RETINA NEWS

 

1st EDITION 2003

 

RETINA SOUTH AFRICA – MOVING INTO THE LIGHT

A National Management Committee Meeting of Retina South Africa was held in Johannesburg on the 28th and 29th of March.  Delegates from Bloemfontein, Cape Town, Durban, Hermanus, Johannesburg, Port Elizabeth and Pretoria approved a dynamic fresh approach to Fighting Retinal Blindness in South Africa.  Some of the focus points of this new strategy will be: -

·        An aggressive membership drive to recruit thousands of South Africans with Retinal Degeneration and their families,

·        To obtain blood samples from all these patients for the gene tracking project at the University of Cape Town,

·        To ensure that all South Africans are eligible for future therapies and treatments,

·        To empower members to create an independent and rewarding life style,

·        To encourage an attitude of hope and enthusiasm in all our members to participate fully in the Fight against Retinal Blindness,

·        To ensure that membership, staff and management of Retina South Africa is a true reflection of the demographic and geographic profile of South Africa,

·        To establish dynamic youth groups throughout the country,

·        To raise sufficient funds to ensure the viability of the organisation and all our projects.

This new strategy is vital to meet the growing demand of patients and their families for the establishment of clinical trials for therapy.  International success in the treatment of various retinal degeneration in animals with similar conditions has confirmed that the application of these results into human trials is now feasible.

To achieve these bold new objectives we need the dedication and support not only of every sufferer of a retinal condition but also of every eye care professional, the corporate sector and provincial and central government.


EMPOWERMENT PROJECT AND INTERACTIVE WORKSHOP

A National project to educate and empower members and relevant workers in the field of Retinal degeneration will be held in Johannesburg in September around World Retina Week.

Retina South Africa National Office and Branches will be sourcing funding for subsidies towards transport and accommodation costs of members.  The project will be held on a weekend and costs will be kept as low as possible.  National and International experts from various fields are being invited.  The interactive workshops will include topics such as: -

·        Understanding your retinal condition

·        Inheritance factors – are my children at risk

·        The gene tracking project – how to participate

·        Treatment and therapies – preparing South Africans to participate

·        Parenting issues – parents of low vision children, low vision parents of sighted children

·        Partners and spouses – they don’t accept or understand

·        Education and career choices – inclusivity and its special challenges

·        Driving and transport issues

·        Employment – the Employment Equity Bill and other issues,

·        Socialising problems

·        Housing

·        Mobility

·        Sport, recreation and hobbies

·        Hi and Low tech assistive devices

·        Youth programme

Please let us know which of these topics (or any other) are of interest to you. If you would like more information please contact us on 0860595959 (local call costs apply) or by fax or email (see front cover).

 

US HELPING YOU

Retina South Africa is a patient action and support group for all South Africans and their families affected by Retinal Degenerations such as: -

·        Retinitis Pigmentosa

·        Macular Degeneration – Age-Related and early onset, Stargardt Dystrophy, Usher Syndrome, Cone and Cone Rod Dystrophy and many others.

The organisation is managed and run by Retinal patients, their families and friends.  Counselling and support is offered at branch level.  Some branches also offer social worker counselling, mobility and skills training.  Retina South Africa offers the following services FREE of charge: -

·        Participation in the gene tracking project

·        Education and information

·        Human services – skills development and assistance with social grants and housing applications

·        Support groups

·        Education and career counselling

·        Mediation in the work place

·        Personal development

·        Referral to specialist services

We are determined to bring these services to the previously disadvantaged communities and the rural areas of out community. In addition Retina South Africa creates public awareness of Retinal Blindness and raises funds to support these services

 

BRANCH NEWS

 

LIBERTY RIDE FOR SIGHT

The 14th Ride for Sight was held in Boksburg on February 23rd.  This is the 4th year that Liberty has sponsored the event and what a great event it was! Over 5000 cyclists participated in either the 57km or 112km event.  The 112km event has been chosen as one of only 12 National events to be a Liberty seeding event for the Cape Argus Pick and Pay Cycle Tour.  Our volunteer portfolio supervisors deserve special credit.  They are Craig Blamey, Lorna Bristol, Jean and Arthur Bowler, Gordon and Dawn Cousins, James and Sandy Cape, Chris Fotheringham, Bevan Langton, Audrey Mans, Gavin Mack, Mike and Ellen McBeath, Colin Nicolson, Michelle and Kobus van der Merwe and Anton van Rooyen.

Our Chief Executive Officer, Ebrahim Patel and his dedicated staff members

ensured excellent administrative back up to the supervisors.  The ride has

once again received excellent reviews from cyclists and is hailed as one of

the best-organised events on the South African Cycling calendar.

 

Springs Wheelers, our organising club, have once again shown their

dedication to Fighting Retinal Blindness.  THANK YOU!

 

Swing for Sight: - Thanks to convenor Tich Cousins For organising the event held in October in Benoni. A total of R18000 was raised.

 

NEWS FROM THE EAST CAPE BRANCH

·        Congratulations to Neville Comley for selling 3500 tickets in the Toyota Christmas Cheer competition for two Toyota Tazz motor cars.  The branch sold a total of 4000 tickets raising a healthy R14 000.00 from the project.

·        Boniswa Banga is being sponsored by the John and Ester Ellerman Trust to attend the International Mobility Congress being held in the Cape in April.

·        Youth group launched. 13 Young RD’s and their parents met in Port Elizabeth on the 12th March to launch the youth group in the Eastern Cape.

·        980 runners participated in the Sportmans Warehouse, Prominent Paint 15km road race and 5km fun run. Retina South Africa (East Cape) were the beneficiaries and received a generous R8000.00 from the event which was held on 22nd March in Port Elizabeth

·        Interactive vision workshops have been launched in 3 Port Elizabeth suburbs – Walmer, Western suburbs and Kwazakhele. The workshops will provide a non-threatening environment for information sharing.  For more

information contact Gail Cillie at the PE office on 041 373 3896.

 

NEWS FROM THE NORTH GAUTENG BRANCH

The Golf tour to Warmbaths raised R3000. The next tour will be to the Wildcoast Sun from 23-27 July.  Places still available.  Thanks to the co-ordinator Kas Walkers.

Art Competition 2003:

          The theme this year will be “Feel me – See me” to encourage 1,2, and 3 dimensional works.

Mamelodi Outreach Project:

          Nkone Mokoele continues to expand this exciting project.  Thanks to Professor Roux at the Pretoria academic Hospital for organising confirmation of diagnosis for this project.  Professor Roux is head of Ophthalmology and a member of our Scientific Medical and Advisory Board.

Contact Poppie Strydom on 012 5460373 for more information.

 

PRETORIA EYE INSTITUTE

Thanks to the management and staff of the Pretoria Eye Institute for their tremendous support of the North Gauteng Branch.  The institute hosts all branch meetings and referrals of Retinal patients from practicing Ophthalmologists and Optometrists is outstanding. Thank You.

 

POLOKWANE MINI-BRANCH

Congratulations to Poppie Strydom and the new convenor in Polokwane,

Stefan Steyn for getting this mini-branch going.  An inaugural meeting was held on the 7th of December 2002.  Blood samples for the gene tracking project

were taken by Genetic Sister Hester van der Berg.  Contact Stefan Steyn on 015 2977666 for more information.

 

NATAL COASTAL BRANCH

Age Related Macular Degeneration:

An educational workshop on Age-Related Macular Degeneration was given by Claudette Medefindt at the La Lucia Mall on Thursday 4th April.  Thanks to Low vision Specialist Joanne Kalil for co-ordinating this AMD group.

Blood Clinic:

Blood samples for the gene tracking project will be taken at the Durban Society for the Blind on May 15th from 9am to 13:00pm. Contact John at 031 3126212 during office hours or Kathy on 031 4222940 after hours.  Our genetic Sister from Cape Town, Lecia Bartmann will be taking blood and answering your questions. Don’t miss this opportunity to start getting your specific gene identified.  Those families whose genes are tracked will be first in line for future therapy trials.

 

WESTERN CAPE:

Nikki de Kok is the new Youth Group covenor. Contact her on 0829044834.

 

FREE STATE BRANCH:

Calling all members in Bloemfontein to become involved in branch activities contact Willie on 051 4361007.

 

UNDERSTANDING SCIENCE

To help you understand the exciting developments in science and research we will be explaining various scientific terms in each newsletter.

Structure of the eye: -

·        CORNEA:  This is the outer protective lining in the front of the eye

·        IRIS:  This is the coloured part of the eye.

·        PUPIL:  This is the opening in the centre of the eye, getting smaller or larger to let in more or less light

·        LENS:  This lies behind the pupil and changes shape to change the point of focus point of light onto the Retina

·        CATARACT:  This is a cloudiness of the lens that causes loss of vision

·        VITREOUS:  This is the white part of the eye that keeps the eye in shape like the air keeps a soccer ball in shape

·        RETINA:  The Retina lies at the back of the eye and acts like a film in a camera.  The Retina changes light into messages which are sent by the OPTIC nerve to the part of the brain where “seeing” occurs – the VISUAL CORTEX

More about the Retina in the next Newsletter.

 

Human Genome: -

·          CHROMOSOMES:  Inside the nucleus (centre) of each cell in your body are two sets of information codes, inherited from each parent.  The codes are broken up into 23 “chapters” known as chromosomes.  The chromosomes are numbered from 1 to 22.  The remaining pair is known as the sex chromosomes.  They are either two X chromosomes in females, or an X and a Y in males.  Collectively these chromosomes are known as the human GENOME.

·          GENE: inside each chromosome are thousands of sets of instructions to produce complex PROTEINS, the building blocks of all cells – these are our GENES.  There are approximately 60 000 genes in each set of chromosomes.  There are often small differences between the maternal and paternal copies of each gene.  Some differences lead to an incorrect copy of a protein being made in the cell.  These differences can be traced through the family.  Genes known to code for proteins that are used by the eye for vision can be checked for errors.  Tracking genes and looking for incorrect copies of a specific gene in a family is known as Gene Tracking.

 

SCIENCE NEWS

Moving to Clinical Trials:

The American Foundation Fighting Blindness (FFB) sponsored a workshop on January 30th to consider the clinical challenges facing the development of treatments and cures for Retinal degeneration.  Leading International Ophthalmologists and Researchers attended.  Extracts from Tom Hoglund’s report:

“The workshop participants spoke frankly about the great potential and the great challenges facing us.  Three major scientific themes emerged.”


Animal Models:

FDA approval to test new therapies in clinical trials hinges on the safety and efficacy of the therapy in animals.  All of the treatment breakthroughs so far have come from such trials.  Unfortunately animal models do not exist for every retinal degenerative condition.  More funding is required to discover or develop animal models for all these conditions.

 

Genotyping: (Genetic Diagnosis)

Imagine the horror of developing a successful treatment but not being able to identify the patients who would benefit from the treatment.  All treatments will require that we know each patient’s genetic diagnosis or genotype.  We have the scientific ability to do this but lack the funding.  Federal support for genetic research ends once a gene mutation is found and private laboratories cannot offer affordable genetic testing services.  To avert a future where clinical trials and treatment go begging for patients we need to increase funding to genotype all patients.

[Editors note: In South Africa genotyping is being offered FREE of charge]

 

Gene and Drug Therapies

Researchers have discovered many new gene and drug therapies that show effectiveness in animal models.  At last we have the knowledge to beat and possibly cure Retinal degenerative diseases. As we head toward clinical trials, research needs will become even greater.  For every promising experimental therapy, toxicity studies must be completed to test the safety of the treatment.  Long term effectiveness and dangers also need to be tested.  The hard work and capital we’ve invested in developing treatments and cures now demands even greater support.  We cannot let these precious discoveries languish on the laboratory bench.

 

Workshop on Artificial Vision: -

The 1st International Symposium on artificial vision will be held in Fort Lauderdale, Florida, USA on May 2, 2003.  Researchers from America, Germany and Japan will meet to present papers and review progress on various methods of artificial vision.  One of the major focuses will be on various forms of the artificial silicon retinal prosthesis.  We look forward to the outcome of this meeting and in the longer term a retinal prosthesis that will restore some form of reasonable vision.

 

Historic Partnership: -

From a report by Tom Hoglund:

The American Foundation Fighting Blindness and OxiGENE INC. recently signed a historic agreement to conduct a clinical trial of Combretastatin.  This promising drug will be tested in the wet form of age related macular degeneration. FFB will fund Phase 1 and 2 clinical trials.  This is the first time that the FFB has funded a human clinical trial.

The drug was tested in a rodent model for wet AMD and appears to inhibit the growth of aberrant blood vessels while not affecting normal blood vessels in the retina.

Editors note: Wet Age Related Macular Degeneration is a particularly devastating form of the disease that can cause rapid and severe loss of vision. Combretastatin is derived from the African Bush willow and is also under trial as a treatment against cancer.

 

NATIONAL GENETICS CONGRESS

The National congress on Human Genetics will be held in Durban from 11th to 14th May, 2003.  Besides presentations from our local researchers, Dr. Bill Hausewirth an international expert on Retinal Gene therapy, will be presenting two papers: -

·      Gene based therapies for Retinal Neovascular Diseases and;

·     Restoration of vision in a canine model of Leber Congenital Amaurosis, an early form of congenital blindness.

While he is here Dr Hausewirth will be addressing Eye Care professionals and members on gene therapy in Johannesburg, Durban and Cape Town. Those wanting to attend should email us at national@rpsa.org.za or call on

011 622 4904

 

GENE TRACKING PROJECT AT UCT

Of the thousands of people in South Africa affected by retinal conditions an alarmingly low percentage have donated blood to the gene-tracking project.  This service is offered free of charge to all South Africans affected by Retinal degenerative conditions.

Without a genetic diagnosis you may be left out of future clinical trials or treatments. Contact the national office for more details.

NEW GENE FOUND FOR MACULAR DEGENERATION

Researchers at the University of Michigan have found that a gene on the X chromosome, known to cause around 70% of the RP3 form of X-linked Retinitis Pigmentosa, can also cause macular degeneration.

Results of this study may help researchers find treatments for age related forms of macular degeneration.

 

GENES ASSOCIATED WITH AGEING IDENTIFIED: -

Dr Anand Swaroop, also at the University of Michigan, has identified genes expressed in the retina that are affected by ageing. Of the 1200 genes that are expressed (active) in the retina, 24 genes were found to be altered during ageing.

“While we still don’t know what causes AMD, we do know that the strongest factors are age and family history” said Swaroop.

Researchers speculate that some people carry genetic weaknesses or variations that only surface later in life.  The ageing process may then trigger or reveal such variations, which may then lead to AMD.

 

 

 

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The success or failure of bringing research successes to actual treatment and therapy is up to us.  Your support is critical.

 

REPORT ON RETINAL REGENERATION DISORDERS RESEARCH AT UCT

 

X-Linked Retinitis Pigmentosa

The DNA of X-Linked families was screened for the RPGR gene. Some families were found to have a mutation in the one section of the gene.

One large family has had the results delivered to them and another family is being counselled at the present time.

A third X-Linked family (RP2) with a different mutation gene has also been counselled.  In X-Linked RP the females carry the genetic defect.  In addition 109 males with no identifiable inheritance pattern were also screened for these X-Linked genes.


Dominant RP17 Gene Research

This gene, in a large family of German origin with dominant RP, has been worked on for many years.  Constant effort and hard work have consistently narrowed the region where the gene is elusively hiding.  The new million rand transgenomic wave DNA analysis machine acquired by the laboratory has helped in the search. A new collaboration with Professor F. Cremers in Holland will speed up the hunt for this important gene.

 

Dominant Retinitis Pigmentosa – RP13

This gene was identified in 2001 in a large South African family of British origin.  Other individuals with dominant RP have been screened but other than members of the original family no other linkage has been found.

 

Stargardt Dystrophy

A comprehensive study of this early onset macular degeneration continues.  Allison September, who is working on her PhD in this project spent a year at Oxford with Dr Richard Callaghan. This gene on chromosome ONE has been linked not only to Stargardt Dystrophy but also to Fundus Flavimaculatus, Recessive Cone Rod Dystrophy, recessive Retinitis Pigmentosa and possibly age-related Macular Degeneration.  A panel of 77 unrelated individuals were used for a mutation-screening programme.  The study has been complicated by the fact that affected individuals may carry 2 different mutations of the gene.  At least one mutation has been found in 54% of the patients studied and at least 2 diseases causing mutations were found in 22% of the patients.

Screening of Stargardt families for another Stargardt gene on chromosome 6 will be undertaken this year.

 

Rhodopsin

The gene on chromosome 3, which codes for Rhodopsin has been shown to account for 30 – 40% of Dominant RP in most western countries.

This gene accounts for a significantly lower percentage of ADRP in South Africans. A total of 114 ADRP patients have been screened for this genetic mutation and an additional 206 other patients have been partially screened.  A Rhodopsin mutation in 5 ADRP families has been found which only accounts for about 10% of identified families.

A Rhodopsin mutation was also found in a recessive RP patient.  All families have been informed and 5 families have received genetic counselling.

 

Usher Syndrome

Past collaboration with Professor Bill Kimberling from Nebraska resulted in 7 families being linked to 3 different disease causing mutations.

A new M.Sc graduate Nathabiseng Motloi will do further research on 44 Usher families this year in the department of Human Genetics at UCT.

 

Results

A total of 21 families have a definite molecular result and 9 of these families have been contacted.

Future plans, besides extra work in these existing areas include a joint project with the Division of Cell Biology at UCT to try and develop a model for repairing damaged retinal cells using Neural Stem cells.

 

EDITORS NOTE: -

Funding for this project is raised by members of Retina South Africa.  How do you support this fund raising?