Emry's story: Compassionate care for complex illnesses

Moments after Emry was born her care team knew something wasn’t right.

She was having trouble breathing.

In the NICU, genetic testing revealed the problem: a breathing disorder called congenital central hypoventilation syndrome (CCHS). That’s when she was transferred to Children's Health℠.

"Basically, her brain doesn’t tell her body when to take a breath," said her mom Myia. "It's like when you are swimming underwater and get the feeling that you need to come up for air. She doesn't have that feeling."

Myia and her husband Daniel couldn't have known that Emry would ultimately spend the better part of her first two years at Children’s Health — and that CCHS was the first of two life-changing diagnoses.

An expert team for a rare disorder

CCHS happens because of a change in a gene that helps control breathing. Babies who have it typically need to be on a ventilator 24/7 to help them breathe. As kids grow up, they may be able to transition to using a ventilator only when they sleep.

The care team soon learned that like many kids with CCHS, Emry also had Hirschsprung’s disease, which requires surgery for their bowels to work properly. CCHS also increases risk for neuroblastoma, a childhood cancer that starts in nerve cells.

Emry’s family was relieved to learn that while CCHS only affects an estimated 2,500 people worldwide, Children’s Health has a team with deep expertise in caring for children with this condition.

"Even though this is a rare disorder, we care for a good number of kids with CCHS," said Andrew Gelfand, M.D., Pediatric Pulmonologist at Children’s Health and Professor at UT Southwestern. "It truly takes a team — pulmonology, gastroenterology, social work, palliative care, cardiology, sometimes oncology — working together to see to every aspect of the child’s health."

Myia and Daniel wrapped their heads around Emry’s diagnosis, wondering what her future would look like.

"We relaxed a bit as we learned more about it from her care team and found out kids can lead fairly typical lives," Myia said. "I also joined an online support group and it was a relief to get to know other parents going through the same thing."

Still, Emry had a long road ahead. She would need her breathing monitored around the clock and routine testing for neuroblastoma.

Her family feared the worst when one of those routine tests came back abnormal. They held their breath waiting for the results of the ultrasound that followed.

Another life-changing diagnosis

Later that day, Myia got the call: Emry had a tumor near her left kidney.

"It was a heartbreaking moment that we knew was possible but wished so hard would never come," Myia said.

A more detailed scan revealed eight tumors in her chest and abdomen. She soon started her first rounds of chemotherapy, followed by two surgeries, two weeks apart, to remove the tumors.

Everyone's hearts sank again when post-surgery testing revealed that some of the cancer was considered high-risk neuroblastoma. That meant Emry needed even more intensive treatment.

"I just told myself 'God doesn’t give you more than you can handle,'" Myia said, "I knew I had to be strong for Emry."

A more complex treatment plan

The road ahead for Emry stretched even longer, with five more rounds of chemotherapy, two stem cell transplants, two weeks of radiation and six rounds of immunotherapy.

Everyone rallied around her: Her 5-year-old brother Noah, who just wanted his new sister to come home. Her parents. Dr. Gelfand, who made a point to make extra visits to her room even when he didn't have to. Tanya Watt, M.D., Pediatric Oncologist at Children’s Health and Associate Professor at UT Southwestern, who led her cancer care. Her aunts, Melissa and Kryssie, who rarely left her side.

Treatment for neuroblastoma weakens the immune system, increasing a child’s risk of infection. Children with CCHS are more likely than other children to have severe complications from infections — and they may not show the typical signs of an infection like a fever.

"We needed to monitor her very closely for infection so we kept her in the ICU for the intensive portion of her treatment," Dr. Watt said. "When it was time for radiation, we had a special transport team bring her back and forth to the radiation center. So many people put in extra thought and hard work to give Emry the best possible care."

The hardest part was the back-to-back stem cell transplants. They aimed to knock out all of the cancerous cells with chemotherapy, and replace them with new, healthy ones.

Myia was pregnant with their third child at the time and had to keep her distance because chemotherapy can be harmful during pregnancy. To make things worse, Daniel was away for Army training. So Myia leaned on her sisters-in-law, Melissa and Kryssie, and Emry’s grandparents, Tanya and Marc. And she was deeply grateful for every nurse and doctor who treated Emry as if she were their own child.

"When you’re taking care of a child who is that sick, the family needs to know you are there for them," Dr. Gelfand said. "You’re there to answer their questions and understand their concerns. My team sometimes laughs at me for making 'social visits' to see patients when they’re in the hospital. But I do it because it helps families know that we really care about their child."

The end of the road, then a U-turn

Emry’s recovery from the stem cell transplants seemed fine initially. But a few days later Myia just knew — something wasn’t right. Her care team agreed and ran some tests. Emry had way too much carbon dioxide (CO2) in her blood.

They tried everything they could to get her CO2 levels down and to improve her breathing. Nothing worked.

"It was 3 a.m. in the ICU and she was in respiratory failure," Myia said. "The doctors said this may be the end of the road for Emry."

Myia asked if they could take Emry off the ventilator. Emry looked miserable and Myia didn’t want her life to end that way.

"When they switched the ventilators, her vitals did a complete 180," Myia said. "It felt like a miracle."

Her health continued to improve steadily and she was ultimately able to finish her treatment. The week of Thanksgiving, Emry’s care team said her cancer was in remission.

"Emry exceeded our expectations at every step of the way," Dr. Watt said. "Our team rallied around Emry and did everything we could to meet her exact needs. It's an honor to be part of a team that is willing and able to provide that level of care."

The end of a 645-day hospital stay

After a year, 9 months and 6 days, Emry finally got to go home.

For the first time in her life, she was home for Christmas — and overjoyed to open presents with her siblings, cousins and extended family.

"It was so amazing to see her there with the other kids," Myia said. "They’re all running around, ripping papers and having a blast. Emry just loved it."

Emry will need ongoing care for her CCHS and regular follow-up scans to make sure the cancer stays in remission. But for now, she’s busy being a kid who loves singing, dancing, bubbles and playing with her siblings.

Myia shares some advice for families going through complex medical treatment: Build a support system. Never be afraid to ask your care team questions. Trust your gut.

"Above all, show your child as much love as you can, every day," Myia said. "That's what we did and that’s what her doctors and nurses did. It's scary but just take it one step at a time. And know that the Children's Health care team is amazing and they'll do everything they can for your child."

Learn more

Children's Health has deep experience treating babies and children with the most complex conditions. Our Complex Care Medical Services program team brings together experts from different specialties to provide expert and compassionate care with the goal of helping your child be as happy and healthy as possible. Learn more about our program and services.