The puzzle
by Cyril Manning
Jennifer and Todd Watson were thrilled when their daughter Kathryn started walking. In that initial month of unsteady steps and potential falls, they had more on their mind than the 14-month-old's pesky cough.
But Kathryn's cough just kept getting worse. After a few weeks she began developing lesions on her face, arms and legs, and the Watsons rushed her to Children's Hospital Boston. Doctors in the Emergency Department were confounded by her strange combination of symptoms and, speculating that Kathryn might have chicken pox, admitted her for close observation. It was Nov. 11, 2000, and neither Kathryn's parents nor the doctors treating her expected the ordeal to last six long weeks, or for Kathryn to end up on a lung-bypass device called extracorporeal membrane oxygenation (ECMO). "It was a parent's worst nightmare," Kathryn's mother remembers.
After Kathryn was admitted to the hospital, her lesions continued to get worse and she began having difficulty breathing. She was also exhibiting stridor, an abnormal, high-pitched breathing sound caused by a constricted trachea. Kathryn's deteriorating condition made the puzzling symptoms much more urgent, so she was moved to the Multidisciplinary Intensive Care Unit (MICU) where critical care experts could manage her airway and breathing problems.
"The MICU is for patients who need close, constant monitoring," explains Adrienne Randolph, MD, assistant in Anesthesia and the pediatric critical care medicine specialist who headed Kathryn's intensive care team. "In the MICU, nurses, critical care fellows and pediatric residents coordinate all care, calling on specialists from throughout the hospital as needed." This team approach was central to Kathryn's treatment, as MICU staff worked hand-in-hand with experts from Otolaryngology, Dermatology, Immunology, Infectious Diseases, Respiratory Care and Pain Management to fit the pieces together.
The team's first urgent task was to keep Kathryn breathing. Stridor is caused by tracheitis, an infection that constricts the airway, so otolaryngologists inserted a breathing tube into her trachea. Because it was so swollen, they had to use the smallest tube available, usually reserved for premature infants.
In the meantime, the team had a second task at hand: diagnosing Kathryn's lesions. They needed to ensure that chicken pox was not the culprit because steroids, which are the typical treatment for tracheitis, suppress the immune system and would threaten a patient with chicken pox.
While the Respiratory Care team monitored Kathryn's breathing, the Infectious Diseases lab tested samples from her lesions. Although the results were inconclusive, they ruled out chicken pox, and the ICU called in another specialist—Stephen Gellis, MD, program director of Dermatology. With one look at Kathryn's lesions, Gellis was certain another puzzle piece had clicked into place. He knew that Kathryn had Sweet's syndrome—a disease so rare in children that it was only the third case he had ever seen. Although he was able to identify the condition, Gellis wasn't able to determine how the Sweet's was related to Kathryn's lung disease.
Nevertheless, Gellis' diagnosis was good news for two reasons: Sweet's syndrome is treatable with steroids, meaning doctors could proceed with a steroid treatment for Kathryn's tracheitis, and, in Jennifer's words, "We had a diagnosis for the lesions. At least we knew what we were dealing with."
With renewed hope, Kathryn's parents could only wait to see if the steroid treatment worked. While the lesions began to disappear, Kathryn still struggled to breathe because her lungs were not supplying enough oxygen or removing enough carbon dioxide from her blood.
After four long days of waiting, the Watsons got news they didn't want to hear: Kathryn's daily chest X-rays were only getting worse and her lungs were showing signs of acute respiratory distress syndrome (ARDS). Characterized by severe inflammation of the lungs, ARDS impedes the ability to transfer enough oxygen to the body to sustain life. "ARDS can be triggered by infections, trauma and many other causes," says Randolph, "and we weren't certain how it was related to Kathryn's Sweet's syndrome." Several times over the course of treatment, they had tested Kathryn for what they suspected might be the missing link — respiratory syncytial virus (RSV), a common childhood infection—but each time the results were negative.
Whatever the culprit, the only treatment for lung disease is time. Kathryn's 14-month-old lungs would have the best chance of healing themselves if they were allowed to rest. But neither a normal ventilator nor an oscillating ventilator, which rapidly delivers very small puffs of air to oxygenate the blood, could adequately sustain her respiratory levels.
"It was just a nightmare," says Jennifer. "On the oscillating ventilator, her body vibrated the whole time. The machine was very loud, and you couldn't move her." As Kathryn's condition failed to improve, it was clear her caregivers were running out of options. That's when Randolph advised the Watsons that Kathryn might need to go on ECMO.
The first time Jennifer heard of ECMO was from another mother in the waiting room whose son almost needed the treatment. "I really felt for her because I knew that it was a last resort," Jennifer recalls. "I remember thinking how lucky she was that, as it turned out, her son didn't need it. I never thought we would be in those shoes." Now that she faced the possibility herself, she says, "I asked the doctor if I should contact a priest to administer last rites, because I wanted her to tell me that I was crazy to think such a thing. But she didn't; instead, she said, 'We're not at that point.' She didn't want me to despair, but she also didn't want to give me too much hope."
At this critical point in Kathryn's care, Randolph set up a team meeting for the Watsons and every specialist involved in the case. "The point of the team meeting," explains Randolph, "was to review every aspect of Kathryn's history and present care in order to determine why her lung disease was worsening and what triggered the Sweet's syndrome in the first place."
Todd says the meeting was another example of how so many experts contributed to his daughter's survival. "The team was incredible. Not just in how they worked together, but also how they worked with us," he says. "We were never left out of the process."
At one point during the meeting, Randolph was called out of the room by Ofer Levy, MD, an infectious disease specialist. Randolph returned with the news that further tests had finally proven early speculations that RSV was indeed behind Kathryn's lung disease. Since viruses like RSV can also trigger Sweet's syndrome, it was likely that the viral illness had also led to Kathryn's rare condition.
Jennifer and Todd were thrilled that the puzzle finally seemed to be solved, and encouraged as their daughter began treatment with RSV-specific antibodies. But the doctors were cautious, warning them not to get their hopes too high. Kathryn's carbon dioxide levels were still fluctuating dangerously and her condition had deteriorated into ARDS, a more serious lung disease than RSV, with at least one in three children with ARDS dying of related complications.
The Center for the
Critically Ill Child
Leading experts of Children's Multidisciplinary ICU have established a new center of excellence: the Center for the Critically Ill Child. This collaborative model fully integrates the MICU's goals of patient care, research and education, and provides the most compassionate and sophisticated intensive care possible. Special components include:
- The only dedicated pediatric emergency transport team in New England, including a new specially designed ambulance
- A state-of-the-art Procedure/Simulator Room for critical care staff to practice and perform life-saving procedures
- The largest center for extracorporeal membrane oxygenation (ECMO) support in the United States
- Enhanced family support services
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Early the next morning, on Thanksgiving Day, Kathryn's lung function got much worse, and the MICU team decided that the time had come to put Kathryn on veno-venous ECMO, which bypasses the lungs but not the heart. It was the final line of defense in the battle to save her life. "That morning was by far the lowest point of the whole ordeal," says Todd. "We had the feeling that we were going into a black hole."
Jay Wilson, MD, senior associate in Surgery, performed the two-hour bypass procedure with help from a team of operating room nurses and the critical care unit. The ECMO catheter was surgically placed in the right side of Kathryn's neck to allow blood to flow through the artificial "lung" of the ECMO equipment, where it would be oxygenated and warmed before returning to her body.
"The first time I saw her on ECMO was heartbreaking," says Kathryn's mother. "The sight of all those tubes in her, her eyes swollen from the medication, was worse than a nightmare." But under the watchful eyes of respiratory care specialists 24 hours a day, the ECMO equipment allowed her lungs to rest and recover. The MICU team kept her sedated so that she would tolerate being on her back and not moving. To ensure that she wasn't suffering, they used narcotics and benzodiazepines to slow down her nervous system. After 10 long days, Kathryn was removed from ECMO and put back on a ventilator, and on Dec. 5, she finally breathed on her own again. "She still had a long way to go," says Jennifer, "but we were so excited that we'd finally turned that corner. We knew we were going to go home."
When they did get home, it took more than a month to fully wean Kathryn from the strong narcotics that had kept her sedated, and since her throat was still healing and she couldn't swallow, Jennifer and Todd fed her through a tube in her nose. "But that whole time," says Todd, "she never complained. She was the same happy, smiling girl she's always been."
Looking back, Jennifer remembers each and every care provider who helped her daughter survive. "The doctors and nurses were nothing but wonderful," she says. "And of course, we were so grateful for Dr. Randolph's presence through the whole thing. Everyone gave us the utmost care in the ICU, and they all worked together so well."
The Center was launched with a generous pledge from the Children's Hospital League. For more information, contact Cindy Zilch of the Children's Hospital Trust at (617) 355-2416.
