NB Online
Eye health special Issue 1
A collection of NB’s
best eye health and
research features
for professionals
rnib.org.uk/nb
Contents
Welcome
03 Guiding you on nystagmus
Essential information and causes of
this eye condition
Eye health special
For many years now, NB
has been providing eye
health and sight loss
professionals with
essential information
about different eye conditions.
05 Shining a light on eye cancer
Understanding the red reflex test can
be key to detecting retinoblastoma in
children
09 Marfan syndrome explained
What you need to know about a
complex genetic condition that affects
the eyes
Now, for the first time, we have produced
a number of our well-received eye health
articles and put them all in one place for
you to read at your leisure and to share
with your patients and clients.
11 Cataracts crisis
Can the NHS cope with the numbers
ready for surgery?
The accessible pdf includes links to help
you learn more about different
conditions, and there is also a fantastic
video trailer for you to watch and listen
to when you get to the back page.
15 Tackling dementia and sight loss
New developments in supporting the
estimated 100,000 people who have
dementia and sight loss
19 Treating thyroid eye disease
The rare condition is often underdiagnosed, but there is plenty of
exciting research underway
Please let us know if you want us to
cover a specific eye condition at
[email protected] and don’t
forget to subscribe online to receive our
free e-alert that details all the latest
news and views for people working in
the eye health and sight loss sectors at
rnib.org.uk/nb
23 Focus on Alströ5m Syndrome
Q&A on the rare condition that can
impact on every organ in the body
25 Latest advances in eye health
Why people with age-related macular
degeneration have difficulty
recognising faces
I hope you find this useful.
Funmi Majekodunmi
Editor, NB online
[email protected]
27 Implications of gene therapy
A round-up of research covering gene
therapy and cataracts
29 Goodbye to injections?
Not quite yet, but there are ground­
breaking treatments for eye conditions
2
Guiding you on Nystagmus
Steve McKay outlines what health professionals
need to know about nystagmus.
What is nystagmus?
Nystagmus means involuntary movements
of the eyes. It can occur at two different
times of life – shortly after birth when it is
known as early onset or congenital and in
adults, it is known as late onset or acquired
nystagmus.
Is it genetic?
Yes, but in many cases there is no family
history at all.
What causes nystagmus?
Congenital nystagmus can occur as part of
a complex health disorder such as albinism
or Down’s syndrome. In other cases, it can
be connected to problems with the eye
such as myopia or childhood cataracts.
Sometimes it occurs for no known reason.
Acquired nystagmus can result from head
injury, stroke, MS and many other causes.
Doctors will carry out a range of tests to
determine the cause.
What are the symptoms?
Nystagmus can affect people in different
ways. For example, some people become
easily tired or suffer headaches and others
do not.
Sometimes the eyes appear to twinkle,
while in other cases, the eyes appear to
sway from side to side. It is often made
worse by tiredness, stress, frustration, busy
environments and unfamiliar places.
3
People with nystagmus:
Where can professionals receive
more information?
• often have difficulty with steps and
•
•
•
•
judging depth of field
need more time to see things and people
around them
struggle with eye contact
are unlikely to be able to legally drive
seldom enjoy participation in ball games.
Nystagmus Network produces a series of
materials such as leaflets and posters for
hospitals and professionals working with
children who have nystagmus. These
promote awareness and encourage
children to talk positively about nystagmus.
Steve McKay is a trustee of Nystagmus
Network (NN).
Can it be cured?
Not yet.
What treatments are available?
Eye muscle surgery and drugs can help in
some cases, but will not “cure” nystagmus.
Glasses and contact lenses do not help
nystagmus, but should be worn to correct
other eye problems.
Useful information
Nystagmus Network provides support,
information and advice on the
condition. It can also introduce adults
and parents to the research projects
mentioned above. NN holds an annual
Open Day and has an active Facebook
page.
What is the null point?
It is the direction of gaze where a person
with nystagmus has the best vision – the
point at which eye movement is slowest.
A person with nystagmus instinctively
turns or tilts their head to make use of it.
The null point can be in any part of the eye
and the head posture can vary from person
to person.
You can email or phone our helpline
service at [email protected],
029 2045 4242 or 0845 634 2630.
Email:
[email protected]
Will I need to see a
rehabilitation worker?
Web: nystagmusnet.org
Rehab officers and similarly qualified
professionals can help with low vision and
daily living aids, registration, personal
independence payments and advice about
other benefits.
People with nystagmus can generally
register as sight impaired and sometimes
even seriously sight impaired. It is generally
at this point that individuals and families
are put in touch with voluntary
organisations for visually impaired people.
4
Shining a light on
eye cancer
Radhika Holmström explores retinoblastoma
and a test that can detect it in children.
Most parents of small children think of “red
eye” in photographs – that bright red pupil
that often shows with flash photography –
as something that is to be avoided. For a
small group, however, it is the absence of
red eye that worries them: because the
“red reflex” test is the most reliable
indicator to date for retinoblastoma, a
malignant cancerous tumour in the retina.
It is often a particularly frightening
condition for parents. “If you stop someone
in the street and ask them which
conditions they fear, in general it will be
cancer first and blindness second,” points
out Robert MacLaren, consultant surgeon
at Moorfields Eye Hospital. However, it is
very rare – the only UK specialist centres
are at the Royal London and Birmingham
Children’s Hospitals – and almost all the 40
to 50 children diagnosed in the UK every
year survive it, although their sight may be
permanently damaged.
The condition
Whereas most sight loss affects older
people, retinoblastoma almost always
affects very young children. “Bilateral”
retinoblastoma in both eyes is usually
detected in the first year (some babies are
born with a tumour) and the “unilateral”
form at around two to three years old. It is
also quite common to develop several
tumours in the same eye.
“If you stop someone in
the street and ask them
which conditions they
fear, in general it will be
cancer first and blindness
second”
Diagnosis: the red reflex
Some children from families with a history
of retinoblastoma are regularly checked
from birth. Most of the time, however, the
first sign that something is not quite “right”
is a white pupil that does not reflect light
– which is particularly evident in flash
photographs, where “red eye” is normally
considered the problem – and/or a squint.
Some children also have red, inflamed
eyes, and possibly deteriorating vision.
5
The causes: gene discoveries
The “red reflex” test, which the Childhood
Eye Cancer Trust (CHECT) is particularly
keen that everyone involved knows about,
involves shining a bright light into the
child’s eyes. Children who reflect white
instead of red should be referred on
immediately for further investigations.
Like all cancers, this is the result of a
breakdown in genetic material. This is
usually a malfunction of some kind in the
RB1 gene, located on chromosome 13.
In some cases (around 40 per cent), the
gene is already faulty and is inherited, while
in others it mutates while the eye is
forming. Inherited retinoblastoma is usually
bilateral, while the non-inherited form only
usually affects one eye.
The test does have its limitations, says
Ashwin Reddy, consultant paediatric
ophthalmologist and retinoblastoma
surgeon at both Barts and Moorfields, who
is also medical advisor to CHECT. “Only a
minority of children who have a ‘white
reflex’ do have retinoblastoma; but it’s the
only test we have for picking up the signs in
children that are otherwise completely
healthy.” Unlike almost all other cancers,
though, the specialists do not need to take
a sample for a biopsy. Instead, they do
further examinations under anaesthetic,
and check out the extent of any tumours
using ultrasound and/or MRI scans.
“We are trying to avoid
using radiation these
days. It is quite traumatic
– you can see from the
faces of people who’ve
had it.”
It is possible to test blood and/or tumour
samples to establish the exact problem
with the RB1 gene to help establish
whether the retinoblastoma is in fact
heritable or non-heritable. If it is heritable,
the rest of the family can be checked; and if
the exact problem with the gene is known,
it is also possible to test pregnancies to
see if a foetus has a chance of developing
the condition as well.
However, Canadian researchers have
recently found that in a very small minority
of cases – under two per cent – the RB
gene is normal, and that an oncogene (a
gene that can help turn normal cells into
tumour cells) called MYCN is driving the
retinoblastoma. “This research completely
challenges conventional thinking and
clinical practice,” explains chief researcher
Dr Brenda Gallie. “We’ve thought for a long
time that all retinoblastoma were caused
by the retinoblastoma gene. Our study now
reveals that’s not the whole story: a new
type of retinoblastoma, with normal
retinoblastoma genes, is instead driven by
extra copies of a powerful cancer gene.”
6
Treatments
The medical team’s first priority is to get rid
of the cancer, especially before it spreads
outside the eye. Their second is to keep as
much sight as possible.
The treatments include cryotherapy, which
“freezes” tumours up to around six mm in
size; laser therapy to “vaporise” the tumour;
and thermotherapy, which uses lasers to
heat the tumour and destroy the cancer
cells. Most children with bilateral
retinoblastoma receive chemotherapy as a
first treatment. That may be followed by
laser treatment as well, either to cut off the
blood supply to the tumour or to heat it up
so that the chemotherapy is taken up
more effectively.
“We are trying to avoid using radiation
these days,” says Reddy. “It is quite
traumatic – you can see from the faces of
people who’ve had it – it damages the
lachrymal glands and it raises the risk of
later tumours elsewhere. If there’s a
relapse, we tend to try to salvage the eye
with ‘intra-arterial chemotherapy’, using a
catheter that goes into the ophthalmic
artery and delivers chemotherapy directly
to the eye. We’re just trying to find the right
dose that will treat the tumour but not
damage the eye.
The future
The survival rate from retinoblastoma is
extremely good – nearly all children who
have it are still alive five years later.
Obviously, they do need regular checkups;
partly to ensure that there is no remaining
malignant tissue, but also because people
with the heritable form of retinoblastoma
have a higher risk than the average of
developing other cancers. This is a
particular concern for Reddy and his
colleagues, who point out that this
demonstrates the importance of genetic
testing; the need for people at risk to avoid
known carcinogens – including radiation in
the form of X-rays and CT scans – and for
ophthalmologists to know the risks too,
in case former patients present with new
eye problems.
“We’re also looking at injecting
chemotherapy agents directly into the eye,
but this is still at research stage (the risk is
that the tumour tissue will be pushed
outside the eye), although preliminary
results are looking good.”
However, some children do lose one or
both eyes, as the only way of limiting the
spread of the cancer. Surgeons put an
acrylic or hydroxyapatite implant into the
eye socket, which should help improve the
cosmetic appearance of the prosthetic eye,
which is fitted around six weeks later. The
eye socket continues to grow as the child
does, and the intention is obviously to have
a prosthesis that is as life-like as possible.
Further information
For more information, visit
chect.org.uk
7
My story
Retinoblastoma in the family
He had six cycles of chemo, and intraarterial treatment too. It stopped the
tumours for a while, but in April 2011 his
left eye filled with blood and they had to
remove it because nobody could see
whether the cancer was progressing –
and in fact, when they tested the tissue
they’d removed they found that it did
have cancerous cells, so they’d done the
right thing.
Kelly Rogers, from Trowbridge, has
retinoblastoma and so has her son Ollie.
“Mine was picked up when I was four
months old. My right eye was removed
within days and I had cryotherapy for four
tumours on the other eye, where I now
have about three-quarters vision. There’s
nothing else on my side of the family, but
my husband’s uncle also realises now
that he had it as a baby too – he thought
he’d lost the eye because of an accident.
For the past 18 months, he has been
stable. He has had a couple of tumours
in the other eye, but he’s fine at the
moment.
I had genetic testing for both my older
children, but neither of them had the
gene problem. Ollie was tested at birth
too, but in fact we knew when he was a
day old that he’d got retinoblastoma. We
accepted it and knew what we had to
deal with – after all, I’ve got a job, I can
drive and my life is pretty normal.
He’s got a wicked sense of humour and
he’s very popular. He’s a very lively,
happy child.”
8
Marfan syndrome
explained
The complex genetic condition with no cure is
explained by Marfan Association UK.
What is Marfan syndrome?
an abnormal shaped narrow chest (pigeon
or funnel deformity), flat feet, hypermobility,
or a high arched palate in the mouth. A
family history is a leading factor for
diagnosis.
Marfan syndrome (MFS) is a disorder of the
connective tissue throughout the body, the
structure that holds us together. It can
affect various parts of the body including
the eyes.
How does it affect the eyes in
particular?
Is it genetic?
The lens of the eye
relies on strands
(zonules) to hold it
in place and
because these
contain fibrillin
which is weak, it
can cause the lens
to slip out of place
(subluxate) and sometimes dislocate
altogether. The Marfan eye is extremely
long from front to back, which causes
shortsightedness and stretches the retina
tight which sometimes leads to
detachment. A squint (strabismus) may
exist and there is a risk of developing
glaucoma.
Around 75 per cent of cases are inherited
and 25 per cent occur as a result of a
new mutation. Each child of an affected
parent has a 50 per cent chance of
inheriting MFS. Even within one family,
the severity and pattern of the condition
varies.
What causes it?
A single abnormal gene on chromosome
15 is responsible. This gene controls
production of fibrillin, a very fine fibre of
connective tissue (the glue or scaffolding
of the body). This gene is mostly inherited
from an affected parent, but less often it
can happen from two unaffected parents
(known as a new mutation).
Can it be cured?
What are the symptoms and
how is it diagnosed?
There is no cure, but it can be managed
and controlled depending on the
symptoms and systems involved.
Medication and surgery can prolong life
and enable a person to live a happy, normal
lifespan within the limits of their condition.
MFS is difficult to diagnose because signs
of the condition vary greatly from person
to person. There are many paths leading
to a diagnosis of this syndrome; very often
it is because of poor eyesight, unusual
tiredness, lung or breathing difficulties,
a tall thin build with long arms and legs or
9
What treatments are available?
As far as the skeleton is concerned, if
diagnosed at the right time, an orthopaedic
specialist can correct the spine with
surgery. A podiatrist can build shoe
supports for flat feet and a rheumatologist
can advise on managing hypermobility and
any consequential conditions. Pulmonary
specialists can help to advise and manage
any breathing or lung problems. Cardiology
has advanced tremendously, and surgery
to replace faulty valves or a dissecting
aorta is now available at early stages
before it becomes life threatening.
Dentists can remove teeth from an
overcrowded mouth.
Where can health
professionals receive
more information?
Marfan Association UK provides free
information on all aspects of the
syndrome and welcomes enquiries from
professionals, patients, their relatives
and anyone with an interest in the
condition.
A network of support is available as well
at meetings in schools and hospitals to
bring MFS to the attention of teachers
and health professionals including
ocular specialists, and an annual
Information Day is open to all.
An ophthalmologist is now able to remove
a dislocated lens, replace it and correct
vision with glasses or contact lenses if
necessary.
Further information
What can eye health or sight
loss professionals do to promote
awareness of Marfan syndrome?
Marfan Association UK, Rochester
House, 5 Aldershot Road, Fleet,
Hants, GU51 3NG, visit
marfan-association.org.uk, email
[email protected]
or call 01252 810472
If the build of the person in front of them
suggests any aspect of MFS as well as a
sight problem, they should ensure that
checks are being made for any symptoms
in the other organ systems involved,
particularly the heart as this can be the
life-threatening aspect. However, it is
important to remember this is a variable
condition and not everyone will have all
or any of the other visible aspects. Early
diagnosis is vital.
Encourage colleagues to look at the whole
person when being presented with a
patient who is shortsighted and has signs
of displaced lenses.
Awareness of Marfan syndrome can be
promoted at meetings that focus on
sight issues.
10
Cataracts crisis
Radhika Holmström reports on the bottleneck
queue for cataract surgery, as well as new
research in this area.
What is a cataract?
“For me, the immediate problem will be
dealing with the number of people awaiting
cataract surgery who are now eligible,”
Professor Harminder Dua, former president
of the Royal College of Ophthalmologists
(RCopth), told NB in 2014. “There has been
a drop in the number, but now this is about
to pick up, and these patients cannot be
ignored.”
Lens cells produce a dense mix of proteins
that give the lens its refractive power – its
ability to change focus. These cells are
produced in the embryo; so, unlike the
proteins in the rest of the body, lens
proteins remain and age as the person
does. In order to keep the lens clear, they
are initially kept dissolved and suspended
with water molecules. Cataracts form when
this suspension collapses – and the
person’s vision becomes cloudy.
Cataracts are a serious worldwide threat to
sight (the World Health Organization
estimates that it is responsible for nearly
half the world’s cases of blindness) despite
being a curable condition. In the UK, more
than 330,000 cataract operations are
carried out every year – in fact, it is
probably the most common operation
carried out on the NHS and is usually a
straightforward, relatively minor procedure.
So what is new on the agenda and why has
a bottleneck in patients awaiting surgery
come about?
Many people develop cataracts in both
eyes, but not usually at the same time.
They can form at any age: in fact, they are
the most common cause of childhood
blindness and partial sight in the UK.
However, they’re most commonly found in
older people.
“Lenses are remarkably resilient – after all,
these proteins hang around for years. But
with age these structures eventually start
to change and unravel,” explains Professor
Barbara Pierscionek of Kingston University.
“They release the water molecules, and the
proteins start to unravel and form clusters.
So you have little pools of water and
clumps of protein which have irregular
borders. As a result, the lens stops letting
light through, and scatters it instead.”
She adds: “There is another form of
cataract: cataract of colouring, or nuclear
cataract, where light is absorbed instead,
but the bulk of cataracts in the UK are the
scattering ones.”
11
Practical issues
Apart from age, diabetes, injury, exposure
to ultraviolet light and medication such as
steroids can all contribute to the risk; and
someone with a family history of cataracts
may be more likely to develop them,
pointing to a strong genetic factor.
Because cataracts are such a common
“elective” treatment, people choose to
have it done. There have been huge
debates over what stage the health service
should shoulder the costs of paying for it.
The marker for access to surgery is “visual
acuity” – being able to read the
optometrist’s test chart – below a certain
level: even if in practical terms their sight
loss is having a very detrimental effect on
their lives.
“Nobody really knows how it develops;
the only real link is with smoking,” says
Moorfields Hospital consultant Alex
Ionides. There may also be a link with
UV light, but this is far from definitive, he
adds. Cataracts can also occur with other
eye conditions.
Treatments
“It has become
increasingly apparent
that commissioners
across England have
been ‘rationing’ access to
cataract surgery”
Surgical treatments of some sort have
been on offer for thousands of years, in
many different cultures. Today, only the
lens is usually removed in Western
countries, leaving the lens capsule
and zonules (the fibres that hold the
lens in place, and which are weakened
in any case by both age and cataracts)
intact.
The surgeon makes a tiny incision on the
side of the cornea and inserts a probe with
a needle-like tip. The lens is broken into
fragments by ultrasonic vibration and
aspirated, a foldable plastic intraocular
lens is inserted, and the incision heals on
its own.
The NHS Action on Cataracts programme,
which finished in 2003, was brought in to
cut waiting times, increase the amount of
cataract surgery available, and cut the
variations in access to service and in
quality. However, over the past year, it has
become increasingly apparent that
commissioners across England have been
“rationing” access to cataract surgery.
This technique is known as
phacoemulsification. “Think of the eye as
an Edam cheese,” Ionides says graphically.
“We remove a circle of waxy coat, scoop
out all the cheese – that’s the lens – and
inject the lens in. It opens up and the
capsule shrink-wraps around it.”
In addition, people with bilateral cataracts
(in both eyes) have been told that they can
only have treatment for one, even though
the evidence is stacking up that both eyes
should be treated – evidence suggests that
the risk of falls actually increases in these
circumstances. The RCopth has always
been opposed to the visual acuity
threshold in any case, feeling that it is more
important to operate when the cataract is
affecting the person’s life. Ionides adds:
“When you’re having problems, that’s the
time to operate.”
It is usually a pretty straightforward
procedure (a laser can be used).
Some people do have complications – and
one in 120 may have a retinal detachment
– but the practice of irrigating antibiotics
into the back of the eye at the end of
surgery has drastically cut down the risk of
post-operative infections.
12
The lens
New techniques?
For someone who has always had short
sight, the new lens can actually mean they
do not need to wear glasses for distances.
However, the artificial lenses usually
provided on the NHS cannot change focus.
It is possible to get multifocal lenses, or
“accommodating” lenses which do, but
these are not particularly good in any case.
“The minute you start etching onto a lens,
you reduce the quality of the vision,”
Ionides explains. “We need a lens that can
mimic the action of a young healthy lens.”
Building on this work, Professor Rory
Duncan and his colleagues at Heriot-Watt
University, Edinburgh, have been
developing a new diagnostic test for
cataracts by looking how the lens proteins
change by illuminating the proteins in the
eye using lasers.
“Proteins are made of amino acids. Some
amino acids, particularly tryptophan, are
known to be fluorescent,” Duncan explains.
“We have identified three specific changes
in the tryptophan in the lens as a cataract
forms, and now we’re trying to quantify
those changes.”
In fact, a number of researchers are doing
some significant work on lens proteins and
the changes that happen within these
proteins – which may well eventually give
rise to new sorts of treatment.
Importantly, this approach also makes it
possible to identify a cataract long before
the symptoms start – about 10 years
earlier than the usual tests, which use a slit
lamp to examine the eye.
Pierscionek and her colleagues are getting
right down to the molecular level in their
analysis. “We’ve been able to look at the
entire lens and see how the refractory
index shape changes with age. In fact,
there are markers of subtle changes from
year to year that may indicate all kinds of
things, including improvements in age
function. Lenses aren’t all the same either:
in many ways, we’ve realised how much we
have to learn.”
“We’re trying to develop an objective test,”
adds Baljean Dhillon, Professor of Visual
Impairment Studies at Heriot-Watt. “At
the moment, if you go to four different
optometrists you might end up with
four different views on the stage your
cataract has reached because they are
using a mix of visual acuity and “backscatter” of light in the eye. The new
technology should give us an idea of
forward scatter – of what the patient is
seeing and not seeing.”
They’ve also found that the gradient of the
lens may be “stepped” rather than smooth
– and that this may be a key element of its
ability to change focus. “It’s continually
changing and curved. We’re working on the
optimal gradient quality for an implant.”
Even more excitingly, it looks as if this work
could eventually produce a new form of
treatment. They have found that lasers
used at a certain wavelength can affect
the molecular structure of the lens,
restoring it to its pre-cataract state without
the need for surgery; and they are now
developing a prototype mobile laser device
for doing this.
Similarly, a team from the Technische
Universität München in Munich, Germany,
has discovered an “activation” mechanism
for one of the protective proteins that
keeps the proteins in a dissolved state.
Having identified the structure and
behaviour of these “crystallins”, it may be
possible to target drug therapy that can
also carry out this activation, reverse the
clumping and clear up the clouded lenses.
“We’ve discovered that illuminating the
proteins with specific wavelengths of laser
could reverse the changes,” says Duncan.
13
Useful information on cataracts, sleep and so much more
Sleep
The eye and elsewhere
One of the
unexpected
benefits of
cataract surgery is
often better sleep
– which is a
particularly
important issue for older people, who
frequently have problems with
sleeping. As NB has reported before,
the eye contains photoreceptor cells
that are separate from our vision, but
stimulate our body’s circadian rhythms
including sleep. Cataracts filter out the
wavelength that “kickstarts” this; so
removing the cataract makes it
possible for the cells to get the right
stimulation again.
“The protein in the lens is one of the few
proteins that retain a foetal element;
and if we can learn from the eye about
what happens to these proteins, it could
have exciting implications for other
diseases,” says Dhillon. “All the diseases
that relate to ageing relate to those
proteins. It could be particularly
interesting in terms of the brain. We do
already know that removing cataracts
can improve cognitive function – and in
some senses, the eye is an extension of
the brain in any case.”
Falls
Successive studies by ophthalmologists
have demonstrated that as soon as
cataract patients are given surgical
treatment, they cut their risk of falling.
This is particularly important for older
people, because falls carry a much
higher risk of fractures. According to the
National Osteoporosis Society, fractures
in patients aged 60 years and over
account for more than 1.5 million
hospital bed days in England alone.
A hip fracture is particularly alarming:
overall, 10 per cent of hip fracture
patients will die within a month and
30 per cent within a year.
Driving
Any driver who
develops
cataracts in both
eyes needs to
inform the DVLA
(and if they hold a
bus, coach or lorry
licence, this applies to cataracts in one
eye only). After cataract surgery,
drivers need to meet the legal “visual
standards for driving” before they can
drive again.
14
Tracking dementia
and sight loss
New developments in supporting people with
sight loss and dementia.
Almost two million people in the UK live
with serious sight loss. Most are older:
around 1.7million are aged over 65 years.
One in 10 people over 75 and one in three
people over 90 have serious sight loss.
If we are serious about addressing the
needs of older people with sight loss, we
need to have the ability to address how
sight loss interacts with other conditions.
One major condition that older people face
is dementia – it is estimated that 100,000
people have both dementia and sight loss
in the UK.
These figures may be familiar to you, but
less well known is that a recent study tells
us that up to three-quarters of people over
65 who are blind or have low vision have
three or more other health conditions. So
co-morbidity in this population is the norm.
Dementia and cataracts
The Dementia and Sight Loss Interest
Group (DaSLIG) was formed to promote a
better understanding of the issues facing
people affected by dementia and sight
loss, share good practice, develop
resources, and provide support for
initiatives and research on dementia and
sight loss.
“It is certainly not the case
that having dementia
precludes you
from cataract surgery”
15
• getting around safely – improved vision
The group has produced a factsheet on
dementia and cataracts. RNIB and others in
the group had been receiving enquiries
from carers and family members as to
whether someone with dementia should or
could have cataract surgery.
will help you avoid trips and falls when
you are out and about
If you have mild or moderate dementia,
then you will still be able to cooperate with
the tests before your surgery, during the
surgery and with the treatment you will
need after surgery.
Some had been advised and others just
anticipated that this was the case. It is
certainly not the case that having dementia
precludes you from cataract surgery.
Cataracts are a very common eye condition
in older people and because of this, many
people may face having a cataract
operation while also dealing with dementia.
If you or someone you care for has
dementia that is more severe – meaning
they have difficulties communicating and
cooperating – then cataract surgery needs
to be more carefully assessed. It is
important to consider whether the risk
of the operation itself, the stress it may
cause and the difficulties of aftercare
will outweigh the benefits gained from
the surgery.
Partnership working
DaSLIG has also partnered with Social Care
Institute for Excellence (SCIE) to develop a
web resource on sight loss for their
Dementia Gateway. The resource is aimed
at formal and informal carers. The content
goes over a broad range of issues such as
prevalence, impact, identification of sight
loss, getting eye examinations,
adaptations, hallucinations and
misinterpretations, and how products can
support someone.
Sometimes the benefits of removing
cataracts are not as obvious as the risks,
but they should still be considered. Often
many of the things that people with
dementia enjoy are visual, such as
watching television, for example. Or if
dementia has made someone isolated,
sight problems caused by cataracts is likely
to limit their activities, making them feel
even more confused or isolated.
“The web resource will be
launched at the NB
conference in March”
Facts on dementia and cataracts
The fact sheet covers:
• what a cataract is and its effect on
vision
• how to tell if you or someone you care
for has a cataract
• cataract surgery and the difference it
can make to a person’s quality of life
• how to prepare for surgery
• how to help someone with dementia
cope with surgery
• what happens after surgery
Whether you have surgery or not may
depend on how your dementia affects your
everyday life. If you have dementia and are
living in your own home – coping with most
things on your own or just need some help
with a few things – then removing your
cataracts will probably be of benefit as it
will help you with day-to-day things like:
• reading
• recognising people and places
• confusion – better sight may help with
your understanding
The factsheet can be obtained at
bit.ly/1d4FK3a
16
Find out more about DaSLIG
Should I tell the eye clinic staff
about my dementia?
The Dementia and Sight Loss Interest
Group – led by Alzheimer’s Society,
RNIB, Thomas Pocklington Trust,
Macular Disease Society and the Royal
College of Ophthalmologists – is part of
the VISION 2020 UK Group. For more
details, go to vision2020uk.org.uk
If you have dementia, then let the staff
at the eye clinic know. The
ophthalmology team will be used to
seeing lots of older people, so they
should be experienced in helping
people with dementia. If staff at the
clinic know you have dementia, they can
take this into account when you are
having treatment.
Research
It is always best to let them know of any
problems you have before your
appointment, so that they can be
prepared for your visit. If it is difficult to
get in touch with the eye clinic before
your appointment, you could ask your
carers or your GP to let them know on
your behalf. If you usually have help from
family or carers, it may be best for them
to be involved and attend the eye
appointments with you.
Barnett K et al. Epidemiology of
multimorbidity and implications for
health care, research and medical
education: a cross-sectional study.
The Lancet, Volume 380, Issue 9836,
Pages 37–43, 7 July 2012
Sight Loss UK 2012 is at
bit.ly/1clHNV5
Source: Dementia and Sight Loss Group
17
Factsheet
Cataracts and dementia
Most people have their cataract removed
by a straightforward operation. This
operation removes the cloudy lens and
replaces it with an artificial lens implant.
This makes sight clear again and removes
the vision problems caused by the cataract.
Cataract surgery is one of the most
common procedures performed by
the NHS.
Cataracts are a very common eye condition
in older people. Most people with cataracts
are over the age of 60 and they become
more common as people age.
Dementia is also a condition that mainly
affects people over the age of 65 and
becomes more common as people get
older. It is thought that there are at least
100,000 people in the UK with both
dementia and sight loss.
Is cataract surgery safe for
someone with dementia?
In most cases, there are not any physical
reasons that make cataract surgery more
difficult if you have dementia. Usually, the
decision to perform the operation is made
by judging the benefits you will gain from
having the surgery against the risks it
involves. Often this decision will take into
account how your dementia may affect
your ability to cope with the surgery and
the aftercare. The decision needs to be
made by you, with help from your
ophthalmologist and your family or carers.
Not everyone with dementia has cataracts
nor do all patients with cataracts have
dementia, but a significant number will
have both conditions. Because of this,
many people may face having a cataract
operation while also dealing with dementia.
What is a cataract?
As we get older, the lens inside our eye
gradually changes and becomes less
transparent (clear). A lens that has turned
misty or cloudy is said to have a cataract.
As a cataract gets worse, it can interfere
with your everyday life. It may:
• make things harder to see
• make reading difficult
• make colours seem washed out
• cause problems when getting around,
as stairs and steps become more
difficult to see
• affect your ability to drive safely.
Cataracts can cause problems with bright
light and many people have problems with
glare.
18
Treating thyroid eye
disease early
Radhika Holmström reports on the importance
of early detection and new research.
works). The reason it malfunctions is
usually the result of auto-immune disease,
where the body’s own immune system
starts attacking its own tissues with autoantibodies.
“It’s a very exciting time
for research into
thyroid eye disease,”
says Dan Ezra. “Things
had been pretty flat for
a few decades and
then in the past two or
three years, there’s
been a huge surge in interest.”
It is not known, at the moment, why this
happens, although some research has
found specific problems in the immune
systems of people who have developed
TED (which could potentially lead to
treatments if the researchers can identify a
way to reverse or repair this damage).
Ezra is the research lead for thyroid eye
disease (TED – also known as Graves’
disease) at Moorfields Eye Hospital in
London and was the key person behind
awareness day bringing together patients
and researchers. This follows the
“Amsterdam Declaration” from international
experts and patient representatives in
October 2009, agreeing that healthcare
providers and professional organisations
need to improve care (including
preventative care) for the condition;
because at the moment, a lot of health
professionals still do not know enough
about the disease. “It’s often underdiagnosed or diagnosed late, because it is
a rare condition and people can present
with quite non-specific symptoms like red
eyes,” explains Helen Garrott, consultant
ophthalmologist at Bristol Eye Hospital.
Either an overactive or an underactive
thyroid can cause problems. TED is usually
linked to an overactive thyroid (although
some people with the condition have
normal or low thyroid function). The
auto-antibodies attacking the thyroid gland
also attack the tissues around the eyes
(the “orbital contents”), which are very
similar to those of the thyroid gland.
The effects on the eyes
Because the tissues being attacked include
the lachrymal gland, which produces tears,
the most common problem is dry eyes; the
eyes feel gritty and uncomfortable and at
the same time, they can also water more
than normal. As the tissues become further
inflamed, they become red and swollen; the
lids swell up, and the muscles and fat
around the eye swell as well, pushing the
eyes forward to bulge out of their sockets
and causing them to move at different
rates, which can result in double vision
The disease
The thyroid is a small gland at the front of
the neck that produces different hormones
with two main functions: controlling growth
in children and controlling the metabolism
(the rate at which the body’s chemistry
19
Treatments
(“diplopia”). The eyelid muscles pull back or
“retract”, so that the person appears to
have a constant stare, and this, combined
with the swelling, can also make the dry
eye symptoms worse. All this can be
uncomfortable at best or extremely painful
at worst.
Both the underlying disease affecting the
thyroid gland and the effects on the eye
have to be treated. Treating the eye itself
usually falls into two stages: the “active”
period of eye disease (this is usually two or
three years) and the “remission” or “burntout” phase afterwards, when the disease
has cleared up. During the active phase,
the focus is on keeping as much sight as
possible, and protecting the cornea as
much as possible too, with artificial tears
and/or ointments for dry eyes, and possibly
eye covers or tape to hold the eye shut at
night if the retracted lid makes it difficult to
close. If the patient has double vision, or
problems with seeing, they may be given
anti-inflammatory drugs, immunosuppressants – or even radiotherapy.
“TED isn’t that rare,” Ezra points out. “It
affects around 30 to 40 per cent of people
with an overactive thyroid, even if that is
usually mildly.” Overall, it is much more
common in women than in men, and is
more prevalent in people of white or Asian
origin than in black people. Around 10 to
20 per cent of people get it to the point
where their vision is at risk, either
temporarily or permanently. The
combination of eyes bulging forward and
scarring from retracted lids can mean that
the cornea ulcerates; or the pressure of
thick, inflamed and/or scarred muscles can
damage the optic nerve, and this is
particularly serious.
Photo © Science photo library
20
“Treatment is very effective in most cases;
it doesn’t eliminate all of the soft tissue
changes, but it makes them significantly
less severe, cutting down the need for
surgery later,” says Garrott. “If there’s
pressure on the optic nerve, the patient
may need emergency ‘decompression
surgery’, but that is very rare.”
including treatments that are already being
used in cancer. We’re looking at new drugs
that target the immune system, and also at
the drug rituximab, which has been around
a long time, but is now being investigated
to see if it’s useful for TED. There’s also
encouraging work on the antibodies that
people with TED produce.”
In the burnt-out phase, some people’s
vision returns to pretty well normal.
However, people whose eyes have been
more severely affected may be left with
scarring, staring prominent eyes, retracted
lids and/or double vision.
“Thyroid eye disease is
often under-diagnosed or
diagnosed late, because it
is a rare condition and
people can present with
quite non-specific
symptoms like red eyes”
“The main treatments are surgical
rehabilitation and trying to restore the
tissues to what they were before,” Garrott
explains. “That includes restoring the
position of the eyeball and the lid, and
surgery to straighten up the muscles to
correct double vision (or at least make it
possible for the person to have single
vision when they’re looking ahead).
“At the moment, there is reasonable
evidence for intravenous steroids, but we’re
still working out the optimum period.
“We think it’s probably around 12 weeks for
people with ‘moderate’ TED,” adds Ezra’s
colleague Jimmy Uddin. “We’re also moving
towards the use of stronger, earlier
immuno-suppressants to suppress the
body’s inflammatory responses to the
disease.”
“The most major treatment is orbital
decompression surgery, to correct the
position of the eyeball as well as any
pressure on the optic nerve. We remove
bone from the walls of the eye socket to
create more room for the soft tissues,
including the eyeball, to settle back.”
Surgery is usually the option here: more
orbital decompression surgery to move the
eyes further back; muscle surgery to bring
the eyes back into alignment and correct
double vision, and/or lid surgery.
Uddin is the primary investigator for
the CIRTED trial being run at Moorfields
Eye Hospital, which is investigating the
use of radiotherapy and immunosuppressants in treating TED.
“Radiotherapy is controversial, as studies
show differing results as to whether it’s
effective or not,” he explains. “In this trial,
with newly-diagnosed patients, we’re
looking at three treatment components:
steroids, a two-week course of
radiotherapy, and a year’s course of the
immuno-suppressant azathioprine.
Everyone on the trial gets steroids, but
different groups get different combinations
of the other treatments.”
Research and developments
“At the moment,” says Ezra, “there’s very
little research base for the treatments we’re
using. We don’t have sufficient evidence of
how effective they are, or at what stage in
the disease they should best be used.
However, there are now established
research programmes going on
internationally, and a lot of people who are
focused on producing work that can go
forward to trial stage. A few promising
treatments and drugs are coming through,
21
Photo © Science
photo library
The way ahead
broadly and develop psychological
interventions too.
All the specialists agree that the most
important thing for TED is wider
recognition, and early diagnosis – because
the earlier the interventions, the more
effective they are likely to be. They’re also
keen to stress that those interventions
need to tackle the psychological effects of
the disease.
“A lot of research shows it’s not necessarily
how you look that determines your quality
of life, but also the underlying
psychological issues.
“We’re trying to unravel all that and unpick
the background; so on the one hand we
have the hard science and on the other the
quality of life work.”
“Many people are devastated by what TED
does to their appearance; there are very
high levels of anxiety, depression and social
avoidance,” Ezra points out.
He concludes: “I do think the next few years
will make a lot of difference for patients.
And engaging with patients, and
partnerships with patients, are an
important part of that. We don’t want to be
directing research on our own.”
“We’ve always concentrated on getting the
surgery right in terms of the vision, but we
need to think about quality of life more
22
Focus on Alström
Syndrome
Kerry Leeson-Beevers, Natural Development
Manager at Alström Syndrome UK, explains the
symptoms and research into this condition.
What is Alström syndrome?
What are the symptoms?
Alström Syndrome is an extremely rare,
progressive and complex condition leading
to sight loss. Both parents must carry one
copy of the ALMS1 gene with a significant
mutation to have a child affected by
Alström syndrome. Because of its low
incidence, there continues to be very little
awareness among the medical profession,
often leading to delayed diagnosis.
Cone rod dystrophy is often the first
symptom noticed within the first few
months of life. Parents may notice that their
child’s eyes are “wobbling” and this is later
confirmed by an ophthalmologist to be
nystagmus. Children also develop
photophobia, becoming extremely
sensitive to light. Many young people are
registered severely sight impaired by the
age of five, although they may retain some
useful vision for some time.
Alström Syndrome affects cilia, microscopic
“hairs” that are essential to many of the
body’s organs, so this condition can
potentially impact on every organ in
the body.
Some babies may collapse with heart
failure. This may initially be thought to be
some kind of virus, but later confirmed as
dilated cardiomyopathy. Early treatment is
often successful and babies do recover.
Lifelong monitoring of the child’s heart is
now considered essential. Young people
are at increased risk throughout puberty.
Sensorineural hearing loss is usually
detected before the age of 10, and many
young children experience glue ear and a
constant runny nose.
Children can put on weight rapidly despite
eating similar portions of food to their
peers. Type 2 diabetes is common among
people with Alström Syndrome and regular
blood tests are carried out to detect
early signs.
23
All people with Alström Syndrome are at
risk of developing cardiomyopathy –
disease of the heart muscle – at some
point in their life. Other symptoms may
include liver and kidney disease, and
respiratory problems.
We liaise with all professionals involved
and ensure they have a good
understanding of the condition and its
impact on families and individuals. We offer
a 24-hour helpline and organise an annual
family and medical conference.
However, it is important to note that not
everyone diagnosed will experience all of
these symptoms. Alström Syndrome
should be considered if a person presents
with two or more of these symptoms.
We work closely with schools to ensure
young people are receiving adequate
support from the visual and hearing
impairment teams and some young people
are supported by a multi-sensory
impairment teacher.
What treatments are available?
Mobility and habilitation training is vital to
ensure that people with this condition are
given every opportunity to learn
independence skills.
Alström Syndrome UK (ASUK), Birmingham
Children’s Hospital and Queen Elizabeth
Hospital in Birmingham are working
together to deliver a highly specialised
multi-disciplinary service to Alström
patients, funded by NHS England. Clinics
are available in Birmingham to all patients
throughout the UK, where tests and
reviews can be carried out.
Is there any research being
carried out into the condition?
ASUK has led a three-year research project
funded by the Big Lottery to deepen
understanding, improve diagnosis and
management, as well as investigating the
basic cellular disease mechanisms in
Alström patients.
ASUK provides additional support to
patients during clinic and the Birmingham
clinical teams liaise closely with the family’s
local health professionals.
Along with Cambridge University,
Birmingham and Torbay hospitals, it has
created a database of patients and
developed a primary cell and tissue bank
across the country.
There is no cure for Alström Syndrome, but
these clinics, combined with a healthy
balanced diet and regular exercise, can
improve the health outcomes for people
diagnosed with this condition.
Emotional support is often required for
families. Living with a dual sensory loss in
itself is extremely difficult for young people,
but they must also come to terms with the
condition as a whole and the impact this
has on their health and wellbeing.
Further information
Call 01709 21 01 51
or visit alstrom.org.uk
What support is available?
ASUK, a strong patient-led organisation,
advocates on behalf of all people with
Alström Syndrome and their families. We
were instrumental in the development of
the clinics and continue to strive to ensure
the needs of our families are being met.
24
Latest advances
in eye health
Mary McDonald reports on research into AMD
and concerns with anti-VEGF.
Face perception and AMD
people with social phobias, autism or
schizophrenia.
New research sheds light on why patients
with age-related macular degeneration
(AMD) often report more difficulty
recognising faces than would be expected.
Encouragingly, the researchers suggest
that training people to allocate their
attention to internal features and to
improve control of their eye movements
could improve face perception for
individuals with AMD.
Researchers at New York University School
of Medicine and New York Eye and Ear
Infirmary have discovered that people with
AMD have a different approach to fixating
on faces than a sighted control group.
See the full report in “Optometry and
Vision Science”, the journal of the
American Academy of Optometry, at
bit.ly/1IPKc9T
Participants with AMD were more likely to
fix on peripheral areas of faces, showing
less attention to the distinguishing features
of eyes, nose and mouth.
Sight and spatial development
Working with blind people has helped
researchers at the University of Bath to
gain a new understanding of how the brain
develops its sense of space.
Their patterns of facial viewing were similar
to those associated with others with
difficulties in face perception, including
25
Researchers from the department of
psychology found that people who lose
their sight later in life use a different
method of following directions to people
born without sight.
retinal thinning in the eyes may be
helpful in evaluating how effective those
therapies are.”
Read the full study in the journal
“Neurology”, Volume 80 No. 1 at
neurology.org
Participants who had been sighted and
then become blind remembered locations
of objects in a room as they are positioned
relative to one another. This was the same
as the sighted participants.
Concerns into anti-VEGF
A study into anti-VEGF treatment raises
concerns about potential side effects.
When researchers at Schepens Eye
Research Institute, Massachusetts,
simulated the VEGF-A activity in adult mice,
they found that blocking the protein
decreased fluid pressure in the eye,
impairing the tissue known as the ciliary
body which produces the fluid that bathes
the eye.
In contrast, participants who had been
born blind first remember a starting point
and then store a memory of the locations
from the “home” location.
Dr Michael Proulx, who spearheaded the
study, said: “Having the experience of
vision early in life lays the groundwork in
the brain for the representation of locations
in a different reference frame than that
found in people who never had visual
experience.”
“There is no evidence to
indicate that current
treatments interfere with
the ciliary body”
The team is now investigating how the
texture or sound of the environment might
influence the frame of reference used, and
hope the findings can improve braille and
audio maps in public places.
The full paper, visual experience facilitates
allocentric spatial representation, is
published in Behavioural Brain Research,
Volume 236, 1 January 2013. View it here
at bit.ly/1IPKEF8
Lead researcher Patricia D’Amore explains:
“Our finding indicates that VEGF-A is at
least one of the molecules that play a role
in keeping the ciliary body healthy.”
Anti-VEGF-A therapies are widely used to
treat wet macular degeneration and
diabetic macular oedema. There is no
evidence to indicate that current
treatments interfere with the ciliary body.
Retinal thinning linked with MS
New research at John Hopkins University
suggests that retinal thinning is associated
with active multiple sclerosis (MS).
A total of 164 patients with MS and 59
healthy patients underwent optical
coherence tomography (OCT) and MRI
scans. In this study, thinning of the
retinal nerve fibre layer occurred at
higher rates in people with earlier and
more active MS.
However, the research team believes the
findings could have implications for new
therapies being developed that involve
more continuous delivery of anti-VEGF to
the eye or more potent inhibitors of VEGF.
Expression and role of VEGF-A in the
Ciliary Body is published in “Investigative
Ophthalmology and Visual Science”,
November 2012, 53:7520-752
or visit iovs.org
Dr Peter Calabresi, who led the research,
explains: “As more therapies are developed
to slow the progression of MS, testing
26
Gene therapy trial
could have huge
implications
Hannah Flynn looks at gene therapy and the
treatment of cataracts in our research round-up.
Retinal gene therapy
The implications of the finds were huge,
according to Professor Hendrik Scholl,
Chief of Visual Neurophysiology Service at
The Wilmer Eye Institute at The Johns
Hopkins School of Medicine. There was no
immediate applicability to the disease, but
“the approach to incorporate the healthy
gene into the virus and place it under the
retina to treat an inherited genetic disease
has huge implications”, he explained in
The Lancet.
A trial has shown that six male patients
(aged 35-63) with choroideremia who were
administered with the correct version of
the REP-1 gene had some improvement
in vision.
Choroideremia is caused by defects in the
CHM gene, which produces a protein called
REP-1 and affects one in 50,000 people. In
those who have the disease, a lack of
REP-1 means that cells in the retina stop
working and slowly begin to die off,
causing blindness.
“Retinal gene therapy in patients with
choroideremia: initial findings from a phase
1/2 clinical trial” can be viewed at
bit.ly/1f7l7Yu
“These are early trials and we haven’t
followed up the patients long term yet, so
we don’t know how long the effects of this
therapy will last,” said Professor Robert
MacLaren of the Nuffield Laboratory of
Ophthalmology at the University of Oxford,
and a consultant surgeon at the Oxford Eye
Hospital, who lead the development of the
retinal gene therapy.
Use of intraocular lens in
keratoconus
The treatment of cataracts with toric
intraocular lenses in patients with
keratoconus was found to be a safe and
efficient treatment by researchers in Spain.
Ten patients took part in the study, which is
thought to be the largest of its kind to date.
Keratoconus is a corneal disease and
patients are known to be at higher risk
of cataracts.
“We also need to work out how many cells
we have treated in these patients using the
methods we used,” he added.
There were plans to continue the study and
to carry out other trials to work out the
correct dose of genes needed.
“MICS with toric intraocular lenses in
keratoconus: outcomes and predictability
analysis of postoperative refraction” can be
viewed at 1.usa.gov/1e4oEZC
27
New treatment for aniridia?
Hand-held retina scanner
developed
Researchers have demonstrated that
aniridia can be treated by the topical
application of a drug to the eye.
A tool to scan a patient’s retina in seconds
has been developed by a team of
researchers at the Massachusetts Institute
of Technology (MIT).
Aniridia is caused by a genetic mutation
that leads incomplete formation of the iris
in 40 per cent of patients. This can cause
loss of vision, and usually affects both eyes.
The technology shines beams of infrared
light onto the retina, which reveals the
cross-sectional tissue structure of the
retina, similar to an ultrasound.
By suppressing the mutation that causes
this problem, researchers showed that
disease progression could be slowed, and
defects in the cornea, lens and retina
could even be reversed, by using mice
for the study. Signalling to the brain was
also restored.
It is still very expensive, but researchers
claim the technology could be used to
increase early diagnosis of eye problems.
“Handheld ultrahigh speed swept source
optical coherence tomography instrument
using a MEMS scanning mirror” is at
bit.ly/Mps6Cw
“We were able to get an eye drop
preparation which meant we could get the
drug to the retina. Previously, it wasn’t
known if the drug could get across the
cornea to the retina,” says researcher Dr
Cheryl Gregory-Evans, Department of
Ophthalmology, University of British
Columbia. This finding has implications for
other retinal diseases, she adds.
It also shows that the “eye is remodelling
and responding to signals after birth. We
have shown that reversal of the action of
the genetic mutation has happened
following this therapy”, she says.
Researchers have funding for a clinical pilot
trial that they hope will take place at the
Department of Ophthalmology, University
of British Columbia; Department of
Ophthalmology, University of Virginia; and
Moorfields Eye Hospital in London.
“Postnatal manipulation of Pax6 dosage
reverses congenital tissue malformation
defects” can be read at bit.ly/1bvaMS9
28
Goodbye to injections?
Tim Jackson, ophthalmic surgeon, reports on two
groundbreaking treatments for eye conditions.
Artificial vision
Retinal implants
Retinal implants are designed to help
restore some vision for people who are
blind from retinitis pigmentosa. They have
been under development for more than a
decade, but of late things have moved
forward substantially.
There are several companies working on
retinal implants, all with slightly different
strategies. The two most developed
devices are from Second Sight in the US
and Retina AG in Germany.
Second Sight uses a small camera
mounted in a pair of spectacles that links
wirelessly to a light-receptive chip
implanted on the surface of the macula.
Retinitis pigmentosa is a disease caused by
progressive loss of the light-sensing
photoreceptors cell in the retina.
Photoreceptors create an electrical signal
when light falls on them. This signal is then
processed by a series of cells, and passed
from the eye to the brain via the optic
nerve. The retinal implants aim to replace
lost photoreceptor function in the centre of
the retina (the macula) by initiating
electrical pulses in response to light.
The Retina AG implant uses the eye’s own
optics to focus light on a chip that is placed
underneath the macula.
The chips themselves are similar to those
used in digital cameras, in that they have
an array of “pixels”, each covering a tiny
area of vision.
Vision with both chips is relatively basic
and hence the chips are only used for
those with the most advanced disease.
However, some patients use the chip to
help them recognise objects, see large
letters and navigate.
Both devices have a CE mark, meaning
they are licensed for use throughout
Europe. But they are nonetheless cuttingedge technologies and as with most
treatments built around technology, there
is the exciting prospect of further
advances.
29
Radiation for wet age-related
macular degeneration
Fewer injections
INTREPID found that patients needed a
quarter fewer injections following radiation
treatment. In certain groups of patients, the
injection rate was reduced by almost half,
with a quarter needing no further injection
over the two-year follow-up. Radiation
caused some collateral damage to healthy
macular blood vessels in a minority of
patients, but only 1 in 100 had any vision
loss as a result.
Wet age-related macular degeneration
(AMD), is a leading cause of vision loss in
the UK. People with wet AMD are usually
treated with regular eye injections of
Avastin, Lucentis or Eylea.
These drugs are undoubtedly helpful for
most, but they impose a burdensome
treatment routine, with repeated eye
injections and regular hospital attendance.
Also, some patients fail to respond fully, or
the initial benefits of treatment diminish
over time.
The trial will follow up patients for three
years to see what the safety profile is like
over the longer term, but results to date
offer cautious encouragement. The device
is not yet available in the NHS, but it is likely
some NHS hospitals will start to offer
treatment inthe future.
Intrepid clinical trial
Radiation is an alternative option that aims
to kills off the abnormal blood vessels that
cause wet AMD in a single treatment,
rather than just suppress them (as with the
injections). The results from clinical trials of
radiation vary, depending on how radiation
is delivered.
A trial of radiation delivered using a
surgical approach produced disappointing
results, and interest in radiation waned, but
then a competitor device produced positive
results in a clinical trial called INTREPID.
Further information
INTREPID tested a technique called
stereotactic radiotherapy, using a device
manufactured by US company, Oraya.
Treatment takes 10 to 20 minutes and is
performed in a hospital eye clinic, without
surgery.
Eye implant video at
bbc.in/1buCOSu
Intrepid study research at
bit.ly/1c11Svb
Patients sit with their chin on a rest while a
robotically-controlled device fires in three
beams of radiation to overlap at the
macula. The total dose of radiation is about
the same as a dental X-ray.
Second sight is online at
2-sight.eu/ee/home-ee
30
Meet our vlogger
Suzie Simons
As NB moves online, our resident
columnist Suzie Simons will now be
vlogging (video blogging) for us. Here, the
blind mother of two gives you a little taste
of what’s in store for our readers.
Watch the trailer now at bit.ly/1aIP9Uv
To watch more of Suzie and read about the
latest developments in the eye health and
sight loss sector. Sign up to our receive our
free e-alerts at rnib.org.uk/nb