Big sisters Violet and Ginny with Nora during her not-so-newborn photo shoot. 

Photo provided courtesy of Amy Green

Down Syndrome

Awareness

The Costley family celebrates Nora’s arrival home. 

Photo provided courtesy of Amy Green

March 21, 2022, is World Down Syndrome Day with the theme “inclusion means…” Each year in the United States 6,000 children are born with Down syndrome, equal to 1 in 700 children. With the theme of “inclusion means…” the organization hopes to help people understand Down Syndrome and what ways they can be included in society.

One Monett family has become advocates for Down syndrome when their third child was diagnosed with the disorder before her birth. Matt and Victoria Costley have three girls: Violet, Ginny, and Nora. When Victoria was pregnant with Nora in early 2021 some “soft-markers” were detected on a routine anatomy ultrasound. “After two tests, the high-risk OB called and confirmed that Nora has Trisomy 21, or the most common form of Down syndrome,” Victoria said.

A congenital defect in Nora’s heart, common in 50 percent of Down syndrome children, was harder to diagnose. “Nora’s heart was complicated, especially with the small left chamber, and was initially labeled as having Hypoplastic Left Heart Syndrome (HLHS),” Victoria said. “We learned that she would require surgery within her first weeks of life, and that surgery would depend on her heart’s performance outside the womb.”

Having received the diagnosis of Down syndrome, Victoria said that she and Matt felt at peace. “God placed so many guideposts along my journey to this moment that allowed me to truly have faith that I was made to be Nora’s mom,” Victoria said. “But her heart condition was always the most worrisome part. Nora’s was not one of the more common defects for Down syndrome. We were left not knowing what an exact surgery plan would be other than she would need surgical intervention within her first weeks of life. As a planner, that was one of the hardest things, knowing that Nora would need to be operated on in St. Louis but not knowing how her heart defect would play out in conjunction with her Down syndrome diagnosis.”

Victoria’s pregnancy continued with little complications, only extra weight from extra amniotic fluid, and another commonality with Down syndrome. 

Proud parents in the NICU getting ready to hand Nora over to the Cardinal Glennon transport team as they prepare to fly her from Springfield to St. Louis just hours after her birth.

Plans were made for a trip to St. Louis for Nora’s birth so she’d be in the hands of doctors immediately who would follow through with the first steps for Nora’s first heart surgery. Nora had other plans and on May 15, 2021, she arrived a little early, being delivered in Springfield and life-flighted via helicopter to Cardinal Glennon Hospital in St. Louis. “Even before the transfer, she was put on a life-saving medication to keep her heart functioning,” Victoria said.

At Cardinal Glennon Nora had a cardiac catheter done in the days before her surgery. “It caused her breathing issues and we had to watch her be intubated,” Victoria said. In these early days, and even before she was born, the Costley family began referring to Nora as the Warrior Princess. As her tiny, newborn body was prepared for heart surgery, Nora proved how strong she was with every step. The warrior kept fighting.

“Her open heart surgery plan was made based upon the findings of the catheter, and the original plan was to allow Nora’s left side of the heart to function. However, when Dr. Fiore was in surgery, he realized that Nora needed the Norwood surgery due to her mitral valve being too small; thus Nora’s heart began working with a single ventricle on the right side,” Victoria said. “The Norwood surgery is not commonly done with infants who have Down syndrome due to poor outcomes. Despite not having done a Norwood procedure on a child with Down syndrome in his 30-year career, Dr. Fiore felt it was her best chance at life and so he proceeded with this more extreme plan. Nora was very poorly after surgery, and her chest remained open for several days. She needed a lot of recovery time, but her chest was closed within a week via a second surgery.”

Nora spent her first six weeks of life at Cardinal Glennon. First in the Neonatal ICU then post-surgery she spent nearly four weeks in the Pediatric ICU. Finally, a few days in the Transitional Care Unit, the Costleys were educated in what Nora would need to thrive at home.

Nora holds Momma’s hand after her first open heart surgery.

“Since being released, Nora receives ongoing monthly Synergist infusions to help with RSV prevention,” Victoria said. “This is an expensive treatment, but for children like Nora who are extremely vulnerable this extra level of protection may just save their lives.”

Nora’s fight wasn’t over yet, and the Warrior Princess has continued to awe her family, friends, and church community with her strength. In October 2021, Nora had a second heart catheter to open her pulmonary artery and explore areas that needed attention for her second open-heart surgery that was in November 2021.

Having Down syndrome, Nora faces other challenges too. “Nora will need surgery soon for her congenital esotropia,” Victoria said. “She will likely need glasses and possibly patching to help with the weaker of her two eyes. She also has nystagmus, which involuntarily causes her eyes to move or shake from side to side. And Nora will need a third open-heart surgery called the Fontan. This is the last in a series of three surgeries that allow the single ventricle pathway to have increased blood flow to the lower extremities.”

Nora’s heart will never be “fixed.” She will always have to be monitored for issues since there is no cure for her heart condition. The Costley family praises the team at Cardinal Glennon for taking on Nora’s unique case knowing that they were treading unchartered waters.

The peace the Costleys had at Nora’s initial Down syndrome diagnosis continues. “The popular phrase in the Down syndrome community is that we are ‘The Lucky Few’, and I would agree,” said Victoria. “As a family we have been touched by the love found in both online Down syndrome groups like, Down Syndrome Diagnosis Network, and local organizations like Down Syndrome Group of the Ozarks.”

Nora’s sisters, Violet and Ginny, have learned about the diagnosis and shared with their friends what it means for Nora to have Down syndrome. “Violet in particular is an advocate for her sister and the Down syndrome community,” Victoria said. “Violet has shared a book with her first-grade classmates and teacher titled Hannah’s Down Syndrome Super Powers, as well as telling anyone she meets about her sister Nora. Violet is the first to speak up and say ‘sprinkle kindness like confetti’.”

Matt and Victoria want those in the community to know that there is nothing “down” about Down syndrome. The term comes from Dr. Down who first identified this syndrome. It refers to having a third copy of the 21st chromosome. This extra chromosome creates many common features for people with Down syndrome. Some features that Nora has, that are common, include: missing nasal bone, single palm crease, small ears, almond shaped eyes, sandal toe gap.”

As Nora grows, the Costleys are enjoying seeing her develop and express herself. “Holding hands is Nora’s all-time favorite thing to do,” said Victoria. “I believe this stems from back in the hospital when all we could do was to hold her hand, as picking her up was not always an option. She loves to be ‘tossed’ in the air, and this almost always elicits a smile. Mama can kiss her cheek and that makes her smile too. She loves when Ginny sings to her and when Violet rocks her or plays with her on the floor.”

Nora may be medically fragile, but she is strong. “She has overcome more in her first months of life than many will in a full lifetime,” Victoria said. “Nora has opinions, and can definitely express her likes and dislikes. She is going to write her own story and we cannot wait to see where it takes her.”

The Costley family are thankful for the First Steps program for children birth to age three with special needs in Missouri. This is open to children with a variety of needs. For Nora, she receives physical therapy, occupational therapy and speech/language therapy weekly, which have made a huge difference in her quality of life. 

Nora, the Warrior Princess, waves “bye, bye” to Cardinal Glennon on her last day after her second open-heart surgery.

Through this whole experience from diagnosis to bringing Nora home and all that will come in the future, the Costley family rely on their faith and recognize the ways God has been with them at every step. Nora’s heart surgeon prayed over Nora before her surgery. The hospital chaplain at Cardinal Glennon shared daily Bible verses and prayers, a comfort during the Costley’s six-week stay at the hospital. But early on, the morning they were scheduled to get results of the initial screeners before Nora was born, Victoria and Matt were waiting in line behind a young gentleman who had Down syndrome. “It was at that moment I knew, beyond a shadow of a doubt, that God was calling us to be parents of a little girl with Down syndrome. Matt and I spoke with the young man and his big sister who was there with him,” Victoria said. “I can’t even explain the sense of peace I had as we waited in line to get our results.”

If you haven’t encountered a person with Down syndrome, I hope you do one day. Expect smiles and hugs to abound and your heart to be touched. In the meantime, let’s all cheer on Warrior Princess Nora as she continues to amaze us all with her strength fueled by the love and prayers of her friends and family. ”