Beyond the Muted Darkness

BEYOND THE MUTED DARKNESS

The photo book Beyond the muted darknesswas launched in Pakhuis de Zwijger on 31 August 2017. This book contains photographs of forty people suffering from Usher Syndrome taken by forty photographers. Beyond the muted darkness shows’ positive and strong pictures of the people suffering from Usher Syndrome. Each photo is accompanied by a short story of both the photographer and the person on the photo.

Photo book ‘Beyond the muted darkness’ was established by initiator and publisher, Marco Groenewegen. Forty top photographers, including Linelle Deunk, Koos Breukel, Reinier Gerritsen, Julie Vielvoije and many others, cooperated in making this book. Humberto Tan had his photograph taken by Sacha de Boer and wrote a foreword.

The blind leading the blind

THE BLIND LEADING THE BLIND

“A hilarious boost for the spirit for everyone in a time of crisis

This is a story about Niek and Joyce.

Niek has a spinal cord lesion and Joyce suffers from Usher Syndrome, which will slowly make her both deaf and blind. Of course, these things are not easy to live with, but these two are coping well. They sure do: they literally draw full houses as inspiring speakers.

Until the coronavirus suddenly puts a stop to these meetings. Niek and Joyce are in sackcloth and ashes. Or rather in a walk-in wardrobe and at the kitchen table. Their work is gone, their health is threatened, all the news is bad news and hugging is no longer allowed. Niek withdraws into himself under his duvet and Joyce turns to chocolate.

However, after a while they are cannot suppress their natural optimism any longer and so they start searching for new ways to make themselves useful and to tell their stories. This goes by trial and error. Niek tries singing for a while (not a good idea) and Joyce tries doing odd jobs (not a good idea either). Still, their search does lead them somewhere eventually and they gain a lot of insights along their ways. It also results in this book: a hilarious boost for the spirit for everyone in a time of crisis.#sterkercoronadoor

Not hear, Not see, do speak

NOT HEAR, NOT SEE, DO SPEAK

If your world becomes dark and quiet, will you be quiet as well?

When Joyce was seventeen years old, a physician told her that she would be of no value for society. He advised her to learn a language. “Then you can perhaps translate a book some time.” That was all that was in it for her, according to him.

At that moment, Joyce has just found out that she suffers from the very rare Usher Syndrome, which would gradually deteriorate her hearing and seeing in the course of time and cause her to be deaf and blind by the time she would be forty years old. Because of this diagnosis she was in a hurry: she wanted to see everything, learn everything and experience everything before her world would be definitely dark and quiet. She continuously fought against her disease and she desperately searched for a way to see the good of it – until she realised that she could never win this fight. Then she started to believe that she had something to tell the world. The fact that she would no longer be able to hear or see some time in the future did not necessarily mean that she would also not speak.

Now she is married, mother of two children, entrepreneur and a popular speaker. She cannot stop the approaching darkness and quietness, but she can use her story to inspire others and to learn how to better cope with changes.

And no, she never translated a book. She did, however, write one. And some book this is!

Séverine sees it differently

Séverine was diagnosed with Usher syndrome 20 years ago. Her deteriorating lost of sight and hearing does not make everyday life easy. However, she still trains for the alternative Four Day Marches, together with her buddy. #SupportofSport

 

Unfortunately, this film is spoken in Dutch with Dutch subtitles. Turn on automatic subtitles in YouTube

 

For Jackson

Click on the photo to watch the film

 

A documentary made by Luca van Turnhout and Linse Chantrain

How does anyone deal with such a diagnosis? We follow Carolien, Willem and Jackson in their daily lives. Although they are daily confronted with Usher Syndrome, they live from one day to the next and try not to think of the future too much. This is not always very easy, though.

A documentary as a bachelor assignment for the applied audiovisual communication (BATAC) training of the THOMAS MORE HOGESCHOOL in Mechelen, the Netherlands/2020.

An article in connection with this mini-documentary: I have a haste for life, because of him’.

This film is spoken in Dutch and does have English subtitling.

A sense of black

The documentary ‘A sense of black’ takes its viewers along in the world of the blind artist George Kabel (1955) from Eindhoven, the Netherlands.
George is sculptor and one of the 800 Dutch people suffering from Usher Syndrome. For George this means that apart from being blind, he will become deaf as well.

The story of George Kabel is the story of overcoming a major setback and giving life a new meaning.

The film had its première in December 2018 during the Eindhoven Film Festival in the Van Abbe museum.

Script, scenario and production: Gerben Zevenbergen
With English subtitling.

 

How is Machteld Cossee doing today?


Overwhelmed with reactions
The film moved people from Machteld’s wide social circle as well as other viewers Director Hetty called the number of reactions to the documentary ‘overwhelming’. Machteld continues: ‘People around me know that I suffer from Usher, but the film zooms in on this. It shows the rough side of Usher.’ She confesses that she did not pick up the children from school herself the first four days after the broadcast. ‘When I appeared in the schoolyard again, other parents came to me. They offered their help and said they now had a better understanding of what was going on. How often was I called ‘arrogant’ or ‘a muddlehead’? I would never call myself that. The film gave people more insight.’ Hetty adds: ‘People looking at Machteld from a distance don’t have any idea how much difficulties she continuously has to overcome. I saw her on a photo with her husband Lars on the tandem in the JAN and I immediately knew that I wanted to watch at things along with her.’

The film did not only have influence on Machteld’s environment but on that of her fellow sufferers as well. ‘Six months ago, a woman I didn’t know came at my door. She stammered a bit while she was standing there and I really had no idea why she had come. Her son appeared to suffer from Usher. When she saw me walking one time, she mustered up her courage and rang at my door. In the period after that we had coffee together twice to talk about this.’ Actually, Machteld has become a kind of standard bearer. ‘Usher Syndrome Foundation received a lot of reactions after the broadcast and various young people had their “coming-out” as I call this after the film.’

Put things into perspective because of Usher
Hetty Nietsch made the film with her daughter Lisa Bom. ‘She had just finished the academy of arts. Working on a film for a few years really costs a lot of money, so we took a small camera and started to work with the two of us. The cameras became better in the course of the time.’ Lisa sometimes even slept at the home of Machteld and Lars.

Source: NPO 2Doc.nl
Text: Wieneke van Koppen
Photo: Lin Woldendorp

Before everything will be dark tomorrow

The sisters Lotte (18) and Roos (16) both suffer from Usher Syndrome. This means that they will soon become both deaf and blind. They do not know how fast this will happen, but their eyesight and hearing already are well below the levels of other people of their age.

Still, the sisters do not prevent themselves from getting the most out of life. Roos is working hard for her final exam and Lotte is fully engaged in her course to become a photographer. They also want to do a few special things, such as seeing the Northern Light, before it is too late.

Production: Kim Smeekes

Unfortunately, this film is only available in Dutch.

Run in pace, following a pacer

With a simple gymnastics band, Usher patient Ivonne Bressers (51) from Arnhem keeps direct contact with her buddy, Edith Mulder during the 7 Hills Run.
Mulder: ‘If necessary, I call “high” to let Ivonne know that she has to lift up her knees a bit higher for, for instance, a threshold. Or I call “right” or “left” when we have to go around a hole or a puddle. I love doing this. Everykilometre is exciting, but really beautiful too!’

Out of the 35,000 participants of the 7 Hills Run held on 19 November, 144 people ran in the Run4Usher team to collect money for research for the benefit of people suffering from Usher Syndrome. This hereditary disease can lead to full deafness as well as blindness. In this fourth year of their participation in the running event, the number of participants was doubled and at this moment twice as much money has been collected as well. Apart from runners without an impairment there also is a small number of people with an impairment, like Bressers.

About 1.5 metre before and after Bressers and Mulder three ‘pacers’ are running, who keep the path clear for a total of seven badly hearing and seeing runners of the Run4Usher team. Bressers: ‘I thoroughly enjoy this. Because of my impairments everything goes slowly in my life. When running, I can give myself a free rein. Besides, we enjoy each other’s company.’ Bressers fully trusts her buddy and the pacers.
‘This was my first 7 Hills Run and it went very well. Fortunately, it was only raining exactly before and after the run. One time someone was grumbling about the pacers until she saw who we were and she excused herself a thousand times.’ Bressers was very satisfied with the wonderful route along the hills.

Annouk van Nunen (41) from Leiden also ran with her permanent buddy, Leen Ooms. Ooms thinks it is great to be able to offer blind and visually impaired people this experience. ‘It is a really responsible task. People cannot see straight away that people with an impairment are running here. Especially not at a crowded event like this. The runners and their buddies are running widely and therefore have to pass widely. Sometimes we really have to elbow our way through the crowd, when necessary with a lot of noise, to clear the way for our runners.’

Four people suffering from Usher Syndrome also ran the 7 Hills Night. This was extra difficult because of their night-blindness. The disease is progressive and shows itself in a lot of variations. It often begins with being hard of hearing until night-blindness is developed in puberty and later tunnel vision. Bressers: ‘It is like she is looking through a straw. I cannot see anything outside that central point. For my hearing I now have implants, but there is no remedy yet for the blindness. I really hope more research will be done soon. The solutions may also be applicable to other eye disorders.’
The woman from Arnhem finds it hard to be forced to accept the growing loss of two senses. ‘The possibilities to compensate the one impairment with the other are decreasing as well. Still, I try to find a balance between asking for help and being independent over and over again. Fortunately, more and more actions are organised and Usher Syndrome is becoming increasingly widely known.’

Source: De Gelderlander, Nijmegen e.o.
By: Loes Wijffels
Photo: Gerard Verschooten

‘I do not see myself as a person with restrictions. I am just Jeff.’

He was born deaf. And he will be blind as well. Jeff Horsten sings a self-written song about his restrictions on YouTube. The 23 years old man from Waspik, the Netherlands, suffers from Usher Syndrome, a very rare disorder. Still, Jeff surprises the people around him with his laughter, his creativity and his optimism. ‘I want to inspire people. I do not see myself as a person with restrictions. I am just Jeff.’ The film of his song ‘Welkom in mijn wereldje’ [Welcome to my small world] has been watched thousands of times and dozens of people have shared it on Facebook. The film was put on-line a couple of weeks ago. ‘Tears’, ‘respect’ and ‘goosebumps’ are responses to the touching song.

Not alone
‘I want to show other people suffering from Usher Syndrome that they are not alone’, says Jeff. He sings the song together with singer Jorien Habing. The text is about the ‘tunnel’, as the man from Waspik experiences his life. It refers to his deafness and poor eyesight: Jeff looks through two holes of half a centimetre in diameter. ‘People usually can see 180 degrees.’ When Jeff looks at someone, he must move along his head. His eyesight is blurred: now 50 to 60 percent. Jeff will eventually become blind. With the help of his friend, Joep Rijk, Jeff had the song recorded in the Swamp studio in Raamsdonk. The idea came up in rehabilitation centre Het Loo Erf in Apeldoorn, where Jeff has spent nine months by now. He hated music for years. Just because he could not hear it. “One moment I heard Ring of Fire by Johnny Cash. I managed to listen to the rhythm. Later I started to experiment with music. I sometimes take a guitar and play a little. However, it remains difficult because I cannot listen well myself.’ Jeff has plenty of texts. He writes a lot. ‘Writing is my way of release. It provides a means to express myself. As a teenager I often picked up the pen. It was a kind of game to me. Suddenly all emotions are lost.’ Jeff’s parents think that the song is really confrontational. Father Pierre: ‘I listened to the first lines but I let the rest of the song rest for a few days.’ ‘Of course, this is not new to us, but many tears were wept in our house when we heard the song’, according to mother Marja. Jeff’s parents experience their lives as a struggle for their son. To get him to the right school, to give him the proper guidance. His poor eyesight was discovered only after seven years. ‘As a boy, Jeff often had quarrels. I was at school almost every day to stick up for Jeff. Now we know that he could not express himself. He felt misunderstood.’

Clicks
Jeff often stumbled over things, he became night-blind and fell over other children while playing. Marja and Pierre later heard in the Radboud Hospital in Nijmegen that Jeff suffers from Usher Syndrome. ‘We had looked up a lot of things on the Internet. Then everything clicked. We were quite sure about it. When then things are actually confirmed, the world stops turning.’
It is hard to accept that Jeff’s restrictions are increasing. He cannot participate in things that are self-evident for other people of his age. Marja: ‘Like riding a bike or a moped. Jeff loves cars, but he will never be able to drive one.’

I am always mentally tired.
I seem to get up with an energy level of 10 percent.

‘It is putting up with things again and again’, Jeff describes the setbacks. His resilience has grown in the course of the time. ‘I give things a place and I have become more flexible. Now I can say “I cannot do it”. I have no choice. In the past, I was only surviving. Now this has been changed into living. I am living. I have switched over.’ Thanks to a cochlear implant he can follow conversations fairly well. He can also talk. His voice is not really bright, because he missed sounds in his early childhood. Jeff manages to follow an education at the Prinsentuin College in Andel and is a qualified animal carer. Jeff can enjoy things, such as Johnny Cash, films of cars on YouTube and the dog that he hopes to have soon. However, despite his humour and positivism, Jeff is struggling with his feelings. ‘I have my difficult moments. Sometimes these last for a day. Then I withdraw into myself and I want to be alone. I do not want other people to see me like this. Shit happens. Everything has its price and I have to pay mine.’ At this moment, the man from Waspik is going through the last phase of his rehabilitation. Jeff is prepared to becoming blind at Het Loo Erf. It is still uncertain when this will happen. ‘I do not notice any major changes between one day and the next. It goes in tiny little steps.’ The man from Waspik is teaching himself some practical things, such as doing the laundry, cooking, shopping or read Braille. ‘Only 10 percent is about the restriction, whereas 90 percent of the rehabilitation is about mentality. They give me self-confidence. I can do much more than I think.’ Marja: “I am surprised about how cheerful everyone is at Het Loo Erf. Its; incredible. We can learn a lot from them.’ Jeff nuances: “In our tiny world there is no place for grey, as many people experience days in their lives. We are either happy or sad.’ His tunnel vision costs really a lot of energy. ‘The greatest problem is the fatigue. I am always mentally tired. It feels like I get up with an energy level of 10 percent. When I do something for a few hours, I have to go to bed. I have to prevent getting a burnout.’ When Jeff is ready with his rehabilitation, he has to take a big step. He will go and live by himself. In the centre of Waalwijk. 100 percent independent. ‘I do not want to live under supervision. Never. My parents are helping me really a lot. They do everything.’ Jeff will probably move house next year. This will be another difficult moment for the Horsten family. ‘We are afraid of the loneliness. This is our major concern’, Marja says. Jeff: ‘I am not easily frightened. I know this will be a struggle, but I have had to fight for everything in my life. I am convinced I will manage.’ His mother endorses this: ‘It has cost a lot of energy. Still we look ahead with a positive mind. We have already struggled, struggled and struggled for 23 years …’

Watch and listen to ‘Welkom in dit wereldje’

Source: Brabants Dagblad
By: Sjoerd Marcelissen