Causes of Blindness & Methods of Prevention & Treatment

The relative importance of various causes of blindness differs according to the level of social
development in the geographic area being studied. In developing countries, cataract is the
leading cause, with trachoma, glaucoma, leprosy, onchocerciasis, and xerophthalmia also
being important. Corneal ulceration is also a significant cause of monocular blindness in the
developing world. In more developed countries, blindness is to a great extent related to the
aging process. Cataract is still important despite the availability of facilities for its treatment,
along with age-related macular degeneration and glaucoma. Other causes are diabetic
retinopathy, herpes simplex keratitis, retinal detachment, and inherited retinal degenerative
disorders.
In terms of the worldwide prevalence of blindness, the vastly greater number of people in the
developing world and the greater likelihood of their being affected mean that the causes of
blindness in those areas are numerically more important. Cataract is responsible for more
than 22 million cases of blindness and glaucoma 6 million, while leprosy and onchocerciasis
each blind approximately 1 million individuals worldwide. Interestingly, the number of
individuals blind from trachoma has dropped dramatically in the past 10 years from 6 million
to 1.3 million, putting it in seventh place on the list of causes of blindness worldwide.
Xerophthalmia is estimated to affect 5 million children each year; 500,000 develop active
corneal involvement, and half of these go blind. Central corneal ulceration is also a
significant cause of monocular blindness worldwide, accounting for an estimated 850,000
cases of corneal blindness every year in the Indian subcontinent alone. As a result, corneal
scarring from all causes now is the fourth greatest cause of global blindness.
WHO estimates that up to 80% of blindness in developing countries is avoidable, ie,
preventable or treatable. The worldwide eradication of smallpox demonstrates what can be
achieved in the area of infectious disease and the superiority of prevention over treatment.
Similar efforts are being made to prevent the infectious diseases trachoma, leprosy, and
onchocerciasis as well as the noninfectious xerophthalmia. The sheer numbers of individuals
blinded by cataract continues to overwhelm the resources available. In all programs to reduce
blindness in the developing world, cooperation between governments and nongovernmental
charitable organizations has proved to be essential. The WHO Prevention of Blindness
Program has established centers in about 60 developing countries to undertake collaborative
studies, particularly generating epidemiologically sound information to form the basis for
rational planning, implementation, and proper evaluation of programs for prevention of
blindness.
In more developed countries, the causes of blindness are less amenable to prevention. In
general, it is necessary to rely on recognition and treatment of the early stages of the disease.
This depends on education of ophthalmologists, nonophthalmologic medical personnel, and
lay people about the necessity for screening for glaucoma and diabetic retinopathy and about
the importance of the early symptoms of retinal detachment, age-related macular
degeneration, and herpes simplex keratitis. The inherited conditions are amenable to
prevention by genetic counseling.
Cataract
Cataract accounts for at least 50% of cases of blindness worldwide. As life expectancy
increases, there is a continuing rise in the total number of people affected. In many parts of
the developing world, the facilities available for treating cataract are grossly inadequate,
hardly sufficient to cope with the new cases arising and completely inadequate for dealing
with the backlog of existing cases, which is conservatively estimated to be 10 million
worldwide.
It is not clearly understood why the frequency of cataract in different geographic areas varies
so greatly, although exposure to ultraviolet radiation and recurrent episodes of dehydration,
such as occur in severe diarrheal diseases, are thought to be important. If medical means
could be found to delay the development of cataract by 10 years, it is estimated that this
would reduce the number of individuals requiring surgery by 45%. Unfortunately, there is no
method of preventing or retarding the growth of cataracts. Although the oral administration of
antioxidants was considered promising, clinical studies have now shown conclusively that
they have no effect on cataract growth. Mobile eye camps have aided in identifying patients
for surgery, but surgery is no longer performed in a camp setting and there are too few well-
equipped hospitals and trained surgeons in many developing countries to keep up with the
load. In a number of blindness surveys, the problem of uncorrected aphakia is particularly
apparent. It is now accepted that intraocular lens implantation at the time of surgery, although
requiring greater expertise, is a better solution than relying on the subsequent provision of
spectacles.
Trachoma
Trachoma causes bilateral keratoconjunctivitis, generally in childhood, that leads in
adulthood to corneal scarring, which, when severe, causes blindness. About 400 million
people have trachoma, most of them in Africa, the Middle East, and Asia. Trachoma can be
treated with various antibiotics, including tetracyclines and erythromycin, but azithromycin is
proving to be the drug of choice. It is estimated that 70 million individuals currently require
treatment, but in the past 10 years, the number of individuals blind from trachoma has
dropped from 6 million to 1.3 million. This is an obvious tribute to current WHO-supervised
treatment programs and probably to the effectiveness of azithromycin. However, to eliminate
the disease will depend on global implementation of WHO's SAFE strategy (surgery for
trichiasis, antibiotic treatment, face washing, and environmental changes such as latrine
building). Prevention of spread of infection will require provision of proper sanitary facilities,
including clean water for drinking and washing, waste disposal, fly control, and behavioral
change in hygiene.
Leprosy
Leprosy (Hansen's disease) affects 14 million people in the world and has a higher percentage
of ocular involvement than any other systemic disease. Up to 10% of leprosy patients are
blind or visually impaired from the disease. The social stigma attached to leprosy has greatly
hindered its treatment, but there are now highly effective chemotherapeutic agents that in
most cases eradicate the infection. Effective treatment programs using triple drug therapy
(dapsone, clofazamine, and rifampin) have markedly reduced the number of cases of leprosy
worldwide as well as prevented the deformity and morbidity associated with the disease.
Onchocerciasis
Onchocerciasis is transmitted by bites of the blackfly, which breeds in clear running streams
(hence the name river blindness). It is endemic in the greater part of tropical Africa and
Central and South America. The most heavily infested zone is the Volta River basin, which
extends over parts of Dahomey, Ghana, Ivory Coast, Mali, Niger, Togo, and Upper Volta.
Worldwide, 1520 million people are affected by onchocerciasis, with 20% of individuals in
hyperendemic areas blinded by the disease.
The major ophthalmic manifestations of onchocerciasis are keratitis, uveitis,
retinochoroiditis, and optic atrophy. The disease is prevented by insect eradication and
personal protection by screening. Treatment with ivermectin is extremely effective in killing
the microfilaria and sterilizing the adult females residing in nodules in the body. The effect of
the mass distribution of ivermectin in areas where onchocerciasis is endemic is a public
health success story. Like leprosy, onchocerciasis is definitely decreasing in its importance as
a worldwide cause of blindness because of successful treatment programs.
Xerophthalmia
Xerophthalmia is due to hypovitaminosis A. Clinically, there is xerosis of the conjunctiva
with characteristic Bitot's spots and softening of the cornea (keratomalacia), which may lead
to corneal perforation. Protein malnutrition exacerbates the condition and renders it refractory
to treatment. Xerophthalmia is a common cause of blindness in infants, particularly in India,
Bangladesh, Indonesia, and the Philippines. Affected infants often do not reach adulthood,
dying from malnutrition, pneumonia, or diarrhea.
Xerophthalmia can be prevented by general dietary improvement or vitamin A
supplementation. If the problems of distribution and administration were solved, the cost of a
quantity of the vitamin sufficient to prevent blindness in 1000 infants would be only about
$25.00. Measles immunization is also important in this regard because of the close
association of measles epidemics with the blinding complications of xerophthalmia.
Other Causes
Glaucoma, retinal detachment, diabetic retinopathy, and herpes simplex keratitis are
discussed in greater detail elsewhere in this text. The incidence of blindness due to glaucoma
has decreased in recent years as a result of earlier detection, improved medical and surgical
treatment, and a greater awareness and understanding of the disorder by the lay population.
However, in many developing countries, glaucoma is the second most common cause of
blindness after cataract. This is especially the case in West Africa, where untreated open-
angle glaucoma is extremely common. In China and Southeast Asia, there appears to be a
preponderance of narrow-angle glaucoma. Glaucoma now blinds 6 million individuals
worldwide, and a simple easy method of detecting patients at risk still does not exist.
Treatment is also a major problem because of the poor compliance of most patients for taking
daily eye drops. A simple but safe surgical procedure may ultimately be the only solution for
reducing the needless burden of blindness from this disease. More research in this area is
essential.
Diabetic retinopathy is an increasingly more common cause of blindness everywhere in the
world. Recent advances in surgical treatment (vitrectomy, laser therapy) are of some help, but
many patients still suffer from proliferative retinopathy, recurrent vitreous hemorrhages, and
eventual bilateral blindness. A vast research effort directed at all aspects of diabetes is in
progress, and there is justification for hoping that the next generation of diabetics will benefit
greatly from what is being done now.
Hereditary conditions are important causes of blindness but should gradually decrease in
incidence in response to the efforts of genetic counselors to increase public awareness of the
preventable nature of these disorders.
As is true also in other countries where medical care and social services are widely available,
blindness in the United States is to a great extent related to the aging process, and about half
of the legally blind people in this country are over age 65. The leading causes of blindness in
this age group are degenerative retinal disorders, glaucoma, diabetes, and vascular diseases.
Costs of Avoiding Blindness
Some examples of what can be achieved for modest outlays of scarce funds are as follows:
1. To cure one person of trachoma in Saudi Arabia: $1.25.
2. To restore vision to one person in India blinded by cataracts: $30.00.
3. To prevent blindness due to xerophthalmia in one infant in Indonesia: 30 cents.
On the advice of WHO experts, the World Council for the Welfare of the Blind and several
international professional ophthalmic societies and agencies agreed to take the initiative,
which led to the establishment in 1974 of the International Agency for Prevention of
Blindness (Vision International), with Sir John Wilson, a blind barrister, as president. The
aim of this agency is to work with groups formed for the purpose of preventing blindness. Its
theme, Foresight Prevents Blindness, was brought into prominent display when WHO
celebrated the first World Health Day on April 7, 1976. Its goal was stated as follows: "In
every donor country during 1976, every family should be askedin thanksgiving for sight
to give $10.00 to save the sight of its fellow countrymen or of the millions in the third
world."
Rehabilitation of the Blind
Although no completely reliable statistics are available, the most widely used estimates place
the legally blind population of the United States at 2.24 per thousand (ie, approximately
500,000). Approximately 50,000 become legally blind annually, and many others have
enough visual loss to constitute a serious employment problem.
Blindness does not necessarily imply helplessness. Individual adjustment to marked visual
impairment or total blindness varies with age at onset, temperament, education, economic
resources, and many other factors. The older patient, for example, may accept blindness quite
stoically, whereas for the younger patient, the vocational or social impact of blindness is
often catastrophic. Blindness is accepted more easily by persons who are born blind and by
persons of any age who lose their vision gradually rather than suddenly.
The aim of rehabilitation is to enable the patient to lead as nearly normal a life as possible.
Approximately 5000 blind persons in the United States are rehabilitated and obtain paid
employment each year. An additional larger number of blind homemakers are able to perform
their household duties without assistance or are able to live independently of others.
Rehabilitation must be individualized. Many special services (see Appendix III) and
increasingly complex optical and nonoptical aids (see Chapter 22) are available, but they are
not universally helpful. Different categories of the blind have different needs, and some blind
people simply cannot benefit from a number of services or aids available. It has been said that
over half of the blind people in the United States are over age 65. The elderly widowed
housewife may need or want no more than mobility training in home care and a steady supply
of Talking Books. A young person facing blindness in later life due to retinitis pigmentosa
requires the full range of social services, including educational assessment, job rehabilitation,
and psychological counseling as well as a number of sophisticated aids.
The responsibility of the physician clearly does not end with the diagnosis, prevention, and
treatment of ocular disorders that might result in blindness. The physician caring for the
patient who is suddenly faced with actual or imminent blindness is in a position to be of great
assistance. When blindness is a possibility but is not inevitable (eg, during acute ocular
inflammation), optimism and reassurance are warranted. However, it is unwise to offer false
hope or to delay "breaking the news" when blindness is inevitable. If it is certain that
blindness will occur, it is important to extend to the distraught patient as well as to the
patient's family the warmth, understanding, encouragement, and assistance so desperately
needed. The physician should be alert to the severe depressive reactions that may occur.
It is especially important to assist the patient in making the adjustment to blindness while
some vision is still present. Early referral to rehabilitation agencies is essential for recently
blinded adults and those with irreversible progressive visual loss. Training programs or
reeducation for the many changes involved in daily living and employment are greatly
simplified if the patient has the partial support provided by even limited vision.
The physician should work actively with both the patient and the family and with other
professional people concerned with rendering services to the blind. The physician must know
what referral sources are available and how to use them skillfully. Medical social workers,
public health nurses, and counseling services and agencies serving the blind and visually
handicapped are common sources of reliable information. It may be valuable to have the
patient talk with a blind person who has made a satisfactory adjustment to blindness.
Mobility Training & Guide Dogs
Mobility training is most important in rehabilitation of the blind. Many state commissions for
the blind offer a wide variety of mobility training courses, either directly or in cooperation
with private agencies. The courses are offered on an outpatient and residential basis and have
varied objectives according to the special needs of the people who apply for help. The
curriculum commonly includes self-care, home functions, and mobility within the
community. Several universities
1
have undergraduate and postgraduate programs in mobility
training for the blind.
The usefulness of guide dogs is limited by their daily care needs and the physical strength
required to hold them in check. They are most useful for students and professional men and
women in good health who lead fairly well-organized lives. At this time, less than 2% of
blind people in the United States use guide dogs. Sonar sensor canes may ultimately be a
better answer to the mobility problem even for those who are now using a dog successfully.
1
Undergraduate-level programs are offered at Cleveland State University in Ohio, Florida
State University in Florida, and Stephen F. Austin University in Texas. Graduate programs
are available at Boston University, California State University (Los Angeles), Northern
Colorado University, San Francisco State University, University of Arkansas, University of
Wisconsin, and Western Michigan State University.
Braille
This remarkably effective system of reading for the blind was introduced in 1825. The braille
characters consist of raised dots arranged in two columns of three. The system is so simple
that a blind child can quickly learn to read braille, and proficient readers can learn to read
braille as fast as they can talk. The system has been adapted to musical notation and technical
and scientific uses also. An international braille code was introduced in 1951.
Braille is used less commonly now than formerly, since many blind people prefer auditory
aids both for informational and recreational purposes. But the recent availability of portable
data storage systems with braille-encoded input and conventional or braille form printed
output has brought about a resurgence of interest. Braille continues to be essential on tags
attached to items in common personal use even for people who do not wish to use it for
reading.
All paper money in the Netherlands and Switzerland is braille-printed to show the
denomination.
Electronic Devices
Optacon is an electronic device that converts visual images of letters into tactile forms. It is
easily portable and can be used with almost any kind of reading matter. Auditory aids are
becoming increasingly important (eg, talking calculators, clocks, paper money identifiers).
Financial Assistance Programs
It is unfortunate that over half of the blind people in the United States are essentially
dependent on Social Security and whatever local supplemental aid may be available to them.
For the younger blind population, rehabilitation programs are commonly administered at the
state level by a division of the department of education specifically set up to serve blind
people in the state. Some of these programs are better than others, and all physicians should
support efforts to increase the effectiveness of such programs in their geographic area of
influence. The programs are of wide scope and offer preliminary counseling followed by
academic or vocational training as the circumstances warrant. Once a realistic vocational
objective has been established, full financial support is commonly available. This single
resource is probably the most crucial referral available to the ophthalmologist, particularly in
the case of young patients. Counseling services are available as early as the junior high
school years to ensure compliance with a curriculum consistent with measured aptitudes and
interests. In many states, such rehabilitation programs as mobility training are administered
under state auspices but contracted to private agencies for operational purposes.
In many countries, the blind receive no financial or other support from their governments and
are either cared for by their families or left to manage by themselves in any way they can.
Special services available to the blind in the United States are listed and discussed in
Appendix III.
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