Diane Schuur lost her sight to the eye disease retinopathy of prematurity.

The great jazz singer Diane Schuur's latest CD Midnight is a complex, stunning arrangement of 13 well-crafted songs. Even more complex is the disease, retinopathy of prematurity, which robbed Schuur of her sight as an infant.

Retinopathy of prematurity is a disorder of the retinal blood vessels that primarily afflicts babies of low birth weight and prematurity.

"Back then ROP was called RLF," says Schuur, born in 1955, who has mesmerized audiences for four decades. "It stands for retrolental fibroplasia and it was caused because I was born two months prematurely."

In 1953, Schuur, and her twin brother, were born dangerously early and "very small." While their very survival is somewhat miraculous, the technology that saved their lives ultimately caused the ROP that blinded Schuur.

"I was born sighted but my twin brother and I were both placed in an incubator for six weeks," Schuur explains. "It was the oxygen that I received in the incubator that destroyed my vision. Those were the days when they didn't monitor the oxygen so I got too much. Fortunately, my brother's eyes are fine."

But many infants are not as lucky as Schuur's brother.

The National Institutes of Health estimate that approximately 10,000 infants develop some ROP. According to Harley's Pediatric Ophthalmology, ROP annually causes between 500-550 new cases of blindness in the United States.

But this data may not accurately reflect the reality many surgeons and retina specialists are witnessing. The increases being seen may be explained by the rise in multiple births as well as the success of neonatal intensive care units to sustain younger and younger preemies.

"I definitely think the incidence is higher," reports Khaled Tawansy, director of pediatric retina surgery at Children's Hospital Los Angeles and the University of Southern California. "My best estimation is ROP causes about 2,000 - 2,200 cases of significant vision loss in neonates annually."

"Because of the improved science of neonatology, ROP is shifting to smaller and younger babies," says Sharon F. Freedman, associate professor of ophthalmology and pediatrics at Duke University Eye Center in Durham North Carolina. "Twenty years ago these babies would not have survived. So we are seeing less severe disease in the so-called heavier premature babies and more in the lighter, younger newborns."

ROP basics

Premature birth is a critical risk factor in ROP because the retinal blood vessels begin to grow from the optic nerve towards the edges of the retina normally around 16 weeks. These vessels complete their journey across the peripheral retina -- the very edge -- around 38 weeks.

"The process of retinal blood vessel growth is programmed to occur in utero in a relatively hypoxic, or oxygen poor, environment," says Tawansy. "When an infant is born and exposed to either ambient oxygen or higher concentrations of oxygen, like an incubator or ventilator, the vascular growth process immediately stops."

In many cases the vessel growth resumes after birth. But in some cases, the growth is aborted and does not return.

"After a few weeks the doctors noticed that my eyes weren't focusing, so then they were able to diagnose me as RLF but by then the damage was irreparable," Schuur says.

When vessel growth fails to resume, the peripheral retina waiting for the blood vessels begins to demand the oxygen that the vessels ought to be carrying. Lacking sufficient oxygen, the peripheral retina produces vascular endothelial growth factor (VEGF) and other stimulants which can cause a new set of abnormal blood vessels to grow.

"The vessels can grow off the surface of the retina - this is the most severe form of ROP - and can ultimately lead to a retinal detachment which is the mechanism that causes blindness in this disease," Freedman notes.

ROP is diagnosed by five stages:

Stage 1: If the retinal vasculature has stopped growing, there is a demarcation line, basically a white line where the vessels end.

Stage 2: The demarcation line becomes elevated to form a ridge.

Stage 3: The ridge extends via new abnormal blood vessels that grow on the surface of the retina and into the vitreous gel.

Stage 4a: The abnormal vessel growth has pulled the retina to an elevated position, but the central part of the retina, the macula, is in tact. 

Stage 4b: The elevation has extended into the center of the retina.

Stage 5: The retina is completely detached and vision is lost.

Sight-saving technology

Schuur's retina was completely detached, leaving her blind. And she reports that the damage was severe enough to affect her optic nerve.

But today, careful monitoring of oxygen levels can reduce damage. And should damage begin to occur, timely laser surgery on the peripheral retina can be performed to prevent further VEGF production and reduce the disease process.

Freedman says this frequently stops the disease in the majority of cases receiving laser treatment, which is widely regarded as less invasive and gentler on the eye than the previous cryosurgery treatment for ROP.

Even Stage 5 damage can be corrected.

"We do surgery for stage 5 ROP routinely," Tawansy says. "At USC - Doheny Retina Institute, we have developed special instruments that are 25 gauge that are more appropriate to the size of the child's eye. This allows us to enter the eye and remove the abnormal vasculature from the eye in order to relax the retina so it can return to its normal position. We can either drain the fluid that is underneath the retina to attach it or we can let it reabsorb."

Tawansy reports a success rate for anatomic reattachment - ranging from 45%-50% for stage 5 detachments and up to 92% for a 4a detachment. How much vision is restored is case dependent and ranges for complete restoration of sight to no improvement in vision.

Freedman stresses that prenatal care is essential to preventing ROP from becoming an issue.

"Everything a mother can do to carry her baby to term in extremely important," Freedman urges. "But when a baby is born prematurely, it is important for the parents to realize that the eye exam is critical. And it would be very appropriate for the parents to ask if the eye exams have been done."

Despite her blindness, Schuur says she thanks God she was born in this era. And appreciates the increased public awareness about her disability and the advances in technology that make the tasks of daily living far easier today than when she was growing up.

"I am also grateful for my sobriety of over 13 ½ years," says Schuur, whose Midnight CD was produced by Barry Manilow and went on sale August 12, 2003. "My music and my health are better than ever. I have a lot of music left in me. I am very proud of Midnight."

Spotlight Health is the leading creator of celebrity-featured health-issue awareness campaigns, connecting consumers with impassioned celebrities whose personal health battles can open eyes, dispel myths and change lives. Spotlight Health helps sufferers and caregivers meet the challenges of difficult health circumstances with understandable, in-depth medical information, compassionate support and the inspiration needed to make informed healthcare choices.

Created: 8/17/2003  -  Donnica Moore, M.D.