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Baby born with malformed organs wins her fight for life

Alannah Jane Pionessa is a spirited 22-month-old, and she has been determined since she was born nine weeks prematurely at Women & Children's Hospital.

It's a good thing. Some thought she would never survive.

"I saw my daughter pray and fight for two months for this baby [before she was born]. She never wavered once," said Kathleen Rust, Alannah's grandmother.

Alannah was born with one kidney and some malformed internal organs.

Medical and surgical specialists at Children's have been following Alannah's case since the problem was discovered in utero. Her parents were so worried about her, they had her baptized when she was minutes old.

"She's doing so well for a kid they thought wouldn't survive," said Dr. Rita Ryan, chief of the division of neonatology.

Alannah was a blessing from the start, said her mother, Heather Rust. Rust was diagnosed with endometriosis, in which tissue normally lining the uterus grows in other parts of the body, and did not think she could get pregnant without medical intervention, but she did.

Things were going fine until she was 24 weeks along, when a sonogram showed fluid collecting in the baby's abdomen. Her obstetrician sent her to Women & Children's Hospital, which sent her to Children's Hospital of Philadelphia.

It was discovered Alannah had only one kidney, and she was diagnosed with persistent cloaca, a condition where the urethra, vagina and anus are fused as one opening instead of three. In Alannah's case, her rectum is normal, but her bladder apparently empties into her uterus and then out of her body.

With fluid collecting in her abdomen before she was born, the diagnosis was critical, and the baby was deemed not viable.

Heather Rust returned to Buffalo knowing the diagnosis but not the prognosis.

"I never gave up faith. I knew she would be fine. You know that mother's instinct that kicks in? Mine kicked in right away," she said.

Thirty weeks into her pregnancy, there was very little amniotic fluid, and she entered the hospital. Alannah was born by Caesarean section at 2:42 p.m. June 8, 2009, with her father, Robert Pionessa, in the delivery room.

Also, there was Sister Brenda Whelan, director of pastoral care at Children's. She met the family before the birth and baptized Alannah as soon as she was born. "My role here at the hospital is to support the families," she said.

The nondenominational department helps those of any faith, or no faith, she said.

"We don't have to do anything medical, other than just stand with them," Whelan said.

Weighing 4 pounds, 13 ounces, Alannah was feisty at birth and was whisked away to the neonatal intensive care unit, where she stayed for just over 11 weeks.

Her belly was so swollen when she was born that she looked pregnant, and 200 cubic centimeters -- more than six ounces -- of urine was drained from her abdomen.

She was on a ventilator for weeks and didn't come off intravenous nourishment for 2 1/2 months. She went home Aug. 26 but five days later was admitted to the intensive care unit at Children's. Two weeks later, she was home and has been back to Children's just for checkups.

"Her parents were very dedicated. They were here all the time," Ryan said. "We love having parents who are very supportive."

Alannah receives physical therapy twice a month, and she just started getting speech services. Her time on the ventilator left a groove in her soft palate, and her speech is delayed.

Doctors aren't sure if Alannah will develop bladder control, but Heather Rust said the early signs of potty training are there, and she has successfully used the potty chair. At some point surgeons plan to reconstruct her urethra.

The condition apparently developed several weeks after conception when cells did not properly divide, Rust said.

While Alannah is a joy, she does have the moments of a typical almost 2-year-old, and she has the Irish and Hispanic temperament of her parents combined, Heather Rust said. But they are grateful for every minute of her life and can't help but smile when the independent streak appears.

"Every time she has a tantrum, I thank God she's having a temper tantrum," she said. "We haven't figured out why she's here, but I think it's to bring joy and happiness."

e-mail: bobrien@buffnews.com

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