Lila's lung disorder was so rare, it was the first case of its kind at Stony Brook. It took a dedicated neonatalogist, working with an expert team, to finally diagnose her and provide appropriate treatment.
For the most part, Natalie N’s second pregnancy was progressing normally–until she reached about 30 weeks and her belly started filling up with fluid. At 32 weeks, Natalie’s water broke. An ambulance took her to a local hospital but due to the likelihood of preterm labor, she was transported directly to Stony Brook University Hospital.
At Stony Brook, an ultrasound revealed that the baby’s chest cavity was filled with fluid, compressing her lungs, and she was also in heart failure. Doctors ordered an immediate Caesarean section. Lila N was born September 27, 2008, with severe edema, fluid around her lungs, and unable to breathe on her own.
That’s when the Stony Brook NICU team, under the direction of Shanthy Sridhar, MD, Chief, Division of Neonatal and Perinatal Medicine, and Medical Director of the Neonatal Intensive Care Unit (NICU) and Neonatal Transports, took over the case.
In the operating room, attending neonatologist Dr. Joseph DeCristafaro immediately intubated Lila, and gave her the ventilatory support she desperately needed. Lila was immediately admitted to the NICU, where bilateral chest tubes were inserted to drain the fluid from her chest cavity. No one knew exactly what was wrong with Lila, and for weeks her condition was extremely unstable. Even with multiple chest tubes, she became so swollen that she was incapable of bending her arms and legs. Eventually fluid no longer drained from the chest tubes, and even though Lila was on a ventilator, her lungs barely maintained minimal oxygen exchange. She could no longer urinate, and her kidneys shut down. Lila appeared to be fading.
Natalie and her husband Kirk signed a DNR order because they just didn’t know how much more they could put Lila through. But the Stony Brook NICU team was not ready to give up. Nurse practitioner, Patricia Mele, needle-aspirated fluid directly from Lila’s chest, reinserted a chest tube, and later was able to insert a catheter to finally drain urine. Lila dramatically improved over the next couple of weeks, but eventually declined again, requiring the placement of additional chest tubes.
After Lila again stabilized and showed signs of increased strength, Dr. Sridhar gave Dr. Thomas Lee approval to perform general surgery on Lila, which included performing a pleurodesis and a surgical biopsy of her right lung. The biopsy revealed that Lila had an extremely rare lung disorder, primary pulmonary lymphangiectasia, the first case of its kind at Stony Brook. The neonatologists there reached out to world experts who specialize in the disorder. With that input, Lila’s care plan was adjusted and monitored by the Stony Brook team of neonatologists, nurse practitioners, NICU nurses, fellows, residents, and faculty.
Each day the team met with Lila’s parents to update the plan and report progress. Two weeks later, Dr. Lee performed a second pluerodesis, this time on the left side of Lila’s chest cavity. Week after week, Lila slowly improved, and was discharged to go home on January 30, 2009.
Today, Lila is developing appropriately, with mild chronic respiratory issues. She is a happy, vibrant toddler who loves music, books, and her big sister Sophie. “I really believe that Lila would not have made it if she hadn’t been at Stony Brook,” says Natalie. “It is more than a hospital. The best word I can use to describe the NICU staff is that they are like family.”