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Tuesday, March 15, 2011

What You Should Tell Your Child About Usher Syndrome

by Mark Dunning

In the last post we discussed when to tell your child that he or she has Usher syndrome. The conclusion of that post, in short, was when you are emotionally ready to discuss it, discuss it. I don’t care if your child is 15 months or 15 years. Talk about it as soon as you, the parent, can handle it because your child is already able to handle it. You just learned your child has Usher syndrome but your child has had Usher syndrome their entire life.  It's new to you.  It's not new to them.  They just don't have a name for it yet.  I can’t tell you how many Usher adults I have spoken to who either a) wished they had talked with their parents earlier and/or b) felt some level of resentment that their parents kept the diagnosis from them for a period. Remember, the most important thing in a parent-child relationship is trust and honesty.


Of course, when you do speak to your child about Usher syndrome, you don’t want to freak them out. I can give you some guidelines on what to say, but I can’t tell you the specific words or tone to take. That’s because every child and every situation is different. How and what you would say to a teenager is different than what you would say to a young child. Kids have different personalities and families have different ways of speaking to each other. I’ve known perfectly functional emotionally stable families that love each other who were most comfortable when they were yelling at each other. I can’t tell you what works best. It’s your family.

But I can give you advice on the message you should deliver. So here goes:

Be positive and don’t put limits on them.

Usher syndrome will not prevent your child from living a happy life. Don’t give them the impression it will. There is no reason that they can’t accomplish anything they want to do. When my daughter was born deaf I assumed she would never appreciate music. Now she sings in the chorus. She’s probably not going to win American Idol any time soon, but if I had told her she’d never be able to sing she might never have tried and I would have been wrong.

What to say? “You can accomplish anything you want to accomplish. You just might have to work harder than most to achieve it. But if you really want to accomplish something, if you really are committed to it, no matter what it is, you can do it.”

Now somewhere out there a reader is saying, um, my kid is NOT going to be able to be an airline pilot. I strongly disagree. It might not turn out exactly as they expect, but who’s to say the technology doesn’t change enough to allow them to do it. Heck, if your child is committed enough to doing it, they might be the one that invents the technology that allows them to do it. Don’t limit what they can do.

Tell them the truth and don’t try to predict the future.

The truth does NOT include telling them they will go blind. That is the future and the future is not the truth. The future is a possibility. And don’t even get me started on what, exactly, constitutes blindness. The truth is today and only today. Here’s the truth I told my daughter:

“You have Usher syndrome. You were born with it. It’s not catchy so your brother and Mommy and Daddy won’t ever get it. It’s the reason you are deaf. It’s the reason you have trouble with your balance. It’s the reason you have trouble seeing at night. It also means that your eyesight might get worse over time so we need to see the doctor regularly to check your eyes.”

That’s it. That’s the truth of Usher syndrome as it pertains to my daughter. My daughter might have severe vision issues later in life. We might also find a cure and she never loses her vision. I can’t predict the future. When you tell your child they have Usher syndrome is not the time to turn in to Nostradamus.

Talk about it a lot and talk about it a little

I told my daughter she had Usher syndrome when she was 9 years old (because she was diagnosed when she was 8). She didn’t have any questions about it at the time because, as I said, she was 9 years old. She just went back to playing with her model horses. But that wasn’t the end of the conversation.

When she wanted to go trick or treating with her friends, we said “sure, but remember, you have Usher syndrome and you don’t see well at night so be careful.” When her class held a roll-a-thon to benefit the Decibels Foundation (and raised $20K, by the way), we talked about Usher. we said, “It will be noisy and dark and you’ll be on roller skates. You have Usher syndrome so that will be hard. Do you want to do it?” She did and was better than half the other kids.

The point is we talk about Usher syndrome all the time. We make it part of our lives but we don’t dwell on it. It’s easier to discuss in short snippets. Someday, maybe, she’ll want to have a long conversation about it. If she does, fine. We’ll talk about it for as long as she wants, but that will be her call.

It’s OK to say ‘I don’t know’.

We’re all dummies when it comes to Usher syndrome. No one knows everything about the disease. When your child asks a question and you don’t know the answer, don’t make one up. Tell them you don’t know. Then tell them that you know how to find the answer, because you do know how to find the answer. You know how to get in touch with the leading experts in the world and if they don’t know, well, no one knows. Then contact the Coalition for Usher Syndrome Research. They know all the experts.

Use language to which your child can relate

This part is for Jennifer. If you are a molecular biologist and your child is comfortable discussing the Myosin 7a protein at the age of 3, well, have at it. Most kids won’t get it. Use metaphors they understand. My son Jack asked if Bella was going to go blind. He likes sports. I told him that was a possibility, but Bella had a long time before that would happen and a lot could change. I used the analogy that we were only in the second inning when it came to Bella and you can’t tell how a game will end in the second inning. I’d like to think he understood that language, but by the time I finished my sentence, he’d changed the subject to Pokemon. Which brings us to…

Why are you crying?

You are most likely taking this harder than your child will. I fretted over telling my daughter for months. When I told her, it was over in 30 seconds, she shrugged, and that was it. You just learned that your child has Usher syndrome. You think something has changed. They’ve lived with it their whole lives. Nothing is different from their perspective. Keep it that way. By the way….

Don’t limit them!!!!

Did I mention this already? I did. You know why I’m repeating it. BECAUSE IT’S IMPORTANT! A young woman I know said it best. Again, I'm paraphrasing.  She said yesterday you didn’t know I had Usher syndrome. I was allowed to ride my bike at night and I was fine. Today you know I have Usher syndrome and suddenly it’s too dangerous. What changed from yesterday to today besides what a doctor told you?

Exactly. Remember the diagnosis of Usher syndrome is just a diagnosis. It’s not a fortune. It’s not a sentence. To your child Usher syndrome is the only thing they have ever known. Just because you’re putting a name to it and calling it a diagnosis doesn’t mean that anything has really changed for your child. Remember that when you talk to them. Their star hasn’t faded. They can still be anything they want to be.

Friday, March 4, 2011

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

by Mark Dunning

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 here, here, 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 (you can read the transcript here). 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. In the next post I’ll give some advice on what you should say and how you should say it.