This Baby Was Born With A Rare Disorder That Made Her Tongue Twice As Big As Her Mouth

The birth of a baby should be one of the most joyous events in any mom or dad’s life. But in 2015 Madison Kienow and her partner, Shannon Morrison-Johnson, were immediately faced with alarm and fear at their daughter’s arrival. Even the South Dakota delivery doctors reacted with shock at the newborn’s appearance. Unfortunately, baby Paisley was born with a very rare genetic disorder which had caused her tongue to grow twice as big as her mouth.

The birth of a baby should be one of the most joyous events in any mom or dad’s life. But in 2015 Madison Kienow and her partner, Shannon Morrison-Johnson, were immediately faced with alarm and fear at their daughter’s arrival. Even the South Dakota delivery doctors reacted with shock at the newborn’s appearance. Unfortunately, baby Paisley was born with a very rare genetic disorder which had caused her tongue to grow twice as big as her mouth.

The then 21-year-old Kienow and Morrison-Johnson, 23, lived in the small city of Aberdeen, SD, and were very much looking forward to welcoming a new face in June 2015. At seven months pregnant, Kienow underwent a cesarean section at the Sanford Aberdeen Medical Center to deliver baby daughter Paisley Faye. However, that rapturous moment of joy came to an abrupt end when it became clear something was seriously wrong.

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Prior to Paisley’s birth, Kienow had undergone two ultrasounds, each time noting her daughter’s seemingly normal features. Nevertheless, there was one thing that stood out – literally. The medical student spoke to CBS News in October 2016, and remembered thinking of her baby, “Oh cute, she likes sticking her tongue out.”

However, following the c-section procedure, Paisley was immediately put on a ventilator precisely for this reason. The sheer size of her overdeveloped tongue was putting the little mite in huge danger. The muscular organ was twice the size of the baby’s mouth, and was hampering her ability to breathe. Unsurprisingly, manufacturing technician dad Morrison-Johnson and Kienow were utterly distraught in the chaotic minutes after Paisley’s birth.

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Indeed, it was a traumatic time that the mom remembers only too well. “It was really emotional,” Kienow told CBS News. “It’s still emotional. I never got to touch her. I got to see her, but I was being stitched up and they just wheeled an OmniBed over to me. And there I saw her, you know – just tubes and cords.”

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Furthermore, the worried Aberdeen doctors did not know what Paisley’s problem was. The baby’s tongue was so massive, it was impossible for her to feed. Consequently, a tube was fitted so that she could receive milk directly into her tiny tummy. Three days after her birth, Paisley was transferred 200 miles away to specialists at the Sanford University of South Dakota Medical Center in Sioux Falls. Despite this move, however, the baby’s condition remained a mystery, leaving Kienow and family crestfallen.

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But despite this, and the fact that Paisley was still in neonatal intensive care, there were still some encouraging signs. One week on from the cesarean, the baby girl began to breathe on her own. And a few weeks later, Kienow and Morrison-Johnson finally got some answers. Specialists diagnosed their daughter with Beckwith-Wiedemann syndrome.

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A mercifully rare genetic condition, Beckwith-Wiedemann syndrome is an overgrowth disorder seen in premature babies. Thankfully, only about one in every 14,000 births worldwide are affected. Symptoms of the syndrome normally include an over-sized tongue, liver tumors, enlarged abdominal organs – which can include heart defects – and excessive development for one half of an affected baby’s body.

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Under the circumstances, it would appear that Paisley had got off lightly. Nevertheless, her mom felt guilty. “I was blaming it on me,” Kienow admitted to CBS News. “What did I do during my pregnancy that was wrong? But it was nothing that I could have prevented. It was just what she was born with. Jesus wanted me to have something a little extra special.”

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But if Paisley’s problem seemed largely cosmetic, it was still a huge worry for Kienow and Morrison-Johnson. “Her tongue filled up her whole mouth,” Kienow told U.K. tabloid the Daily Mail in October 2016. “It was very thick and protruding out of her mouth. It looked [like] she had an adult’s size tongue inside the mouth of a baby.”

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The mom continued, “Her tongue was constantly sticking out, she was always chewing on her tongue because it took up so much room in her mouth.” Paisley’s parents were told that there was really only one option. As Kienow explained, “Doctors worried it was going to affect her airways and cause her to suffocate. They told us she had one of the largest tongues they had ever seen, and that the sooner she had reduction surgery the better.”

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With this in mind, Paisley underwent reduction surgery at six months old, with doctors removing two inches of her tongue. The procedure saw them cut through the middle of the muscular organ, before removing slices from around the edges. Speaking to CBS News, Kienow remembered seeing the surgeon immediately after the operation. Apparently, he told her, “Wow, that was quite the surgery!”

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Sadly, this was not an end to the matter. Paisley’s tongue started to grow back soon after the procedure, which led to a second date with the knife four months on. Following that, doctors were more confident that the growing infant would not need such radical surgery again because the symptoms of Beckwith-Wiedemann lessen with age. Meanwhile, Kienow and Morrison-Johnson were loving the new-look Paisley.

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Indeed, they had noted an immediate change in their daughter’s appearance. “Since recovering, she smiled for the very first time,” a thankful Kienow told the Daily Mail. “I couldn’t believe it and was shocked by how beautiful my little girl looked. Being able to see her smile is absolutely huge for us, and now she’s not even having difficulties eating which is a massive relief.”

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And it wasn’t just Paisley’s appearance and appetite that was thrilling Kienow and Morrison-Johnson. “She is even getting close to saying her first words,” the mom went on. “She couldn’t even make the sounds to say words like ‘mama’ and ‘dada’ before, because of the size of her tongue, so this feels like a massive achievement.”

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Nevertheless, although the reduction surgeries eventually proved successful, the continued risks of Beckwith-Wiedemann syndrome were still present. This meant that Paisley was still being monitored by her doctors. Indeed, the infant attends hospital for an ultrasound and blood test every three months, but this schedule is set to end when she reaches her eighth birthday.

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It is true to say that as Beckwith-Wiedemann sufferers grow older, the risks associated with the condition recede considerably. Experts suggest that most go on to lead perfectly normal adult lives. But there is still a seven-to-25 percent chance for children with the syndrome to develop cancerous tumors, so Paisley will be closely attended to by her medical team.

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But the oral problem in more severe cases should not be overlooked. Tricia Surles, a manager with a U.S. Beckwith-Wiedemann syndrome support group, was quoted in the Daily Mail article. “Problems caused by macroglossia, an enlarged tongue, can be more severe and require immediate surgical intervention such as tracheotomy in order for the child to breathe,” she explained. “While many children do not need any intervention, some will need speech therapy, tongue reduction surgery or jaw realignment.”

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But as for Kienow and Morrison-Johnson, they were delighted with their daughter’s progress, and they praised Paisley’s resolve in the months following her dramatic birth. “She is one determined little girl,” her proud mom told CBS News. “She has a lot of fight in her.” However, Paisley’s parents also deserved praise. They had faced a massive trauma in the immediate aftermath of their daughter’s birth and months of fear and worry. Thankfully, due to the skill and expertise of medical specialists, Kienow, Morrison-Johnson and baby Paisley can all now smile and look forward to life together.

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