Coughs, giggles, grit
A family’s quest for normalcy, despite a chronic illness
When you meet Owen, a joyful, energetic and silly 6-year-old greets you. You’d never know he has a chronic illness. For him, each breath is a victory in his ongoing fight for health and a “normal” childhood.
When Richard and Keenan Thornton found out their firstborn would be a little boy, they were ecstatic. Keenan had a smooth pregnancy and labor, but after delivery, Owen started having difficulty breathing. Owen went to the Neonatal Intensive Care Unit (NICU) at Keenan’s delivery hospital, and Richard and Keenan were told not to worry.
Once Keenan was in recovery, she and Richard visited Owen in the NICU.
“I remember being in a wheelchair through all of this,” said Keenan. “When we saw Owen for the first time, he was covered in tubes and stickers, and monitors were beeping. The neonatologist came over and said, ‘We need to talk.’ We immediately knew something was very wrong. As the doctor talked to us, the Teddy Bear Transport team arrived to take him to Cook Children’s.”
Once they arrived at Cook Children’s Medical Center in Fort Worth, they met with a new neonatologist, a pulmonologist, surgeons, IV specialists, anesthesiologists and nurses. Owen was diagnosed with cystic fibrosis (CF), as well as meconium ileus, which would require reconstructive surgery.
CF is a progressive genetic disease that affects the lungs, pancreas and other organs. The hallmark feature of CF is the accumulation of thick mucus in the lungs, resulting in difficulty breathing. Meconium ileus is a bowel obstruction that occurs when the meconium in a child’s intestine is thicker and stickier than usual, creating a blockage in the small intestine. Most infants with meconium ileus have cystic fibrosis.
“I watched as my 2-day-old son was wheeled back for a major surgery,” Keenan recalled. “He was in the hands of a wonderful surgeon who took great care of Owen. They were able to clear the blockage and resection his bowels.”
Once the meconium ileus was resolved, the family faced addressing and creating a care plan for Owen.
Richard and Keenan met Karen Schultz, M.D., for the first time when Owen was 2 days old.
“Dr. Schultz had Owen immediately start receiving CF treatments,” said Richard. “We believe this is why he’s so healthy today.”
While in the NICU, the Thorntons received incredible support from the Cook Children’s staff.
“We were inundated with information, textbooks and people ready to mentor us, as we wondered what Owen’s life would look like,” said Richard. “Cystic fibrosis isn’t the same diagnosis as it was 20 years ago. I remember Dr. Schultz told us that Owen won’t know anything different. To him, this is normal, and that’s how you should treat him. I took that to heart.”
Treatment for Owen started small with pancreatic enzymes and gentle chest percussion therapy to loosen mucus. Now, in addition to taking enzymes before every meal and percussion therapy with a specially designed vest, Owen also does breathing treatments every day.
At 2 years old, Owen took part in a joint medical trial at Cook Children’s and the Cystic Fibrosis Foundation. He was the first child in his age group in the U.S. to take ORKAMBI®, a CF modulator, a drug that aims to restore function in the mutated gene that causes CF.
“Trials are the only way for us to know if a new therapy actually does what it should without untoward side effects,” said Dr. Schultz. “The Cystic Fibrosis Foundation has a legacy of improving the lives of patients with CF and an ultimate goal of curing CF. New CF medications are aimed at correcting the underlying problem rather than controlling consequences of the disease. Like all medications, they are first approved in adults. We know that with CF, the younger these medications can be started, the better a patient’s long-term outcomes are. Owen taking part in the ORKAMBI® study meant it could be approved for younger children. Without the willingness of families like the Thorntons to participate in trials, the goal of curing CF would never be reached.”
Because there is currently no cure for CF, Owen will have to continue treatment for the rest of his life, and the Thorntons are confident that Cook Children’s is the best place for his ongoing care.