Health

A 3-year-old boy whose tongue does not stop growing needs a second surgery to stop the dilation of organs

A 3-year-old boy born with his tongue four times larger than his mouth needs surgery to prevent further organ expansion.

Little Owen suffers from a rare disease called Beckwith-Wiedemann Syndrome (BWS). It is a disease that affects 1 in 15,000 babies.

Little Owen suffers from a rare illness called Beckwith-Wiedemann SyndromeCredit: Jam Press

One of the main symptoms of this condition is a large tongue size (macroglossia).

This condition makes it difficult to eat, speak, develop teeth and jaws, and increase drooling.

Some children may need to have tongue reduction surgery to prevent the tongue from blocking the airways.

Little Owen has already had surgery once for this condition and needs another surgery to help with potential jaw problems caused by the disease.

Her mother, Teresa Thomas, from Pennsylvania, USA, was diagnosed with an infant in 2018, just two weeks after her birth.

She now wants to raise awareness about her condition and help other families who are experiencing the same struggle.

At first, doctors explained to Teresa that Owen’s tongue was “slightly swollen.”

However, a 30-year-old child said further examination revealed that the mouth was four times as large, revealing that the organs were blocking the child’s airways.

He underwent long surgery to remove 2 inches, eradicated obstructive sleep apnea, leaving the tongue that did not occupy the entire cavity of the mouth.

What is Beckwith-Wiedemann Syndrome?

BWS is an inborn error of metabolism syndrome

Caused by a chromosomal abnormality
It fluctuates because some children have more symptoms and some have fewer symptoms.
Usually diagnosed using a blood test
Increased risk of Wilms tumor, a type of kidney cancer
BWS children grow up to be healthy adults

What are the signs and symptoms of BWS?

Great Ormond Street Hospital The following states that it is a sign of BWS:

Large tongue size (macroglossia)
Height and weight gain at birth or in childhood
Growth asymmetry, eg, one arm or leg larger than the other (hemihypertrophy)
Hypoglycemia in the first few days or weeks after birth (neonatal hypoglycemia)
Umbilical hernia or other abdominal wall defects, such as umbilical hernia, where other organs such as the intestine and sometimes the liver are inside the umbilical cord but remain outside the abdomen at birth

Theresa said she felt sick in her stomach when Owen was diagnosed.Credit: Jam Press

Unfortunately, this is not a permanent solution. Owen’s tongue continues to grow to this day, and doctors are now looking for ways to slow it down and operate on boys.

Teresa, a full-time housewife, said: “Owen was formally diagnosed by the BWS Genetics team at the Philadelphia Children’s Hospital.

“I knew from the bottom of my heart that this was how to make Owen and he had BWS, but even if I knew it, the official diagnosis was not easy.

“I remember my stomach hurting. I almost vomited on promise.

“Before Owen, I had never heard of his syndrome. Even the doctors I worked with at the time had never heard of it.”

The syndrome was discovered in the 1960s and doctors are still learning about the condition.

Teresa said the official diagnosis of Owen would not make things easier.Credit: Jam Press

Owen gave birth by caesarean section because he weighed 12 pounds when he was born.Credit: Jam Press

During her pregnancy with Owen, Teresa said everything was normal-except for a few scans that showed him a large measurement.

On February 7, 2018, Owen was born by Caesarean section and weighed less than 12 pounds.

Teresa said: “He was so big that I had a hard time controlling my bleeding.

“My umbilical cord and placenta were also much larger than usual. I lost a lot of blood during childbirth and became anemic.

“His tongue grew larger at birth and they wiped it off and thought it was swollen.

“But my nurse told me I should push the problem, and the doctor started investigating if he had any signs of BWS.”

Lifesaving

After leaving the hospital, the family went home, but Teresa and her husband Mike had to use a breathing system to monitor him.

When the family first returned home, Teresa said she couldn’t sleep.

She added: “Fortunately, while we were waiting for him to undergo plastic surgery, we purchased Owlet socks to monitor his oxygen levels while he was asleep.

“I am very grateful for purchasing that little device. It surprised me several times to tell us that he wasn’t getting enough oxygen while he was asleep.

“It saved his life many times.”

Teresa couldn't sleep when they first got home with Owen — Teresa couldn’t sleep when they first got home with OwenCredit: Jam Press

Teresa said Owen's condition tools had a huge impact on her husband's marriage.The photo above shows husband Mike, Owen, Teresa, and son Michael. — Teresa said Owen’s condition tools had a huge impact on her husband’s marriage.The photo above shows husband Mike, Owen, Teresa, and son Michael.Credit: Jam Press

After his first surgery, little Owen spent 10 days in the hospital.
Teresa said Owen’s recovery had hit her marriage as mom and dad were having a hard time dealing with it.

Teresa said: “It was probably more difficult for me and my husband Mike to recover than Owen.

“We had a very different way of dealing with it.

“He handled it by behaving everything as it worked, it’s difficult for me, so there was a lot of debate at first.

“But we grew up and learned how to communicate and be there for each other, despite his different treatment of illness.

“I sometimes felt helpless. You don’t want to see your child experience pain.”

Owen's tongue continues to grow because of his condition — Owen’s tongue continues to grow because of his conditionCredit: Jam Press

Owen is in the photo above using a breathing deviceCredit: Jam Press

She added that “it’s one of the worst things” that a mother can see, and said she learned a lot about “the strength that her son has.”

Teresa said: “It’s really great to see how well he is doing every day.

“When they took Owen’s breathing tract out, he ate like a champion from the bottle and didn’t behave like he was in pain.

“His seams took weeks to completely melt. Before surgery, his tongue was always out of his mouth.

“Many times he held his breath during sleep and suffocated hard enough to vomit.”

follow-up

However, family distress did not stop there, and Teresa said Owen was at risk of developing cancer as a result of his condition-she described it as “follow-up syndrome.”

Teresa said: “Every child is different and is affected in different ways.

“Owen has a high risk of developing cancer and is screened for ultrasound and blood tests every three months.

“His type of BWS (UPD) means that his tongue keeps growing.

Little Owen must be screened every 3 monthsCredit: Jam Press

His constant treatment did not prevent him from breaking his bond with his brother MichaelCredit: Jam Press

“It was so big that it was very difficult for us to strain our stomachs.”

Last summer, the family met with Owen’s plastic surgeon to discuss the best options for young people.

Despite this major setback, Owen is one step ahead when it comes to his speech and has worked with a speech therapist.

Language and language problems are one of the major problems with the macroglossia.

Theresa says she’s trying to make Owen no different than her six-year-old brother Michelle.

Michael and Owen are drawn together aboveCredit: Jam Press

The family said Owen was “very well done” despite his conditionCredit: Jam Press

She states: “Owens’ tongue influences his conversation and diet. He was receiving speech therapy very early and it was very helpful. He also eats to help eat solids. I also received therapy.

“He’s drooling more than most kids, and in the summer his tongue actually burns, so you have to try to put on a hat in the sun, but otherwise he’s very much I’m doing well.

“He’s far ahead of where he should be. We couldn’t be proud of him.”
Teresa uses BWS’s personal experience to defend people who are not provided with the same level of support that their families have received.

The mother also has a blog about Owen and her family’s life in a rare disease, as well as co-sponsoring a podcast.

She added: “My hope for the future is that standard treatment for all BWS children will be universal.

“Currently, depending on the country you live in, standard treatment is not the same for all BWS.

Owen's tongue influences his speech and diet — Owen’s tongue influences his speech and dietCredit: Jam Press

“Some places, such as the United Kingdom, do not screen children with BWS who belong to the group with the lowest risk of cancer.

“Knowing that my child is at risk for cancer and no screening is offered, it hurts the mother I’m involved with.

“We also hope that more is discovered about BWS and adulthood, more doctors are more educated at BWS and know how to help BWS children and their families.”