By Angelica Leicht
By Dianna Wray
By Aaron Reiss
By Camilo Smith
By Craig Malisow
By Jeff Balke
By Angelica Leicht
By Jeff Balke
Here's what can happen when you're the parent of a primordial dwarf:
First of all, you don't know you're the parent of a primordial dwarf. The words "primordial" and "dwarf" have rarely surfaced on their own in conversation, let alone together. So you're not aware that this is a thing, and your doctor just shrugs his shoulders and refers you to a "specialist" when you ask him why your kid looks like she was shrink-rayed.
As your kid grows up, she doesn't actually grow. Not much, anyway. So when you take your bite-sized, underweight kid and her normal-sized twin sister and their older brother to the grocery store, people threaten to call Children's Protective Services because you obviously don't like one of your daughters and are therefore starving her to death. Or perhaps they'll approach you with the belief that it is entirely appropriate to ask about the small one's life expectancy.
Such were the first six years after Chrissy Bernal gave birth to Sierra and Sienna. Because it wasn't until then, after seemingly endless trips to Texas Children's Hospital to see a procession of endocrinologists, cardiologists, pulmonologists and neurologists, that they wound up in the office of a geneticist visiting from Belgium who took one look at Sienna and said, "Wow. I've only seen two others like her."
That's when he explained: Sienna was one of maybe 200 people in the world who fell under the umbrella of primordial dwarfism, a set of conditions sharing the characteristics of poor growth while in the womb and slow growth after birth; a birth weight roughly under three pounds and length less than 16 inches, among others. They were a select group of people with the distinct honor of once being labeled "bird-headed dwarfs" in medical literature.
Sienna eventually became part of ongoing studies at Texas Scottish Rite Hospital in Dallas and the Nemours/Alfred I. duPont Hospital for Children in Delaware. Along with about 40 other primordial dwarfs in the U.S., Sienna has contributed her DNA and medical history to help doctors understand the genetics behind this rare form of dwarfism, as well as how this dwarfism relates to other medical conditions. But when Sienna's not helping solve an incredible genetic mystery, she's simply trying to be a regular 11-year-old girl.
At three-feet-ten, Sienna (who also goes by "Sinny") is taller than most primordial dwarfs. And at 27 pounds, she's one of the slightest.
She's a bony bundle of energy who'd like a nice pair of red heels and thinks her glasses and hearing aids make her look dorky. Or, even worse, intelligent.
"I don't want to look, like, too smart," Sienna explains to her mom and sister, who are sitting with Sienna in the family's Tomball home on a Sunday afternoon. "I don't want to be too smart for a guy."
"You can never be too smart for the guy," Chrissy corrects. "If you're with a guy that you're too smart for, you don't need to be with him."
This was appended to an earlier conversation about guys — or one guy in particular, who may or may not be dating Sienna.
"Does he know you're dating him?" Sierra asks with a grin.
Sienna pauses, shakes her head. "But he knows my name, though."
Sierra: "Okay, so if I know your name, does that mean I'm dating you?"
This kind of ribbing apparently began in utero; like security-cam footage of a stickup, an ultrasound photo shows Sienna kicking her sister in the head. Of course, this was when Sienna finally came out of hiding. It wasn't until six months into Chrissy's pregnancy, in October 1998, that a bemused ultrasound technician discovered that Sierra was not a sole occupant.
The doctor explained to Chrissy that sometimes twins' heartbeats are so in sync that two sound like one. Baby B, as the doctor called the new arrival, was simply mirroring her sister. But there was another, more worrisome reason that Baby B hadn't been detected: Developmentally, she was five weeks behind her sister.
The existence of an additional, undersized fetus wasn't the only shocker: Baby B's heart had a hole in the wall separating the ventricles. Perhaps more alarmingly, there appeared to be a deformation of the cerebellum, the fist-sized region of the brain integral to motor function.
Eventually, doctors diagnosed the brain defect as something called Dandy-Walker. Not exactly reassuring, but now Chrissy knew exactly what to type in a search engine and fret over. What stumped the doctors was Baby B's small size. An amniocentesis could help solve the mystery, but there were of course risks. Infection. Fetal trauma. Miscarriage.
Chrissy would later write of the experience, "Then they said something [I] would have never expected to hear: 'We'll need you to let us know which baby you would like to root for, in the event that either one of them starts to go into distress or their condition worsens.'"
Chrissy wrote that her answer was very quick and very clear: "'That is in God's hands.'"
The girls were born three months premature. Whether by providence or simple tenacity, Sienna defied doctors' expectations. Weighing 23 ounces at birth, tiny enough for her arm to slide through a wedding band, Sienna was not expected to survive. If she did pull through, physicians predicted, she would be a vegetable. She spent her first 108 days in intensive care, first subsisting on sugar water pumped through a tube in her nose, then through a feeding tube snaked straight into her stomach.