Living with Usher - Brothers and Sisters

Usher syndrome - The experiences of brothers and sisters

Growing up with a brother or sister who has Usher syndrome can be challenging but also bring rewards. The story below, which is fictional, illustrates many of the issues that siblings deal with.

Tom’s story

Tom is 19 years old and lives with his parents and his older brother James and his younger sister Sandra. He has recently passed his “A” level examinations and is due to go to university soon.
When Tom was about eight years old his brother James was diagnosed with Usher syndrome Type 2. Sandra, who like James was hard of hearing from birth, was diagnosed with the condition a few months later.
Tom is now aware of how life changed at that time. His parents always seemed to be taking James or Sandra to a hospital, which was some distance away. When these appointments were during the school holidays, Tom had to spend many hours in the car and then sit quietly in the hospital waiting room. Strangers would often come to the house to talk to his parents. They were friendly enough, but Tom was soon sent out of the room to do his homework or play football in the garden. Nobody ever told him who these people were or what was happening.
Tom remembered how his mother and father often seemed sad or upset and would argue a great deal over little things. As a family, they no longer did the things together that they had previously enjoyed, such as going on camping holidays or cycling trips at the weekend.
One day, when he was still quite young, Tom left his bicycle on the ground in the back garden. His sister Sandra tripped over it and badly sprained her wrist. His mother shouted at Tom and called him “stupid”. He later heard his mother crying in her bedroom.
Tom had heard the word “Usher” mentioned a great deal, but did not understand what it meant. As he got older he became aware that as well as being hard of hearing, both James and Sandra had a problem with their eyes; they didn’t see very well at night and tended to trip over things. Tom’s friends would tease him about his brother, saying how “rubbish” James was at football.
Recently, Tom looked up “Usher syndrome” on the Internet. He found out that it is a genetic condition and that he might be a carrier. Although their sight doesn’t seem to be getting worse at the moment, Tom also found out that James and Sandra could lose more of their vision.
Tom’s ambition for the future is to train to be a commercial pilot, but he worries that because of the Usher syndrome in his family he would not pass the medical or be accepted for training.
Although he is trying to think about his future career and his new life at university, Tom worries that if his brother and sister lose their sight, he will have to look after them and maybe support them financially.
Tom is not interested in having children at the moment, but wonders whether he should have a family in the future, as his children may have Usher syndrome.
Tom has never really talked to his parents or siblings about his feelings and anxieties. He has been with his girlfriend for just over a year and although they have talked about his brother and sister, Tom has not mentioned that he may be a carrier of Usher syndrome or his concerns about his future career prospects. 
Having a brother and sister has certainly been challenging for Tom but he also recognises that there have been positive sides to this. For example, his girlfriend notices that he is understanding  and caring towards people who are experiencing difficulties. And he still has a lot of fun with his siblings and enjoys their company – just like most siblings.

The impact of Usher syndrome on the whole family

Parts of Tom’s story may seem familiar to any young person who has a brother or sister with Usher syndrome.
Often parents are the first to notice that there is a problem with their child’s vision. Their son or daughter may not like moving around in poor light, may not be good at ball games or may trip over the vacuum cleaner if it is left in the living room. 
Some time may pass between these initial concerns and a confirmed medical diagnosis. Clinical investigations may take several months and involve several trips to a hospital eye clinic. This can be tiring and stressful for all family members involved.
As a small child, Tom knew that something was wrong, but his parents did not talk to him about what was happening. They were worried that he either would not understand or would be upset. Tom learnt about Usher syndrome through overhearing conversations between his parents and professionals visiting the house, or by reading some of the information that had been given to his parents.
As James’ and Sandra’s vision changed and they had difficulty seeing in the dark, or seeing objects outside their field of vision, activities like overnight camping trips, or cycling and football were less enjoyable. There was also an impact on Tom who, as a child, sometimes felt that he had done something wrong or was being punished. For example, his parents no longer took him camping, he was now told off if he left his bike or other things on the ground and his mother sometimes seemed to be angry with him for no reason.
As a young adult Tom began to understand more about the condition but became more concerned about the direct impact of Usher syndrome upon him and his future relationships and family life.


Benefits of sharing information – some considerations for parents

At different times in his childhood Tom experienced many different feelings in relation to Usher syndrome. For example:
Anxiety – why were his brother and sister always going to hospital? Why did his parents appear sad or upset?
Confusion – why did strangers come to the house? Why did his mother tell him off when he hadn’t done anything wrong?
Feeling pushed out – why were his brother and sister getting all the attention?
Guilt – why did James and Sandra have Usher syndrome, while he was unaffected?
Clearly the decision about how much to tell a brother or sister about Usher syndrome and when to do so lies with parents or guardians. However, younger children such as Tom often pick up more information than adults realise, in his case through overheard conversations or reading information left around the house. This may cause confusion or anxiety for the child.
Although it can be challenging for parents to involve everyone, creating a culture of openness and inclusion within the family can help to deal with many of the negative feelings that siblings can experience. The main challenge is to know how much information to give to siblings and when. 
Parents may like to consider spending time with all their children, telling them about Usher syndrome and providing each child with the chance to ask questions. The Usher Team at Sense can provide advice on how to approach this and can also help parents select information that is appropriate for their children. It may also be possible for a member of the team to be present to talk to the family, and help parents to respond to any questions or concerns that their children may have

Improved understanding

Ideally all family members can help to maintain an environment where the person with Usher can move around safely and independently. As an older child, Tom would certainly have understood this and been able to take some responsibility for keeping his brother and sister safe – for example by putting his bike out of harm’s way.
When he was younger though, Tom needed to know that the reason that his parents no longer took the family camping was not a punishment for him. It was due to their concerns about his brother and sister having poor night vision.
Although some young people with Usher may prefer not to take part in certain activities – such as football or camping – there are plenty of things the whole family can enjoy - for example, swimming, bowling or canoeing. It may be useful to discuss this as a family.  Siblings can continue to participate in activities they enjoy by, for example, joining the Scouts or a local sports club. This also gives them the chance to develop friendships and interests outside the family.


Older children and teenagers

As children grow up they have questions about all aspects of life and this will no doubt include Usher syndrome. Parents may chose to continue to address questions or concerns directly, or teenagers and young adults can talk to an independent person, such as a Sense worker or a youth counsellor.
This support can help a sibling to understand the changing practical needs of their brother or sister (perhaps relating to communication or mobility), and also help them to explore their own feelings about Usher syndrome.


Adult siblings

Siblings who do not have Usher syndrome may be a carrier of the faulty genes for this condition. Their vision, hearing and balance should not be affected however. In Tom’s case, his possible carrier status would not influence his medical suitability to train for a career such as a commercial airline pilot.
Brothers and sisters who want to start a family may wish to find out more about the risk of their children having Usher syndrome or being carriers. Usher syndrome is a recessive condition, where both parents have to carry the same gene for their children to be affected. The condition is relatively rare within the general population and in turn the risk of having an affected child is relatively low. However any person with Usher syndrome, or who may be a carrier, should seek specialist advice if they are concerned about having a family. GPs can refer patients for genetic counselling, which aims to help families understand more about the condition and how it is passed through the generations. 
Updated: February 2016
Review due: February 2018

First published: Thursday 4 February 2016
Updated: Monday 16 January 2017