Eye Hope Foundation

Autumn 2019 update

Dear Wolfram society,

It’s the time of the year again for a short update on our activities during the past 6 months. Thanks to your continued support we have continued to fund a couple of very interesting projects in Belgium, the USA and Estonia. Without your contributions to our fundraising this wouldn’t be possible.

During the past months, an initiative has been initiated in the US to create a Wolfram Syndrome Research Alliance bringing together researchers, patients and medical doctors. As part of this work, an overview was created of currently ongoing research and treatments in Wolfram Syndrome with the different phases.

Figure 1: Wolfram Syndrome Research and Treatment Pipeline, made by Sarah Gladstone.

Many of the projects in the overview, have been initiated or supported by our Foundation.

Since the announcement that Victor is currently taking Victoza (also called Liraglutide or GLP1 agonist), I have been contacted by numerous parents of WFS1 kids. Several dozens of kids are now on Victoza. According to my knowledge, the results are uniformly positive resulting in an increased quality of life at least. Especially the diabetes part becomes manageable. In order to gather the knowledge and experience at these different individuals, I created a Facebook group called “GLP1 for WFS1 kids” (https://www.facebook.com/groups/517126908859489/). Before starting however, its recommended to visit your endocrinologist.

Despite the positive results we observe, there is little known on the neuroprotective effect (eyes, hearing and brain) of liraglutide on the longer term. Therefore, we initiated since the beginning of this year a project in Estonia (Tartu) to investigate the long-term effects of liraglutide on WFS1-deficient rats. This project is running for almost 2 years according to the lifetime of the rat. During the past week, Victor and me visited the research director Dr. Mario Plaas and his co-workers Dr. Anton Terasmaa and Kadri Seppa in Tartu. The project investigates the effects of liraglutide on most phenotypes (diabetes, eyes, ears and brain) and is unique in its kind. Currently it's sponsored monthly by Eye Hope Foundation and we are always looking for funding in order to continue this project. I believe it's key to our society and the results will be presented at conferences of neuroscience.

Liraglutide is in best case slowing down the degeneration which is still our main priority. We discussed also several ideas to go to the next step which is recovery of functions. It's my ultimate hope we can initiate projects on this as well in a near future!

Figure 2: Victor entering the cleanroom in Tartu with A. Terasmaa

Figure 3: Inspection of WFS1 and Wild-type rat by Victor

Figure 4: Visionary drawing as an inspiration to the team, made by Victor.

Figure 5: Final dinner in Tartu, Estonia, with research team.

As a last point, during March next year, the French Wolfram association will organise its bi-annual Wolfram syndrome workshop in Paris where researchers have a chance to interact. The research that is sponsored by Eye Hope Foundation will again be strongly represented and provide major highlights to the conference!

To conclude, things on Wolfram research are moving, still too slow for me, but at least faster than ever before. Time is our worst enemy!

Thanks for reading and take care,


Eye Hope Foundation – Autumn 2019 update


Eye Hope Foundation– Spring 2019 update

Dear Friends, Dear Wolfram Syndrome Community,


In this newsletter, I wanted to give an overview of the research initiatives we took with our foundation during the last six months.

These initiatives are always discussed and evaluated internally. During the last months, I received significant assistance by Dr. Ir. Yvan Gilliams, a retired engineer that is devoting a large part of his pension to understanding the mechanisms underlying Wolfram Syndrome and supporting Eye Hope Foundation.


During January 2019, we decided to donate money to a project in the labs of Prof. G. Bultynck (GBIOMED - KU Leuven) at the KU Leuven. The project will investigate the role of Ca2+ signalling in cell models of Wolfram Syndrome. It will allow us to conclude the underlying mechanism and hence allow us to focus on those molecules that primarily target that pathway. The project will be executed by an experienced Ph.D. student and will also receive European funding.


The lab in Brussels is continuing their research with GLP1 agonist on a cell and a mouse model of Wolfram syndrome. Their work was the basis to put my son last October on Victoza (a commercial GLP1 agonist). Their results as well as the results on Victor were presented last week at a conference in the UK. Marianas work won a presentation award (Figure 1). Victor his diabetes is currently managed by Victoza only, allowing us to remove the insulin pump. For effects of GLP1 on the neurodegeneration its too early to conclude anything both on animal and on “Victor’s” level.

Figure 1: Mariana Esteve presenting the work on Wolfram Syndrome at the Islet Study Group and Beta Cell Workshop
Therefore, we have started a project in Estonia in the beginning of the year. The group led by Dr. M. Plaas (www.etis.ee/CV/Mario_Plaas/eng?lang=ENG&tabId=CV_ENG) and Dr. A. Terasmaa (https://www.etis.ee/CV/Anton_Terasmaa/est?lang=ENG&tabId=CV_ENG) will investigate the effects of GLP1 agonist on a rat model of Wolfram Syndrome. This project will cover almost 2 years and allow us to make conclusion on the neuroprotective effects of GLP1 agonist. The animals were born in January and started the administration of GLP1 agonist during last month. Both researchers have a long experience within the Wolfram field and were driving forces behind the various animal models.


Figure 2: Anton Terasmaa with the WFS1 rats that are receiving GLP1 agonist


The projects that we are sponsoring are closely followed by the Wolfram community. The groups we are sponsoring are visited by other foundations that are evaluating to sponsor them as well. I am also pleased to have received a meaningful donation from another Wolfram foundation to fund our Estonian project. The more such collaborative funding we receive the more useful our research will be!

Furthermore, it a great pleasure to note that several WFS1 patients other than Victor have starting to take GLP1 agonist to slow down progression of Wolfram Syndrome. Patients from Iran, Italy, Germany, Spain and USA have been contacting me to know our experiences. In my humble opinion it’s the most promising available drug currently on the market to combat Wolfram Syndrome. The projects that we are doing now will allow us to conclude on the degree of protection it offers against the various symptoms.

Thanks to all the supporters and volunteers helping our foundation grow towards a meaningful organisation. The results thus far prove that a small foundation like Eye Hope can make a difference when the scarce resources are used wisely! And on the latter, you can be sure that I will guard over it!


With best regards,

Lode Carnel

Eye Hope Foundation – Spring 2019 update


Eye Hope Foundation – Christmas 2018 update

Dear friends,


During our last letter, I reported on the increasing popularity of GLP1-agonist to treat Wolfram Syndrome in animal models. These results triggered me to arrange a workshop in Leuven during last week (19-20th of November) where our researchers from Belgium met with Dr. M. Plaas and Dr. A. Teresmaa for Estonia. The goal of the meeting was to align the different ongoing research projects on GLP1 agonist on mice, rat and human 😊. Prof. G. Bultynck and Prof. C. Verfaillie presented their projects and where introduced to our growing Wolfram research community (close to 15 people in Belgium only!).

Prior to the meeting, I asked Victor to draw a picture for the researchers. Below is what I call an artist impression of the research world, a mouse getting an injection with some medicines by the doctors… The drawing is also signed and dated, the original stayed behind at the labs!

The workshop concluded with a general impression that GLP1 agonists are probably the most promising drugs for Wolfram patients that are currently available. Mice and rats don’t develop diabetes if the GLP1 agonist treatment is started early enough. Furthermore, Victor has now had a significant number of days where he has been insulin and pump-free like he had no diabetes. Of course, we still need to monitor the food and it doesn’t allow for “un-healthy” kind of lifestyle. However, we are short of words to describe how it feels after 4 years with meal-to-meal management of carbohydrates and insulin. We can only wish that this will continue for ever, but that probably asking too much. As a side-effect we rather must fight against vomiting and hypoglycaemic events, although those have been diminishing during the last weeks. I am happy to discuss our findings with other potential parents of WFS1 children, don’t hesitate to contact me on lode.carnel at gmail.com.

Besides diabetes, the community is still hopeful for the neuroprotective effects of GLP1 agonists that could delay the vision and brain related issues.

Since Victor is the first kid with Wolfram Syndrome that is on the drugs, the result also impressed the researchers. Dr. M. Plaas described Victor as his hero when he found out what his animal-based research has led to. The initial results from Victor seem to go beyond what the researchers thought was possible.

I am happy that I took the risk of putting my son on this medication. I hope that this short time optimism will translate into long term rewards as well.

After my 38 years on this planet, I am however very down-to-earth, and I realise that Wolfram patients will need more than a pharmaceutical treatment. The best result I am willing to accept is a status-quo due to medication. Therefore, it was interesting to meet with Prof. Bultynck who will analyse wolfram syndrome on a molecular level. This will be the basis on which we will find better and more targeted molecules in the future (https://gbiomed.kuleuven.be/english/research/50000618/50753344).

Finally, we met Prof. Verfaillie her team consisting of four persons: Catherine, Arefe (principal researcher), Kirsten and Thomas. All of them or working on a CRISPR CAS9 gene therapy technology suited for Wolfram Syndrome. While this project carries probably the highest risk and the longest timeline, it also has the highest potential and hence should be started now. It has the potential to cure the genetic defect on a cell level. Initial results are exciting, and we need to investigate how we can fund this project going forward. It carries also a potential for several other rare diseases!


With this I hope I gave you some insight into our research and I want to thank all the researchers that contributed to this event. Thanks Mariana, Evy, Lies, Geert, Tim, Lode, Lieve, Mario, Anton, Catherine, Arefe, Kirsten and Thomas!  Keep up the good work, going from research to having Victor on a medication in 1,5 years must be close to record-breaking! It proves that Eye Hope Foundation is making a difference already now.

I hope to reflect at the end of next year with the same satisfaction about our projects as has been the case this year.


Therefore, I also want to wish all readers and contributors to Eye Hope Foundation a happy end of the year and the best wishes for 2019! Go and spread the Eye Hope Foundation word!


With best regards,


Eye Hope Foundation – Christmas 2018 update