Parents, Community Support Child Stricken With Rare Disorder
Two area parents are hoping to make every day count for their daughter born with a rare genetic disorder known for its high mortality rate.
Dane Bush and Erica Webster have been together for three years and live in Kennedy. After a difficult pregnancy, Erica Webster had a C-section caused by a placenta previa. Their daughter, Scarlett, was born April 22 at Oishei Children’s Hospital in Buffalo, where she spent the first 42 days of her life in the NICU.
“At first, they thought everything was fine,” Scarlett’s grandmother, Sandra Webster, said.
However, the doctors put Scarlett in Oishei’s newborn intensive care unit as a result of her lacking good muscle tone. While Scarlett was in the NICU, she suffered a seizure.
“The second day after birth, they diagnosed her with something called Polymicrogyria,” Sandra Webster said.
Polymicrogyria is a condition characterized by abnormal brain development prior to birth.
“The first thing they got hit with was that she was probably going to have disabilities,” Webster said. “The next day, they got hit with a genetic condition called Zellweger syndrome.”
According to the National Center for Advancing Translational Sciences, Zellweger syndrome is caused by both parents passing on a recessive gene to their child, causing an autosomal recessive genetic disorder.
Erica Webster has three older children, ages 6, 11 and 12. Scarlett is Bush and Webster’s first child together.
“It’s just the strangest thing that the two of them happened to have this gene and find each other,” Webster said.
Symptoms of Zellweger syndrome often include seizures, poor muscle tone, hearing loss, vision loss and problems feeding. Children diagnosed with this disease often develop fatal problems with the heart, liver, and kidneys.
“Zellweger is very rare, and she definitely will not live past her first birthday,” Webster said.
Webster said Scarlett is not able to keep her oxygen saturation levels up, which means she is required to be on constant oxygen.
“She’s in respiratory failure now,” she said, “so she has to be suctioned all the time.”
Although Scarlett is able to move her body fairly well, Webster said she continues to struggle with poor muscle tone.
Scarlett is also not able to be fed like most babies.
“She is tube-fed through an NG tube that goes down through her nose,” Webster said.
The child’s tube-feeding requires the parents to feed Scarlett roughly every three hours.
“She’s thankfully growing. She went from 4 pounds 2 ounces. She’s up to 8 pounds 4 ounces now,” Webster said.
Scarlett’s body cannot process certain fats, which causes the fats to collect around the organs and inside the organs and destroy the body over time.
Despite the serious medical challenges facing the family, Webster said Dane and Erica are doing everything they can to support Scarlett and take care of her.
“The two of them are like a well oiled machine taking care of this baby,” Webster said. “They just go right to it. They know what to do.”
Webster also said Erica’s older children are wonderful siblings to Scarlett and helpful to the parents.
While Scarlett is not expected to live past her first birthday, the family intends to make every day count.
“The expectation is to do things as normally as possible,” Webster said. “They’re just trying to make it as normal as possible for Scarlett and for the other three kids and for each other, while also knowing that every day is precious.”
While the parents know what the outcome will be for their daughter, Webster said they try not to focus on that. Instead, the family is focused on creating a loving and comfortable environment for Scarlett.
“You’re praying for comfort,” Webster said. “It’s brought everybody so close. She means the world to everybody.”
While the experience for the family has been very difficult, the family is trying to enjoy every moment with Scarlett.
“She’s just beautiful. You look at her and you think, ‘Wow that’s just a normal baby, but she’s not. She has tubes coming out of her face and everything,'” Webster said.
A fundraiser for Scarlett and her family is planned for Sunday at the Gerry Rodeo grounds. The fundraiser will include a motorcycle ride around the lake, a pulled pork dinner, basket raffles, children’s activities and a DJ.
Participants can register for the motorcycle ride at 9 a.m. The ride will take place at 10 a.m. and will conclude at 1 p.m. The family is asking participants take pictures along the ride and upload them to the Love 4 Letti Facebook page.
“My daughter would like to have memories. That’s what she wants out of this,” Webster said.
The money raised at the event will help the family pay for Scarlett’s medical bills. Scarlett’s specialists are located in Buffalo, which adds extra expenses to the family.
“It gets expensive,” Webster said.
In addition to the fundraiser, the family has a GoFundMe page (https://gofund.me/b4ab6baa) and has received support and encouragement from people all over the world. Webster said social media has even helped the family gain support from people as far away as Uganda.
“Just the love that has been put out there has been tremendous,” Webster said, “I’m just amazed at how far this has reached.”
Webster said she prays no one ever has to go through what their family is going through. However, she offered words of encouragement for anyone facing a similar situation.
“Keep your family strong.” she said. “Be up front with everybody right from the start, including children. No secrets, and just love the daylights out of them. Make yourself aware of what’s going on. Do everything you can to learn as much as you can. Education is the key.”