The Best Time

March 28, 2013

by Mark Dunning

Here is a simple question for you. How old were you when you learned you had Usher syndrome and would you have wanted to have known at a younger age? 

Just to be clear, I’m not asking “Is it important to diagnosis Usher syndrome in children at as young an age as possible.” I already have strong feelings on that point (the answer is “yes, it is important to diagnosis children with Usher at as young an age as possible”). No, I’m asking about when you first knew for certain that you had Usher syndrome.

You remember that day. I know you do. Maybe you felt like someone tore your heart out. Maybe you were stunned, stunned for days, for weeks, unable to process what it meant. Or maybe you dismissed it. You could see perfectly fine. What did the doctor know? Or maybe you were relieved. You knew there was something wrong, even when others didn’t believe you. Now you knew. It had a name. Maybe you were even empowered by the knowledge, able to address the condition now that it was defined. 

You may have reacted in any number of ways, but I know you remember it. I know the day you learned you had Usher syndrome is stabbed in to your memory like a banner on a hill. 

What I’m asking is, knowing now that you have Usher syndrome, knowing how it felt to find out, would you have wanted to have known the diagnosis earlier than you did?

Your answers are important. They are important to parents. They are important to physicians. They are important to school systems and communities and extended families. And they are important to me. 

I know of a number of families that have gone to great lengths to protect their children from the diagnosis. Just think of how hard that is to do these days. The doctor can’t say anything. The school system, if they know, can’t say anything. That means the guidance counselor, the nurse, the teachers, the principal, the special education director, all of them, have to act in the best interest of a kid with Usher while being careful to never give a hint of the diagnosis to the child. Aunts and uncles and friends can’t ask about the child’s diagnosis at family events without first making sure the child isn’t around. And as the child gets older and becomes more curious about themselves, they have to somehow be kept from finding out on Wikipedia or Facebook or, gulp, this site. I can’t tell you how many stories I have heard have ended that way, with the kid confronting the parents about something they read on the internet. 

The reasoning is sound. The parents want their child to grow up happy. They don’t want to burden the child unnecessarily. Sometimes they succeed. Sometimes the child even makes it well in to adulthood unaware of their condition.

 

But is that the right course of action? 

I ask this in part for myself. My daughter, Bella, is now 14 years old. We have been talking to her, in dribs and drabs, about Usher syndrome since she was first diagnosed six years ago. She goes to the Usher Syndrome Family Conference every year so she knows a number of adults with Usher syndrome and has a whole host of friends with Usher. She is on a first name basis with several guide dogs. There are no secrets for Bella.

She seems to handle the knowledge perfectly well. I have never seen the slightest hint of any emotional damage the diagnosis has caused her. In fact, I feel her knowledge of it has made her happier. She loves her friends with Usher. They understand her. She has a reason for her difficulties and she can address them. And those around her can support her when she needs it and marvel at her when she doesn’t it. She is a happy kid. 

Still there are times I wonder how well the diagnosis sits in the recesses of her mind. Bella talks in her sleep. We can hear her in the next room babbling away just about every night. My wife does it, too, so it’s not that strange for us. Every night is like a cocktail party in our house. Usually it’s just mundane drivel. Sometimes it funny, like when she shouts at her brother Jack for something he has done in a dream. Sub-conscious Jack is every bit the tease that real life Jack is.

But some nights are different. Sometimes, once every couple of months maybe, Bella will wake up screaming for me or my wife. It is heart stopping to awaken from a dead sleep to your child screeching for help. Invariably, though, Bella is surprised when we thunder in to the room and shake her awake. ‘I was just dreaming,’ she’ll say as she waves us away, ‘what is the big deal?’ 

And she is probably right. There are a million stresses on a teenage child. Any one of them could be leaking out in the form of a scream in the night. Usher syndrome is to Bella what mortality is to all of us. It’s something terrible that is supposed to happen far in the future, but it also so incomprehensible that she just tucks it away and ignores it most of the time. But I wonder if, in the dead of night, when she is no longer holding back, if her fears about her future leak out.

So what do you think? You know what it is like to live with the diagnosis. Either you have it or a family member does. Is it better to know at a younger age or an older age? Would you have preferred to have known earlier or not? I’d love to read your answers. You can leave them in the comments below or, if you’d like to do it more privately, you can e-mail me at m.dunning@lek.com.

I look forward to your responses.

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