Published On May 3, 2012
At my son Jack’s funeral, one of his many doctors apologized, teary-eyed, for having failed to save him. That this world-renowned specialist would even attend my son’s funeral, let alone shed tears for him, moved me beyond words. Yet mixed in with my immense gratitude was puzzlement at his remark.
Jack, who was five, had suffered from Kohlmeier-Degos disease, in which the lining of a patient’s blood vessels proliferates, blocking the flow of blood. Systemic Degos disease is always fatal; there is no cure. Despite those stark facts, the doctor seemed unable to accept that his training and persistence had not been enough, that he could not make the story end well.
Jack began developing severe headaches and stomachaches around his fifth birthday. An MRI showed a subdural hygroma, a fluid collection between the tough inner lining of the skull and the thin membranes that enclose the brain. A hole was drilled in his skull to extract the fluid and, when it accumulated again soon afterward, a shunt was implanted to transfer this mysterious fluid from his brain to his abdomen. All of this happened in less than two months, and though Jack had innumerable tests, they rendered no diagnosis. Eventually he was hospitalized because he was so sick—his blood pressure was high, he suffered severe head and stomach pain, and a large amount of fluid had collected in his abdomen.
Then the lesions appeared—small red indentations all over his body. They would turn white, then black, which gave his expanding medical team at MassGeneral Hospital for Children additional clues to what had been a conundrum. Jack died eight months after first experiencing symptoms, only a month and a half after he was diagnosed.
Mixed with the doctor’s grief, and perhaps exhaustion, was a sense of failure, but I count his successes, as well as those of his dedicated, talented colleagues—residents, chaplains, subspecialists of every variety, social workers, physical therapists, nurses, respiratory therapists and child psychiatrists. Degos disease is so rare that many who die from it probably never receive a diagnosis. My son’s illness was diagnosed because we consulted a neurologist trained in both adult and pediatric medicine. He had seen the disease only once before, in the 1960s. Jack was given an innovative treatment that showed promise but ultimately did not provide a cure. Now, inspired by his case, one of his doctors is conducting a clinical trial in hopes of developing a treatment for Degos disease.
What I wanted to offer to that doctor, as I remembered the long hours in the pediatric intensive care unit, the frantic phone calls to specialists all over the world, the tests after tests, the intubations and extubations, and the tears, were words of profound gratitude—but my grief wouldn’t let me formulate them. There were some who stopped coming to Jack’s room once they learned that we were no longer pursuing aggressive treatment. Most likely they thought there was nothing left for them to do. But the ones who continued to come let us know, by their presence alone, that we had not been abandoned. The wisest ones were able to detect our deepest fears and to assure us we were not failures as parents and that they admired our courage. They reminded us of every night we had spent at Jack’s bedside, advocating for him every step of the way, even when we had to make decisions no parent should ever have to make.
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