Meaningful patient representation informing Canada's cancer drug funding decisions: views of patient representatives on the Pan-Canadian Oncology Drug Review.

In this interview with the patient representatives on the Expert Review Committee (perc) of the Pan-Canadian Oncology Drug Review (pcodr), those representatives offer their views about how to be a valuable contributing member of Canada's national cancer drug funding recommendation committee. The article seeks to inform readers, and especially clinicians, about pcodr from the perspective of the patient representatives.

A prospective randomized study to evaluate the renoprotective action of beating heart coronary surgery in low risk patients.

OBJECTIVES: Cardiopulmonary bypass (CPB) is widely regarded as an important contributor to renal failure, a well recognized complication following coronary artery surgery (coronary artery bypass grafting (CABG)). Anecdotally off-pump coronary surgery (OPCAB) is considered renoprotective. We examine the extent of renal glomerular and tubular injury in low-risk patients undergoing either OPCAB or on-pump coronary artery bypass (ONCAB). METHODS: Forty low-risk patients with normal preoperative cardiac and renal functions awaiting elective CABG were prospectively randomized into those undergoing OPCAB (n=20) and ONCAB (n=20). Glomerular and tubular injury were measured respectively by urinary excretion of microalbumin and retinol binding protein (RBP) indexed to creatinine (Cr). Daily measurements were taken from admission to postoperative day 5. Fluid balance, serum Cr and blood urea were also monitored. RESULTS: No mortality or renal complication were observed. Both groups had similar demographic makeup, Parsonnet score, functional status and extent of coronary revascularization (2.1+/-1.0 vs. 2.5+/-0.7 grafts; P=0.08). Serum Cr and blood urea remained normal in both groups throughout the study. A significant and similar rise in urinary RBP:Cr occurred in both groups peaking on day 1 (3183+/-2534 vs. 4035+/-4079; P=0.43) before reapproximating baseline levels. These trends were also observed with urinary microalbumin:Cr (5.05+/-2.66 vs. 6.77+/-5.76; P=0.22). Group B patients had a significantly more negative fluid balance on postoperative day 2 (-183+/-1118 vs. 637+/-847 ml; P=0.03). CONCLUSIONS: Although renal complication or serum markers of kidney dysfunction were absent, sensitive indicators revealed significant and similar injury to renal tubules and glomeruli following either OPCAB or ONCAB. These results suggest that avoidance of CPB does not offer additional renoprotection to patients at low risk of perioperative renal insult during CABG.

Do physiological changes in pregnancy change defibrillation energy requirements?

Resuscitation during pregnancy is uncommon, with approximately 70 deaths occurring during pregnancy in the UK per annum. Physiological changes during pregnancy may affect transthoracic impedance (TTI), which determines transmyocardial current. Increased blood volume, cardiomegaly, haemodilution, changes in lung volume and changes in thoracic volume may alter impedance in ways that are difficult to predict. We measured TTI at term and after delivery once physiological changes had resolved. Mean (SD) TTI was 91.3 (15.8) Omega at term and 91.6 (11.8) Omega 6-8 weeks after delivery; the difference was not statistically significant. We conclude that current energy requirements for adult defibrillation are appropriate for use during pregnancy.

Primary pulmonary hypertension in pregnancy; a role for novel vasodilators.

We describe the case of a 28-week pregnant woman presenting with severe primary pulmonary hypertension (PPH). She had an elective Caesarean section under general anaesthesia at 32 weeks gestation. Pulmonary artery pressures (PAP) measured from a pulmonary artery catheter before anaesthesia were in excess of 100 mm Hg. Intraoperative nitric oxide was used to reduce PAP. After the delivery of a healthy infant PAP was controlled with nebulized iloprost and a prostacyclin infusion. Seven days later she was discharged from intensive care taking an oral calcium antagonist and warfarin. She developed intractable right heart failure and died 14 days after delivery. Despite increasing experience in the use of drugs to reduce PAP, the clinical course of pregnancy complicated by severe PPH is usually fatal.

Outcome at age 4 years in offspring of women with maternal phenylketonuria: the Maternal PKU Collaborative Study.

CONTEXT: Untreated maternal phenylketonuria (PKU) increases risk for developmental problems in offspring. The extent to which this risk is reduced by maternal dietary therapy at various stages of pregnancy is not known. OBJECTIVE: To determine whether dietary treatment during pregnancy of women with PKU affects developmental outcomes of offspring. DESIGN: The Maternal PKU Collaborative Study, an ongoing, longitudinal prospective study begun in 1984. SETTING: A total of 78 metabolic clinics and obstetrical offices in the United States, Canada, and Germany. PARTICIPANTS: A total of 253 children of women with PKU (n = 149), with untreated mild hyperphenylalaninemia (n = 33), or without known metabolic problems (comparison group; n = 71) were followed up to age 4 years. INTERVENTION: Women with PKU were offered a low-phenylalanine diet prior to or during pregnancy with the aim of maintaining metabolic control (plasma phenylalanine < or =10 mg/dL [< or =605 micromol/L]). Women with mild hyperphenylalaninemia, who had plasma phenylalanine levels of no more than 10 mg/dL (605 micromol/L) on a normal diet, were not treated. MAIN OUTCOME MEASURES: Children's scores on cognitive and behavioral assessments (McCarthy Scales of Children's Abilities, Test of Language Development, Achenbach Child Behavior Checklist, Vineland Adaptive Behavior Scales, and Home Observation for Measurement of the Environment), compared by maternal metabolic status at 0 to 10 weeks', 10 to 20 weeks', and after 20 weeks' gestation. RESULTS: Scores on the McCarthy General Cognitive Index decreased as weeks to metabolic control increased (r = -0.58; P<.001). Offspring of women who had metabolic control prior to pregnancy had a mean (SD) score of 99 (13). Forty-seven percent of offspring whose mothers did not have metabolic control by 20 weeks' gestation had a General Cognitive Index score 2 SDs below the norm. Overall, 30% of children born to mothers with PKU had social and behavioral problems. CONCLUSIONS: Our data suggest that delayed development in offspring of women with PKU is associated with lack of maternal metabolic control prior to or early in pregnancy. Treatment at any time during pregnancy may reduce the severity of delay.

Education and debriefing: strategies for preventing crises in crisis-line volunteers.

Telephone crisis lines offer an important service to individuals in crisis. The accessibility as well as a lack of other means of support leads many individuals to call the line. The role of the volunteer is to listen and support the caller as well as provide information and referrals to other agencies. Agencies are presented with a high turnover of volunteers and are then faced with the task of recruiting and training replacements. Volunteers are often exposed to horrific accounts of human pain and suffering which may affect their personal thoughts, feelings, beliefs and actions and influence the decision to quit. Compassion fatigue is one term used for this inherent "cost of caring." Many factors contribute to this cost including the nature of crisis calls, the repeat caller, and personal coping mechanisms. Educating and debriefing the volunteer are two strategies that may prevent the onset of compassion fatigue and volunteer resignation. Debriefing is viewed as an effective strategy for volunteers as it has been found to be successfull in assisting other helpers in many different contexts to cope and deal with the traumatic events that they experience or hear about.

Anaesthesia for emergency caesarean section in a parturient with bleeding placenta praevia and a potentially malignant hyperthermia-susceptible fetus.

A parturient who was 35 weeks' pregnant by her husband who was known to be susceptible to malignant hyperthermia, required anaesthesia for caesarean section for bleeding placenta praevia. The patient was considered to be haemodynamically stable and the procedure was carried out uneventfully under subarachnoid block. Anaesthesia was conducted as for an individual who is susceptible to malignant hyperthermia. The combination of the potential susceptibility to malignant hyperthermia of the fetus, and the problems of bleeding placenta praevia, produced an unusual clinical situation with potential conflict of interests when choosing the anaesthetic technique.

Hospital utilization of Saskatchewan people with fetal alcohol syndrome.

We describe the hospital utilization of 194 Saskatchewan persons with Fetal Alcohol Syndrome (88% Aboriginal), born between 1973-92. Complete provincial hospitalization data were obtained for 128 patients; partial data for 29 patients. Proportionately more persons missing data were adopted, not living with biological family members or were deceased. The hospital separation rates for the children with FAS, pooled from 1987-91, compared to the 1989-90 Saskatchewan rates were significantly higher (95% level of confidence) for males and females < 1 year, 1-4 years and 5-14 years of age. Relative to Saskatchewan Registered Indians, significantly higher hospitalization rate ratios were observed for males with FAS in all age groups and for females only age 5-14 years. Rate ratios for younger females may not have achieved significance because of missing data. Higher hospitalization rates in children with FAS may not be explained solely by factors associated with ethnicity.

Bone age and growth in fetal alcohol syndrome.

We have found delayed mean bone age in 63 children with fetal alcohol syndrome (FAS). The mean bone age Z-score for boys (n = 31) was -2.12 SDs and for girls (n = 32) was -1.62 SDs. This might suggest that they have potential for catch-up growth. However, experience with children with intrauterine growth retardation suggests that this will not be the case and that FAS children will be of reduced height at maturity. Further support for this assumption was gained from a sample of 26 patients who were followed until at least the age of 14 years for females and 16 years for males. There was no significant change in height Z-scores from early childhood to early adulthood, the mean score being -2.16 SDs and -2.11 SDs at mean ages of 4.83 years and 18.69 years, respectively. On the other hand, there were significant changes in weight and head circumference. The mean weight Z-score changed from -2.10 SDs to -1.14 SDs (p < 0.001). The head circumference mean Z-score in 16 patients was -3.13 SDs at a mean age of 2.79 years and -2.63 SDs at a mean age of 17.37 years (p = 0.013). Short stature can continue to be used as a diagnostic criterion for FAS beyond childhood.

Foetal alcohol syndrome in Saskatchewan: unchanged incidence in a 20-year period.

Despite major initiatives in public and professional education about foetal alcohol syndrome (FAS) in Saskatchewan in the last 20 years, its incidence rate has not fallen. The rate was 0.515 per 1,000 live births in 1973-1977 and 0.589 in 1988-1992. Two hundred and seven (207) cases were ascertained, the majority being patients of the Alvin Buckwold Child Development Program in Saskatoon. These individuals were severely handicapped: 72% had at least one malformation, the mean intelligence quotient was 67.8 (range 35-106) and 45.9% had a behaviour problem. Only 25.6% still lived with their biological parents when last seen, and only 27 of 108 cases were in a regular class at school without additional support being necessary. New approaches are needed to reduce the incidence of FAS. Emphasis should be placed on individual case-finding, counselling for high-risk women, and community development programs. We are currently attempting this through a provincial coordinating committee.

Neuropsychological evaluation of preschoolers with fetal alcohol syndrome.

Children with Fetal Alcohol Syndrome (FAS) manifest a variety of central nervous system deficits which may include mental retardation, perceptual, linguistic and fine motor deficits, as well as behaviour problems. The neuropsychological performance of to preschool-aged children with FAS was compared to age, sex, and race-matched controls. Profile analysis revealed significant deficits in the FAS group on measures of intelligence and linguistic abilities. Comparison of group profiles revealed that the FAS group displayed impaired visual-motor integration in the presence of average visual-perceptual matching. The FAS children were also reported to exhibit a greater frequency of behaviour problems compared to controls. Although the FAS group were significantly growth delayed, the often reported association between cognitive abilities and skeletal growth was not evident. These results indicate that preschoolers with FAS display a distinct pattern of impairments, and understanding of this unique ability profile is important for the development of support and intervention programs for these patients and their families.

Physician awareness of fetal alcohol syndrome: a survey of pediatricians and general practitioners.

OBJECTIVE: To determine the knowledge, clinical experience and perceived needs for resource materials of Saskatchewan physicians in regard to fetal alcohol syndrome (FAS) and alcohol-related birth defects. DESIGN: Mailed survey. SETTING: Saskatchewan. PARTICIPANTS: All 48 pediatricians and half (394) of the family physicians (FPs) and general practitioners (GPs) practising in Saskatchewan received a questionnaire. The numbers of physicians who completed it were 24 and 249 respectively. RESULTS: The pediatricians were more likely than the other physicians to be aware of FAS and to have diagnosed at least one case of FAS. Among the FPs and GPs, the year of graduation from medical school was a significant factor in their knowledge of FAS and their diagnostic practices. Those who graduated before 1974, the year FAS was first described in the medical literature, were less likely than the more recent graduates to be aware of FAS and to ask their patients about alcohol use during pregnancy but were more likely to feel comfortable discussing alcohol-related issues in families. All of the groups reported a need for more information about FAS and for resources on alcohol-related issues in general. CONCLUSIONS: Saskatchewan physicians are aware of FAS but have expressed a need for more information about FAS, particularly for parents, as well as physician training materials and information about where to refer patients with FAS and parents with alcohol-related problems.