Reaching new heights
by Anna Gonski
Throbbing ears, trouble breathing, difficulty swallowing, feeling lethargic... must be a common cold, right? Wrong. What about the flu? Nope, try again. Pneumonia? Survey says... no.
Fifteen-year-old Allison Pollock suffered from all these symptoms and felt just plain "lousy" for most of her young life. But after a long medical journey, including several different diagnoses, four major surgeries and countless medications, she's finally starting to feel better. And she's got a robot named da Vinci to thank for it.
Allison's health problems began very early. "I can remember bringing her in for her two-week check-up," says her mother, Cheryl. "I could feel her wheezing when I held her in my arms, and she was very lethargic." Sure enough, Cheryl found herself in the emergency room at 11 p.m. that very night with a very sick little girl on her hands.
Allison had a bad case of pneumonia; nothing some antibiotics couldn't cure. But even after beating her pneumonia, she was still breathing too quickly, a sign of what her pediatrician thought was cystic fibrosis (CF)—an inherited lung disease characterized by an abnormality in the body's mucus glands that's chronic, progressive and ultimately fatal.
So the Pollocks were referred to a pulmonologist who tested Allison for CF on and off until she was 4 years old, when two, back-to-back sweat tests (a CF test that measures salt concentration in sweat) turned up positive. Allison was formally diagnosed with CF and started on various medications to help control the disease.
"The CF diagnosis was devastating," say Cheryl. "But when the new medications didn't seem to make much of a difference, I knew something wasn't right." Cheryl set about exploring other diagnostic tests for CF, which led her to a new nasal test in clinical trials at The Hospital for Sick Children in Toronto, Canada. Accepted as part of the study, the then-6-year-old underwent a full genetic work-up that revealed that she didn't have any of the CF mutations.
A follow-up sweat test was also negative, which sent the Pollocks and their doctors back to the drawing board, this time exploring the possibility of Immotile Cilia Syndrome, a genetic disorder characterized by abnormal motion of the cilia—the hair-like structures that line the lungs and help clean them out.
Allison underwent several tests over the next few years, but nothing conclusive pointed to Immotile Cilia Syndrome. In the meantime, she continued to have severe sinus, ear and upper respiratory problems, including a few bouts of pneumonia that landed her in the hospital overnight.
"She had six different sets of ear tubes placed for her infections," says Cheryl. "She also had two sinus surgeries, one when she was 7 and one at 12, and at least one or two cases of pneumonia a year for as long as I can remember."
Unsure what else to call it, doctors came to regard Allison's illness as asthma. "Regardless of what other issues she was having, the wheezing in her lungs was always present," says Cheryl.
She continued to see her pulmonologist every three months, and in 2003, a routine CT scan revealed that the middle lobe of Allison's right lung was no longer functioning. After yet another surgery, this time to remove the section of her lung that wasn't working, the Pollocks hoped they had finally seen an end to Allison's ever-present infections and illnesses.
But sure enough, the problem persisted, and the family was referred to Children's Hospital Boston in December 2003 for another opinion.
Allison landed in the right place—Children's Center for Aero-Digestive Disorders. Two years ago, three physicians —Reza Rahbar, DMD, MD, an otolaryngologist, Andrew Colin, MD, a pulmonologist, and Samuel Nurko, MD, a gastroenterologist, began conducting monthly meetings to discuss patients with problems of the airway, lungs or upper digestive tract. These are called aero-digestive disorders, and they include noisy breathing, swallowing disorders and chronic pneumonia. The center has since convened a formal conference, adding additional specialists from Cardiac Surgery, General Surgery and Radiology to take a multidisciplinary approach to these patients.
Gastroenterologist Rachel Rosen, MD, MPH, was the first to see Allison, exploring the possibility that she might be aspirating, or inhaling, stomach acid into her lungs. "Allison was experiencing acid reflux, when acid backs up into your esophagus, causing the burning sensation known as heartburn," she says. "So we started her on an antacid, and she did amazingly well."
But the reflux didn't explain Allison's chronic lung infections, so Rosen referred her to the Division of Respiratory Diseases where she met with Pulmonary fellow Michele Palella, MD, PhD. "One of the first things I noticed about Allison was that she had very noisy lungs," says Palella. "But when we tested her lung capacity, there didn't appear to be any predominantly lung-related issues."
Palella also discovered that Allison often suffered episodes of gagging when swallowing liquids. "Allison's medical history revealed a question of left vocal cord paralysis, which manifests in difficulty swallowing because the paralyzed cord remains open, allowing food or
liquids to slip into the airway and lungs," says Palella.
After reviewing Allison's case with Colin, Palella referred Allison to Children's Department of Otolaryngology and Communication Disorders, where she met with Rahbar, who confirmed the diagnosis. "A unilateral vocal cord paralysis may present as hoarseness," says Rahbar, "but rarely causes severe aspiration to the point of having pneumonia several times per year, so something obviously didn't add up."
Based on her medical history, Rahbar recommended that Allison have a complete aero-digestive evaluation under anesthesia. Rahbar, Rosen and Colin all took part in the procedure, performing tests and collecting samples from Allison's lungs, larynx and gastrointestinal (GI) tract. Her lungs and GI tract were relatively normal, but Rahbar did find a rare abnormality called a laryngeal cleft—a defect in the separation between the larynx, or voice box, and the esophagus.
"When the larynx develops properly, it's completely separated from the esophagus, allowing swallowed foods and liquids go into the stomach," says Rahbar. "A laryngeal cleft creates an opening between the larynx and the esophagus, allowing foods and liquids to pass through the larynx and into the lungs. This leads to problems with swallowing, like coughing, gagging and chronic lung disease."
Once Allison was formally diagnosed, the next step was surgical repair of the opening. While the traditional method has long been an invasive procedure requiring an incision in the neck and opening of the larynx, Rahbar performs a less invasive endoscopic procedure, going directly through the airway without any need for incision.
But about three years ago, he began looking into the possibility of robot-assisted surgery to repair airway abnormalities, working closely with Craig Peters, MD, director of the Minimally Invasive Surgical Unit, and Pedro del Nido, MD, chief of Cardiac Surgery.
Robotic equipment works very much like an extension of the surgeon's hands. While sitting at a console, the surgeon views a three-dimensional, magnified display of the area being operated on. The image is produced by two lenses at the end of a wand inserted into the location. The system's instruments flex in a wrist-like movement near the surgical site, precisely emulating the hand and finger movements of the surgeon at the console. The flexibility lets them work in tighter spaces, meaning that Rahbar could operate through Allison's mouth rather than having to make a small incision for the endoscopic procedure.
Children's Urology, Cardiac Surgery and General Surgery groups have been performing robotic surgeries using a system called da Vinci for about three years, but it was Rahbar who decided to test the equipment in the airway. "It seemed like a natural fit to repair laryngeal clefts—minimally invasive, more accurate and faster recovery," says Rahbar. But there was one problem. The equipment was too big to fit into the airway.
However, recent technological advances have allowed for much smaller equipment, so Rahbar quickly approached the Pollocks about using the robotic procedure to repair Allison's cleft. "Dr. Rahbar called me to explain that he wanted to try and do the repair with robotics," says Cheryl. "I thought it was great, very exciting."
"Laryngeal clefts are typically diagnosed much earlier in life, so the robotic equipment would still be too big for an infant or toddler," he says. "But since she's older, Allison's larynx was larger, making her the perfect candidate for the procedure."
On June 6, 2005, Allison became the first patient ever to undergo a robotic surgery on her airway. The repair was hugely successful, and Allison was on her way home just two days later. "Dr. Peters helped tremendously, offering technical support of the robotic equipment, since he is so experienced with it," says Rahbar. "And Dr. Lynn Ferrari in Anesthesia truly made the whole procedure possible, keeping the patient sedated enough to be operated on, but lightly enough to breathe on her own."
Today, Allison is feeling great and just started the 10th grade in September. She's down to only three medications—an antacid, something for allergies and an inhaler. And she is currently spiking and setting on her school's volleyball team.
"I haven't had to stop and rest at all during volleyball practice so far," says Allison. "This is a first for me."
To learn more about supporting Otolaryngology at
Children's Hospital Boston, contact Brandt Henderson in the
Children's Hospital Trust at (617) 355-5342 or
brandt.henderson@chtrust.org.
