When Should You Tell Your Child That He Or She Has Usher Syndrome?


by Mark Dunning

Original article appeared on Usher Syndrome Coalition Website

Thanks to genetic testing, we are now able to identify children with all types of Usher syndrome at a much younger age. This is a good thing for a lot of reasons (which you can read herehere, and here).). It also presents an unexpected challenge for parents. When should you tell your child that he/she has Usher syndrome and what, exactly, should you tell him or her? I’ve thought a lot about this since a recent conference call we had on the subject. I’ve come to the conclusion that, in my opinion, a child is always ready to learn he or she has Usher syndrome. It is the parents that may not be ready to deliver the news. This does not mean that someone other than a parent should deliver the news. I’m looking at you, doctors. Please don’t deliver the news to the parents in front of the kids or to the kid directly. Let the parents handle. But the parents should only do so when they are ready.

The vast majority of people with Usher to whom I have spoken about the subject say they would have preferred to learn they had Usher syndrome sooner than they did. Not everyone, mind you, but most. That sampling includes teenagers, young adults, and older adults.

One young lady explained it best. I’m paraphrasing here, but she said that before she learned she had Usher syndrome, she felt stupid. She would trip over things the other kids didn’t. She would be unable to see things the other kids saw. She would feel anxious in places like movie theatres when the other kids weren’t. She wondered what was wrong with her. Learning she had Usher syndrome was difficult in many ways, but it was also a relief in others. She wasn’t stupid. She had Usher syndrome. These weren’t personal failings. It was genetic. She could make accommodations to deal with Usher syndrome. She could ask for assistance or bring a flashlight and when the other kids asked why, she could tell them. It wasn’t easier, but it was better.

In my opinion, the earlier a child knows they have Usher syndrome, the better. They become more comfortable with their condition if it’s the only condition they have ever known. They are more willing to advocate for themselves if they have always done so. And the vision issues tend to scare them less if they are very young because 1) they don’t fully comprehend the implications of the disease and 2) their vision is usually pretty good at the time.

Of course, all of this assumes that the diagnosis is delivered to the child appropriately by the parent. You don’t want to explain the molecular structure of the myosin 7a protein to your three year old (Sorry Jennifer). They won’t get it. But telling them that they should take your hand when it’s dark because they have Usher syndrome and that means they might have more difficulty seeing is more than appropriate.

None of this means that a parent who has just received the Usher diagnosis for their child should scoot home and tell the child the news. You shouldn’t. Just because the child is prepared to hear the news doesn’t mean you are prepared to deliver it.

It is hard to understate the strain the news puts on parents. They are in a terribly fragile emotional state for months afterward. Most parents of children with Usher syndrome (and all types of hearing loss, for that matter), do not have a history of Usher syndrome in their family. Few have ever heard of it much less met someone with it. They don’t know what to expect. All they know is that they were told their child had hearing loss and would at some point have vision problems. They hear deaf and blind and think of the lonely child being picked on in school or the homeless man with the cup full of pencils. They feel frightened, uneducated, unprepared, and impotent. In short, they are not in a good position to give sound advice.

So when should you tell your child he or she has Usher syndrome? I think there are two milestones to pass before you discuss it.

Discuss Usher syndrome with your child when you are emotionally prepared to do so.

This isn’t the movies. You don’t need to have the sobbing scene where you choke out the news to your child. Kids can sense when you are upset and it makes them upset. I’ve smashed my thumb with a hammer and started cursing only to see my son go screaming and crying out of the room. When you are hurt, they are hurt. If you are scared, they are scared. Make sure you have your emotions in check before you discuss it.

This does not mean you should be cold or flippant about it. You just shouldn’t turn to jelly before you get to the second syllable of Usher. That’s not as easy as it sounds when you’re looking into the searching eyes of your child.

Just remember that they probably already suspect something is up. They may not know the whole story but they overheard you talking to your husband or your mother or your best friend. They saw you searching the web or reading a pamphlet. They caught you crying in the bathroom or grumpily polishing off a second box of Ho-Ho’s.

I had another young lady tell me the following story. Again, I’m paraphrasing. “My Mom told me that she had something important to tell me. She started to cry which made me start crying and that made my mom even more upset. We were sobbing in each other’s arms and she told me I had Usher syndrome. We cried some more and then I asked her what she wanted to tell me. What was upsetting her so much? My Mom was confused. She said ‘I just told you. You have Usher syndrome.’ I was SO relieved. I already knew that. My brother had heard my parents talking about it a long time ago and told me. I thought I had cancer or something.”

In short, if you are still lying awake every night with tears in your eyes you are probably not ready. You child is ready. You are not.

Learn everything you can about Usher syndrome.

Once you are able to pull yourself off the floor, you should spend some time educating yourself before you talk to your child. This is helpful for a number of reasons.

1) You’ll find out that a diagnosis of Usher syndrome is not the hell you first expect it to be. It’s scary, sure, but most Usher adults are happy and successful. Your child did not get a death sentence. This will help to keep you from losing it when you talk to your child about the diagnosis.

2) You’ll learn that there are a lot of things you can do to help your child right now and that there are even more potential treatments in the pipeline. This will help you should your child get scared when you talk about the diagnosis. It’s good to focus on a hopeful future.

3) You’ll learn how a child with Usher can live safely with few restrictions. Nothing upsets a kid more than to hear that a) they have Usher syndrome so b) they can no longer ride the bike they just rode that afternoon. They can do anything. They just need to be smart about it.

4) You will feel confident that you can answer any question that your child might ask. And remember, ‘I don’t know but I can find out’ is always a good answer.

At that point, grasshopper, you are ready.


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