Research out of the University of Tennessee Health Science Center(UTHSC) examined the ophthalmic impact of sickle cell disease (SCD) in pediatric patients to distinguish how prevalent the condition can be in this younger demographic.
The findings were presented by Barbara Smith, MD, at the American Academy of Ophthalmology (AAO) annual meeting.
Give me some background first.
As a rare, inherited blood disorder, SCD occurs when there are abnormal copies of genes that make hemoglobin—causing red blood cells to become hard and sticky, resembling a c-shaped farm tool called a “sickle.”
Among complications such as frequent infections, hand and foot swelling, periodic episodes of pain, and anemia (to list a few), vision problems can also be part of the symptomatology for SCD patients.
As such, screening for SCD is largely conducted in newborns and older adults.
What causes this?
This occurs when the sickle-shaped cells are trapped within the small blood vessels located within the anterior and posterior segment. Symptoms and sequelae might include blind spots, a sudden onset of floaters, or blurred vision.
In other words: sickle cell retinopathy (SCR), can be considered both a retinal dystrophy and an age-dependent process with older patients primarily at a higher risk.
To note, though, patients with the spectrum of retinal conditions associated with SCD often may not present with such symptoms.
Which brings us to…
The Sickle Cell Clinical Research and Intervention Program (SCCRIP), is an ongoing longitudinal cohort study that originally targeted the younger demographic patients diagnosed within the developmental stages of SCD (birth to 18+ years) to examine potential ocular manifestations.
The goal: To produce care guidelines along with potentially enhanced treatment options.
Now talk about the study.
Investigators conducted a retrospective chart review of 652 SCD patients (ages 10 to 25 years; median age = 14) who had a dilated eye exam from October 2010 to September 2022.
To note, all patients’ eye exams were under surveillance during a grand total of 2,240 visits over the course of the review period.
What types of DR did these patients have?
- 33% = Nonproliferative sickle retinopathy (NPSR)
- 6% = Proliferative sickle retinopathy (PSR)
And what kind of treatment?
Per the presentation, 30 eyes received panretinal photocoagulation (PRP)—largely for PSR stage 3 (43%)—while intravitreal anti-vascular endothelial growth factor (VEGF) therapy was administered to five eyes (also with PSR).
Any complications observed?
According to the authors, retinal detachment (RD) and retinal artery occlusion (RAO) were observed in two patients each.
Further, one patient with a central retinal artery occlusion (CRAO) experienced vision loss—with a final best-corrected visual acuity (BCVA) of 20/60—after SCD complications.
What else?
Per an AAO news release, the effectiveness of various SCD therapies were also analyzed: two treatments—hydroxyurea and chronic transfusions—were correlated with decreased rates of retinopathy, including when accounting for different genotypes.
And overall?
Based on the data, one in three patients were found to have retinopathy, of which only 9% required treatment—suggesting the need for pediatric patients to be screened for vision problems as often as adult SCD patients.
And expert input?
“Our data underscores the need for patients— including pediatric patients—with sickle cell disease to get routine ophthalmic screenings along with appropriate systemic and ophthalmic treatment,” stated Mary Ellen Hoehn, MD, UTHSC lead researcher and professor of Ophthalmology.
The presentation, Evaluation of Pediatric Patients With SCR Enrolled in a Cohort Study at One of the Largest Sickle Cell Programs in the Country, was presented on Saturday, Nov. 4, 2023, during the annual AAO 2023 meeting in San Francisco, California (Nov. 3-6, 2023.)