Living With Spina Bifida: A Historical Perspective

Abstract

On October 11, 1983, “Baby Jane Doe” was born in Port Jefferson, NY, and diagnosed with hydrocephalus and myelomeningocele, the most common and serious form of spina bifida. Doctors advised her parents that she would die without surgery. With surgery, she would live from 2 to 20 years, paralyzed, bedridden, incontinent, and severely “retarded.” Based on this information, the parents declined treatment. Baby Jane survived however, to become a self-fulfilling prophecy; because she was predicted to have brain damage, surgery was withheld, resulting in damaging infections that might have been avoided with early aggressive treatment. Subsequent litigation and media coverage concerning Baby Jane Doe focused on the issue of “quality of life.” (The story of Baby Jane Doe is recounted in many places. See, for example refs 1 and 2.) Before 1960, the survival rate for all forms of spina bifida was 10% to 12%.3 Surgeons typically postponed treatment until age 2, believing that only the strongest would survive that long.4,5 Before the introduction of antibiotics in the 1940s, most infants with myelomeningocele succumbed to meningitis. Hydrocephalus also contributed to mortality; the development of shunts in the late 1950s thus revolutionized treatment of myelomeningocele.6 Those who survived meningitis and hydrocephalus faced renal complications. In the 1960s, procedures for urinary diversion were developed to preserve renal function and allow children to gain social continence.7 By the mid-1960s, improvements in treatment produced higher survival rates and lesser degrees of disability. In 1967, W.J.W. Sharrard, Robert Zachary, and John Lorber, pediatric surgeons at Children’s Hospital, Sheffield, UK, reviewed the cases of 526 children born between 1955 and 1962 and treated for myelomeningocele. They concluded that there was “no place for the selection of patients for conservative treatment rather than operative … Address correspondence to Lisa J. Pruitt, PhD, Campus Box 23, Middle Tennessee State University, Murfreesboro, TN 37132. E-mail: lisa.pruitt{at}mtsu.edu