null point Surgery – Nystagmus Network

Your nystagmus research questions answered – question 9

We asked a group of Nystagmus Network supporters what questions they would most like to put to nystagmus researchers. Then we found researchers to answer them.

Your questions were answered by Jay Self (JS), a Consultant Paediatric Ophthalmologist at University of Southampton and nystagmus researcher and Helena Lee (HL), a Consultant Ophthalmologist at University of Southampton and a nystagmus researcher.

Question 9: How can we best improve the quality of life for those with nystagmus?

(HL) Empowering patients and their families, making sure they have the information they need to understand their condition and that needs to be passed on to their schools or their employers on how best to optimise their circumstances. These are all little things that don’t require a pill or a prescription, but make a big difference. If, say, a child is put into the right place in front of the whiteboard for their null point or given extra time or things are blown up or they’re given an i-Pad. All these little things make a big difference before we prescribe anything or do anything else.

Then there are little things like optimising your glasses, making sure they’re the best they can be, with tints if you need them or checking your vision in the dark if you have a retinal dystrophy, or checking whether bright light makes a difference. It’s actually about understanding all those little things. Then you can get on to other things like contact lenses, surgery for null point if necessary, trying treatments such as the ones we try for acquired nystagmus and for congenital nystagmus. Sometimes there’s prism treatment. There’s quite a lot of stuff that can be done in your local clinic that doesn’t require anything very special, but just requires understanding of the condition.

(JS) I would agree. A lot of it boils down to information sharing support and also doing all the normal stuff in a timely way. I totally agree with the glasses correction. It’s easy to put that to one side when people are stressed about getting the genes tested. You’ve got to do all the normal things we do in a timely way and in a bespoke, sensible way.

A final thing is a massive thing that the Nystagmus Network can do, which they have been doing for the last few years, which is really celebrating good news stories, which I don’t think was a massive focus a few years ago. If you speak to David Katz or Richard Osman they almost say that ‘nystagmus made me,’ ‘it’s made me do the things I’m doing and it’s actually given me super powers.’ You cannot push that message too much, especially when you’ve got new mums with little babies and they think it’s the end of the world.

The Nystagmus Network is enormously grateful to Jay and Helena who gave up their time on a sunny Saturday afternoon to answer questions from the nystagmus community so openly and fully.

Your nystagmus research questions answered – question 3

We asked a group of Nystagmus Network supporters what questions they would most like to put to nystagmus researchers. Then we found researchers to answer them.

Your questions were answered by

Jay Self (JS), a Consultant Paediatric Ophthalmologist at University of Southampton who has been researching nystagmus for around 15 years since studying nystagmus for his PhD. He runs specialist nystagmus clinics and researches primarily cause and diagnosis and treatment of albinism since around 50% of his nystagmus patients seem to have albinism.

Helena Lee (HL), a Consultant Ophthalmologist at University of Southampton and a nystagmus researcher, currently working on developing a treatment for albinism.

Question 3: Would it cause more problems to stop the eye wobble?

(HL) We have very poor understanding of nystagmus, why it occurs and why it’s happening in the first place. We do know that for adults who acquire nystagmus, it’s horrendous because they haven’t compensated for it. The entire world moves around them. So for them it is worthwhile stopping the wobble, because their visual system has developed to work with eyes that are steady.

In some children with nystagmus, we know that when we perform certain kinds of surgery for their null point so that their eyes can be more steady, it does seem to improve their visual function. Just this experience would seem to suggest that reducing the wobble helps to some degree. Eliminating the wobble entirely is a difficult one to answer, because in how many people have we managed to do this? And how can we ask them whether they’re better off with or without it?

(JS) I would agree. I think it’s unlikely that, if we stopped it, it would make visual function worse. People have done experiments with gaze contingent visual tracking so that they look at a screen and wherever their eye looks the image moves to it, so in theory they are simulating removing the nystagmus. It does seem to help with speed of seeing. They haven’t reported having any specific problems, but obviously that’s a simulated scenario. I think it’s unlikely.

The question probably arises because we’ve talked for years about nystagmus being a compensation mechanism for something going wrong. One way of thinking about it is that it’s not an effective compensation. It doesn’t really achieve what it’s trying to achieve, as far as I’m aware.

We will publish more of your questions and the answers Jay and Helena gave over the next few days.

The Nystagmus Network is enormously grateful to Jay and Helena who gave up their time on a sunny Saturday afternoon to answer questions from the nystagmus community so openly and fully.

Guest post: Harry’s back at school!

Over the summer holidays we followed Harry’s progress as he underwent the Anderson Kestenbaum procedure to address the head turn associated with his nullpoint. Today we hear from Harry’s Dad, Ian, how Harry is getting on, 4 weeks after his operation.

Ian writes:

“Harry’s eyes are now nothing but a slight irritation and, apart from eye drops 4 times a day, he doesn’t need any pain relief. Other than a bit of redness around the edges, his eyes show very little sign of the trauma they’ve been through. Harry says they’re still itchy. This is because the stitches still haven’t dissolved and they won’t until around 8 weeks post op.

“Harry’s vision is on a par with pre-op which is really pleasing. He still needs to wear glasses, but now minus the prisms.

“We attended a meeting yesterday with the consultant. 4 weeks post op he was delighted with the healing process (Harry is about 80% through the recovery) and with the results of the operation. I have to say my wife and I and, even more importantly, Harry are delighted with the outcome, too. Harry doesn’t need to see the consultant for 6 months now, so, in effect, he’s signed off.

“The photo was taken the day before his meeting with the Consultant, his first day back at school. You will see he’s looking straight at the camera.

“Even though we had taken our time and researched and thought we knew what to expect, seeing your child going through this surgery is tough. The first few days, in particular, are not nice. There’s the uncertainty over the success of the operation and the torment of seeing your child suffering (Harry was a very good patient). On the Sunday after the operation, when Harry expressed some concerns, we found it really difficult. You start doubting whether you’ve made the right decision and question whether it would have been better to leave well alone. So I don’t want to make it sound likes it’s been a walk in the park, it really isn’t. However, when you see photographs like this, with a beaming smile looking straight at the camera then it does make it feel it was worthwhile.

“Whilst this has been a roller-coaster of a journey, we’re so pleased we have done this. I also know this won’t work for everyone. I just hope Harry’s experience can help others, considering this course of action, understand just what it entails.”

The Nystagmus Network does not endorse or otherwise any particular medical treatment. We are simply sharing one family’s experience. We are grateful to Ian and Harry for sharing their story so generously with us and we wish Harry well as the new school term begins.

Guest post – Harry’s recovery from surgery continues

Harry and his family continue their post surgery journey.

Ian writes:

On day 7 after the op Harry is able to open his eyes for longer periods, the blurry vision is settling down and he is able to see much better. He is still having to have eye drops 4 times a day for the next month and is taking pain relief still. He is really positive about the outcome, although he is a bit bored, spending his summer holidays stuck in the house.

In terms of the pain, Harry has been really brave and coped with it well. I would say that during the first 2-3 days he has been in a lot of discomfort, particularly when trying to open his eyes. Now he would describe it as an ache and a more irritating, itchy feeling (the stitches in the eye). Trying to stop him rubbing his eyes has been a challenge.

After 13 days Harry is able to open his eyes pretty much as he did prior to the operation. The soreness around his eyes has calmed right down. He is still a little bloodshot, but his eyes look so much better than they did the first 7 days. Harry has commented that the pain (which is bearable and more of an ache) is less at the front of his eyes, but more of an internal ache from the back of the eye.

His vision is settling down and he thinks it’s now similar to pre-op, but looking straight ahead.

Harry is getting used to a change in his peripheral vision, following the change of eye position. The consultant said this is natural and his brain will retrain to adapt to this shortly.

The best news is his ‘null’ point is still there, but in the new eye position.

This is the end of Ian’s account, for the moment, of his son’s recovery from null point surgery. The family wanted to share the experience with others. Null point surgery is not for everyone. Please seek advice from your ophthalmologist. The Nystagmus Network does not endorse or otherwise any particular medical treatment. We are simply sharing one family’s experience.

We wish Harry a full recovery.

Guest post – one week after Harry’s null point surgery

Harry has undergone the Anderson Kestenbaum procedure this summer to address his marked head turn. His Dad, Ian, has asked us to share their experiences. A few days after the operation it was time for father and son to have a chat about how things had gone.

Ian continues:

On the Sunday post operation I had a catch up with Harry and he mentioned some concerns he had about the success of the operation, which worried me, too. So I emailed the consultant on Monday morning with a list of these concerns. I wanted to be able to give Harry comfort, but I didn’t know the answers myself. The consultant immediately made us an appointment to go in to see him. He spent 20 minutes with Harry, examining him and discussing all the concerns. The outcome of this assessment was that the operation so far appears to be a huge success and the couple of concerns Harry had are just things that will settle down in time.

These are some of their concerns and responses from the consultant:

His actual head posture was turning slightly to the other side now (by about 5 degrees)

The consultant advised that this is fine. When his muscles settle down post op they will move towards the original position. In 2 months’ time we should pretty much see a straight head posture.

When Harry was opening his eyes slightly and not focusing on anything his eye was turned in towards his nose on the right side.

This is part of the new eye position. Once Harry can open his eyes properly, without pain, his eyes will naturally find their null point with his head looking forward. This is fully expected and no concern.

Harry noticed his peripheral vision to the right was vastly reduced.

This again is naturally going to happen as a result of the new eye position, but, once Harry is fully using his eyes, his brain will adapt to this and he will automatically turn his head to see. In theory he had poor peripheral vision on the other side when he turned his head, so this is just something new.

Null point surgery is not for everyone. Please seek advice from your ophthalmologist. The Nystagmus Network does not endorse or otherwise any particular medical treatment. We are simply sharing one family’s experience.

Guest post – Harry’s null point surgery

Ian is sharing his thoughts and experiences as his son undergoes Anderson Kestenbaum surgery this summer to address the head turn associated with his null point.

In this post he recalls the day of the surgery:

We had to be there at 8.00am and Harry was in really good spirits. He went down to the operating theatre about 10.45am. Then it was the waiting game for my wife and me. He was wheeled in to the room from recovery around 2.00pm, much to our relief. He was very groggy still and couldn’t open his eyes, but we were hugely relieved to hear that he had opened them and had been able to see the anaesthetist standing over him. That was my first fear as a parent overcome. Could he see? Tick. Yes, he could.

Harry felt sick after the operation. Apparently that’s common for people who’ve had surgery on their eyes. He was sick around 4.00pm and also felt dizzy. At this stage they were considering keeping him in overnight. Harry hadn’t opened his eyes yet, as they were really sore. The consultant who operated on him came to the room and got Harry to sit up on the bed and look at me holding some large letters about 4-5 metres away. Harry could open his eyes only for a few seconds before they hurt too much, but, again, to our relief he was able to read what was in my hand, albeit it was a bit blurry at this stage. Even more pleasing was his head posture. Immediately it was noticeable that he was looking straight at me. No head turn. Another tick in the box and another of our fears overcome. After this he seemed to come round and we got him home that night.

His eyes were so sore to look at. He has been super brave, but we know they have been really painful for the first 2-3 days in particular. He has been able to open his eyes only for a second or two. The rest of the time he’s been lying with his eyes shut, as this is more comfortable. His eyes did look very sore. Not a sight for the squeamish.

Guest post – questions for Harry’s consultant

Harry and his parents had many, many questions before finally deciding to go ahead with null point surgery. Here are just some of the questions the family asked, with the answers they received from the surgical team:

Is this a complicated procedure?

Yes. Operating on eyes in general is complicated, but when 2 eyes are to be operated on at the same time it is a complex procedure.

How long does the surgery take?

From going down to the anaesthetist to coming back from recovery takes about 2 and a half hours, between 1 and a half and 2 hours for the actual surgery

What are the risks involved?

The null point may not be picked up again by the brain in its new position, meaning we lose the one thing Harry has to control his nystagmus.

Double vision can result from the eye realignment not being exact. Damage can occur to vision during the operation. Plus there are all the usual risks associated with surgery. In addition the consultant was keen to point out that there is a chance that we go through the surgery and, over time, Harry’s eyes move back to their original position.

What is the recovery timescale?

Everyone is different, but expect soreness for the first couple of weeks, which will ease over time. Full and final results of head posture will be known around 2 months post op when all the swelling has settled down and the brain has worked out its best position to see.

Guest post – Harry’s null point surgery decision

15 year old Harry has undergone the Anderson Kestenbaum procedure this summer. Parents often ask what null point surgery involves. Thorough consultation with the ophthalmologist and the surgical team are key. Harry’s Dad, Ian, asked us to share his son’s experience.

Ian, writes:

My son, Harry is now aged 15 and was born with idiopathic nystagmus. Luckily for us all, Harry has relatively good vision and has a null point which enables him to control the nystagmus and see. The downside to this null point is that he has a 40 degree head turn.

My wife and I made a decision when Harry was young that we would not consider surgery on his eyes and that if this ever happened it would be because Harry wanted to and was of an age that he could sensibly make that decision.

As Harry grew up he tried wearing glasses with prisms to help straighten his head posture, but a combination of constantly losing them and being conscious about wearing glasses with prisms meant that this wasn’t a particular success.

Harry went to the opticians about a year ago on his own and during his chat with the optician the topic of head posture came up. The optician made a referral to Ophthalmology. Over the next 12 months we had multiple meetings with the senior ophthalmologist and after many discussions and questions we agreed to pursue the Anderson Kestenbaum procedure for Harry.

Please see Ian’s next post for just some of the questions the family had and the answers they received from Harry’s consultant.

Introducing Harry’s story

This is Harry. He’s 15 years old. He has nystagmus and, as you can probably tell from the photo, he also has a fairly marked head turn associated with his null point. This summer, Harry and his parents decided it was right for him to undergo surgery.

Known commonly as ‘null point surgery’, because it seeks to move the null point to a more natural, central position rather than off to the side, the Anderson Kestenbaum procedure is sometimes suggested by ophthalmologists where head turns are very marked. Medical professionals do not claim that the surgery can cure nystagmus, simply that it can enable the patient to look straight ahead afterwards.

The procedure involves detachment and reattachment of the eye muscles. As with any surgery, there are risks involved. The result is usually a more natural head position so that the patient can look straight ahead to focus on faces and objects. Sometimes they report that they can read a line or so further down an eye chart. Follow up surgery is sometimes needed to refine any correction. Some people perceive a slowing of the eye movements as a result of surgery, though this is thought to be anecdotal and could simply be due to the change in the position of the null point.

Harry has now had his surgery. His family asked the Nystagmus Network to share their story. Over the next few days we’ll be posting reports from Ian, Harry’s Dad, as Harry recovers from his null point surgery.