Autumn/Winter 2000
Francesca Wolf reports on young people who have Usher
Being a teenager is hard enough. Raging hormones, roller-coasting emotions, a continually changing body and shifting sense of self, self consciousness, school pressure, worries about friends, relationships and the future – all these are experienced by most adolescents at some point.
Although it can also be a lot of fun, adolescence is difficult in other ways too. It’s a time of transition, a bridge between the dependency of childhood and independence of adult life. Along the way this often means a pulling away from parental and family influence, the increasing importance of friends and peer group identity, a testing of boundaries, a trying out of one’s wings. It is a period of exploration of the world and new experiences. In the process, values may be interrogated and fundamental questions asked: who am I? What will I become? What will I do for a career? What is my place in the world?
It’s not easy. Imagine, then, what it must be like if you have Usher syndrome. While navigating the already treacherous waters of adolescence, you also have to cope with deafness or partial hearing and the implications of present or future loss of sight. Just when your life should be opening out, it starts closing in.
A significant impact
Ilene Miner has conducted research into adolescence and Usher and, in the article The Impact of Usher syndrome, type 1, on adolescent development( Journal of Vocational Rehabilitation 6 ( 1996) 159 -166), she states that the impact " is significant in every sphere, i.e. home, school, social relationships, future career, marriage, continuing education, and day-to-day activities."
The impact of diagnosis can be devastating. Often this happens in the early teens – for Usher Type 1 - but even when the diagnosis is earlier, some families choose not to tell their children. When they later find out many Usher adults say they wish they’d known sooner because they felt they were clumsy or stupid, when actually they just couldn’t see, due to deteriorating vision. So while the diagnosis can be traumatic, not having it can be even worse.
Shock, anger, grief, denial are common responses – both for the person with Usher and their family. " Parents are invariably traumatised by the diagnosis and the way it was delivered," says Miner. There can be " a resurgence of grief for parents who are still dealing with having a deaf child " and " renewed struggles at home". Significant vision problems don’t usually kick in until the teens so they come at what is already one of the most emotionally stressful periods of family life.
Miner says "Adolescents may experience guilt and shame because they are dependent on others, and anger and depression because they are experiencing losses almost daily, from restricted conversations to restricted activities … They feel diminished by the constant onslaught to their self-esteem."
The progressive loss of vision can cause depression and mourning, for this loss attacks deaf people at their core – a people who do so much connecting through their eyes. And the mourning is cyclical , says Miner – repeated again and again throughout life at key stages or as vision deteriorates. Yet though depression in adolescents with Usher syndrome is so common it is frequently missed by professionals who tend to concentrate on the practical realities of deaf-blindness.
Anxiety is handmaid to depression and adolescents often have fears about the future. In Miner’s study they worried about jobs, rejection, being a burden on the family and not being able to become independent or form relationships. They worried too about marriage and having children. In fact teens with Usher may have useful vision for quite a long time, and many worries are based on lack of knowledge. " Teens find their futures foreclosed too early," says Miner.
Identity
The whole question of identity is central. Those with Usher Type 1 often already have a deaf identity, use sign language and are exploring " where they will fit into the world of deaf people and into the larger world of hearing people." ( Miner). Discovering that vision will also be affected is an overwhelming blow. People with Usher Type 2 – who are hard of hearing – may already be unsure of their identity, and when the sight problem starts this can get even more confused. So it’s very hard for both groups.
A recurrent theme of older adolescents is that "no-one understands". In general, blindness specialists may know little about deafness and deaf specialists little about visual impairment. Teachers and others may be uncomfortable dealing with the issues. But not talking can increase fears by suggesting that what is coming is so terrible that no one can mention it . Moreover, if a teenager doesn’t know anyone else with Usher, how can he or she conceptualise the future?
Experiences at school may be painful – whether the young person is in mainstream education or at a school for the deaf. Support services are sometimes inappropriate or inadequate. If there is no one else around with Usher he or she can feel isolated, while bumping into things or people may cause embarrassment and be misinterpreted as clumsiness. Sometimes friends are lost because they don’t understand the vision problems or feel awkward talking about what is going on. Any kind of difference also makes you more of a target for teasing or bullying.
Nightlife
Social life is terribly important to teenagers. All the usual activities - pubs, clubs, restaurants, parties - are more difficult for those with Usher. Nightblindness, becoming blind in dim light, can make nights out quite frightening. Lisa Hoya, who has Usher 2, says " As a teenager I could never see at night. You can’t just say ‘ I’ll meet you in the pub’ because you need someone to take you there, or to get a taxi. When you get to the pub you have to find your way but it’s always dark, so are cinemas… everything is so complicated."
Teens may find it progressively harder to follow conversations in groups of people. "It is quite traumatic for teens to have Usher," says Lisa. "You’ve already got this lethal combination of hormones raging and want to have fun. But it’s so easy to miss the joke, miss the moment. People with Usher 2 can’t lip-read in the dark."
Despite this, Lisa says she still managed to have a lot of fun and scrapes ( such as raiding a "haunted house"!). This was largely due to "supportive friends" who looked out for her and these can make a huge difference to teens with Usher as acknowledged by Lindsey and Nicola (see case histories).
Another positive is that new technology - in the form of the Internet and mobile phones – is opening up the world. Young people with Usher can communicate through e-mail, and connect with others through newsgroups. Text messages on mobile phones give them freedom, access, and the ability to keep in touch with friends. All these are important tools in breaking down isolation.
However, one of the biggest blows is not being able to drive. Obtaining a driving licence is such a treasured rite of passage for teenagers – an important milestone on the road to independence. But in most cases peripheral vision is too poor to get the licence so the young person with Usher becomes dependent on others for help getting around. This can lead to tremendous frustration.
An arduous journey
Young people with Usher face "an arduous journey that cannot be made alone", writes Miner. They need to know the truth about their eye problem, have information, and access to counselling. They need ongoing support – from family, friends, the community, professionals – and " they must have access to people who have been on that road already".
She stresses how important it is for teenagers with Usher to meet others in similar situations and also deafblind adults: " How can a child incorporate an image of what he or she might be in the future without ever having seen a grown up version of him or herself?" Meeting others with Usher can provide positive role models ,examples of what deafblind people can do and allow them to ask the questions churning around inside. It can also help develop confidence, counter feelings of marginalisation and provide a sense of community.
Lisa Hoya agrees there should be more opportunities for young people with Usher to meet and share activities. " You wonder ‘ Where do I belong?’ Teens with Usher need a sense of belonging," she says.
Usher comes in many forms, is usually a slowly changing condition, and although young people often fear they will lose their sight within months or a few years, in most cases this isn’t so. Indeed, they may have good useful vision for 20 years or more – longer than most people today stay in a job. Teens with Usher need the freedom to choose the path they want – educationally, in relationships, in their career. Many have tremendous inner strength and resilience, and when this is harnessed to knowledge and optimism, they can take control of their lives.
Then the future becomes something that opens out rather than closes in. Lisa Hoya sums it up: "It’s important to develop self esteem and confidence and realise that Usher shouldn’t stop you doing anything … it hasn’t stopped me."
References
Miner, I The impact of Usher syndrome, type 1 on adolescent development Journal of Vocational Rehabilitation 6 ( 1996) 159-166
Miner,1 and Nuccio, J Mental health and deafblindness: a focus on wellness ( 1997)
Lindsey’s story
When I learned I had Usher I thought ‘I’m going to go blind ‘ and cried a lot. But later I found out more and realised I’m not the only one! Now I’m used to it and know how to handle it, although sometimes I get paranoid about it becoming worse.
Boarding school was difficult. Some people laughed and picked on me because I couldn’t see their faces and I was so depressed I wanted to go home. In the second year I found out about Usher. My close friends helped me when walking outside in the dark but there were other problems. Every break or lunchtime there was a crowd of people and I sometimes bumped into them or tripped over bags. In gym it was hard to catch balls because of their colour and I was made to feel stupid and blind – I burst into tears and yelled to staff that I couldn’t see the stupid balls. One day the fire alarm went off and I was looking for the exit but the stair was painted black. How could I see that?
I can’t stand being at home all day and night and like going out. In the day things like shopping are no problem for me. I love walking my dog in the forest and I also play football for Dagenham – defence is the best place for me. But I can’t go anywhere in the dark alone: it’s impossible just to go to a friend’s house. At night I go with my friend to a local pub which has light and choose a table which suits me. Many places I like, though – nightclubs, tenpin bowling , pubs – are dark inside, but when I ask if they can be a bit lighter the manager says no! It can be hard talking to friends in clubs without eye contact but I know they are listening.
My close friends support me 100% and I feel safe with them. They are great, but sometimes I feel as if I rely on them too much and I’m not sure if it bothers them. My boyfriend, too, supports me a lot.
I communicate through BSL with deaf people, though sometimes I miss something if the signing is a bit low for me to see both the hand and lips. Sometimes I use deafblind signing in the dark – say in bed with my boyfriend. Talking to him in the dark makes me feel as if I am a normal person.
It’s important to meet other Usher people – you feel you’re in the same world, not an outsider. It can make you more confident – that it needn’t stop your life. I really enjoyed the parent day meeting and would love to travel to different places and meet more people with Usher.
I’m not looking forward to the future, though, because my eyes might get worse. Sometimes I feel my life is worthless and I worry that I might not get a job because of Usher. I want my own house and family and I don’t want any guides or social workers .Let them control me? No way! I’d rather be on my own and learn from experience, while still having friends for support and contact with an Usher group. It’s best not to worry too much - just let the future happen and get some support.
Nicola’s story
My family found out I was deaf when I was six months old. At first people communicated by pointing and showing me things, but when sign language was introduced communication became easier.
When I started school I was given a hearing aid, but at around 13 refused to wear it. I said " I was born this way and nothing will change that so just let me live the life I want to." They insisted hearing aids were for my own good, but I wasn’t happy – I felt they were trying to take away my identity as a deaf person. All I could hear when I put on a hearing aid were sounds that made no sense.
I chose to be a member of the deaf community, whilst remaining a part of the hearing community. Being in a hearing community alone could make me feel lonely, but accepting myself as a deaf person enabled me to get on with life and learn the powers of independence.
However, at the same age ( 13), my teachers noticed I was bumping into people and things. I was referred to hospital where I was diagnosed with Usher 1 and told " You will go blind."
I felt as if my fight for independence had been a waste of time. I was shocked, angry and tried to find someone to blame. My life seemed to be going nowhere and I didn’t want to talk to anyone as I felt so much resentment towards their happy problem-free lives. Being an adolescent gave me an excuse to rebel and do the exact opposite of what people said. If nothing in the world could go right for me, why do the right things? I felt confused and isolated.
Once the shock had sunk in, I got on with life as best I could, but still felt scared and alone. On waking, I squinted my eyes to make sure the world was still there, but when I closed my eyes at night there was nothing. It felt like even I wasn’t there - I was going to become an anonymous person who no one saw. I began to worry about the future and how Usher would affect my life. Would I definitely go completely blind and, if so, when? Would anyone want to marry me? Would I be able to see my children when they were born? I had always had confidence but suddenly it had gone.
At this point my mother contacted Sense who sent us much useful information which made me realise I was not the only person with Usher . I went on to meet other people with Usher and gradually my confidence returned.
There were setbacks: at school I was indecently assaulted by students for which I needed counselling for several months. However, gradually I have come to terms with my disability and I’ve done well : I passed 10 GCSEs and a GNVQ in Information Technology. I also attended a deafblind course and learned the deafblind manual alphabet. I’ve sent my CV to Tyne Tees TV for a job as trainee interpreter. Life is getting better.
An ongoing problem though, though, is full Mobility Allowance. Despite going to a tribunal three times, I am still on the low rate and I can’t understand why. I also failed my field vision test which means I can never drive. This is annoying - I long to be able to drive myself around. It’s my independence they are taking away - upsetting, as it makes me feel I am pressurising other people to help, although they don’t mind.
I go out at weekends, usually with my sister, Georgina, or best friend, Gemma. A lot of bars and restaurants have stairs but Georgina makes sure I’m behind her and puts my arm on her shoulder so I know when to step up or down. All my sisters and Gemma are great and sign really well.
Having Usher does cause problems like bumping into things, but I just laugh about it. Sometimes I get annoyed when I’m out with deaf friends because people stare when we’re signing – but that’s because they’re amazed at our communication – not because there’s anything wrong with us. I’ve learned to cope with Usher and now, if anyone says anything to upset me, I laugh and walk away. I’m not the blind one …. they are.
James’ story
Like most 23 year-olds, I really need my independence, so I live on my own – although my parents don’t live far away. I know this may be a problem in the future as my eyesight deteriorates, but it’s impossible to predict exactly when, or how bad it will be, so I just live for the present and enjoy the freedom of pleasing myself.
I spend a lot of time in my office at home, sorting out paperwork for my role as youth co-ordinator for Usher UK, and studying for my City and Guilds in Further and Adult Education. In the future I’d like to teach BSL to hearing students.
I’ve got a real mix of deaf, Usher and hearing friends. It’s good to share experiences with friends who have Usher and I’d say my closest friends are deaf, but I learn a lot more about the "outside world" from hearing friends. The secret of my relationships with my hearing friends is mutual respect. I need them to understand what Usher means and not interfere (even if it’s well-meaning) when it’s my life to control.
When I meet new friends who are hearing or deaf I don’t like to tell them about my Usher immediately because I’m worried they may be put off and give up before they’ve had time to get to know me. I love mixing with all types of people and on the surface no one would realise I have Usher. But this can lead to problems with people not being aware of my communication needs.
For example, I am gay, and living in Chesterfield, there aren’t many places to meet other gay people. When I was younger, I visited a local gay pub, but the lighting was so bad that even if people were speaking slowly I wasn’t able to lip-read, so I gave up. This is the same with a lot of social situations. Deaf and hearing friends don’t realise I need good lighting to communicate with them and I sometimes end up excluded from conversations and ignored - like a pet dog.
On the relationship side, my Usher can also be a problem when I meet someone new. Early on they may not understand why I need to stay in good light, and further down the line arguments can occur over me wanting to go to somewhere well-lit on a night out. I mean, everyone else just wants enjoy a drink in a cosy bar, so I don’t blame them! I don’t want people to treat me with kid gloves, so it’s hard to find a compromise. I prefer now to meet gay friends during daylight until they start to understand a bit more about my Usher. I’ve also met lots of good friends over the internet and outside the "scene".
Having Usher and being gay sort of puts me in a rather small "community". They way I see it, there are three communities - deaf, Usher and gay-Usher. The smallest by far is gay-Usher, but I feel I belong to all three communities. I do know other people who are gay and have Usher and we’ve talked about setting up a social club for other gay-Usher people. Right now I don’t think I’m in a position to do that, but I do encourage the Usher UK youth group to discuss absolutely any topic they feel is important, including sexuality.
It’s so important not to let disability or sexuality stop you from achieving your goals. Last year I surprised a lot of people by running the London Marathon. It was a lifetime ambition, which, interestingly enough, has made a lot of local people treat me with more respect. I feel proud of who I am, and, most importantly, I believe every person with Usher should be proud of who they are too.
James Clarke was interviewed by Georgia Veats
