Introduction:
Ethics, Ethics Everywhere
They say that when you have a hammer, everything looks like a nail. When you are an ethicist, you see ethical problems behind every screening urinalysis. Thomas Welch ends his superb review of selected renal diseases with a brief warning of the problems lurking behind the ability to diagnose autosomal dominant polycystic kidney disease (ADPKD) in childhood. “In the absence of symptoms,” he observes, “one might argue the merits of diagnosing in childhood a disorder unlikely to cause morbidity until adulthood.” This argument will become increasingly pervasive in pediatric practice over the next decade as the Human Genome Project identifies the molecular basis of more and more late-onset disorders. On the one hand, parents and pediatricians will always be tempted by the seductive appeal of knowing over the uncertainty of not knowing. Particularly when a relative has been affected, some parents will wonder and worry whether bad kidneys are the explanation of their child’s headaches or fatigue or uneven temperament. Physicians will be tempted by the intuitively obvious benefit of early treatment for chronic diseases. On the other hand, early labeling of children can lead to early disability. The burden of the label, particularly during childhood, can be worse than the disease. The “vulnerable child,” long familiar to pediatricians, may soon become the “uninsurable, unemployable” child. ADPKD is only one entity on a growing list of predictable late-onset disorders, including Huntington’s disease, neurofibromatosis, and Li Fraumeni syndrome-a condition associated with a 30% risk of cancer by the age of 30 years. There is little evidence as yet that presymptomatic diagnosis and early intervention have medical benefits to offset the psychosocial risks. It will not suffice for the pediatrician to sit on his or her hands, because there is a third hand. Affected individuals may be able to sue for damages if they can convince a jury that presymptomatic detection would have prevented later injury. The pediatrician may be helped by ethical guidelines developed by various professional groups, commissions, and consensus conferences. At the least, parents should be informed of the availability of such tests as they become routinely available. The physician’s duty may be discharged by simply informing; it is not clear there is a duty to actually test for disorders for which effective treatment has not been established. From the esoteric ADPKD, which the practicing pediatrician will rarely encounter, we turn to the ubiquitous question of circumcision. Thomas Wiswell’s concise and complete review tightens the noose around those who claim that the procedure is only a ritual, lacking medical justification. The incidence of penile carcinoma and serious urinary tract infection in uncircumcised boys approaches the incidence of phenylketonurra (PKU). Wiswell concludes that the growing evidence for serious illness in uncircumcised boys imposes an increasing obligation on the pediatrician to counsel in such a way that refusal is truly informed. But just as PKU screening has become mandatory in most states, Wiswell’s review raises the question of whether refusal of circumcision should also be considered sufficiently dangerous as to be beyond parental discretion and near the boundary of medical neglect. A major difference as compared with PKU screening is that one of the ma-
422
Current Problems in Pediatrics / November/December
1992
jor sequelae-penile cancer-does not typically occur until after the child is an adult and can decide for himself whether to be circumcised. Whether that is time enough to prevent the cancer is unclear. The more common complication of urinary tract infection, while sometimes associated with very serious sequelae, is not so uniformly associated with a devastating outcome as is the case with PKU, nor will circumcision prevent all such cases. NF
Current Problems in Pediatrics / November/December
1992
423
Ethics, Ethics Everywhere
They say that when you have a hammer, everything looks like a nail. When you are an ethicist, you see ethical problems behind every screening urinalysis. Thomas Welch ends his superb review of selected renal diseases with a brief warning of the problems lurking behind the ability to diagnose autosomal dominant polycystic kidney disease (ADPKD) in childhood. “In the absence of symptoms,” he observes, “one might argue the merits of diagnosing in childhood a disorder unlikely to cause morbidity until adulthood.” This argument will become increasingly pervasive in pediatric practice over the next decade as the Human Genome Project identifies the molecular basis of more and more late-onset disorders. On the one hand, parents and pediatricians will always be tempted by the seductive appeal of knowing over the uncertainty of not knowing. Particularly when a relative has been affected, some parents will wonder and worry whether bad kidneys are the explanation of their child’s headaches or fatigue or uneven temperament. Physicians will be tempted by the intuitively obvious benefit of early treatment for chronic diseases. On the other hand, early labeling of children can lead to early disability. The burden of the label, particularly during childhood, can be worse than the disease. The “vulnerable child,” long familiar to pediatricians, may soon become the “uninsurable, unemployable” child. ADPKD is only one entity on a growing list of predictable late-onset disorders, including Huntington’s disease, neurofibromatosis, and Li Fraumeni syndrome-a condition associated with a 30% risk of cancer by the age of 30 years. There is little evidence as yet that presymptomatic diagnosis and early intervention have medical benefits to offset the psychosocial risks. It will not suffice for the pediatrician to sit on his or her hands, because there is a third hand. Affected individuals may be able to sue for damages if they can convince a jury that presymptomatic detection would have prevented later injury. The pediatrician may be helped by ethical guidelines developed by various professional groups, commissions, and consensus conferences. At the least, parents should be informed of the availability of such tests as they become routinely available. The physician’s duty may be discharged by simply informing; it is not clear there is a duty to actually test for disorders for which effective treatment has not been established. From the esoteric ADPKD, which the practicing pediatrician will rarely encounter, we turn to the ubiquitous question of circumcision. Thomas Wiswell’s concise and complete review tightens the noose around those who claim that the procedure is only a ritual, lacking medical justification. The incidence of penile carcinoma and serious urinary tract infection in uncircumcised boys approaches the incidence of phenylketonurra (PKU). Wiswell concludes that the growing evidence for serious illness in uncircumcised boys imposes an increasing obligation on the pediatrician to counsel in such a way that refusal is truly informed. But just as PKU screening has become mandatory in most states, Wiswell’s review raises the question of whether refusal of circumcision should also be considered sufficiently dangerous as to be beyond parental discretion and near the boundary of medical neglect. A major difference as compared with PKU screening is that one of the ma-
422
Current Problems in Pediatrics / November/December
1992
jor sequelae-penile cancer-does not typically occur until after the child is an adult and can decide for himself whether to be circumcised. Whether that is time enough to prevent the cancer is unclear. The more common complication of urinary tract infection, while sometimes associated with very serious sequelae, is not so uniformly associated with a devastating outcome as is the case with PKU, nor will circumcision prevent all such cases. NF
Current Problems in Pediatrics / November/December
1992
423
