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Parents’ Role in Ophthalmic Care for DS
Ophthalmic Disorders Among Children with Down Syndrome: An
Insight Into Parents’ Knowledge, Awareness Level and Attitude
ABSTRACT
Objectives: This study attempts to probe into parents’knowledge, awareness and
attitude regarding management of ophthalmic disorders among children with Down
syndrome (DS).
Patients and Methods: This cross-sectional study was conducted during the World
Down Syndrome Day event on March 21, 2013 in Jeddah, Saudi Arabia. Parents of
children with DS who attended the event were requested to participate in the survey
by responding to a questionnaire. The questionnaire assessed parents' knowledge,
awareness level and attitudes toward common eye diseases that affect children with
DS. Descriptive and analytical statistical methods were used to analyze data, data
were collected on 21st of march, analyzed and written between that day and February
2014.
Results: A total of 72 parents accepted to respond to the questionnaire. Only 49
parents (74.2%) reported taking their children for ophthalmology visits, and 27
(37.5%) among these reported taking their children for routine visits at varying
intervals. A total of 34 parents (51.3%) did not perceive common ophthalmologic
disorders in their children with DS.
Conclusions: Most parents are not aware of the ophthalmologic disorders that affect
children with DS. This highlights the need to embark upon large scale awareness
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Parents’ Role in Ophthalmic Care for DS
campaigns to educate parents of children with DS. Parent’s awareness is a crucial
factor of early diagnosis and proper follow up of ocular disorders in children with DS.
Keywords: Down syndrome, eye disorders, ocular diseases, ophthalmology, trisomy
21
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Parents’ Role in Ophthalmic Care for DS
INTRODUCTION
Down syndrome (DS), also known as trisomy 21, is a genetic disorder caused by the
presence of an extra copy of chromosome 21. It is the most common chromosomal
abnormality in humans. In Saudi Arabia, the incidence of DS has been reported to be
1 in 554 live births1; while in the United States, 1 in every 691 babies is born with DS
each year, according to the Centre for Disease Control (CDC).2
Common eye problems that occur in any group of children are more frequent in those
with DS and sometimes tend to be more marked.3 Children with DS have obliquely
placed eyes. The internal canthi are more than normally distant from one another and
the palpebral fissure is very narrow. In addition to these external ocular features,
several ocular manifestations; including strabismus, nystagmus, keratoconus, cataract,
hyperplasia of the iris, and refractive errors occur more frequently in children with DS
than in their healthy counteraprts.4, 5. The prevalence of refractive errors (hyperopia,
myopia, and astigmatism) reported in the medical literature ranged between 3% and
62.3%.5, 6, 7, 8, 9, 10; while that of strabismus ranged between 5% and 35% 5,10, 11, 12 and
that of esotropia was higher than that of exotropia in all the studies.5, 10, 11,12 Other
ocular disorders reported in patients with DS include conjunctivitis (5.6%–6.7%,6, 13
and glaucoma (6.7%).13
Another study conducted at the University Eye Hospital of Ljubljana,
Sloveniashowed that ocular findings in children with DS included nystagmus
(29.2%), esotropia (26.1%), epiphora (21.5%), Brush field spots (16.9%), lens
opacities (12.3%), abnormalities of the retinal vessels, foveal hypoplasia or retinal
pigment epithelium hyperplasia (32.2%), and optic disc pallor (7.6%). Hyperopia
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Parents’ Role in Ophthalmic Care for DS
(36.9%) was the most frequent refractive error in the group, followed by astigmatism
(29.2%) and myopia (24.6%).15
DS is almost always diagnosed at birth and it requires lifelong parenting and
supervision. Hence, parents’ awareness is crucial in the prevention and treatment of
eye conditions in children with DS. However, hitherto, there seems to be a serious
dearth of medical evidence highlighting the awareness and knowledge levels of
parents of children with DS about ocular diseases and their attitude towards this
frequently reported complication of DS.
The current study was carried out to investigate awareness and knowledge levels and
attitudes of parents of children with DS toward ophthalmic disorders that are
frequently encountered among this category of children.
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Parents’ Role in Ophthalmic Care for DS
MATERIALS AND METHODS
A cross-sectional design, questionnaire-based survey was conducted among parents of
children with DS who attended the World Down Syndrome Day event, a public
awareness campaign organized on March 21, 2013 at King Abdulaziz University
Hospitalin Jeddah, Saudi Arabia. The questionnaire was presented to all attendees and
explained by the investigators, to be auto-filled by volunteering participants. The
informed consent was orally obtained from all participants prior to inclusion and the
study was approved by the Biomedical Ethics Research Committee of King Abdulaziz
University.
In an attempt to increase the response rate, the questionnaire was designed in a simple
fashion including the following items: 1) child’s age and gender, 2) history of visits to
an ophthalmologist, 3) frequency of routine visits to a physician, 4) eventual
participants’ source of information on ophthalmic diseases, 5) awareness of
participants about 15 listed eye conditions (e.g. up-slanting eyes, epicanthal folds,
myopia, hyperopia, strabismus…etc.) and 6) their knowledge about the past or present
existence of each of these conditions in their respective children. The questionnaire
underwent face and content validity prior to study.
Statistical Analysis
Data were analyzed using the Statistical Package for the Social Sciences (SPSS Inc.,
Chicago, IL, USA), version 20.0. Descriptive statistics were performed to analyze all
variables including response patterns of the participants. According to the number of
eye conditions acknowledged, participants were divided into three knowledge-level
categories: A) weak (0 to 4 conditions); B) fair (5 to 9 conditions); and C) good (10 to
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Parents’ Role in Ophthalmic Care for DS
15 conditions) knowledge. Cross-tabulation was performed to correlate the level of
knowledge with the frequency of ophthalmology clinic visits; as well as with the
existence of ophthalmologic history in the affected child. Chi-square test was used to
analyze the correlation between categorical variables. Results were considered
statistically significant for a p-value of < .05.
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Parents’ Role in Ophthalmic Care for DS
RESULTS
All of the attendees (N=72) of the awareness campaign who were approached by the
researchers on a convenience sampling agreed to complete the questionnaire,
representing a response rate of 100%. The age of the participants’ children affected
with DS ranged between 1-36 years, with a mean (SD) = 9.2 (6.9) years and a gender
distribution as 31 (43.1%)males and 36 (50.0%) were females; while gender of the
child was not indicated in 5 (6.9%) of cases.
Half of the study participants (n=36; 50.0%) reported that their children had or had
experienced ≤ 2 of the listed eye conditions and 30 (41.7%) reported ≥3;while only 6
responded that their child had not experienced any ophthalmic disorder. Amongst the
participants, 59 (81.9%) reported a history of eye surgery in their children and none of
them reported that their child was blind.
Forty-nine participants (74.2%) reported taking their child for a visit to an
ophthalmologist; however, only 27 (55.1%) of these reported regularly taking them
for a follow up visit (Table 1). The most common and notable sources of information
reported by a sizeable number of parents were the internet (n=41; 56.9), following by
ophthalmology clinics (n=28; 38.9%), and books (n=22; 30.6%). Other sources
reported by parents included television, media publications and “word-of-mouth”
information.
Table 2 shows the frequency of responses to questions that assessed the participants’
knowledge of an ophthalmic disorder in their child with DS. Low awareness levels
were noted among participating parents, particularly regarding the common
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Parents’ Role in Ophthalmic Care for DS
ophthalmologic conditions encountered in children with DS, namely; glaucoma,
cataract, strabismus, nystagmus, lacrimal duct obstruction and corneal and retinal
abnormalities.
Table 3 shows the frequency of ophthalmic conditions perceived or understood (terms
were explained by participants that matched the true definition or near full definition
in text books) by the participants and those reportedly experienced by their children.
Notable findings include epicanthal folds, up-slanting eyes and myopia to be the most
frequently perceived ophthalmologic disorders and, at the same time, the most
frequently experienced by the participant’s children. Cataract, glaucoma, strabismus
and lacrimal duct obstruction were also perceived as potential ophthalmologic
problems by a sizeable number of parents in the study; although they reported that
their children had never suffered from any of these disorders.
Many parents did not perceive or understood common ophthalmologic disorders in
their children with DS, as suggested by the responses to questions that assessed the
participants’ awareness of common ophthalmologic conditions—the proportion of
parents who were knowledgeable about common eye disorders that affected children
with DS was 48.5% (Table 4); however, the result did not reach statistical
significance.
Regarding the site of diagnosis, 41 of 63 participants (65.1%) who responded to the
question reported that their child’s ophthalmologic disorder was diagnosed at an
ophthalmology clinic; 5 (7.9%) at a pediatric clinic; , and only one case at a family
medicine clinic, while the remainders 11 (17.5%) of cases were diagnosed at other
locations. Eleven parents (17.5%) reported the disorder was diagnosed at birth.
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Parents’ Role in Ophthalmic Care for DS
DISCUSSION
This study is novel in its objective as this is the first attempt ever to assess parents’
awareness levels and perception of common eye disorders among their children with
DS. As a result, the findings of this study could not be contrasted with other previous
comparable data. However, findings emanating from studies performed among the
general population revealed that parents generally fail to recognize the
commencement of visual disturbances in their children. A recent study by the Vision
Council of America reported that 27% of parents and guardians who had not taken
their children for an eye exam believed that their children were too young to develop
vision problems.16 Probably one of the most alarming findings of the previous study is
the misplaced belief among the common masses that eye check-ups are only necessary
when a vision problem appears or when a pair of spectacles is needed.16
In our study, 49 of 66 parents (74.2%) reported taking their children for a visit to an
ophthalmologist and only 27 (55.1%) admitted taking their children for a follow up
visit. This leads us to infer that DS-related ophthalmologic disease may be unreported,
and thus undiagnosed, in several cases. In several other instances, cases might be lost
to follow-up. Underdiagnoses, under-reporting and delayed diagnosis of visual
disorders is likely to be the tip of the iceberg among all the medical concerns in DS.
Consequently, children with DS may be at greater risk for developing end-stage eye
complications including impaired vision.
Although our questionnaire was administered in the local language, using simple
words and avoiding as much as possible the use of medical terminology or
professional jargon, we found that ocular conditions were unfamiliar to some parents
whose children might have experienced or were experiencing such conditions at the
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Parents’ Role in Ophthalmic Care for DS
time of the survey. On the other hand, with regards to the main objective of our study,
we observed variable levels of awareness and knowledge between the specific eye
disorders when each was investigated separately.
We demonstrated that common ophthalmic disorders were insufficiently known by
parents of children with DS, as evidenced by the low frequency (48.5%) of their
accurate identifications in the study participants. Regarding knowledge sources,
parents mainly obtained information about ophthalmic disorders from the internet and
ophthalmology clinics.
Because eye disorders are more common in children with DS 4, 5, it is crucial for
parents of patients with DS to be more aware of ophthalmic diseases that may affect
their children. 3 Health care providers should organize public awareness campaigns to
promote routine regular follow up visits in children with DS, including
ophthalmologic visits. Education programs could be organized at ophthalmology
clinics, using visual and audio material focusing on eye health in children with DS.
Parents’ participation is crucial to the care of patients with DS. Therefore improving
parents’ knowledge and raising their awareness about the increased ophthalmologic
risk in their children is a critical determinant in the primary and secondary prevention
of ophthalmic disorders, ensuring early diagnosis and proper follow up.
This study has some limitations that warrant discussion. First, the small sample size of
72 is not representative of the population of children with DS in Jeddah. In addition,
participants were recruited during an awareness campaign, which may constitute an
important selection bias as the voluntary attendance of such events suggests the
preexistence of a basic level of awareness. Moreover, we did not collect participants’
demographic data, mainly for the sake of simplification. However, findings of a study
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Parents’ Role in Ophthalmic Care for DS
conducted in families of children with DS showed that family demographics were
likely to be important factors for healthy family function and consequently for the
quality of care of children with DS.17 Further large scale multi-centric evaluations are
warranted to reach more robust results and conclusions regarding the issue, which
may provide decisive and constructive information to improve the management of
patient with DS in the future.
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Parents’ Role in Ophthalmic Care for DS
Conclusion
Major lacunae exist in knowledge and awareness about the ophthalmic risk associated
with DS, among parents of afflicted children. Epicanthal folds, up-slanting eyes and
myopia were the best known among the investigated ophthalmologic disorders;
probably because they were the most frequently experienced by the participant’s
children. On the other hand, more common ophthalmologic conditions, such as
glaucoma, cataract, strabismus, nystagmus and lacrimal duct obstruction were
associated to lower awareness levels.
Given that parents’ participation is crucial to the care of patients with DS, educating
parents and raising their awareness about the increased ophthalmologic risk in their
children is a determinant factor for early diagnosis and proper follow up of
ophthalmologic disorders in these patients. We recommend the use of Down
Syndrome Medical Interest Group guidelines, which were developed for people with
DS to outline the basic medical essentials of follow up of their disease.18
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Parents’ Role in Ophthalmic Care for DS
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