IlliniThon News Story – Part 4 (Brenna’s Story)

For my final (and maybe favorite) story, I wrote about miracle child, Brenna Westlake, her family, and her rare skin condition. The people I got to interview for this story were wonderful and I’m looking forward to seeing the Westlakes at IlliniThon in just 10 days!!

donor drive cover photo

When Courtney Westlake takes her four-year-old daughter Brenna to the grocery store or the library in Springfield, they are used to getting stares and questions.

“We were walking to the library and this old man passed us. He stopped and turned around and stared at us the whole rest of the way we were walking,” said Courtney. “Her skin is just a surprise to people, they don’t expect to see this little girl who looks like she has a severe sunburn because they’ve probably never seen it before.”

11713840_1128342500525747_7206501105167832282_oBrenna has a very rare genetic skin disorder, harlequin ichthyosis. Harlequin is the most severe form of ichthyosis, a group of skin disorders. Brenna’s genetic code is missing a gene, causing her skin to form incorrectly. Because of this, her skin grows very fast and is very thick. It also has to shed more rapidly, causing her red, flaky skin that can look like a peeling sunburn.

Dr. Joseph Conlon, the Westlake’s pediatric dermatologist, explained the medical issues beyond Brenna’s unique appearance.

“[Our skin] keeps everything in that’s supposed to stay in and everything out that’s supposed to stay out,” explained Dr. Conlon. “[Brenna’s] skin is not able to do everything that it needs to do.”

He went on to explain that our skin regulates our body temperature and moisture. It also keeps infections and other harmful things out of our body. Since Brenna’s skin cannot do all of those things, she is vulnerable to dehydration, overheating and infection.

To avoid those dangers, Brenna and her family have to take special care of her skin. They apply Aquaphor, a thick lotion, all over her body four to five times a day. Every evening, Brenna takes about a 45-minute bath. The long bath allows her skin to soak up the moisture and helps exfoliate the skin that has built up in the last 24 hours.

There are also lots of little things that the Westlakes constantly have to be thinking about. They wash Brenna’s hands a lot to help her avoid germs and infection. They closely monitor her temperature to make sure she is not too hot or too cold. In the winter, you will often find her wrapped up in a blanket and in the summer she has a special vest that helps keep her cool and prevents overheating.

Brenna eats a lot because her body uses so much energy to produce her extra skin. She also has to drink a lot to stay hydrated.

Courtney explained that sometimes Brenna’s care can be exhausting and says it is difficult to think about how Brenna will have to do these things every day for the rest of her life. She also knows that her daughter will always be explaining to people why she is different and what harlequin is.

Evan Westlake, Brenna’s dad, described the condition with the ‘bricks and mortar’ analogy. He explained that everyone’s skin is made of bricks and mortar, but Brenna’s skin is missing the mortar. Her body knows there are gaps in her skin so it tries to produce more skin to heal those gaps, which is what normal skin would do for a cut. However, the new skin that her body produces is also faulty, leading to thicker skin that still doesn’t seal like it should. Brenna then has to get rid of the extra skin, which is why it flakes off and peels like it does.

Harlequin ichthyosis is a recessively inherited genetic disorder, meaning that a baby must inherit one recessive gene for the disorder from each parent.

“The chance of both of us having the genetic mutation for this is one in a million,” said Courtney.

The condition is so rare that accurate statistics cannot be calculated and the Foundation for Ichthyosis and Related Skin Types (FIRST) simply lists harlequin ichthyosis as “very rare.”

The Westlakes said they know of about 20 other people in the United States who also have harlequin ichthyosis. They have had the opportunity to meet these people through FIRST.

FIRSTFIRST was started in 1981 in California for families who are affected by ichthyosis. It started as a volunteer organization but is now run by a team of full-time staff members in Philadelphia. The foundation raises money for ichthyosis research. It also helps provide support for affected families by connecting them via the Internet and at the National Family Conference every two years.

“I think a combination of [having full time staff and the age of the Internet] really has gotten us to where we are today,” said the Chief Executive Officer of FIRST, Jean Pickford. “We now have over 5,000 connected families in our database.”

The Westlakes are grateful for the support of FIRST and love to help other families that have babies born with ichthyosis. There are only a few official medical documents that talk about harlequin and a few doctors that have treated it before. The Westlakes and their medical team used all of the resources available to them to help Brenna and are happy to share their experiences to help others.

When Brenna was born, her parents’ first thought was that she just needed to be wiped off, but the thick, white coating on her little body couldn’t be wiped off. Within an hour, Brenna was rushed to the neonatal intensive care unit at St. John’s Hospital, and Springfield’s only pediatric dermatologist was sent a picture of Brenna. Dr. Conlon immediately diagnosed Brenna with harlequin ichthyosis.

“I look at those pictures and I just remember the fear and the worry and the stress that we had at that time,” said Courtney as she looked through a photo album. “I’m glad that we can look back at those pictures from her birth now and have her with us today. Now it truly is a big celebration; it didn’t feel like a celebration back then.”

Brenna is just starting to notice her baby pictures, but she doesn’t realize that they are different than anyone else’s. She doesn’t ask about her skin or see it as something that makes her different; it’s just a part of her world.

“We don’t talk about [Brenna’s condition] that much, not because it’s something we don’t want to talk about, but it’s just not an issue for our family right now,” said Courtney. 12510454_1239170502776279_854214510338747551_n“We always want her to know that no matter how the world outside is treating her, at home she’s just part of our family.”

Evan and Courtney want Brenna to know that she is an amazing girl, and that has nothing to do with her skin.

“Above all, it has always been this huge love for her and this gratitude that she is a part of our family,” said Courtney. “With that being the top emotion, it’s easy to help her see that.”

“Who’s the most beautiful girl in the world?” Courtney asked as the family sat in the living room.

“Me!” exclaimed Brenna, as she pointed to herself and danced around.

Brenna is a spunky and outgoing four-year-old, according to her parents. She is very social and loves school and running errands with her parents.

Courtney describes her six-year-old son, Connor, as sensitive, cautious and thoughtful. Most importantly, he loves his little sister.

“He is just the perfect brother for her,” said Courtney.

Connor, who has normal skin, is also starting to defend his sister and explain to other kids that she just has a “skin addition.”

“I just like to play with her!” said Connor with a big grin, as he hugged his sister, almost knocking her over.

Connor isn’t the only one who enjoys spending time with Brenna.

“I spent a lot of time with Brenna last year at [IlliniThon],” said IlliniThon’s family relations director, Lauren Adrian. “I was just drawn to her. She’s such a cutie, how could you not love dancing with her?”

Brenna loved being in the spotlight at her first IlliniThon in 2015. IlliniThon is an annual Children’s Miracle Network event at the University of Illinois that raises money for St. John’s Children’s Hospital in Springfield, Ill. Evan and Courtney were amazed by how excited all the students were and how much money they raised.

“We know first hand what that money actually does go to. We’ve used so many of the resources that they’re raising money for so that’s really neat to be a part of,” said Courtney.

The members of IlliniThon are happy to have Courtney on board, too. A few days after Brenna was born, Courtney started her blog, Blessed by Brenna. Adrian explained that the members of IlliniThon love to be able to share links on social media to blogs like Courtney’s.

“[Courtney] being so connected with the Internet makes it easier for people to understand why we do what we do,” said Adrian.

The students who plan IlliniThon have been working to make this year’s event the biggest and best one yet. IlliniThon will take place on April 23, 2016 and many miracle children, like Brenna, and their families will be in attendance. The IlliniThon executive team is passionate about what they are doing and it’s all for the kids and families.

“What always inspires me about the families is their ability to adapt to the situation and only find positives within it,” said Adrian.

The Westlake family has definitely done that, as Courtney uses her journalism degree to write about the importance and beauty in differences. She wants others, especially her daughter, to see and celebrate the beauty in the world.

“Life doesn’t always look like what we planned,” Courtney wrote on her blog. “But it doesn’t have to be perfect to be wonderful. Beautiful can be found everywhere, when we take the time to see it.”

 

To join me in supporting IlliniThon and kids like Brenna, make a donation!

Also read my other IlliniThon News Stories:

IlliniThon News Story – Part 1

IlliniThon News Story – Part 2 (Trick or Treat For the Kids)

IlliniThon News Story – Part 3 (Peyton’s Story)

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