Libby and David Durkee were in the midst of a big life change as they drove along a lonely stretch of highway near the border of western Nebraska and northeastern Colorado in August 2012. They’d departed their native Illinois for the Denver area, where David had accepted a new job with Molson Coors. The packed truck held their belongings and son Nathaniel, then 17 months old.

Excitement mixed with a bit of anxiety as the truck sped along the road. Outside of people David had met at Molson Coors, the Durkees didn’t yet know anyone in Colorado, nor had they finalized their housing. They were expecting their family of three to grow in a few months: Libby, then 33, was 23 weeks pregnant. That fact suddenly made all other concerns vanish. Libby began having contractions.

“I knew something was very wrong,” she said.

With little but corn and wheat fields surrounding them, the Durkees had no choice but to push on to Denver to get help. They checked into Lutheran Medical Center in Wheat Ridge, then transferred to UCHealth University of Colorado Hospital, where Libby delivered Oliver Patrick at 24 weeks, 2 days on August 28 – more than three months short of term. He weighed 1 pound, 5 ounces, roughly the heft of an inflated regulation basketball.

Stunning blow, but rays of hope

Shock set in as Libby gazed at the tiny Oliver, and she fell into silence for several days. “I didn’t think he had a chance,” she recalled. “You can’t look at a 1-pound baby and think this is going to be okay.” Meanwhile, a team of providers in the hospital’s Neonatal Intensive Care Unit (NICU) worked to save Oliver, who suffered from chronic lung disease and required mechanical ventilation to breathe.

Libby and David would soon learn that Oliver’s providers had another concern – one that they had never heard of. He was at high risk for retinopathy of prematurity (ROP), a disease that causes abnormal blood vessel growth in the retina, the light-receiving tissue at the back of the eye. The disease is a primary cause of blindness in children. The primary risk factors for ROP are gestational age of 30 weeks or earlier and birthweight of less than 1,500 grams, or 3.3 pounds.

Oliver’s ROP warning signs were blinking red. In addition, his stressed lungs required high concentrations of supplemental oxygen, another risk factor for ROP. In one important way, however, he and his parents were lucky. They would get care from an interdisciplinary team at UCH and nearby Children’s Hospital Colorado with long-term experience identifying, treating and studying ROP. Each infant at risk for ROP born at UCH is screened for the disease, carefully monitored and their information entered into a registry started in 2014 by the Colorado Retinopathy of Prematurity Research Group (CO-ROP).

Pools of data

The registry contains information on more than 2,000 infants and their mothers, said Dr. Anne Lynch, professor and director of the Division of Ophthalmic Epidemiology in the University of Colorado School of Medicine’s Department of Ophthalmology, who founded CO-ROP with support from her department’s leadership.

“With the size of the registry, we have the power to do good epidemiological studies,” Lynch said, noting that CO-ROP has thus far produced a dozen manuscripts based on research into the disease. In addition, the group gathers and studies data collected from mothers that could yield clues to the reasons for pre-term births, such as inflammation of the placenta or conditions like preeclampsia (high blood pressure during pregnancy) that can lead to a medically-indicated preterm birth.

“The reasons for pre-term births could potentially be related to [ROP] as well,” said Lynch, a specialist in perinatal epidemiology.

Shining light on ROP

Information gleaned from the registry could help providers tread a delicate clinical line: identifying babies most at risk for ROP while ensuring that none who are at risk are eliminated from screening, said Dr. Emily McCourt, an associate professor of Pediatric Ophthalmology and Adult Strabismus at Children’s Colorado and the CU School of Medicine.

“We want to find out what other risk factors there are for ROP other than infants being born early and small,” McCourt said. “The more we know about who is at risk, the more we can make an efficient screening tool.”

McCourt noted, for example, that CO-ROP work initiated by Dr. Jennifer Cao, a former resident in pediatric ophthalmology at CU, produced a screening algorithm for ROP that added a net weight gain of less than 650 grams (about a pound and a half) during infants’ first month of life to the gestational age and birth weight criteria. A study published by Cao, McCourt, and Lynch in 2016 demonstrated that the algorithm could successfully identify all severe cases of ROP while reducing by nearly a quarter the number of infants who would require screening.

The CO-ROP model has not yet been adopted as the standard for ROP screening, McCourt said. But she sees the work as an essential step toward ensuring that infants whose ROP progresses get treatment to arrest the blood vessel growth, either through laser surgery or, more rarely, injections while limiting the number of infants who need exams that can be stressful.

An uncertain journey

All of these questions and concerns played out in the real world for Libby and David Durkee. At seven weeks, weighing 2 pounds, Oliver lay still while his providers lifted and clamped open his eyes to examine the retinal blood vessels for signs of ROP.

The exam itself was “traumatic,” Libby said, but the results produced more anxiety: a diagnosis of moderate ROP. The disease can resolve without treatment, but Oliver wasn’t so lucky. At 11 weeks, the ROP had become severe, putting him at a 30% risk of total blindness. McCourt and Dr. Scott Oliver, associate professor of Ophthalmology and medical director of the UCHealth Eye Surgery Center at the Sue Anschutz-Rodgers Eye Center on the Anschutz Medical Campus, told the Durkees that Oliver would need laser surgery to prevent his retina from detaching from the blood vessels that nourish it. But even successful laser surgery, they added, would likely cost him a portion of his peripheral vision.

However, there was an alternative, McCourt added. Oliver could get injections of a drug called bevacizumab (trade name Avastin) to blunt the blood vessel growth in the retina and allow healthy retinal cells to grow before a later laser surgery. The option could save his peripheral vision, McCourt said, but there were risks. First, the CU team had never done the injections for this indication. In addition, data on the long-term effects of the treatment were – and remain – sketchy.

With little time to make a decision, Libby began Googling Avastin and contacted a pharmacist friend for advice. She found little information, but after a day of discussion, she and David decided to go with the injections, with the understanding that if they didn’t work, Oliver could still get the laser surgery.

An eye-opening first

With that, Oliver became the first patient in Colorado to receive injections for ROP. The first – Libby admits she couldn’t watch – was in the right eye. After exams confirmed the treatment had reversed the abnormal blood vessel growth in that eye, injections in the left eye followed. He got a second injection in the right eye at the end of December, then left the NICU on January 19, 2013 after a 141-day stay. In March, Scott Oliver performed laser surgery that completed the vision-saving treatments.

He’s still the exception, McCourt noted. “Laser is our preferred treatment,” she said. The team limits injections to infants who have very little blood flow to the retina – this was true in Oliver’s case – or those so sick that they can’t endure the time required for laser surgery, she explained.

After the surgeries, many challenges remained for Oliver. He got glasses at nine months and still wears them. He required oxygen when he came home, as well as a feeding tube that he used for more than two years. Libby and David had to keep him separated from Nathaniel, who was in day care, to protect his still-fragile lungs from colds and flu. Those worries remained for his first few winters. He needed frequent follow-up, including surgeries by McCourt to correct strabismus, a condition that prevents the eyes from aligning properly.

Today, though, Oliver has left his uncertain start far behind. Libby and David held him back from school one year and he’s still a bit small for his age, but he began first grade in August. Libby says he’s picked up reading quickly, plays sports and competes with older brother Nathaniel on math problems.

“We’re past the point of worrying about him,” Libby said.

Aftermath

She and David have had three more children since, all of whom she carried to full term, including Willa, born in May. But Libby acknowledged that the ROP ordeal left its mark. Like many caregivers, she devoted so much attention to Oliver and her family that she neglected to care for herself.

“I never looked at what [the ROP] could do to us,” she said. One day during a follow-up clinic visit with Oliver, the emotional toll suddenly swept over her. As she washed her hands, the smell of the soap evoked powerful and traumatic memories of the dark days in the NICU.

“I had a complete breakdown,” she said. “I probably should have been in therapy.”

She and David also had to learn to stand their ground when family and friends questioned their decisions about Oliver’s care. For example, they got approval from their providers to travel back to Illinois in the spring after his birth – with the proviso that they needed to protect him rigorously from infection. They asked those they visited not to hold him, which caused some hard feelings.

“We had to stand up to family,” Libby said. “I wasn’t expecting that to be so hard.”

Her advice to others who confront a similar situation? “All your friends and family are going to have opinions,” she said. “Listen to your physicians and trust your gut. Don’t let somebody guilt you into doing something you are not comfortable with.”

Peering into the future

Lynch and McCourt both underscore the importance of having a strong clinical team to support the Durkees and other parents confronting the risk of ROP and other dangers of premature birth. Lynch, for example, noted that neonatologists, obstetricians, pediatricians, retinal specialists, pathologists and biostatisticians all play a role in research related to ROP.

“We can’t do it in isolation,” she said.

McCourt also pointed to the high-quality care delivered in the UCH NICU, where ROP rates are low. Data flowing into CO-ROP can help keep them that way, Lynch added. “We continue to monitor trends and risk factors,” she said. “If the rates go up, we will ask why. Do we need to investigate practices in the NICU or on the obstetrics side that could be impacting a change in trend?

Libby Durkee is grateful for that attention. She remembers the NICU staff easing the loneliness and fear that permeated her and David’s first Thanksgiving and Christmas in Colorado. McCourt, whom they saw many times in Oliver’s early years, provided “huge support” for them.

She and her family have indeed traveled a long way from the dark road where her contractions began in 2012.

“We feel so lucky that he was born at University and not somewhere in rural Nebraska,” Libby said.