In this guest blog post, John Sanders, CVI Society Associate has written an imaginary scenario of a hospital appointment from the perspective of the family of a child with nystagmus and CVIs. Consultant Emma and Orthoptist Atif offer some great advice and reassurance to Mia's mum and dad.
'From the thousands of conversations and email exchanges I've had over the past 30 years with parents of children who have nystagmus (and adults with the condition too) one thing stands out. After receiving a diagnosis of nystagmus most people leave the eye hospital with more questions than answers. The following is a suggestion for how a conversation between patients and professionals might go. Some cases are of course more complex than the fictional dialogue below. But the example is based on typical reports from parents where nystagmus is the "only" diagnosis or a main part of the diagnosis.'
Evidently an additional diagnosis of cerebral visual impairments (CVIs) adds another layer to the mix. Many of the issues for children with CVIs are also the same for children with nystagmus. People who have nystagmus may also have excellent acuity (clarity of vision) just as is the case for many with CVIs.
I would like to thank Professor Chris Harris of the University of Plymouth and Nikki Espiner of the Nystagmus Family Foundation for their help in writing this article.
Ophthalmologist Emma: Thanks for coming in and sorry you had to wait so long. Last time we saw Mia we talked mainly about the fact that she has Cerebral Visual Impairment-- or CVI as it's generally known. But you also have questions about nystagmus,don't you? Don't worry. You're not alone. Most parents are confused by nystagmus. And, to be honest, we're still learning about it in the medical profession too. But Atif and I will do our best to give you answers.
Mia's dad: Thanks. I suppose the first question we have is: do you know why Mia has nystagmus?Obviously we've searched nystagmus online and seen that it can occur with a lot of conditions, some of them quite worrying.
Ophthalmologist Emma: Yes, I know. And I'm very aware that parents - quite understandably -- get anxious when they read about nystagmus. Fortunately, in Mia's case, I can reassure you that none of the tests we've done suggest anything other than nystagmus and CVI. It's true that we still don't know precisely why Mia has nystagmus. But it's highly unlikely that there's any other serious underlying cause or condition. Other than nystagmus and CVI, Mia is a healthy 18 month old.
Mia's mum: Something else we wanted to ask too. You've done the electro-diagnostic thingummy (1)
Ophthalmologist Emma: Yes, we've done the VEP, ERG and OCT (2)tests and they've ruled out lots of possible causes and linked conditions. Based on what we know about Mia, her symptoms, the way her eyes move, etcetera, it's very, very unlikely that an MRI scan would tell us anything we don't know already. And there is a slight risk from the anaesthetic we'd have to give Mia for an MRI. However, to be on the safe side, I suggest that I refer Mia to a bigger hospital for an eye movement recording test which we can't do here. It's a very simple and safe test. All Mia has to do is look at a screen and cameras will record her eye movements. Hopefully that might show us why she has nystagmus and that the cause is quite benign. In the highly unlikely event the test shows something else, then the sooner we know about it the better, because sometimes we can treat underlying conditions.
Mia's dad: If, as you say, it's probably a harmless, benign cause, then how will knowing why Mia has nystagmus be of any use to us? I'm just thinking: do we really want to put her through another test?
Ophthalmologist Emma: I take your point and the choice is yours. But knowing why Mia has nystagmus does have benefits. Firstly, most parents tell me they find it really helpful simply knowing why their child has nystagmus. It's much easier to talk about nystagmus with family and friends for instance if you know what the cause is. And I know from experience that people will ask you that question. Secondly, if we know exactly why Mia has nystagmus, we can tell you more about how it's likely to affect her vision when she's older. Thirdly, in the future,there may be treatments for certain kinds of nystagmus. So, if we know now why Mia has nystagmus then as treatments hopefully come along we'll know whether or not they might work for her.
Mia's mum: We'll definitely go for the eye movement test then. But you just mentioned something else we wanted to ask: what can you tell us now about how nystagmus will affect Mia in the long term?
Ophthalmologist Emma: Well, the first thing to say is that Mia can see a lot. As I'm sure you can tell from how she behaves around you and at home. But my colleague Atif can perhaps tell you more than I can, as he's tested Mia's vision.
Orthoptist Atif: That's right. Mia is of course too young to read letters on an eye test chart. But as you know we've done other tests showing her pictures and patterns which give us a good idea of what Mia sees. Her vision is fairly typical for a child her age with nystagmus. And, if anything, Mia's distance vision may yet improve a bit over the next few years.
Mia's dad: But from what I've read on the Internet, Mia probably won't be able to drive, will she?
Orthoptist Atif: It's too early to be sure, but you're right. It's probably wise to work on the basis that Mia won't drive. Now, I know that probably comes as a shock,but most adults with nystagmus tell us it's not as bad as people think. They know from childhood that they'll be unlikely to drive, so they factor that in when they're choosing where to live, what kind of job to do and so on. When you're ready I suggest you talk to adults with nystagmus. They can probably do a lot more than I can to put your mind at rest about issues like driving.
Mia's mum: That's OK. We'd sort of worked out the bit about driving. But, apart from distance, how else will nystagmus affect Mia's vision? It's really frustrating just now, because she's too young to tell us. And we want to help her if we can.
Orthoptist Atif: You've told me before that you've noticed Mia's eyes flicker more when she's tired, hungry, upset, anxious or in strange places. The more her eyes flicker, the less well she sees. That means that what she sees changes throughout the day. So, although nystagmus in children isn't usually a degenerative condition, it can vary a lot from hour to hour. Simply being aware that how well Mia sees is likely to vary quite a bit means you can help her a lot. For instance, let her rest if she's tired. And whenever you go anywhere new describe it to her. It will also probably take Mia longer to see things, especially in busy or cluttered surroundings. And it's very hard for people with nystagmus to scan.So, help her by pointing or telling her which direction to look. And, there are lots of other little - but important - things you can do. For example, what every parent learns is: stand in the same place when you pick her up from school. That makes you easy to find and will stop her panicking that she can't find you.
Mia's dad: And what about this null zone or null point Mia has? I'm still struggling to understand that.
Orthoptist Atif: Don't worry. Everyone does at first. Think of the null zone like a torch beam in a dark room. You see best where the beam of light points. The null zone is a bit like that. Mia sees best in a narrow beam to her left because that's where her eyes move least. The only way she can move that beam - the null zone - is by moving her head. That's why she holds her head to the right to look straight ahead. She's not aware she's doing it. That's simply how it's most comfortable for her to see. It's just like I'm right-handed, so I never even think about picking up a pen with my left hand. But, of course, the null zone means that Mia's field of vision is a bit different from people with ordinary vision.
Ophthalmologist Emma: If I could just add another important point we sometimes forget to tell parents. Most people with nystagmus say they generally have a stable image of the world around them. But, as Atif said, if they're tired or stressed - or if they can't look at something using their null zone - then for a while at least they may see the world jumping about.
Mia's mum: So, nystagmus doesn't just affect how far Mia can see, but how quickly she sees ,how well she can see in different directions and it changes depending on how she feels? And sometimes the world isn't still for her. Nystagmus is much more complicated than I realised.
Ophthalmologist Emma: I know it's a lot take in at one go. Atif has some printed information you can take away explaining all these things. There's also useful information online - try the Nystagmus Network for instance. And we'd like to see Mia every 6 to 12 months for the time being too, so you can ask us questions at those appointments. Sadly, there are no miracle cures or treatments for nystagmus as yet, but the more you understand it, the more you can do to help Mia.
Orthoptist Atif: That's absolutely right. In our experience, parents can usually do far more than they think. I'll give you information about the local specialist teachers who work with children who have a vision impairment -- that's a horrible phrase I know, but parents get used to it. And I'll put you in touch if you want with parents of older children with nystagmus. Almost every parent I know tells me that talking to other families in the same situation helps more than they ever imagined.
Mia's dad: Going back to education for a moment. Will Mia be able to go to a mainstream school? What support is available? Will she be able to see the board, take part in sports? There are just so many questions. What should we be doing and when and where do we start?
Orthoptist Atif: Taking your questions one at a time, there's no reason why Mia shouldn't go to a mainstream school. Support is available in theory, although parents tell us it's often harder to get in practice. So, one thing we can do to help you is give Mia a Certificate of Vision Impairment, or a CVI as it's known. That will strengthen your case when asking for support for Mia in school. I'll give you the contact details for the people you need to talk to about support in school. Being honest about it, children with nystagmus do often struggle with somethings at school. Like you say, seeing what's on the board, taking part in sport, even things like seeing what's for lunch in the school canteen or taking part in assembly are all often much harder for children with nystagmus and CVI (cerebral visual impairment) will also make life much more challenging at times. But the specialist teachers - and parents - tell us there are ways around all these challenges.
Mia's dad: That's helpful. We hadn't really thought about those little, everyday things. It shows how much there is for us to think about. But you've answered some of the big questions we had. And it's good to hear about the steps we can take to help Mia. At least the future doesn't sound quite as scary now. So, thank you both.
Ophthalmologist Emma: That's OK. We know it's a worrying time for parents of very young children. But honestly most of the children we see with nystagmus grow up to lead ordinary, normal lives. I'm not saying it's always easy along the way. As Atif says, you might have to work hard to get Mia help in school. Every parent I know says they're forever having to remind teachers about how nystagmus affects their child. But remember that support is available and we can point you towards it.
Orthoptist Atif: And there are a few more practical things we can do as Mia gets older. Glasses, for instance, if Mia needs them. And we can also refer you to people who can advise you about magnifiers and technology that might help.
Ophthalmologist Emma: But for now the main messages we'd like you to take home are that firstly, nystagmus is not the end of the world; secondly, we're always here to answer your questions and signpost you to other sources of support; and thirdly - and above all -- there's a lot you can do to help Mia on a daily basis. You're with her every day, so you know what she can and can't see better than people like Atif and me. In many ways that makes you the experts.
(1) Electro-diagnostic tests often used to try to find out why someone has nystagmus are VEPs (visual evoked potential) and ERGs (electroretinogram).
(2) OCT (optical coherence tomography) is a non-invasive scanning or imaging technique for examining eyes.
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