established with members from OIFE, OIF and Mereo Biopharma in addition to 5 medical professionals from US and Europe, including both pediatricians and adult doctors.
Webinar on new treatments in OI
Part two took place on Zoom from 15.00 to 17.00 and included talks about:
– News on BOOSTB4 (Cecilia Götherström)
– News on setrusumab project (Mereo Biopharma)
– Access to treatments for rare bone diseases (Oliver Gardiner & Paul Connor)
The event has received a lot of positive feedback in retrospect and many people were positively surprised how well an event on Zoom could work. 37 people answered the evaluation form and 95% of the participants gave a score of “Very good” or “Excellent”, which means that OIFE AGM 2020 can be considered a success.
Rare Revolution Magazinegives a voice – and a platform – to patients, industry professionals, clinical and healthcare providers and the patient groups and charities that support over 8000 different rare conditions. Created by BMA award-winning, featured writers and sisters; Nicola Miller and Rebecca Stewart, who identified a huge gap in the industry, Rare Revolution is a space where all of these rare disease parties can come together in one place to all become better informed and cross-promoted, while benefiting from shared experiences and best practice. The slogan on Rare Revolution Magazine is “It’s time to turn the tide and make our collective voices heard.”
Which better opportunity than Wishbone Day to start a collaboration between OIFE and Rare Revolution Magazine? This is what we thought, and we have been lucky to be heard and welcomed by the Rare Revolution Magazine Team who offered OIFE the chance to take over the social media channels of the magazine for one whole day and use their tools to reach out for a greater, and for sure interested audience.
What is Wishbone Day?
Wishbone day is the collective voice of people with OI, talking about OI and the things that matter most to them, in their own way. Wishbone Day draws awareness to the contributions of people with OI, the challenges of living with OI, and the important ways that people can contribute to our journey to live full and happy lives.
In this perspective, OIFE took this opportunity as a perfect fit in the Wishbone Day 2020 communication strategy that aims to collect the wishes of the OI community through our #wish4OI campaign, adding some kind of educational, explanatory activities aimed to raise awareness on OI, on OIFE and on some of the topics that matter the most to the OI community.
For the whole day tomorrow 5th May, several OIFE branded contents and post will be shared by Rare Revolution Magazine according to an editorial plan suggested by OIFE itself. We strongly encourage you to follow Rare Revolution Magazine social media and share what you find interesting for yourself and your OI community
May 6th is Wishbone Day the International Day of OI celebrated all over the world by the OI communities to improve and boost the awareness around OI.
We have seen many incredible ways to celebrate this day, some created by individuals and some guided by National organizations’ initiatives.
As the international umbrella for OI Organizations OIFE has always been watching, enjoying and promoting the many different and creative inputs given all over the world to celebrate this unique day.
This year OIFE decided to get even more involved!
As usual, we have launched a Fb picture Frame we invite you all to use and share. It is easy but really relevant as it will show the yellow ribbon of the Day to all your friends and contacts on social media and help improve the visibility of the day of the OI community.
The first big brand new news is that on 5th May OIFE will take over the social media channels of Rare Revolution Magazine a digital magazine giving a voice to those affected by Rare conditions and the charities that represent and support them. So stay tuned as we will release some brand new contents about OI, OIFE and Wishbone Day on that date.
In addition to this, we have launched an interactive social media-based campaign called #Wish4OI.
The aim is to collect the wishes of the OI community to get to know what they wish there could be more knowledge and awareness about in the future about OI.
It could be anything from treatments to policy work, from very basic to complicated issue you want to address or would like the OIFE to work more on.
How to do get involved?
Very simple…take a photo of yourself with a paper and write on it your #Wish4OI.
You can either share it online tagging or mentioning OIFE and using the hashtag #wish4OI or you can send it to firstname.lastname@example.org.
Our idea is to collect how many wishes as we can and create a video or a poster with them all.
What are you waiting for? Let us know your #wish4OI!!
Each year all delegates are invited to the Annual General Meeting (AGM). Anyone from our member organizations can attend the AGM. In addition OIFE volunteers, volunteers from other organizations, professionals and other people with an interest in OIFE can be invited. At the AGM the EC accounts for OIFE’s activities in the past year, future plans and policies are discussed and members exchange news and ideas.
AGM ON ZOOM
This year our AGM will be different. Our meeting that was planned to take place in Valencia, will take place on the video platform Zoom in two different parts:
OIFE AGM PART 1 (BUSINESS PART) Time: Saturday 9th of May from 09.45 – 12.15 CET
The title and graphic identity of the Latvia meeting Jean Moitry and I attended in Riga is a reference to the Chinese tale of the three monkeys. One of them doesn’t see, the second doesn’t hear and the third hides his mouth. This is an optimistic message because the meaning of the parable is that if you choose not to listen to evil, not to look at it, nor to repeat negative ideas to others, you are a wise-man/wise-woman.
More literally, when one is a person with OI, one may have in addition to orthopedic problems, issues with one’s sight, or one’s hearing, or with jaw deformities or one’s dental system that may cause pain, teeth fragility all the way to affecting one’s self-esteem especially during teenage years (smile). Behind this humorous and welcoming entry, the rich program Ingunn and OIFE had devised contained arduous scientific material not always easy to apprehend for a neophyte like me, when it comes to all the background knowledge about our pathology. Let’s try to summarize and indicate a few tracks.
Introductory words the organizers were followed by a general presentation of OI by Dr. Antonella Forlino, explaining different ways to classify OI, the Sillence classification and the classification with the 19 different genetic types. Research pertaining to the mutation classification could lead to more specific treatments and follow up in the future.
Day one was first devoted to eye pathologies which can occur due to thinner sclera and cornea and then to dental treatments and jaw function problems pertaining to OI patients suffering in these areas. We learnt that serious vision problems such as glaucoma or cataract seem to affect less than 5% of OI people of any type, and are therefore reported as less prevalent in OI patients than in the average population. Dr Janna Waltimo Siren and Dr. Suken Shah then approached skull base deformities and basilar-invagination which may appear in as much as 25% of the OI population (30% of types III and IV). Concerning dentinogenesis imperfecta (=DI), according to an Eastman Institute study, among 50 patients of all types of pathologies suffering from DI 28% had this condition due to OI. DI mostly affects OI types III and IV, much less type I. In Northern Europe, several studies have been devoted to its various forms notably within the TAKO and the Eastman institutes networks. Dr. Agnes Bloch-Zupan, a pediatric dentist and biologist at the IGBMC Centre of reference on mouth and dental disorders in Strasbourg (France) then spoke of her research within the European program Interreg V RARENET on rare dental diseases, among which OI had been included. That same day we learnt from Dr. Manuel Joaquin de Nova Garcia that the effects of anti-resorptive treatments such as bisphosphonates on the evolution of dentinogenesis have been studied: they seem to be causing dental malocclusions in some patients. Studies about the consequences of such treatments in relation to hearing loss apparently have not yet been made.
On the second day, we were given an overview of the various forms of hearing deficiencies in OI. It is commonly said that about 50% of us will suffer from hearing loss. Within the framework of a Danish study, 60% of examined ears in O.I. patients were affected. A lot was learned about the prevalence of deafness due to transmission failure (middle ear) in comparison with hearing impairment caused by perception defects (i.e. inner ear, cochlea affections). Optimal ways of detecting, treating and following up the various types of deafness in OI, were detailed by audiologist Freya Swinnen.
I noted that there appear to be less studies produced nowadays about “deafness and OI” than in the 1970 to 2000 period. A few existing recent studies however reveal that the most severe hearing losses associated with OI originate not specifically in middle ear — of which the small bones deformations and possible surgical treatments have been explored extensively, as Dr. Pedersen reminded us —, but rather in the inner-ear or in a combination of both conduction and perception problems (i.e. middle and inner-ear deterioration) adding up with age. As far as we know, “fortunately”, very severe malfunctions and this combination factor occur in less than 5% of OI patients.
The causes of mutations and conditions inducing malfunction in the cochlea in some OI patients whilst not in others have not yet been identified. What we know is that, like the middle-ear bones, a fragile cochlea is likely to suffer from micro-fractures and chaotic bone remodeling. As the temporal bone is also prone to demineralization with age, perceptory cells finding themselves in a hostile environment, begin to atrophy. In the case of severe hearing loss — affecting patient’s capacity to understand spoken conversations even while wearing external hearing aids —, one may have recourse to cochlear implant (=CI) provided one’s cochlea hasn’t been obstructed. Implantation consists in the surgical insertion of a silicone electrode carrier in the inner-ear, coupled with a microprocessor implanted under the skin of the head (see Illustration).
As surgeon Gunta Sumeraga, Freya and myself emphasized, it is absolutely indispensable to be examined by a surgeon who already knows OI or similar bone pathologies before making the decision to get an implant. With a cochlea wall as thin as ours, risks of cochlear perforation and meningitis are greater, as well as risks that the facial nerve gets stimulated (in addition to the auditory nerve aimed at) when the electrical processor of the implant is turned on, causing facial paralysis or jerks.
We still have some way to go before a solid body of work exists to improve the quality of life of OI people affected by deafness. Although this may be considered a secondary disability because it appears, in its stronger expression, after the age of 40, deafness should be treated as early as possible, when it is still mild. Regular (possibly annual) audiograms should be performed starting in early childhood and throughout adulthood. It is important also to be prescribed and to wear hearing aids as soon as medium range deafness is diagnosed, as well as to get a professional psychological follow up: these are key factors for avoiding the serious psycho-social damage untreated deafness may cause.
Each section of the Riga program began with patient testimonies that had been carefully and sensitively selected by members of the OIFE board, who read them aloud to us. The conference’s audience of about 80 people consisted primarily of medical professionals and secondly of patients and OI family members of all ages, converging from all over Europe and Russia. Such diversity among the attendees surprised Canadian Pr. Jean-Marc Retrouvey who made a rather arduous presentation on 3D modelizations of cranio-facial characteristics in OI. He expected to have a mostly professional audience! However this is very much the specificity of OIFE: mixing audiences to allow active patients to document themselves whilst providing an opportunity for professionals to meet the people who make their practice meaningful, and not just remain among themselves.
The Riga meetings were successful at that, taking place in a beautiful setting and in a very friendly atmosphere. Thumbs up and a BIG thank you to Ingunn, Taco, Ute, Anna, Dace and her husband for their warm welcome. Many thanks also to Zita for her skillful simultaneous transcription that allowed deaf people such as me to follow the conferences.
OIFE youth event 2019 was hosted by the Spanish OI Society AHUCE and saw 49 participants between the ages of 18 to 35 from all over Europe (10 different countries in total) come together for a variety of activities and socialising. Gareth Cumming from the UK attended the event and here is what he wrote:
Getting There This was my first time visiting Bilbao and due to limited flights I had to fly the day before giving me the opportunity to scope out the city before the others arrived.
I was a bit apprehensive at first due to the fact that Bilbao is situated on a very mountainous area meaning that it was very hilly and difficult to get around by manual wheelchair. The weather also didn’t help as Bilbao is located in the very north of Spain and combined with the time of year meant that the first few days was torrential rain – worse than British weather!
Day One On the first day of the youth event I arrived at the BBK Bilbao Good Hostel. It was chosen as it is a hostel which has been designed to cater for many disabled guests, and it was the perfect venue to cope with all 25 wheelchairs that attended. Due to everyone arriving at different times from different countries there was only time for socialising and getting to know each other through ice-breaker activities.
Day Two The rain had eased off a little, so we all made our way to the city centre. To get there we had to get the metro train which was a short walk from the hostel and was fully accessible however it did involve going in several lifts which was only big enough for 2 wheelchairs at a time, so with 25 wheelchairs this did take a while! Once we all arrived in the city centre the rain was too heavy for the free city tour that was organised so this gave us all free time to explore the city’s shops and bars and sample the local Pintxos – the region’s version of Tapas, very tasty!! When we returned to the hostel the group collected for the first organised activity, a very in-depth talk about relationships.
Day Three The sun had started to come out and we went on our next activity, a visit to the world famous Guggenheim Museum. To get there we all boarded a large wheelchair accessible coach – which again took forever to load 25 wheelchairs on and get strapped in. Once at the museum we split up in to 3 groups for a guided tour around the museum and talk about some of the museum’s most famous pieces of art. The works ranged from very traditional paintings from artists such as Van Gogh and Monet to a giant metal spider sculpture and a giant puppy made out of flowers. Once the museum visit was over most of us decided to make our own way back to the hostel as loading the bus took too long, so groups of us made our way to the metro station with several stops at the local bars to get some more Pintxos (Tapas) and Cervezas (beer), the evening was finished off by more “socialising” on the hostels roof balcony.
Day Four The Spanish sun had finally come out to play so we all made our way to a nearby park where we all took part in the “Gymkana”; we all split up into teams and took part in team activities such as quizzes, riddles and physical challenges, ending with a picnic on the grass where it was warm enough to strip off and top up our tans. In the afternoon the majority of us went back into the city centre where we were able to go on the free city tour that had been rearranged from earlier in the week. We were able to take in the amazing views of the city and hear about its fascinating history. The evening culminated in the main party night where the organisers had reserved a local night club where we partied until the small hours. It may not have been wise to hold the main party on the last night as many people had early flights the next day and we all had to check out of the hotel by 10:30am!
Goodbyes The last day was just enough time for early morning goodbyes through teary eyes and painful hangovers, which was an encouraging sign that everyone thoroughly enjoyed the week and that it was really well arranged and executed by the organisers and their volunteer helpers. The whole trip was fantastic, even with the dodgy weather, and there was only one OI related injury. Unfortunately, being 35, this was the last year that I was young enough to qualify to attend, which is a shame as 2020 will be somewhere in Poland, therefore I would encourage anyone reading this with OI between the age of 18 – 35 to sign up as soon as it’s advertised.
Writter by Gareth Cummings, Trustee Brittle Bone Society
From August 3rd – 6th 2019 the Chinese OI society (China-Dolls) celebrated their 10th anniversary in Yantai in the east of China. They organized a medical conference two days before the meeting. During this conference three international experts (Dr. Frascini from Italy, Dr. To from Hong Kong and Dr. Semler from Germany) gave lectures and discussed treatment strategies with 50-60 colleagues from China who are interested in OI.
6th national conference for people with OI in China
On the last day of the conference the doctors were seeing 30-40 people with OI, answered their questions and gave advise for further treatment. The questions covered all areas of OI therapy from bisphosphonates to dental problems and scoliosis. It was interesting to see, that many patients had a quite good surgical treatment, but the situation regarding wheelchairs and walking aids was really poor. There are no adapted wheelchairs available in China and also no walkers can be used. No equipment is adapted to the size of the person and no special devices are available for children. Many children are sitting in wheelchairs for adults and are not able to move on their own because they can not reach the wheels or the chairs are too heavy for the muscle power of a child.
The medical system in China offers a very basic health care to all people and differs from province to province. In some parts of the country a treatment with bisphosphonates or rodding of extremities is reimbursed but in other area this has to be paid. Because these treatments are expensive people with OI are often depending on support from charity organizations. China-Dolls as the national patient organization is very active in fundraising and charity events to collect enough money to offer support for medical treatment to as many OI people as possible.
10th anniversary of China-Dolls
The annual meeting of the society took place at a nice hotel close to the Chinese sea in Yantai. About 200 people were participating in the conference. People with OI of all ages and their parents or partners were present and discussed how they handled their problems in daily life. The meeting started with an official opening ceremony which took more than 3 hours. A lot of certificates were handed to people who supported China-Dolls in the past – and hopefully in the future. During the next 2 days many workshops were held including topics like “independent living” and “sexuality”.
I had the honor to represent OIFE at the meeting and to give a talk about activities of OIFE during the meeting. It was interesting how much the aims of China-Dolls differ to those of OIFE. In China the main goal of the patient organization is to provide a good medical care for all people with OI. Therefore, fundraising is extremely important but also political work on the national or regional level. High ranked representatives of the administration have the possibility to include some specific treatments into the national health plan and then these drugs or surgical procedures will be covered by the national insurance. Therefore, a lot of political work is still required to provide sufficient medical treatment to all people with OI in China. Other topics like selfcare and participation in social and economic life were also discussed but are not that much in the focus of the society at the moment as they are in Europe.
Collaboration for a better future
The whole meeting expressed a very positive mood and China-Dolls has a lot of reasons to be proud what they have achieved in the past. During the tea-breaks (coffee was very hard to get) there were very lively discussions between the participants, the officials and everybody around. In the hall and on the corridors, there was the usual chaos which is the same at all OI meetings and seems to be a characteristic of OI.
Altogether it was an interesting trip and impressive to see what China-Dolls have already achieved in a communistic country. I am sure that the situation in China will improve very fast in the future and that a closer cooperation between China-Dolls and OIFE can be beneficial for both sides in the future.
Written by Dr. Oliver Semler, Chair of medical advisory board of OIFE