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)