The decision to forgo life-sustaining treatment is frequently made for children dying in French PICUs. Guidelines must be available to help the medical staff reach this decision. Knowledge of the decision-making process in French PICUs provides the experts with information needed to elaborate such recommendations.

The aim of our study was to evaluate pain frequency, intensity, and disability levels in a population with neuromuscular disorders (NMD). Of 862 questionnaires mailed to outpatients treated at 10 centers, 511 (300 men and 211 women) responded with answers suitable for analysis (response rate: 59.3%). Patients had Duchenne or Becker muscular dystrophy, type 1 myotonic muscular dystrophy, facioscapulohumeral muscular dystrophy, metabolic myopathy, or myasthenia gravis (MYA). The questionnaire packet included numeric scales for pain intensity and relief, the Brief Pain Inventory, the Saint Antoine Pain Questionnaire, and a scale to assess disability. More than two-thirds of the 331 patients (67.3%) suffered pain during the last three months. The mean number of days with pain was 18.4+/-15.1 days. The mean pain intensity was 4.8+/-2.5. Pain was usually diffuse (153 patients, 44%) and intermittent (228, 71%). Pain intensity varied by the NMD diagnosis; the most severe pain was observed in metabolic myopathy (13/27 patients suffered severe pain, 49%) and in MYA (16/42, 38%). Approximately three-quarters of patients had fewer than 10 days of inactivity due to pain during the last three months, and 98% had fewer than 30 days. Our study indicates that pain is frequent in hereditary muscle disorders and MYA. Mean intensity is moderate. Pain in NMD patients should be systematically assessed.

We conducted a descriptive study on a given day on all inpatients requiring palliative care in a French university hospital. In each department, a collaborative team made up of physicians and nurses identified and described the clinical signs, the treatment protocols, the social and family characteristics and the outcome for each patient using a standardized questionnaire. Study subjects were inpatients in the hospital and presented advanced or terminal-stage life-threatening conditions. Two-hundred-and-forty-five patients were included in the study. These patients represented 13% of the total number of inpatient beds available in the hospital on the day of the survey. Sixty-six per cent of study subjects suffered from physical discomfort and 80% suffered psychologically. Patients still received specific treatment for their condition in 45% of cases. Social problems were identified principally in medium- or long-term care department inpatients who made up 36% of the total inpatient population. A request for transfer to another care structure had been completed for 24% of patients. Assistance from the Palliative Care Unit's support team had been requested in 25% of cases, mainly to provide psychological support for the patient and the health care providers. These results have led us to reconsider the general organization of palliative care in the health care system.