‘Pillow Angel’ debate continues

The debate over growth and puberty attenuation in children with profound developmental disabilities continues. From the medical perspective, growth reduction by dramatically increasing circulating estrogen levels is not innovative, said Michael Kappy, MD, PhD, of The Children’s Hospital, Denver.

“Estrogen can attenuate height if given in high enough doses,” he told the PAS meeting in Honolulu. “We have seen the same effect in males and females.”

The public debate began with patient Ashley, born in 1997. She was born with static encephalopathy of unknown etiology, explained Douglas Vanderbilt, MD, of Children’s Hospital, Los Angeles. Ashley is unable to turn over, feed herself, or care for herself. She has the mental development of a three-month old. Her parents have nicknamed her “Pillow Angel.”

Life expectancy for severely developmentally disabled individuals is 45 to 50 years, Dr. Vanderbilt said

After extended consultation with medical and ethical experts, Ashley’s parents opted for growth attenuation to limit growth so that she would remain small enough for home care. The girl has reached her full height of about 4ft 6in and 75 pounds.

The parents also decided on a hysterectomy and double mastectomy to eliminate concerns about malignancy and other physical issues, including a family history of large breasts and fibroids.

The debate over Ashley’s treatment stems from ethical considerations, explained Norman Fost, MD, MPH, of the University of Wisconsin, Madison. While Ashley’s parents acted in what they believe is their daughter’s best interests, critics suggest otherwise.

Dr. Fost said the primary objections include the unknown benefits and risks from growth attenuation, unauthorized human research in the guise of treatment, a slippery slope leading to future problems, and child abuse.

None of the objections stand up to scrutiny, he said. “It takes courage to do the right thing.”