“I cracked an egg with two yolks and immediately thought it was a sign – I am having twins,” says Rose Oge. “But, three years ago, we welcomed a solo Lemuel and never expected to have twins next. I thought ‘what?’ when they told me, but was happily surprised.”
Before the twins’ birth, Rose and her husband Emmanuel had another shock. Prenatal ultrasounds and an MRI showed one baby may have an intestinal blockage, though a definitive diagnosis couldn’t be determined until birth. When Mihaly and her brother, Darren, were born 5 weeks premature, the diagnosis was confirmed: Mihaly’s abdomen was swollen – a clear sign of internal pressure.
Additional tests were quickly performed showing twisted intestines – a potentially life-threatening condition. Just hours after her birth, Mihaly was rushed to the operating room, leaving her parents stunned. “Our doctors did their best to explain what was happening, but I couldn’t grasp an operation the day after their delivery,” says Rose. "We were just overwhelmed."
In the operating room, Allan Goldstein, MD, surgeon-in-chief and director of the Pediatric Neurogastroenterology Program at MassGeneral Hospital for Children, diagnosed the newborn with jejunal atresia. This rare disorder is thought to result from lack of blood flow while a baby is developing the small intestine, which results in a disconnection between the upper small intestine and the lower small intestine, colon and rectum. Mahily’s intestine was also wrapped around itself and prevented blood flow to the bowel, a condition called midgut volvulus.
The combination of jejunal atresia and midgut volvulus is rare. The major risk is losing all of the small intestine, leaving a baby without the ability to digest food. The operation itself also carries significant risk in a newborn, with potential for bleeding, infection and poor healing.
After an extensive procedure to untwist and remove dead bowel, Mihaly spent 4 months in the Patty Ribakoff Neonatal Intensive Care Unit and on the Ellison 17 pediatric unit where she began the slow rehabilitation process.
Mihaly had a second operation at 7 weeks old to lengthen her bowel. This procedure is not commonly performed, but Mihaly’s small intestine was very short - a result of her first life-saving procedure. Without the elongation procedure, Mihaly would have difficultly surviving without intravenous (IV) nutrition throughout her whole life.
“A typical infant of Mihaly’s age would have roughly 160 cm of intestine. So she struggled to absorb enough nutrients from her mother’s breast milk to grow,“ says Lauren Fiechtner, MD, director of nutrition in Pediatric Gastroenterology and Nutrition and the Center for Feeding and Nutrition. “She needed a tube to feed her IV nutrition in addition to her mom’s breast milk.” This treatment remained until she was 5 months old, when she finally was able to grow without the IV support.
“We’re accepting things now and I feel more educated thanks to Dr. Fiechtner and MGHfC,” says Rose. “I have the courage to do my own research to learn as much as possible. We’re learning how to cope with her needs.”
As Mihaly continues to mature, so will her treatment needs. Research shows that by feeding infants like Mihaly orally, the intestine will become used to absorbing more and more nutrients. Mihaly still is seen at the MGH Center for Feeding and Nutrition, and the team meets with her family monthly to closely monitor how her intestine is processing nutrition while increasing the amount of food she eats.
“Many children who are as medically complex as Mihaly have an aversion to eating by mouth,” says Fiechtner “but she’s progressing quickly and loves to eat green beans and purees with her brother.”
All photos courtesy of the Oge family.