A baby who was born with an adult-sized tongue due to a rare genetic condition can finally smile after having surgery to stop it poking out.
Paisley Morrison-Johnson had such a large tongue that she needed help breathing for the first week of her life to stop her from choking to death.
She was born with Beckwith Wiedemann syndrome (BWS) - a rare overgrowth disorder affecting around one in every 14,000 births worldwide.
It caused her tongue to grow more than twice the size of her mouth, which meant she struggled to feed.
So Paisley, from Aberdeen in South Dakota, had to be fed through a tube in her stomach until she was six month's old.
Parents Madison Kienow, 21, and Shannon Morrison-Johnson, 23, agreed to surgery to slice parts of it away.
But her tongue continued to grow back and still hung out of her mouth.
Six months ago, Paisley had her second tongue reduction and has smiled for the first time after having a total of more than six inches of muscle removed.
Paisley, who is 16 months old, is now enunciating the sounds that will allow her to speak her first words.
'Her tongue filled up her whole mouth, it was very thick and protruding out of her mouth,' said Ms Kienow, a medical student.
'It looked she had an adult's size tongue inside the mouth of a baby.
'Her tongue was constantly sticking out, she was always chewing on her tongue because it took up so much room in her mouth.
'When she was born, it was so enlarged that 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.
Paisley was incubated on a ventilator for her first three days and then given nasal oxygen - but by the time she was a week old she was breathing on her own.
Attempts to bottle feed her were unsuccessful so she had a tube fitted so milk could go directly to her stomach.
When she was six months old, surgeons operated and removed two inches of her tongue.
They cut the tongue through the middle and took cut portions out of the sides.
But after her first surgery, her tongue appeared to grow back and she had a second operation four months later.
'Since recovering she smiled for the very first time.
'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.
'She's like a completely new baby - her facial features look different, she smiles a lot and she has even getting close to saying her first words.
'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.'
Before having her reduction surgeries, Ms Kienow noticed that people would stare at her daughter because of her enlarged tongue - which is one of the main symptoms of BWS and medically known as macroglossia.
Other symptoms of the condition include:creases behind the ears, one side of the body being larger than the other and an above average birth weight.
'Because her tongue protruded out of her mouth and constantly stuck out it always looked like she was making silly faces.
'Quite often, strangers would stare at her and some would make comments.
'They would always ask me why she looked so different and why she had such a huge tongue.'
Doctors now hope Paisley won't need further surgery and are monitoring for other risks associated to Beckwith Wiedemann syndrome.
Children with the condition have a 7 to 25 per cent chance of developing cancerous tumours so Paisley will have ultrasounds and blood tests every three months until she is eight, when the risk significantly drops.
'Since her second surgery, her tongue hasn't grown back as much and it isn't affecting her eating or drinking out of a bottle which is great.
'She seems like a very happy baby and is being well taken care of, so we're really confident about her future.'
Approximately 90 percent of children with BWS have an enlarged tongue, which can lead to breathing, feeding and speech problems.
Tricia Surles, co-manager of support group How Big BWS, said: '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 breath.
'While many children do not need any intervention, some will need speech therapy, tongue reduction surgery or jaw realignment.'
For more information, visit www.howbigbws.com
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