Not so ‘incompatible with life’ after all

For years, healthcare providers have debated the effectiveness and ethics of medical interventions for children diagnosed with Trisomy 13 and 18, a pair of rare but severe genetic conditions that can affect multiple organ systems. Most babies born with these conditions die within the first weeks of life. Parents often are told their baby’s defects are “incompatible with life,” and therefore should receive only palliative care. But mounting research now questions whether such a grim prognosis is certain.

Eight years ago, doctors diagnosed Bella Santorum, daughter of former U.S. Sen. Rick Santorum, R-Pa., with Trisomy 18. Santorum, who also ran for president in 2012, told LifeNews about the day they brought 10-day-old Bella home from the hospital.

“Doctors prepared us for how she was going to die,” he recalled. “Frankly, this made us angry. … We were going to fight to give her the opportunity to do as well as she could.”

New Canadian research led by the Hospital for Sick Children (SickKids) and the Institute for Clinical Evaluative Sciences (ICES) lends support to the Santorums’ choice. Researchers evaluated 174 children with Trisomy 13 and 254 with Trisomy 18, who were born over a 10-year period. They found while early mortality is the most common outcome for these children, those who survived for at least six months and received special medical care lived much longer than predicted. Medical care for the children ranged from minor procedures to complex cardiac surgical repairs. Among those who had surgery, one-year post-operative survival was near 70 percent. The study is published in the July edition of JAMA.

“Long-term survival is more common than was previously expected,” the study’s senior author, Astrid Guttmann, staff pediatrician and scientist at SickKids, told HealthCanal.com.

In a separate study, researchers in nine states found five-year survival rates of 10 percent to 12 percent for children with Trisomy 13 and 18. Aggressive treatment produced the highest survival rates, according to the research, published in the American Journal of Medical Genetics in April. Both studies caution the viability of each child can vary depending on many factors.

Despite the promising findings in multiple studies, many in the medical community still nudge parents to consider abortion and hesitate to encourage more aggressive treatment.

“More research is also needed about quality of life in these populations and about the risks and potential benefits of surgery,” said lead author Katherine Nelson, a staff pediatrician at SickKids.

In an editorial accompanying the Canadian study, John Lantos, a medical ethicist at Children’s Mercy Hospital in Kansas City, wrote it is “ethically justifiable” to withhold medical treatment and let some infants die while offering aggressive treatment to others. But he insisted parents should drive the decision.

The Santorums, who had faced the heartbreaking loss of another child, engaged their faith in the process but answers didn’t come overnight.

“All I can think about was, ‘I can’t go through this again,’” Karen Santorum told LifeNews. “I was praying … asking why.”

In his book Bella’s Gift, Rick Santorum admitted he emotionally held Bella at a distance, at first.

“I remember holding that finger, looking at her and realizing what I had done. I had seen her as less of a person,” he told LifeNews.

Their decision came at great cost. In 2012, Santorum dropped out of the Republican presidential primary race, citing the demands of caring for then 3-year-old Bella. But the family has no regrets.

“Bella makes life in the Santorum home an experience of love, hope, and beauty in a way that we could never have imagined,” Santorum told National Review.

Karen Santorum frequently speaks against the push for abortion for babies diagnosed with certain disabilities before birth.

“What we got a lot was ‘lethal diagnosis incompatible with life.’ They really have to stop using those words. Because when they do it leads to lethal outcomes,” she told LifeNews.