Siblings and Usher Syndrome
February 3, 2014
by Mark Dunning
I have two children. If you read this blog frequently, you know about my daughter Bella. She has Usher syndrome and I write about her a lot. I also have a son named Jack who is now 12 years old. He does not have Usher syndrome and so I don’t write about him very often. He tends to be a supporting character in the stories.
That’s the plight of siblings without Usher. They are loved. They are not forgotten. But it is easy to let them slip in to the background when dealing with all that Usher has to offer.
In Jack’s case, this is particularly unfair. He deserves the same recognition and attention as Bella because he does some pretty amazing stuff on his own. Since I run the place, I thought I’d take this opportunity to tell you how wonderful my son is while also offering some advice about siblings of an Usher child.
Around the time Jack was born, my wife, Julia, started the Decibels Foundation to help kids with hearing loss. The core program is a playgroup/parent support group. Kids with hearing loss between the ages of 0-3 get access to a language rich playgroup with other hearing loss kids. They get both speech therapy and access to sign language. In parallel the parents meet with other parents and professionals. They can grieve for their kids while they learn about hearing loss (since most have no experience with it) and about their education and medical options. The goal is for the parents to address their emotions around hearing loss so they can make lucid decisions for their kid’s future while leaving open as many communication options for the child as possible. The program has produced tremendous results for a great many families.
Jack has been involved with the program from the start and still actively participates with the little kids in the playgroup when he can. We held a recent alumni event that was attended by about fifty people. Jack spent the day entertaining the little kids, at one point walking through the kitchen with a giggling kid on each leg and one on his back. He was wearing a kid suit.
Jack cares about these kids with hearing loss. He cares enough that a few years ago he came up with the idea for Stink Week to ‘make a stink about hearing loss’. Stink Week runs from February 2nd to February 9th. The idea is that you set a fundraising goal then you wear the same shirt every day for the week or until you meet your fundraising goal. It’s fun, it’s easy, and the kids love it. All the money goes to benefit the Decibels Foundation and the programs that Jack cares so much about. This is the third year and Stink Week has raised over $40,000 in the first two years. You can join Jack in stinking at www.stinkweek.org. Or, if you’d prefer, you can support him (and hopefully get him to change sooner).
Usher syndrome has had a profound impact on Jack’s life. He’s never known anything other than having a sibling with Usher and a mom who runs a hearing loss program. He doesn’t have Usher, but it defines who he is, at least in part. This is true of every kid that has a sibling with Usher. As you’ll see below, it forever changes who they are.
Bella has had a lot of doctor appointments over the years and this has been a source of friction for Jack. On the one hand, he sees Bella getting a day out of school. He sees Daddy taking a day off of work. And he sees his parents and Bella going in to Boston and leaving him behind. It used to upset him that he didn’t get to do that as well.
So we flipped the script and started taking him with us. That wasn’t much fun either. He didn’t want to go through the tests that Bella would go through, but at least she got to do something. He just sat there and got scolded for fidgeting or disturbing the examination.
This doesn’t even take in to account the speech therapy and the cochlear implant surgeries and the meetings with the school. We do a lot for Bella because we have to do a lot for Bella. Finding the time to do the same for Jack has been difficult.
One way we’ve addressed this is I coach his baseball team and I attend all of his hockey practices and games. I built a small rink in front of our house for the winter (ah, New England!) and we skate together once or twice a week. Sports are his special time, the compensation for all the extra time I spend with Bella. Of course, that’s not all that fair to Bella who gets a doctor’s appointment in exchange. And so on the struggle goes…
Let’s take the bathroom. There is a light switch outside the door. When one of the kids is in the bathroom with the door closed, it is very tempting to click off the light. Should Bella turn off the light on Jack, it’s annoying. Should Jack turn off the light on Bella, it’s potentially dangerous. Combine balance issues with night blindness and wet floors and she could really get hurt. So when Bella turns off the lights, she hears an exasperated ‘don’t do that, it’s not nice.’ Or, rather, don’t do that because I don’t want to hear him screaming. But when Jack turns off the light, he gets a much more serious tone and probably a lecture. ‘You can’t ever do that. We’ve talked about Bella’s issues. It’s too dangerous.’ Same crime, different punishment.
The trick is recognizing this as a parent and meting out the same punishment to both of them. Bella gets the same lecture as Jack. However, this needs to be combined with an explanation. They both know that the punishment for Bella hardly fits the crime. So we need to make it clear why the rules are the way they are. We usually do this by sitting them both down and explaining why the lights, in this case, are an issue and why we will respond the way we do. They both hear the same message, they both have the same rules, they both have the same repercussions. There is no gray area. ‘Thems the rules’, as they say in Westerns, which is fine with the kids. All they really want is fair treatment under the law.
Different rules and added responsibilities
Bella is an excellent lip reader. Jack uses this to his advantage all the time. When he is mad at her, he will mouth something nasty to her. She’ll flip out and yell which will draw the ire of Julia and me. Bella will plead for justice, Jack will claim he didn’t say anything which is technically true. So we have a new rule. No mouthing nasty things to each other.
That goes on a list of items that Jack is never allowed to do that includes 1) never whisper something behind Bella that others can hear and she can’t, 2) never touch her implants unless she asks for help, 3) never use her vision loss against her (see the turning off the lights story above). In short, Jack can never use Usher syndrome to gain an unfair advantage against Bella. We don’t like fights in our house, but if there is going to be a fight, it’s going to be a fair one.
But note that none of those rules apply to Bella. Jack doesn’t have hearing loss or vision loss. He doesn’t have cochlear implants. So he has different rules than she does and he doesn’t always consider that to be fair. I look at it as leveling the playing field, like adding weight to a racehorse to compensate for a lighter jockey. Just don’t expect your kid to agree with you on that point.
He also has added responsibilities. Bella is about as independent as can be imagined. She doesn’t want help very often. But if they are together with a bunch of kids and she is missing part of the conversation, it’s his responsibility to make sure she is included. If they are together and it is dark, it’s his responsibility to make sure he guides her. These are heavy responsibilities for a younger brother more comfortable bickering with his sister than helping her. Other kids don’t have to do this for their siblings. Because of Usher syndrome, Jack does.
Usher syndrome is scary. Siblings feel it, too. They often have anxiety. Many parents try to shield the siblings from the diagnosis but that often can make things worse. Now the sibling knows something is wrong but also feels excluded. We try to include Jack in our discussions about Usher syndrome, particularly now that he is becoming more of an adult. But even when he was younger, we tried to share an equal amount of information with him. We would just put it in language he would understand. We have found that it’s best to ask him what he wants to know. Often it’s nothing. But sometimes it’s big. When he was around seven he asked me if Bella was going blind. He had put the tidbits together and come to a conclusion that was eating at him. Kids figure it out. It’s usually best just to include them from the start so they don’t have to guess what it means.
Talking frequently about Usher also helps with feelings of guilt. Siblings do stupid things to each other. Jack has knocked off Bella’s implants. He’s pushed her only to have her topple over much more easily than he expected. He’s abandoned her in certain situations. In other words, he’s a little brother. All siblings feel some guilt in those situations. But with an Usher sibling, it’s magnified. Not only did they do something to hurt their sibling, it was amplified by the condition in a way they did not intend. When things do go wrong, we try to put a border around the scolding. You shouldn’t have pushed her, for instance, but it’s not your fault that she fell over. That’s the Usher syndrome. Just remember that next time.
I guess that’s the message for everyone. Remember all of this next time you’re dealing with your non-Usher kids. They are impacted, too. Talk to the siblings about the disease and encourage them to ask questions about it. Do it with the Usher sibling present, if possible, so there are no perceived secrets or taboos. As with anything else Usher related, don’t predict the future. The Usher sibling is not destined for anything. And, most importantly, provide hope. In Jack’s case we tell him all the time that Bella is lucky and we know she’ll be happy in the future. We know this, we say to Jack, because she has you.