Baby with genetic syndrome smiling after tongue-reduction surgery

Image 1 of 7

Courtesy Madison Kineow

Baby Paisley is all smiles after having surgery to reduce her enlarged tongue, which was a result of Beckwith-Wiedemann syndrome

Paisley's mother, Madison Kienow said at ultrasound appointments, her doctor would comment that the baby was sticking out its tongue. It was not until she went into pre-term labor at 26 weeks pregnant that she learned her baby had the syndrome. 

Beckwith-Wiedemann syndrome causes some parts of a baby's body to grow faster or larger than normal. The syndrome is thought to be the result of a gene abnormality. Beckwith-Wiedemann babies may have an enlarged tongue - or macroglossia - as well as concerns with their kidneys, abdominal organs and belly-button, and blood sugars at infancy. 

In Paisley's case, her tongue was so enlarged, her doctors said it was the largest they had ever seen. 

Paisley's doctors, father-son duo, Doctors David and Patrick Munson at Sanford Health in Sioux Falls, S.D., worked with Paisley's parents on a plan to reduce the baby's tongue size, and to address other Beckwith-Wiedemann symptoms including an umbilical hernia.

Mother Madison Kienow said she recently began school as a pre-medical student and was so happy to have such supportive family and friends, as well as her team of doctors. After two surgeries to remove parts of her tongue, little Paisley is all smiles, and her mom knows, even though the syndrome makes things more difficult, there's a reason her family was chosen for the journey. 

"Jesus Christ never thought Paisley was perfect without Beckwith-Wiedemann," Kienow said. "I ask, 'why us,' but now I know why. Because we're strong, and because we have love." 

Today, Paisley is about 17-months-old and her mom is connecting with other moms of Beckwith-Wiedemann babies, which sometimes have mismatched limb growth, known as hemihyperplasia or hemihypertrophy. Kienow said one of Paisley's legs and foot are growing faster than the other.

That means two different size shoes. But representatives from companies like Nike and Adidas have sold Kienow two different sized shoes for Paisley, making life a little easier. She also found a Facebook group for moms of kids with hemihyperplasia, many of whom arrange swaps for the single shoes their child doesn't need. 

Kienow said baby Paisley will have to undergo ultrasounds and tests to be sure she does not develop tumors as an adolescent - another symptom of the syndrome - but her long-term prognosis is good. The life expectancy for Beckwith-Wiedemann babies is usually normal.

And with a smile like hers, Paisley is sure to live a long, joyous life.