First Act
Within minutes of her birth, it became apparent that Rose was unable to eat. Frothy secretions built up in her mouth and she started to choke. Rose was affected by one of the most severe foregut malformations, esophageal atresia with tracheo-esophageal fistula. During the first month of her embryonal existence, poorly understood disturbances in molecular, cellular and morphogenetic pathways had led to a failure of the esophagus to form as a continuous organ and separate from the trachea.
On day two of life, Rose underwent a four-hour long operation to connect her esophagus and disconnect it from her trachea. Despite successful repair, her long and arduous odyssey had only just begun and she continued to suffer severe pneumonias. Following a battery of tests, her parents were told the problem was tracheomalacia- Rose’s efforts to clear secretions from her airways were frustrated as her excessively floppy trachea would collapse with every cough, trapping the mucous in her lungs. With time, they were told, the trachea would stiffen up and things would improve. Only they didn’t. Rose continued to require daily intensive airway clearance, frequent hospital admissions and almost constant antibiotic treatment. She was barely able to attend school during most of her childhood. Rose’s set of challenges resembled those of Odysseus, Homer’s tragic hero: Bloody battles, vicious fights with life forces, years of isolation, and imprisonment.
With these struggles in mind, it was suggested that Rose receive psychotherapeutic help. She arrived in the psychologist’s room with her hair disheveled, concealing her face. Initially reticent, she slowly opened up, primarily through her drawings and songs. Underneath the shy exterior, was a very funny, talented young teen. Her drawings featured images of people with masks on their faces and long necks tied in a knot (Figure 1). She felt choked and unable to truly express herself.
Her inner experience mirrored her external reality. Like Rose’s airway secretions were stuck deep down, unable to reach the surface, so could her emotions not be expressed, let alone exorcised. Rose developed a sense of identity founded on her illness- defective, isolated and an object of pity. “Everyone looks at me and feels sorry for me”.
But help was at hand. A novel surgical approach for tracheomalacia had been developed at Boston Children’s Hospital. This involved suturing the back wall of the trachea to the anterior spinal ligament, thereby achieving airway patency and facilitating effective secretion clearance. In a case series of nearly 100 children who underwent this repair, significant and clinically important improvements in all respiratory outcomes were observed. (1)