Josie’s story: Breathing on her own and smiling big

When Kacie drops her 5-year-old Josie off at school, Josie smiles and waves at all the other kids on her way to her classroom.

“It’s like she’s the unofficial mayor,” Kacie says. “She’s always high-fiving people and giving them hugs. Other kids go out of their way to say ‘good morning’ to her, she just loves it.”

She also greets the team at Children's Health℠ Multidisciplinary Pediatric Aerodigestive Clinic (MPAC) as if she were the mayor. She goes out of her way to smile and wave at the doctors, nurses and therapists who work together in her care.

Seeing Josie thrive means everything to her family – because she spent the first eight months of her life in the hospital and needed medical equipment to breathe until she was almost four.

Cardiac intensive care

Josie’s parents first learned she’d be born with Down syndrome through a prenatal blood test and with a heart defect on a prenatal ultrasound.

“We knew she’d have medical difficulties, but we didn’t realize how sick she’d actually be,” says Josie’s dad, Mark.

After she was born, they learned that Josie had multiple heart conditions make it difficult for the heart to pump blood through the body and typically require surgery early in life. She had a successful open heart surgery at 3 months old. But then she experienced hypoxic respiratory failure, a serious condition when you don’t have enough oxygen in your blood.

Josie spent the months that followed in the Pediatric Cardiac Intensive Care Unit, where she needed a machine to help her breathe. She grew bigger and stronger but still couldn’t breathe on her own. That’s when her family met Josie’s primary cardiologist, who became a huge advocate for Josie, Matthew Lemler, M.D., Pediatric Cardiologist and Professor at UT Southwestern – and where they were introduced to the MPAC team.

Thoughtful conversations

MPAC brings together experts from Otolaryngology (ENT), Pulmonology,Gastroenterology and other specialties to care for kids with complex breathing needs. Kacie and Mark got to know Yann-Fuu Kou, M.D., Pediatric Otolaryngologist and Associate Professor at UT Southwestern, who would become a crucial part of Josie’s care team. Dr. Kou recommended a tracheostomy, a surgical procedure that involves creating a hole in the front of the neck, to help Josie breathe.

“Some kids who have significant heart problems will need breathing support for months or years,” Dr. Kou says. “A tracheostomy can be a good option for kids to continue to need breathing support but also be able to go home.”

Mark and Kacie were overwhelmed as they wrapped their heads around the procedure and follow-up care. Most of all, they wanted to make sure they were doing what’s best for Josie. They had discussions with many care team members, including one they’ll never forget with Andrew Gelfand, M.D., Pediatric Pulmonologist at Children’s Health.

Dr. Gelfand gently explained that sometimes babies are so sick that a trach is not recommended, but that wasn’t the case for Josie. He was honest that there was a long road ahead. But he was hopeful and optimistic for Josie’s future.

“That conversation brought me so much confidence that we were doing the right thing,” Kacie says. “We leaned on the compassion and expertise from the care team, our faith in God, and the support of family and friends.”

Dr. Kou performed Josie’s tracheostomy shortly after, and the care team worked closely with the family to learn how to manage Josie’s trach.

Just as important was explaining everything to Josie’s older sister, Eleanor. Then 4 years old, Eleanor had only met her little sister a few times because Josie had been so sick. The Child Life team explained to Eleanor what her sister’s medical equipment would look like using a teddy bear with a trach and a feeding tube. When Josie moved to Children’s Health Pediatric Inpatient Rehab and Integrated Therapy Services Center, Eleanor finally got to spend more time with her sister.

“Eleanor came with me to give Josie baths and just do things that big sisters do. It was so beautiful,” Kacie says. “This peace washed over Eleanor as soon as she was involved in her care. She didn't care that Josie had all of these cords and attachments, she was just excited to be with her sister.”

A surreal moment

When she was about eight months old, Josie finally got to go home. Her first birthday was a huge milestone. Still taking COVID-19 precautions, her family held a drive-by birthday party that included friends, family and even some members of Josie’s care team.

“A child’s first birthday is always a big occasion, but Josie had spent 300 days of her first year of life in the hospital and we'd seen her almost go to heaven more than once. So it felt momentous,” Kacie says.

Still, it wasn’t easy caring for a baby with complex medical needs and her sister at the same time.

“Our living room looked like an ICU with a ton of medical equipment. We’d wake up every day and do the best we could,” Kacie says.

Kacie distinctly remembers an appointment when the care team at the Complex Airway Clinicanswered all her questions and then told her: “Just keep doing what you're doing. You're doing a great job and I really think she’s going to thrive.”

Shortly after Kacie and Mark welcomed their third daughter into the world, they started talking to Dr. Kou about removing Josie's trach. Dr. Kou explained that Josie would likely need airway reconstruction to breathe on her own.

Josie (left) with your little sister Ruth (center) and big sister Eleanor (right).

“Sometimes the airway can get weak around a tracheostomy,” Dr. Kou says. “But we can do a procedure where we take a portion of the cartilage near the rib to help strengthen the airway and then we can remove the trach.”

Just before Josie’s 4^th birthday, after having her trach for three and a half years, Dr. Kou and the care team performed the procedure to remove it. And, for the first time in her life, Josie came home from the hospital without any breathing equipment. It felt surreal.

“We just couldn’t believe it that first morning,” Mark says. “There she was, like any other kid, sleeping on a pillow, breathing on her own.”

Loving and enjoying every moment

These days Josie spends her time at school, playing with her sisters and trying to convince her parents to give her ice cream.

“Our girl can devour some ice cream,” laughs Mark.

She still sees Dr. Kou for regular checkups at the MPAC clinic and comes to Children’s Health for physical therapy, occupational therapy, speech therapy and feeding therapy.

Josie (center) with her family: her mother Kacie (top left), father J Mark (top right), big sister Eleanor (bottom left) and little sister Ruth (bottom right).

Big sister Eleanor has taken a particular interest in helping her sister with feeding therapy, and recently wrote on her school assignment that she wants to be a feeding therapist when she grows up, just like Josie’s therapist Miss Kyla.

Their family would encourage other families going through similar diagnoses to hold onto hope, and to not let the worries of tomorrow – or even the next few hours or minutes – stop you from loving and enjoying your child in that moment.

“I wish Josie hadn’t had to go through so much medically, but it has transformed our family in such a positive way,” Kacie says. “We have come out better on the other side, and we are so proud of Josie. She is just this beautiful, incredible, vibrant girl.”

Learn more

Children’s Health provides expert, compassionate care from birth through young adulthood, including care for our smallest and most medically complex patients. Our specialists work together across disciplines to care for every aspect of a child’s health, helping children with complex needs thrive.

Our Ear, Nose and Throat (ENT) Program cares for children with a wide range of breathing and airway conditions. For children who need specialized or multidisciplinary airway care, our programs include the Multidisciplinary Pediatric Aerodigestive Clinic Airway Management Program and our Complex Airway Clinic.