Rights, respect for dignity and end-of-life care: time for a change in the concept of informed consent.

The current concepts of autonomy, surrogate autonomy and informed consent often lead to futile and expensive care at the ends of life. They may impinge on the dignity of the patient as well as subject society to unwarranted expense. In order to provide affordable healthcare for all, these concepts are in need of modification.

The ketogenic diet: seizure control correlates better with serum beta-hydroxybutyrate than with urine ketones.

The objective of this study was to determine the relationship between beta-hydroxybutyrate levels and seizure control in children on the ketogenic diet. Seventy-four children on the ketogenic diet presenting for routine follow-up visits had blood levels of beta-hydroxybutyrate correlated with their seizure control. Forty-two children admitted for initiation of the ketogenic diet had urine ketones measured by dipstick and correlated with simultaneous blood levels of beta-hydroxybutyrate. Blood beta-hydroxybutyrate levels statistically correlated with seizure control (P = .003). Children with blood beta-hydroxybutyrate levels greater than 4 mmol/L were significantly more likely to have a decrease in seizure frequency than those with levels less than 4 mmol/L. Urine ketones of 4+ (160 mmol/L) were found on dipstick when blood beta-hydroxybutyrate levels exceeded 2 mmol/L. Seizure control correlates with blood beta-hydroxybutyrate levels and is more likely when blood beta-hydroxybutyrate levels are greater than 4 mmo/L. The traditional measurement of urine ketones by dipsticks in children on the ketogenic diet provides a less than optimal assessment of the degree of blood ketosis. Three to four plus (80-160 mmol/L) urine ketones are necessary, but not necessarily sufficient, to achieve optimal seizure control in children on the ketogenic diet. At present, however, urine ketones are the only readily available inexpensive approach to ketone assessment.

Medication cost reduction in children on the ketogenic diet: data from a prospective study.

In a prospective study of the efficacy of the ketogenic diet in children with severe, refractory epilepsy, data were collected on medication changes over 1 year. Cost reductions in medications were calculated over the first year and estimated for a second year. Fifty-seven percent of the children stayed on the diet for 1 year, and 74% of these children had their number of medications reduced. Forty-eight percent of children who stayed on the diet were on no medications at 12 months follow-up. Daily medication costs were reduced by nearly 70%.

Why would you remove half a brain? The outcome of 58 children after hemispherectomy-the Johns Hopkins experience: 1968 to 1996.

PURPOSE: To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. METHODS: Charts were reviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Sturge-Weber syndrome or other congenital vascular problems. Seizure control alone did not seem to adequately describe the outcomes of the procedure. Therefore, a score was constructed that included seizure frequency, motor disability, and intellectual handicap. This burden of illness score better described the child's handicap before and after surgery. RESULTS: Perioperative death occurred in 4 out of 58 children. Of the 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) have nonhandicapping seizures, and 23% (12/54) have residual seizures that interfere to some extent with function. Reduction in seizures was related to the etiology of the unilateral epilepsy. Eighty-nine percent of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandicapping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness. In 44 the procedure was very successful, in 7 it was moderately successful, and in 3 it was minimally successful. Success was related to the etiology, and early surgery was preferable. CONCLUSION: Hemispherectomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children with severe or progressive unilateral cortical disease. Early hemispherectomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the child to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development.

No-fault cerebral palsy insurance: an alternative to the obstetrical malpractice lottery.

Sixty percent of malpractice premiums paid by obstetricians go to cover suits for alleged birth-related cerebral palsy (CP). Yet substantially less than half of that money goes to CP victims, and less than 10 percent of children with CP receive any compensation at all from tort suits. This paper proposes a system that would compensate all children born with CP for most handicap-related expenses, in exchange for which the children would be foreclosed from bringing suits alleging birth-related malpractice. Malpractice would be policed by a state board, which would investigate all CP cases. This proposal would be more equitable than current systems. It would also be less expensive, since it would avoid costly litigation and decrease the cost of obstetrical malpractice insurance.

Epilepsy and the inner city schools: a school-based program that makes a difference.

A program was developed within the Baltimore City School System to comply with U.S. Public Laws 94-142 and 94-484. The program provided assessment, appropriate placement, counseling, work experience, and epilepsy education. Self-identified students were primarily black and poor, more likely to be either 1 year behind in reading or in special education. Seventy percent had psychosocial problems but for less than half were the problems directly related to seizures. The program decreased the nonpromotion rate and the dropout rate to less than half of that for the school system as a whole, and cut in half the percentage of youths who were unemployed or not in school or training 1 and 2 years after graduation. The per pupil cost was 10% greater than current expenditures for the system as a whole.

Anticonvulsant serum levels are useful only if the physician appropriately uses them: an assessment of the impact of providing serum level data to physicians.

The ability to measure serum levels of anticonvulsants has been a significant advance in the treatment of epilepsy. This technique enables practitioners to monitor a patient's plasma concentration, to detect potential toxicity, and to assess compliance with the prescribed regimen. A retrospective study of 164 adults with epilepsy was conducted to evaluate how serum anticonvulsant determinations were used by physicians in their treatment of epilepsy. Results indicate that the availability of test results did not improve the degree of seizure control, nor did it diminish patient reports of toxicity. In 17% of therapeutic decisions, prescribers did not appear to use the blood levels appropriately in their therapeutic decision-making process. When physicians did appropriately utilize information from serum levels, the degree of seizure control improved significantly compared with when the prescribers did not use this information. The cost of determining serum levels of anticonvulsants is justified only if the information is appropriately utilized.