Rare disease doesn’t define 11-year-old Mykela

At 11 years old, Mykela Shields has experienced more hospital stays, tests and procedures than most people will in their lifetime. Her memories consist of the pinch of a blood draw, the beeping of hospital equipment and the sterile smell of the facility.

When she was 4 years old, Mykela was diagnosed with lysinuric protein intolerance, a genetic condition so rare there isn’t a recorded number of Americans with the metabolic disorder.

With LPI, the body cannot properly absorb or use specific amino acids found in protein.

This was just the beginning of a lengthy list of conditions and ailments she would come to be diagnosed with in the coming years, including lupus, failure to thrive, adrenal insufficiency, osteopenia, interstitial lung disease, pulmonary hypertension, rheumatoid arthritis and more.

For many parents, having a sick child is one of the most difficult situations imaginable. But Becky Shields knew what she was getting herself into when she chose this life of hospital stays and sleepless nights.

“She came to live with us at two and a half,” Becky said. “We adopted her at three and a half.”

Becky’s plan had been to have four biological children and then adopt one. She ended up with three biological children, four adopted children and more than 80 kids have come through the house through fostering.

There’s a large age range in the Shields family with ages of 37, 33, 30, 24, 23, 20 and 11.

By the time Mykela was adopted, she had already been to numerous doctors to try and figure out a myriad of symptoms and ailments.

Small in stature at less than 4-feet tall and just 55 lbs, Mykela uses a wheelchair to get around and is typically on 4 to 6 liters of oxygen. She is on 29 medications and uses a feeding tube, but she doesn’t let it slow her down.

A normal kid

“She’s goofy, silly. She can make anybody laugh,” Creston Middle School teacher Becki White said. “And ornery — very ornery. She’s just a normal, typical fifth-grade child outside of being sick."

Sassy and yet ornery is the consensus among people who know Mykela.

“She’s just so full of life, and she always teases me like ‘I’m going to run you over,’” Middle School Principal Lesa Downing said. “She wants to be here. She loves school, loves being with her friends. You never see her without a smile.”

Becky said her daughter is wise beyond her years. “So very spunky, and she tells you what she wants and when she wants it,” she said. “She’s got a big personality. She’s a Sour Patch Kid.”

Corina Hoepker has Mykela in social studies, and sees her personality shine.

“She’s ornery,” Hoepker said. “She’s always got little one-liners to spit out. The other kids just flock to her.”

Watching Mykela tackle every day with strength and resilience is an inspiration to those around her.

“There is something deeply humbling about watching an 11-year-old carry more courage than grown adults,” Mykela’s para Shyla Stow said. “She doesn’t complain. She doesn’t ask for sympathy. Instead, she makes jokes. She tries to make everyone laugh. She checks on other people. She makes it easy to come to work because you know when you see her, you’re going to be reminded of what resilience actually looks like. She has already lived through things no child should have to understand. She has faced realities that most of us are lucky enough to never know. And yet she still chooses joy. That’s what makes her extraordinary, an idol, that girl.”

Mykela is more than a diagnosis. She is a child who loves “Lilo and Stitch,” collecting trinkets and coloring.

“She is very much an artist,” White said. “And she’s a collector of all things. I have so many little knick-knacks that she has and carries around with her.”

White’s room serves as a sort of home base for Mykela, complete with a small couch, heated blanket, fan and things for her to do when she gets depleted during the day.

Mykela has always been good at advocating for herself when she’s tired herself out.

“She’ll tell us,” White said. “So we’ll just come in here, and my lights dim. She’ll put something on my smart board, and she’ll sit there and just get comfortable and relax and take the time until she builds the strength to go back out there.”

Downing loves watching Mykela enjoy life as a middle schooler.

“When she was at the dance last Friday, I love to see her here having fun with her friends,” she said. “She had a smile on the whole time.”

Stow is the one who pushes Mykela around in the wheelchair, spending more time with her than anyone.

“It’s hard to even know where to begin when it comes to Mykela because she isn’t just “special” — she is the kind of soul that changes the entire room without even trying,“ Stow said. ”Mykela is one of one. Truly. Life has placed mountains in front of her that most adults would crumble under, and yet every single day she shows up, on time, with the best outfits, the brightest smile and an attitude that somehow says, ‘I’m still here.’ And that alone is powerful. What amazes me most isn’t just what she faces physically — it’s who she chooses to be anyway."

Ongoing treatments

In October, Mykela was sent to the University of Iowa Children’s Hospital in Iowa City because she was coughing up blood and her oxygen levels went up.

A major reason for Mykela’s struggling to breathe is protein buildup in the lungs, a complication associated with LPI.

“We had talked with the doctors in Iowa City about doing a lung wash,” Becky said. “They go in, put water in your lungs and clean it out. They were trying to get the protein out of her lungs.”

During a whole-lung lavage, the patient is placed under anesthesia while 20 to 30 liters of saline is flushed through one lung at a time. Mykela underwent this procedure at the Cincinnati Children’s Hospital, one of the top programs in the nation for lung diseases and conditions.

“When they did the lung wash, they didn’t get as much protein out as they thought they would,” Becky explained. “I was thinking, OK, we can go have a lung wash every year, two years, whatever, to get the protein out of her lungs and then she won’t need as much oxygen.”

But after taking a piece of lung for a biopsy showed Mykela to have inflammation in her lungs, she was flown back to Iowa City to start Cytoxan, a medication for the inflammation.

The goal is to avoid Mykela having to be upgraded to high-flow oxygen, potentially eliminating her attending school.

Along with having gone through more than most will in a lifetime, Mykela knows more than most, staying very aware of her condition and procedures.

“She knows what’s going on. She understands,” Becky said. “She knows exactly what she wants, where she wants it, and she takes her own dressings off of everything.”

The hospitals and communities rally to help bring joy to sick children everywhere. She has participated in the iconic “Iowa Wave,” a college football tradition where at the end of the first quarter of every Hawkeyes home game, more than 70,000 fans, coaches and players turn toward the adjacent Stead Family Children’s Hospital to wave to patients and families on the top floor.

Mykela has met Herky the hawk, Drew Sample from the Cincinnati Bengals, George Kittle from the San Francisco 49ers, the Hawkeye girls basketball team, Iowa cheerleaders and more.

After her three-month hospital stay, Mykela is back at home with monthly visits to Iowa City for her Cytoxan. Fortunately, after thousands of needle pokes and many blown veins, Becky advocated for Mykela to receive an implanted port in her chest for easy access to veins.

But still, even just the monthly trip is difficult. “A year ago, in December, she was on 0.5 liters of oxygen, so we could have little tanks,” Becky said. “But in a year’s time, we’ve gone from 0.5 to 4 to 6 and sometimes 8 liters.”

Just on the drive to and from Iowa City, they used five big tanks of oxygen.

Mykela has donated blood, skin and lung tissue for research on her condition in the hopes it will help future children with LPI and its accompanying complications.

Currently, there are only four papers on the condition, and two of them are referencing the other two papers.

“So there are really just two papers that have been done on this,” Becky said. “It’s very low research. They think probably more children die from it because they don’t know. More kids have it than what we realize.”

The future

Though LPI is rare, patients can experience a near-normal lifespan through strict, lifelong dietary protein restriction and supplementation. The issue arises from complications like the inflammation and protein buildup in the lungs. Without an effective treatment, the prognosis could be grim.

“This is a little girl whose body fights battles daily. A body that feels weak. A body that has endured more procedures, more hospital stays, more uncertainty than most people experience in a lifetime,” Stow said. “And yet, her spirit? Untouched. Unshaken. Unmatched. Her heart is the strongest thing in the world.”

In 2020, Mykela was given the chance to Make a Wish. Though she wanted to go to Disney more than anything, the COVID pandemic made travel impossible through the program.

While she enjoys the bunk bed and iPad that came of her shopping spree, she never stops talking about her desire to go to Disney.

“Disney is at the top of her list,” Becky said.

Right now, Mykela would need to fly with a paramedic and a lot of oxygen. If the Cytoxan works, this could become easier. However, flying with oxygen is never easy. Driving would also be difficult as they would likely need a van full of oxygen tanks.

Becky has hope that the Cytoxan and lung washes will help Mykela get back to a more normal life.

If interested in assisting the family in making Mykela’s dream come true, there is a GoFundMe to donate.

In addition to her dreams of Disney, the family would like to get her a more appropriate wheelchair as her current one is one donated from the hospital and built for an adult, not a child. They’d like for her wheelchair to be motorized so she has more independence.

They are also looking at getting a night nurse so Becky isn’t up many times a night adjusting Mykela’s oxygen or helping with an upset stomach. There could also be a benefit to having a nurse go to school with Mykela so Stow isn’t checking her oxygen at all times.

It’s not just Mykela’s family who worries for her wellbeing.

“It’s exhausting and sad. And she does take a lot of our time, which I would not trade for anything because she’s alright and she’s fun and exciting to be around,” White said. “But yeah, mentally, you just want the best for her. It’s wanting her to get better.”

Downing said she views all the students as her children.

“You can’t separate it. They just do, they become your kids,” Downing said. “When she’s sick, you just worry. You don’t stop worrying when they leave these doors.”

Regardless of what happens, Mykela will continue to be the spunky girl she is today.

Stow said Mykela is her hero.

“She teaches strength without preaching it. She teaches gratitude without saying a word. She teaches perspective without ever trying to give a lesson. Just by being here — by showing up — she reminds us that life is fragile, yes, but it is also beautiful and worth showing up for,“ she said. ”A child who has walked through suffering and still chooses kindness. A little girl who needs oxygen to breathe but somehow gives everyone around her room to breathe easier... She may be small in size, but her impact is enormous."