Candidate fought for daughter with Trisomy 18

Isabella Santorum is one of the most compelling aspects of U.S. presidential candidate Rick Santorum’s campaign. Nicknamed Bella, Santorum’s three-year-old daughter has been the focus of media attention because of her father’s care of and pride for her. Bella suffers from Trisomy 18, a rare and often fatal genetic disorder that affects one in 3,000 live births in the United States; less than 10 per cent survive to celebrate their first birthday, although a small number, often girls, do live to their 20s and 30s. The disorder is caused by an extra copy of the 18th chromosome, which may lead to heart defects, kidney problems, respiratory and gastrointestinal anomalies, as well as severe developmental delay.

Recently, when Bella developed pneumonia in both her lungs and came close to dying, Santorum took time off from his campaign to be with her in the hospital, even cancelling campaign plans for the January 29 Florida Republican primary. Her condition has been depicted as Bella’s “struggle” or “fight” against the disorder, even though it was the parents who would have fought the most to ensure that she had the opportunity to survive.

Robert Marion, the chief of genetics and developmental medicine at the Montefiore Medical Center’s Children’s Hospital in New York said to the Washington Post that trisomy 18 is “almost always diagnosed prenatally, and those pregnancies are almost always terminated.”  The Wall Street Journal reported that Santorum and his wife, Karen, only received the diagnosis after Bella’s birth and that doctors advised them to “let her go” – a eupthemism to let her starve to death or refuse further medical interventions. The couple refused, wanting to give their newborn daughter the best life they could. In a video about Bella released last year, Santorum calls her “the centre of the universe for our family.”

The practice of withholding live-saving treatment from children born with trisomy 18, however, is widespread. Most people facing this situation “are not told about the options they have,” said Alex Schadenberg, executive director of the Euthanasia Prevention Coalition, to The Interim. Parents are often told there is no hope and that medical treatment only prolongs suffering. As for Rick Santorum, Schadenberg said, “he knew better… and he demanded treatment for his child.” Children suffering from trisomy 18 and 13 are classified as “incompatible with life” explained Schadenberg. “It’s a values decision the medical community has made,” he said.

Studies have highlighted the prevailing medical attitudes towards the condition. The April 1992 Lancet study, “Avoidance of emergency surgery in newborn infants with trisomy 18,” led by A.P. Bos declared that: “A newborn infant with trisomy 18 should be considered as a patient with a hopeless outlook who ought not to be subjected to invasive procedures.” More recently, the report, “Attitudes of Neonatologists Toward Delivery Room Management of Confirmed Trisomy 18: Potential Factors Influencing a Changing Dynamic,” by Melanie P. McGraw and Jeffrey M. Perlman published in Pediatrics in June 2008 described findings of a survey of doctors dealing with newborns diagnosed with trisomy 18. McGraw and Perlman said: “Of respondents, 44% indicated that they would be willing to initiate resuscitation.” More than nine in ten (92 per cent) of those willing to resuscitate would do so based on “maternal preference” and only 11 per cent are influenced by “previous obstetric management decisions or personal medical belief.”

Ultimately, what is at stake is the parents’ ability to decide on the future of their child. The report, “Lethal Language, Lethal Decisions,” written by Tracy K. Koogler, Benjamin S. Wilfond, and Lainie Friedman Ross of the Hastings Center, published in March-April 2003, states that “lethal anomalies” such as trisomy 18, trisomy 13, and anencephaly “are usually treatable… what makes them sometimes ‘lethal’ then, is the decision not to repair the anomalies or treat the disabilities.” The authors report that doctors often rate the state of health of the child lower than the parents do and that what doctors tell parents “is influenced by the providers’ attitude towards neurological impairment.” In the end, “the phrase “lethal anomaly” obscures the normative nature of the decision and interferes with authentic parental autonomy.”

Bella Santorum is a testament to the flaw in this system of classification. And as Schadenberg notes, while political pundits applaud Bella’s courageous fight for life, it should be remembered that it is her parents, Rick and Karen Santorum, who have fought for her, giving their daughter the rare opportunity for a person with Trisomy 18, to live past their third birthday.