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August 2001, Number 10
What a week this has been for people like us! First, the Report of the Royal Commission on Genetic Modification was released at the end of July. The 1,500 page report which cost $6,000,000 and took 14 months to be released received 10,000 submissions, which indicated the intense interest that the NZ public had in this subject. There were 49 recommendations, one of which was that an Ethics Council be established. It seems that no one is against medical research done in the laboratory but we will have to wait another three months until the end of November before the Government decides which recommendations it wants to adopt.
In America, President Bush has to make his most difficult political decision since he was elected. He has to decide whether his Government will fund research on embryonic stem cells derived from human embryos, which could lead to cures for retinal diseases. America's best research scientists work with Federal funding and in the meantime all FDA funding for this type of research has been put on hold. After he has made his decision, Congress then has to approve which way to go and it is uncertain as to which way they will vote, no matter what Bush decides, as the Congress is very evenly split numbers wise.
As I write this the "Catching the Knowledge Wave" conference has just concluded in this country. Professor Robert Lord Winston, who fronted the two fascinating TV series "The Human Body" and "Super Human", stressed the need for more support for tertiary students and the need for more support for scientists in New Zealand to do basic scientific research.
All of these things will make a difference one way or another in due course to people with retinal degenerations and it seems that we will have to patiently wait even longer for the powers that be to make up their minds what to do.
Meantime the "No Nonsense Eye Sense" Expo will be held in Auckland on the 22nd September and our Annual General Meeting is to be held in Wellington on World Retina Day, 29th September.
This issue features New Zealand eye research and Cataracts, which affect many of our members at some stage. We salute Erik Weihenmeyer, the first blind man to conquer Mount Everest, the world's highest peak, and a man with a retinal disorder like ourselves. Martine Abel, one of Retina's peer supporters, writes of her loss of colour vision and Dunedin member Helen Adams publishes her first book. Retina President Tony Haas in his last letter of his first two year term, looks ahead to what he sees might be the future for the Society.
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FROM THE PRESIDENT's DESK
By Anthony Haas
Planning is underway, facilitated by the Wellington branch of Retina New Zealand, to do more at the Annual General Meeting than review the society's work programme on peer support, public education, research and blindness prevention and awareness. The AGM could build on the success of the "Reaching Out" theme when the AGM was last in Wellington to reach out to professionals and other people who can influence the policies of concern to the voluntary consumer society.
Insufficient progress has been made on designing policies to address the challenge of increased numbers of New Zealanders being affected by Age-related Macular Degeneration. The Ministry of Health's policy development work could take up the issue through a number of channels:
* in its studies of policies for the ageing society
* in the implementation of the NZ Disability strategy
* in disability committees and boards of District Health Boards.
Retina New Zealand and the RNZFB Blindness Awareness and Prevention Unit (BAP) could assess whether this is one of the issues they could each progress in partnership. It could assist if specialists reviewed such strategies for blindness awareness and prevention - for example to assess if the statistics on which policy cases are built can be mobilised. The partnership between the Blindness Awareness and Prevention Unit and Retina New Zealand - reflected perhaps in BAP's strategic plan as well as project cooperation such as the 22nd September Retina Week Eye Expo, is also ripe for management, planning and development so that this society's and other interests can be advanced by BAP's work.
Retina New Zealand, committed to strategies of coping and hoping, should give appropriate attention to which scientific and medical issues it wants to advance. The AGM has been asked to consider policies on genetic modification. There are also policy choices of a medical and ethical nature to be acknowledged for stem cell research. There is the question as to whether diabetic retinopathy, and the concerns of the Diabetes Association, the needs of people with glaucoma and rare eye diseases need more attention by Retina New Zealand or those with whom it could work.
The society might want to widen its international sources of information - the US Foundation Fighting Blindness and the Lighthouse are two American connections that may respond if asked to deepen relations.
Retina New Zealand's membership is less than the number of New Zealanders who have retinal dystrophies - a reaching out programme at the AGM to Wellingtonians in the policy and communications process could give new impetus to what should be a continuing reaching out programme from the branches and active members.
DONATIONS TO RETINA NEW ZEALAND INC.
From Kaye Newton, Retina NZ Treasurer
At last our society has received approval from the Inland Revenue Department as a charitable organisation from 1st April 2001.
This means that any donations you make to the society which are over $5.00 can be claimed as an income tax rebate. You can claim one third of the donation as a rebate up to a maximum rebate of $500.00 (or $1,500.00 worth of donations). Please keep your receipt as proof of the donation if you want to claim the rebate.
REVIEW OF VISION RESTORED IN A CANINE MODEL OF CHILDHOOD BLINDNESS
By June Ombler
In our last newsletter (No.9, May 2001) we featured an article on Vision restored in a canine model of childhood blindness.
This began - "In one of the single most important advances in the history of retinal degeneration research, a group of Foundation-supported scientists used gene therapy to restore vision in a canine model of severe childhood blindness, known clinically as Leber Congenital Amaurosis (LCA). This finding, published in the May (2001) issue of Nature Genetics, represents the first time researchers successfully restored vision in a large animal model of retinal degeneration".
In our November 1997 newsletter I said in my President's letter that our society has supported and closely co-operated with the RP research team of Dr Michael Denton, Dr Marion Maw and Robyn Bridges at Otago University. Now the Otago research team has made a major genetic breakthrough which will alter the direction of some of the research into treatments for RP.
Papers from the Otago laboratory, the collaborating German group of Professor Andreas Gal and a third research team were published in the same issue of Nature Genetics. The research groups had each found defects in genes found in the retinal epithelium, a layer of cells found at the back of the eye. The genes were RPE65 and RLBP1 and they both play a role in vitamin A pathways in the retina. Dr Maw and Professor Gal were studying DNA from 20 families in India which had been collected through many years of dedicated effort by Dr Denton and a number of Indian collaborators.
Identification of RPE65 as a retinal degeneration gene paved the way for the recent gene therapy studies. For the first time vision was restored in an animal model with eyes that approach the human eye in size. Previously progress had only been made on mouse models, which does not necessarily transfer to human sized eyes.
This shows that small research teams in a country like New Zealand can achieve outstanding results and that identification of genes is an important first step towards the development of effective treatments. So, once again I would like to congratulate Dr Marion Maw and Dr Denton, who nowadays is based mostly in Pakistan collecting pedigrees of families for DNA analysis by the Otago University Retinal Degeneration research team.
AUCKLAND STUDENT WINS FIRST PRIZE FOR WORK ON CATARACTS
From Prof Joerg Kistler, School of Biological Sciences, University of Auckland
Ms Kaa-Sandra Chee, a second year PhD student at Auckland University School of Biological Sciences has won the overall National Foundation of Research, Science and Technology First Prize award, which was presented to her in Christchurch by Nobel prizewinner Professor Alan MacDiarmid at the end of June.
Miss Chee expects to complete her PhD by mid 2003 and several publications are likely to arise from her work.
Ms Chee's award winning work is in the area of molecular vision research, focusing on the mechanisms that the lens has evolved to maintain transparency. One of the key features that minimizes light scatter in the lens is that cells are arranged in a highly ordered fashion, leaving very little space between them. Furthermore, there are no blood vessels interfering with the path of light. This poses two problems that the lens has to overcome: it needs to regulate cell volumes very precisely to not disrupt the crystalline cell order, and also needs an alternative mechanism of delivering nutrients to inner portions of the lens. Indeed, many cataracts start with cortical opacities, which develop when the cells or the space around them swells uncontrollably by taking up excess water.
Ms Chee has discovered several transporters which can move chloride ions across the cell membranes. Because water follows the ions, these transporters play a major role in keeping cell volumes precise and thus help maintain lens transparency. Ms Chee's aim is to identify the molecular isoforms of these transporters and to learn how they function.
Ms Chee is a member of a larger team that works towards the common goal to better understand normal lens functions and determine what goes wrong in cataract. A consortium of three research groups, led by Professor Joerg Kistler in the School of Biological Sciences, Dr Donaldson in the School of Medicine, and Professor Peter Hunter in the School of Engineering, respectively, has made major progress in defining the molecular parameters of lens function, and is now in the process of developing a computer model. This model will be the first of its kind to help identify new drug targets aimed at preventing cataract, and also, to predict cataractogenic side-effects of drugs in clinical use.
In the longer term, therefore, this basic research has the potential to generate new knowledge that could lead to the development of novel anticataract therapies. With the increasing prevalence of cataract associated with longer life expectancies, and the enormous numbers of cataract surgeries required, new anticataract strategies are a major health goal world-wide.
INTERESTING FACTS ABOUT CATARACTS
Compiled by June Ombler
Recently I have had several enquiries about cataracts and the safety of having cataracts removed if you have RP. A search of my files revealed some interesting information.
What type of cataracts are found in people who have RP? Research tells us that more than 50% of people with RP develop cataracts caused by general ageing and exposure to ultra-violet light. Professor Gislin Dagnelie, Director of Low Vision Services at Johns Hopkins University School of Medicine in the U.S.A. says that "Cataracts that are commonly found in combination with RP affect the rear central part of the lens and are called posterior subcapsular cataracts or PSC's. Cataracts caused by sunlight, on the other hand, affect primarily the centre of the lens and are called nuclear cataracts".
Laser treatment and Cataract extraction: Several members have expressed concern about possible damaging effects of laser treatment to remove remnants of the capsule after cataract removal. Professor Alan Bird of Moorfields Hospital in London has set out below the facts surrounding this treatment.
In modern cataract extraction the posterior capsule of the lens is left intact. This leaves the eye divided into two compartments. There is overwhelming evidence that leaving the posterior capsule intact reduces the incidence of complications and the eye appears to be much healthier. Unfortunately, the posterior capsule occasionally becomes opaque due to growth of cells, such that it appears similar to frosted glass and causes the vision to become blurred. This sequel to cataract extraction is more common in patients with retinitis pigmentosa (RP) than in eyes that do not have retinal disease. The problem is relatively easily resolved. A small hole can be created in the posterior capsule using a laser. The procedure is undertaken with drops through a contact lens, takes less than 5 minutes and does not cause pain. There was some concern that making a hole in the posterior capsule might be followed by macular oedema, but experience suggests that if it is undertaken more than three months after the original cataract surgery, then the risk is extremely small. From: BRPS News, Winter 1999.
Can Cataract development be slowed? A question asked of Professor John Marshall at the British RP Society's AGM in June 2000 was:
Q Is there any medicine that will slow cataract development?
A No, but if it comes to an operation, it is comforting to note that it is one of the most successful operations performed, with a success rate of around 98%. Everyone, if they lived long enough would develop cataracts but the process can be accelerated by environmental effects, one being short wavelength light, from blue through to ultra-violet. Protect your eyes from sunlight by wearing sunglasses that cut out blue light, for example the orange tinted "blue bloc" lenses now worn by many members (In the U.K.)
Dr Dianne Sharp, Retinal specialist from Auckland and member of the Retina NZ Scientific & Medical Advisory Board comments: There is an increased chance of developing cataracts as we get older. The risk of age-related macular degeneration (AMD) also increases with age and both conditions are affected by exposure to UV light, while some people have a genetic predisposition.
There is not a specific type of cataract associated with AMD or macular degeneration. Nuclear sclerosis is a gradual yellowing of the lens. It may have little effect on vision in the early stages but be associated with increasing shortsightedness. Posterior subcapsular lens opacities tend to have a more significant effect on vision particularly in bright sunlight.
While modern micro-incision surgery has a high success rate, people with retinal disorders such as RP or AMD and cataract should be guided by their ophthalmologist to assess their retina before proceeding with cataract surgery.
OUTCOME OF CATARACT SURGERY IN PATIENTS WITH RETINITIS PIGMENTOSA
Published in British Journal of Ophthalmology, August, 2001, 85:936-8 By Jackson H, Garway-Heath D, Rosen P, Bird AC, Tuft SJ Department of Clinical Ophthalmology, Institute of Ophthalmology, Cayton Street, London, EC1V 9El, UK.
AIM: To determine the visual benefit of cataract extraction in patients with retinitis pigmentosa and to identify risk factors for poor outcome.
METHODS: A retrospective analysis was undertaken of a continuous series of 142 eyes of 89 patients with retinitis pigmentosa undergoing cataract surgery between 1985 and 1997.
CONCLUSIONS: Cataract surgery for relatively minor lens opacities is beneficial in patients with retinitis pigmentosa, and most report subjective improvement of visual symptoms. The incidence of capsular opacification is high and anterior capsular contraction may occur. The number of eyes with poor vision due to macular oedema was unexpectedly low.
RESULTS: Mean age at surgery was 47.5 years (range 24-81 years). In 100 eyes there was posterior subcapsular lens opacity alone, 37 eyes also had moderate nuclear sclerosis, and five had only nuclear sclerosis. All patients had central visual fields of <10 degrees.
Overall, mean visual acuity improved from 1.05 (SD 0.38) preoperatively to 0.63 (SD 0.49) postoperatively on the logMAR scale. Significant postoperative capsular opacification occurred in 88/139 eyes (63%) and 45.1% required capsulotomy.
Anterior capsulotomy was undertaken in 5/52 (9.6%) eyes undergoing phacoemulsification.
Postoperative macular oedema was noted in 20 (14%) eyes. Visual acuity improved in 109 eyes (77%), was unchanged in 29 eyes (20.5%), and worsened after surgery in four eyes (2.5%). 86/89 patients reported major improvement of visual function.
WHAT'S IN A COLOUR
By Martine Abel, Auckland Branch member
To see colour, or not to see colour? Well, most people I've dealt with so far, are of the opinion that, if you can see as little as I, losing your colour perception should not matter, as it didn't mean the difference between using a mobility aid or reading print or not, but this is so untrue!
I could never see much, couldn't even count fingers, but I felt blessed in a way, for up to 5 years ago, I could distinguish colours: white, red and black especially, but even yellow, green and brown at a push. My mother, meaning well and probably not realising how much this would mean to me later, would let me build leggo toys and insisted that I make the walls, the cars, or whatever a specific colour "so that your eyes don't get lazy", she used to say and I did struggle, but I did as I was told.
And when thinking back, it's always the colour contrasts that helped me a great deal and that now forms a large part of my memories: A dark vague spot on a pavement, meaning water; A light round object on a dark lawn, being a ball; A brownish object on a white plate, another biscuit left for me; Something shining on a table, a glass forgotten; A lightish blob on a dark carpet by my feet, my dearest golden guide dog, Hetty. And this list could go on and on...
But then, five years ago, Glaucoma snuck up on me like the proverbial thief in the night, for, as most already know, it is so hard to catch it in its first stages, the symptoms being: Night vision loss and perceiving colours very vaguely. So, I just thought my retina is playing tricks on me. When Glaucoma was diagnosed at last, I took heaps of medication before going for an operation to relieve the eye pressure and although my eyes are totally comfortable now, I've lost that extra spark called colour forever.
So, is this a warning to all to be cautious on vision symptoms or just me, reminiscing or even a tribute to my "colourful" past? Well, maybe all of the above. So, let me ask again: What's in a colour? A colour by any other name, still brings sweet enough memories.
About Martine Abel
Martine has Lebers Congenital Amaurosis, a rare hereditary condition of the Retina where there is extensive loss of sight in early childhood. Four years ago she contracted Glaucoma as a secondary eye condition, which took away most of her contrast and all of her colour perception.
She is an RNZFB Communications Instructor which involves tuition of Braille, touch typing and basic word processing. Martine also does Closed Circuit (CCTV) assessments and gives instruction in all sorts of devices to do with reading and writing adjustments for the blind and vision impaired.
Photograph: Martine Abel with her dearest golden guide dog, Hetty.
She is a qualified teacher and counsellor and has always enjoyed studying although it entails a great deal of extra work, taking adaptive technology and blindness-related matters into account. Martine is also involved with a few consumer and sports groups such as Retina NZ (for which she is a Peer Counsellor), ABC NZ, the Guide Dog Society, Blind Cricket and Blind Indoor bowls.
Martine is in her twenties and has a guide dog called Hetty. She has been living in NZ for the last five years after emigrating from South Africa.
As from 1st August 2001, Martine has been appointed for six months by the RNZFB as the Project Leader for the Braille Literacy Project Stage 2.
BLIND MAN CONQUERS MOUNT EVEREST
By June Ombler
In its 18th June issue Time Magazine devoted its cover photo and ten pages to an amazing feat by a man blind since he was 13 with a rare hereditary retinal disease. Erik Weihenmayer, 33, knew that blindness was inevitable due to his diagnosis of Retinoschisis, a progressive degenerative blinding disorder of the retina for which there is no treatment or cure. He accepted this in his teens and decided to face the challenges that came his way and see what he could achieve in spite of his blindness.
Erik Weihenmayer climbed Mount Everest, the world's highest mountain of 8,850 metres. He achieved this in spite of all predictions that it could not be done by a blind person. Injury from a fall into a crevasse and suffering altitude sickness caused him doubts even at Camp One, about facing the unknown hazards of the dreadfully difficult climb ahead of him. He used two special poles to feel his way forward and after making ten trips carrying equipment up the mountain to the higher camps through the Khumbu Icefall, his climbing speed and confidence increased and altitude sickness abated.
Erik was no couch potato. He played basketball and competed in the National Junior Freestyle Wrestling championship in Iowa while still at school. Needing more challenges, he took up rock climbing at sixteen and found it was a sport that he could participate in equally with the sighted. This led him to mountaineering and he has led teams of climbers up sections of the El Capitan cliffs in the Yosemite National Park in the USA and Mount Denali in Alaska. He has also climbed Mount Kilimanjaro, the highest mountain in Africa, Aconcagua in Argentina and many other peaks. Another sport he has tried many times is sky diving.
Erik followed behind sighted climbers using his two poles to feel his way ahead and listened for voices and the bells on other climbers' packs to help him with orientation. His climbing team of nineteen sponsored by the National Federation of the Blind and others consisted of a documentary filming team who were all experienced climbers and trusted friends.
The team's first attempt to reach the summit was foiled by bad weather. On 24th May, in spite of fast running out of time and terrible weather conditions the team eased their way up the near vertical mountain to the South summit and across the knifelike ridge and up the 12 metre Hillary steps. Then on up a snow slope to triumphantly stand on the summit of the highest mountain in the world almost 48 years to the day after Sir Edmund Hillary and Tenzing Norgay were the first people to achieve this feat.
Back home in the USA, his wife Elly and their one-year-old daughter greeted the news with great relief and joy, awaiting his homecoming as an American hero, to rate with Helen Keller as an inspiration and icon to all blind people. With his great tenacity, Erik Weihenmayer has proved that blind people can achieve almost anything if they make up their minds to do so.
OTAGO/SOUTHLAND BRANCH CHAIRPERSON PUBLISHES HER FIRST BOOK
Helen Adams, Chairperson of Retina NZ's Otago/Southland Branch recently jointly published her first book, "Vi Fraser's Country", the life and times of her mother, who was born in 1904 and died in 1999. Spanning the 20th century, it tells of the life of a farmer's wife who lived in the high country backblocks in the Hakataramea Valley beyond Kurow, to Weston, Five Forks and Oamaru in the South Island.
Vi Fraser was well-known in North Otago for her short stories and poems which she wrote for magazines, newspapers and the radio. In her later years she won many prizes in the Charles Croot Creative Writing competition and for her poetry in the Third Age Poetry competitions.
Written notes and tapes made by her mother and some genealogical research by her husband Geoff enabled Helen Adams to put the book together as an autobiography of her mother. Published by themselves, the book gives both an interesting life story and a particularly comprehensive picture of the life of a farmer's wife living in the high country backblocks in the days before electricity, and when there were very few motor cars.
Auckland Branch News:
Auckland Branch members will be manning a booth at the "No Nonsense Eye Sense" Expo to be held on Saturday, 22nd September from 10.00 am to 4.30 pm in the Lordship Lounge, Alexandra Park Function Centre, Alexandra Park Raceway, corner of Greenlane and Manukau Road. The function centre is located inside the grounds of the Alexandra Park Raceway.
Retina New Zealand will have available concise and easy to read information which will help people "hope and cope" with their retinal or allied eye disorder. Retina NZ representatives from the Auckland Branch will be in attendance and happy to answer a range of questions such as:
* Where do I get clinical information on my condition?
* Is there any research into treating my condition?
* Are there people with similar conditions to my own who I can talk to?
* What should I do about my mother or friend who appear to be losing their sight?
Retina New Zealand is proud of its programmes that deliver peer support services and clinical and research information dissemination services to a steadily growing number of New Zealander's. So why not come and find out who your Auckland Branch are and whether Retina NZ can assist you or your partner, friend or relative.
Organised by the Royal NZ Foundation for the Blind and Retina NZ, there will be around 15 to 20 exhibits staffed by optometrists, sunglasses firms and interest groups such as Retina NZ, and the Eye Bank. There will be expert speakers on eye conditions, a variety of free eye tests, glasses frame advice and free information kits. It should be huge!! The Expo is targeted at the over 50 age group but everybody is welcome. Make sure you go with all your older relatives and friends. There is no charge to enter.
How to get there:
* If travelling north on the Southern Motorway, exit at Greenlane. At Greenlane Interchange turn left onto Greenlane Road past Greenlane Hospital to Alexandra Park Raceway on the right to the main gates of Auckland Trotting Club and Alexandra Park Function Centre. Take Gate B or C to drive into the complex.
* If travelling South on the Southern Motorway, take Greenlane exit and turn right into Greenlane Road past Greenlane Hospital. On the right is Alexandra Park Raceway taking you to the main gates of Auckland Trotting Club. Take Gate B or C to drive to the function centre.
Disabled car parks are clearly marked outside Centennial and Members stands and other areas. Ample ordinary car parks available.
Central Auckland routes 304, 305, 312 and 328 get you to within 300 metres. Consult the journey planner on http://www.rideline.co.nz
From Central Auckland take Tranz Metro train route STH to Remuera Stn and walk or taxi 2028 metres to venue. Refer to journey planner on http://www.rideline.co.nz
For more information, please contact Fraser Alexander on (09) 3556-914 or (025) 840 937 or e-mail Fraser on firstname.lastname@example.org
Wellington Branch News:
This year Wellington Branch will be hosting the Society's Annual General Meeting on World Retina Day, Saturday 29th September. The meeting will be held in the premises of the Royal NZ Foundation for the Blind, 121 Adelaide Road, Wellington.
Please come with your family and particularly your older relatives as the Society is launching its latest booklet "About Macular Degeneration - a New Zealand Guide to Macular Degeneration", which includes information about Age-related Macular Degeneration.
The Annual General Meeting will begin at 10.15 am and there will be a break for lunch provided by Wellington Branch from 12.45 to 1.45 pm. After lunch Dr Marion Maw, Chair of the Retina Scientific & Medical Advisory Board will lead a discussion on Genetics.
Following the afternoon session there will be a wine and cheese "Reaching Out" function to which everyone is invited.
This is a draft of the day at the moment but the final agenda will be sent to you nearer the time with the remits to be discussed, Proxy forms and C.V.'s of candidates for the President for the following two year term.
Please write this date in your diary to reserve the day and be sure to come. Wellington Branch and the Retina Executive look forward to meeting you there!
CHANGE OF RETINA INTERNATIONAL WORLD WIDE WEB ADDRESS
We would like to inform you that the International Retinitis Pigmentosa Association (IRPA) was renamed to become Retina International.
In the meantime the www.irpa.org domain has expired and was taken over by a domain name broker. He unfortunately links it currently to some sites which are not really related to our cause.
May we kindly ask, if you could update your link (www.irpa.org) to point to our new web address http://www.retina-international.org
Philipp Schlaeppi, Retina Suisse
Email : email@example.com
DO YOU NEED HELP OR ADVICE?
The Retina NZ Peer Support programme is a free and confidential service, operating nationwide. To make contact, telephone 0800 243 33 33, press 1 for General enquiries and then ask the call centre operator to put you in touch with a Peer Supporter in your area.
Auckland Genetic Hotline (Ask for Dr Julie McGaughran) 0800 476 123
Wellington Genetic Hotline 0508 364 436
Christchurch Genetic Hotline 0508 364 436
(South Island callers ask for Dr Caroline Lintott)
(Please note a recent change of phone number for the RNZFB in Wellington as listed above). The fax number remains the same).
CLOSING DATE FOR RECEIPT OF ARTICLES FOR THE NEXT ISSUE IS FRIDAY 9 NOVEMBER 2001.