Rare, extraordinary, one in a million. All could describe the journey of Anna and Isabella. The girls have persevered through difficult starts to life, with the help of our pediatric experts.
Five months into her pregnancy, Sohyun and her husband Wise learned they were having monoamniotic twins, a rare type of twins who share the same amniotic sac. They also received the sobering news that the most common complication is umbilical cord entanglement and the mortality rate is 50 percent.
While in the womb, Isabella was diagnosed with mirror image dextrocardia, meaning the heart formed on the opposite (right) side of her body. Anna was diagnosed with an encephalocele, a rare sac-like protrusion, or “bump,” of the brain through an opening in the skull.
“We probably had a better chance at winning the lottery than to have monoamniotic twins with these conditions,” says Wise. The main concern for Anna was whether the encephalocele was covered with skin or exposed, which couldn’t be confirmed until she was born.
“It was all so shocking. I never thought we would have twins, and certainly didn’t anticipate all these complications,” says Sohyun. “Everyone we knew was having perfectly healthy babies."
Care for the Most Fragile Newborns
The Division of Neonatology, led by Robin Steinhorn, MD, is devoted to treating newborns like Anna and Isabella who require the highest level of expertise and care. Each year, the neonatal intensive care unit (NICU) team cares for approximately 600 critically ill newborns, who are transferred from area hospitals that cannot meet their complex needs.
The physicians work closely with colleagues at Prentice Women’s Hospital, rotating service between the two hospitals’ NICUs. Additionally, Children’s neonatologists provide coverage at a number of community hospitals, including Central DuPage Hospital, Silver Cross Hospital and Lake Forest Hospital. Together they serve as the safety net for the most fragile newborns in the Chicago region.
When the girls were born six weeks early in January 2008, Isabella was not able to breathe on her own and needed to be transferred to the NICU at Children’s. Anna, whose encephalocele fortunately was covered, remained at Prentice to grow and gain strength. “It was heartbreaking to have the twins separated,” says Sohyun. “The girls hadn’t met each other outside of the womb.”
Leading Expertise for Rare Conditions
At Children’s, Sohyun and Wise learned that Isabella had only one lung as well as complete cartilage tracheal rings, causing an extremely narrow trachea, which made it impossible for her to breathe unaided. They met with Dr. Steinhorn, who arranged a consultation with Carl L. Backer, MD, head of the Division of Cardiovascular-Thoracic Surgery, and Lauren D. Holinger, MD, head of the Division of Otolaryngology.
Because babies in the NICU often have complex conditions affecting different areas of the body, the NICU team works closely with a variety of subspecialists. “The distinct advantage of our hospital is the breadth of pediatric specialists under one roof, who are just a call away,” says Dr. Steinhorn.
The family learned that Dr. Backer is one of the world’s leading experts in treating Isabella’s extremely rare condition. Dr. Backer explained that he would perform a slide tracheoplasty. In this procedure the trachea is initially cut in half and then each half is cut vertically. The two sections “slide” together to create a new trachea that is half as long but with four times the internal diameter.
Although he cited a 90 percent chance of survival, Dr. Backer had only performed the procedure eight times previously because of its rarity. Dr. Holinger cautioned Sohyun and Wise of a tough road ahead with a host of potential complications and a long recovery for Isabella. The primary concern was the formation of scar tissue, which could necessitate repeated surgeries. “We were nervous, but the fact that Dr. Backer and Dr. Holinger are so well-known definitely made us feel a lot better,” says Wise.
At only five days of age and weighing less than five pounds, Isabella underwent an intensive six-hour surgery performed by Dr. Backer and Dr. Holinger to reconstruct her trachea. Isabella was supported with a heart-lung machine during the delicate procedure, which required great precision given how narrow her trachea ring was — about as tiny as a Cheerio®.
The Annals of Thoracic Surgery recently published a paper co-authored by Dr. Backer and Dr. Holinger and others, detailing their results of treating nine patients with this same combination of conditions. This is the largest known group of infants with one lung and severe tracheal narrowing. Patients have come from far distances seeking the expertise available at Children’s.
Despite the risks and expected complications, Isabella’s recovery was phenomenal. “Her's was one of the best recoveries I’ve ever seen for this procedure,” says Dr. Holinger.
New Hope on the Horizon
When she was 2 months old, Isabella came home for the first time and was finally reunited with her sister. Anna, who had been home since she was 3 weeks old, underwent surgery at 5 months old to remove the encephalocele on her head.
Isabella and Anna, now 2½ years old, love to play together and speak their “twin talk.” They are catching up to their peers, thanks to early intervention through speech, physical, developmental and occupational therapies. Dr. Steinhorn recently ran into Anna and Isabella in the hospital lobby and says she could not believe her eyes. “It was amazing to see these girls, who were so ill as infants, running around as toddlers.”
Because she is living with one lung, Isabella can become winded if she overexerts herself, but is just as active as her sister, if not more so. All in all, they are healthy, happy and show few signs of their complex medical journeys.
“As neonatologists, our mindset is different than it was 10 years ago. Survival is no longer the only desired outcome; it’s about finding ways to increase the quality of children’s lives,” says Dr. Steinhorn. “We want to see former NICU babies running around on the playground and thriving at school. Anything less than a full life is unacceptable to us.”
This article first appeared in the fall 2010 issue of Heroes magazine.