Physician views regarding the benefits and burdens of prenatal surgery for myelomeningocele.

OBJECTIVE: Examine how pediatric and obstetrical subspecialists view benefits and burdens of prenatal myelomeningocele (MMC) closure. STUDY DESIGN: Mail survey of 1200 neonatologists, pediatric surgeons and maternal-fetal medicine specialists (MFMs). RESULTS: Of 1176 eligible physicians, 670 (57%) responded. Most respondents disagreed (68%, 11% strongly) that open fetal surgery places an unacceptable burden on women and their families. Most agreed (65%, 10% strongly) that denying the benefits of open maternal-fetal surgery is unfair to the future child. Most (94%) would recommend prenatal fetoscopic over open or postnatal MMC closure for a hypothetical fetoscopic technique that had similar shunt rates (40%) but decreased maternal morbidity. When the hypothetical shunt rate for fetoscopy was increased to 60%, physicians were split (49% fetoscopy versus 45% open). Views about burdens and fairness correlated with the likelihood of recommending postnatal or fetoscopic over open closure. CONCLUSION: Individual and specialty-specific values may influence recommendations about prenatal surgery.

Guidelines for Growth Hormone and Insulin-Like Growth Factor-I Treatment in Children and Adolescents: Growth Hormone Deficiency, Idiopathic Short Stature, and Primary Insulin-Like Growth Factor-I Deficiency

BACKGROUND/AIMS: On behalf of the Drug and Therapeutics, and Ethics Committees of the Pediatric Endocrine Society, we sought to update the guidelines published in 2003 on the use of growth hormone (GH). Because idiopathic short stature (ISS) remains a controversial indication, and diagnostic challenges often blur the distinction between ISS, GH deficiency (GHD), and primary IGF-I deficiency (PIGFD), we focused on these three diagnoses, thereby adding recombinant IGF-I therapy to the GH guidelines for the first time. METHODS: This guideline was developed following the GRADE approach (Grading of Recommendations, Assessment, Development, and Evaluation). RESULTS: This guideline provides recommendations for the clinical management of children and adolescents with growth failure from GHD, ISS, or PIGFD using the best available evidence. CONCLUSION: The taskforce suggests that the recommendations be applied in clinical practice with consideration of the evolving literature and the risks and benefits to each individual patient. In many instances, careful review highlights areas that need further research

What factors are important to parents making decisions about neonatal research?

BACKGROUND: Although parents of neonates with congenital heart disease are often asked permission for their neonates to participate in research studies, little is known about the factors parents consider when making these decisions. OBJECTIVE: To determine the reasons for parents' decisions about participation in research studies. METHODS: Qualitative analysis of the unsolicited comments of 34 parents regarding reasons for agreeing or declining to participate in research studies. Parents' comments were offered spontaneously during interviews about clinical care decisions for neonates with congenital heart disease. RESULTS: Parents cited five types of reason for or against permitting their newborn to participate in research studies: societal benefit (n = 18), individual benefit for their infant (n = 16), risk of study participation (n = 10), perception that participation posed no harm (n = 9), and anti-experimentation views (n = 4). CONCLUSION: Addressing parental decision making in the light of these reasons could enhance the parental permission process for parents of critically ill neonates.

Role conflicts of physicians and their family members: rules but no rulebook.

OBJECTIVES: To elucidate the difficulties physicians have when a family member becomes ill and to elicit their underlying causes. DESIGN: Using a key informant technique, we solicited chairs of family medicine departments for their experiences with the health care provided to seriously ill family members. We then conducted in-depth, semistructured telephone interviews that were then transcribed, coded, and labeled for themes. SUBJECTS: 8 senior family physicians whose parents had experienced a serious illness within the past 5 years. All of the subjects reflected on experiences stemming from their fathers' illness. RESULTS: These physicians faced competing expectations: at an internal level, those of their ideal role in their family and their ideal professional identity; and at an external level, those originating from other family members and from other physicians. Reconciling these conflicting expectations was made more difficult by what they deemed to be suboptimal circumstances of the modern health care system. CONCLUSIONS: Conflicting rules of appropriate conduct, compounded by the inadequacies of modern health care, make the role of physician-family member especially challenging. The medical profession needs a clearer, more trenchant understanding of this role.

Ethics and communication: does students' comfort addressing: ethical issues vary by specialty team?

Ethics education aims to train physicians to identify and resolve ethical issues. To address ethical concerns, physicians may need to confront each other. We surveyed medical students to determine if their comfort challenging members of their ward teams about ethical issues varies by specialty and what attributes of students and their teams contributed to that comfort. Compared to other specialties, students felt significantly less comfortable challenging team members about ethical issues on surgery and obstetrics/gynecology. We suggest that ethics education must address the atmosphere on ward teams and give students skills to help them speak out despite their discomfort.

Deaths attributed to pediatric complex chronic conditions: national trends and implications for supportive care services.

BACKGROUND: Children with complex chronic conditions (CCCs) might benefit from pediatric supportive care services, such as home nursing, palliative care, or hospice, especially those children whose conditions are severe enough to cause death. We do not know, however, the extent of this population or how it is changing over time. OBJECTIVES: To identify trends over the past 2 decades in the pattern of deaths attributable to pediatric CCCs, examining counts and rates of CCC-attributed deaths by cause and age (infancy: <1 year old, childhood: 1-9 years old, adolescence or young adulthood: 10-24 years old) at the time of death, and to determine the average number of children living within the last 6 months of their lives. DESIGN/METHODS: We conducted a retrospective cohort study using national death certificate data and census estimates from the National Center for Health Statistics. Participants included all people 0 to 24 years old in the United States from 1979 to 1997. CCCs comprised a broad array of International Classification of Diseases, Ninth Revision codes for cardiac, malignancy, neuromuscular, respiratory, renal, gastrointestinal, immunodeficiency, metabolic, genetic, and other congenital anomalies. Trends of counts and rates were tested using negative binomial regression. RESULTS: Of the 1.75 million deaths that occurred in 0- to 24-year-olds from 1979 to 1997, 5% were attributed to cancer CCCs, 16% to noncancer CCCs, 43% to injuries, and 37% to all other causes of death. Overall, both counts and rates of CCC-attributed deaths have trended downward, with declines more pronounced and statistically significant for noncancer CCCs among infants and children, and for cancer CCCs among children, adolescents, and young adults. In 1997, deaths attributed to all CCCs accounted for 7242 infant deaths, 2835 childhood deaths, and 5109 adolescent deaths. Again, in 1997, the average numbers of children alive who would die because of a CCC within the ensuing 6-month period were 1097 infants, 1414 children, and 2548 adolescents or young adults. CONCLUSIONS: Population-based planning of pediatric supportive care services should use measures that best inform our need to provide care for time-limited events (perideath or bereavement care) versus care for ongoing needs (home nursing or hospice). Pediatric supportive care services will need to serve patients with a broad range of CCCs from infancy into adulthood.

Ethics and immunization policy: promoting dialogue to sustain consensus.

The societal consensus that has supported the United States' universal childhood immunization programs for the past 50 years shows signs of eroding. This article proposes a systematic approach to evaluate immunization policy options. Through a unifying framework that combines epidemiologic, economic, and ethical concerns, this approach promotes a clearer understanding of underlying issues and inherent tradeoffs between alternative policies. Such a systematic examination of policy options could facilitate the public dialogue necessary to continually recreate a broad consensus on immunization practices and enable us to choose policies most in accord with our fundamental values.

Continuity and quality of care for children with diabetes who are covered by medicaid.

BACKGROUND: Poor and minority children with Type 1 diabetes mellitus are at increased risk of severe adverse outcomes as a result of their disease. However, little is known about the quality of care that these children receive and which factors are associated with better quality of care. OBJECTIVES: Our objectives were as follows: 1) to describe the utilization of services associated with quality of care for children with Type 1 diabetes mellitus who are covered by Medicaid and 2) to test the hypothesis that increased continuity of primary care is associated with better care for these children. DESIGN: Retrospective cohort study. METHODS: Washington State Medicaid claims data for 1997 were used to determine what proportion of children with diabetes had 1) an inpatient or outpatient diagnosis of diabetic ketoacidosis (DKA), 2) a glycosylated hemoglobin (HgA1c) level that had been checked, 3) a retinal examination, and 4) thyroid function studies. Continuity of care was quantified using a pre-established index. RESULTS: Two hundred fifty-two eligible patients were identified. During the observation year, 20% had an outpatient diagnosis of DKA, 6% were admitted with DKA, 43% visited an ophthalmologist, 54% had their HgA1c checked, and 21% had their thyroid function assessed. Children with high continuity of care were less likely to have DKA as an outpatient (0.30 [0.13-0.71]). Children with medium continuity of care and high continuity of care were less likely to be hospitalized for DKA (0.22 [0.05-0.87] and 0.14 [0.03-0.67], respectively). For preventive services utilization, high continuity of care was associated only with an increased likelihood of visiting an ophthalmologist (2.80 [1.08-3.88]). CONCLUSIONS: The quality of care for Medicaid children with diabetes can be substantially improved. Low continuity of primary care is an identifiable risk factor for DKA.

Patients' knowledge of options at the end of life: ignorance in the face of death.

CONTEXT: Effectiveness of legislation promoting advance directives and legalizing physician-assisted suicide depends on patients' understanding their legal options about end-of-life care. However, outpatients' understanding of their legal options at the end of life has not been studied. OBJECTIVES: To estimate the percentage of outpatients who are informed about 4 areas relevant to end-of-life care: refusal and withdrawal of lifesaving treatments, physician-assisted suicide, active euthanasia, and double effect; and to determine whether authoring advance directives, experiencing illness, acting as a proxy for health care decisions, and caring for an ill loved one are associated with better knowledge in end-of-life care. DESIGN: Cross-sectional survey. SETTING AND PARTICIPANTS: One thousand consecutive English-speaking, adult patients attending 1 university-based internal medicine clinic and 3 community-based, university-affiliated, mixed internal medicine and family practice clinics in Oregon during May and June 1999. MAIN OUTCOME MEASURES: Percentage of correct responses in the 4 topic areas and total knowledge score, adjusted for demographic (eg, age, race, educational level, income level, marital status) and experiential (eg, health, proxy decision making, advance directives, and death of a loved one) factors. RESULTS: Of the 1000 patients invited to participate, 728 (73%) consented and completed the questionnaire and were included in the analysis. A total of 69% of respondents answered correctly regarding refusal of treatment, 46% for withdrawal of treatment, 23% for assisted suicide, 32% for active euthanasia, and 41% for double effect. Sixty-two percent of respondents did not distinguish between assisted suicide and euthanasia. After adjustment for other covariates, better knowledge was significantly associated with white race (odds ratio [OR], 2.3; 95% confidence interval [CI], 1.3-4.2), having at least a college degree (OR, 3.0; 95% CI, 1.4-6.7), and having been a proxy for health care decisions (OR, 1.8; 95% CI, 1.2-2.6). Personal experience with illness (OR, 1.0; 95% CI, 0.6-1.5), death or illness of a loved one (OR, 1.6; 95% CI, 1.0-2.7), and authoring an advance directive (OR,1.3; 95% CI, 0.9-2.0) were not associated with better knowledge. CONCLUSIONS: A significant proportion of outpatients at university-affiliated clinics in Oregon appear to misunderstand options in end-of-life care. Our results suggest that greater public knowledge about end-of-life care is needed, and advance care planning must be preceded by education about options in end-of-life care. JAMA. 2000;284:2483-2488.

The predicaments of "dangerous safety": living with juvenile diabetes in 20th century America.

Remedies are not useless because they fall short of their full scope. It is better to keep a man on the edge of a precipice, if you cannot pluck him away from it, than to let him fall over. And many diabetic patients are kept in this predicament of dangerous safety. Lectures on the Principles and Practice of Physic, 1836-1837 SirThomas Watson

Pediatric deaths attributable to complex chronic conditions: a population-based study of Washington State, 1980-1997.

OBJECTIVES: Advances in medical technology and public health are changing the causes and patterns of pediatric mortality. To better inform health care planning for dying children, we sought to determine if an increasing proportion of pediatric deaths were attributable to an underlying complex chronic condition (CCC), what the typical age of CCC-associated deaths was, and whether this age was increasing. DESIGN: Population-based retrospective cohort from 1980 to 1997, compiled from Washington State annual censuses and death certificates of children 0 to 18 years old. MAIN OUTCOME MEASURES: For each of 9 categories of CCCs, the counts of death, mortality rates, and ages of death. RESULTS: Nearly one-quarter of the 21 617 child deaths during this period were attributable to a CCC. Death rates for the sudden infant death syndrome (SIDS), CCCs, and all other causes each declined, but less so for CCCs. Among infants who died because of causes other than injury or SIDS, 31% of the remaining deaths were attributable to a CCC in 1980 and 41% by 1997; for deaths in children 1 year of age and older, CCCs were cited in 53% in 1980, versus 58% in 1997. The median age of death for all CCCs was 4 months 9 days, with substantial differences among CCCs. No overall change in the age of death between 1980 to 1997 was found (nonparametric trend test). CONCLUSIONS: CCCs account for an increasing proportion of child deaths. The majority of these deaths occur during infancy, but the typical age varies by cause. These findings should help shape the design of support care services offered to children dying with chronic conditions and their families.

A trade-off analysis of routine newborn circumcision.

BACKGROUND: The risks associated with newborn circumcision have not been as extensively evaluated as the benefits. OBJECTIVES: The goals of this study were threefold: 1) to derive a population-based complication rate for newborn circumcision; 2) to calculate the number needed to harm for newborn circumcision based on this rate; and 3) to establish trade-offs based on our complication rates and published estimates of the benefits of circumcision including the prevention of urinary tract infections and penile cancer. METHODS: Using the Comprehensive Hospital Abstract Reporting System for Washington State, we retrospectively examined routine newborn circumcisions performed over 9 years (1987-1996). We used International Classification of Diseases, Ninth Revision codes to identify both circumcisions and complications and limited our analyses to children without other surgical procedures performed during their initial birth hospitalization. RESULTS: Of 354, 297 male infants born during the study period, 130,475 (37%) were circumcised during their newborn stay. Overall 287 (.2%) of circumcised children and 33 (.01%) of uncircumcised children had complications potentially associated with circumcision coded as a discharge diagnosis. Based on our findings, a complication can be expected in 1 out every 476 circumcisions. Six urinary tract infections can be prevented for every complication endured and almost 2 complications can be expected for every case of penile cancer prevented. CONCLUSIONS: Circumcision remains a relatively safe procedure. However, for some parents, the risks we report may outweigh the potential benefits. This information may help parents seeking guidance to make an informed decision.